diseases_conditions.id,diseases_conditions.ts,diseases_conditions.title,diseases_conditions.diseases_conditions_detail,diseases_conditions.last_update,diseases_conditions.category_1_x_diseases_conditions_id
41,"2018-02-02 05:14:16",Hemochromatosis,"What Is Hemochromatosis?
Hemochromatosis (HE-mo-kro-ma-TO-sis) is a disease in which too much iron builds up in your body (iron overload). Iron is a mineral found in many foods.
Too much iron is toxic to your body. It can poison your organs and cause organ failure. In hemochromatosis, iron can build up in most of your body's organs, but especially in the liver, heart, and pancreas.
Too much iron in the liver can cause an enlarged liver, liver failure, liver cancer, or cirrhosis (sir-RO-sis). Cirrhosis is scarring of the liver, which causes the organ to not work well.
Too much iron in the heart can cause irregular heartbeats called arrhythmias (ah-RITH-me-ahs) and heart failure. Too much iron in the pancreas can lead to diabetes.
If hemochromatosis isn't treated, it may even cause death.
Overview
The two types of hemochromatosis are primary and secondary. Primary hemochromatosis is caused by a defect in the genes that control how much iron you absorb from food. Secondary hemochromatosis usually is the result of another disease or condition that causes iron overload.
Most people who have primary hemochromatosis inherit it from their parents. If you inherit two hemochromatosis genes—one from each parent—you're at risk for iron overload and signs and symptoms of the disease. The two faulty genes cause your body to absorb more iron than usual from the foods you eat.
Hemochromatosis is one of the most common genetic disorders in the United States. However, not everyone who has hemochromatosis has signs or symptoms of the disease.
Estimates of how many people develop signs and symptoms vary greatly. Some estimates suggest that as many as half of all people who have the disease don't have signs or symptoms.
The severity of hemochromatosis also varies. Some people don't have complications, even with high levels of iron in their bodies. Others have severe complications or die from the disease.
Certain factors can affect the severity of the disease. For example, a high intake of vitamin C can make hemochromatosis worse. This is because vitamin C helps your body absorb iron from food.
Alcohol use can worsen liver damage and cirrhosis caused by hemochromatosis. Conditions such as hepatitis also can further damage or weaken the liver.
Outlook
The outlook for people who have hemochromatosis largely depends on how much organ damage they have at the time of diagnosis. Early diagnosis and treatment of the disease are important.
Treatment may help prevent, delay, or sometimes reverse complications of the disease. Treatment also may lead to better quality of life.
For people who are diagnosed and treated early, a normal lifespan is possible. If left untreated, hemochromatosis can lead to severe organ damage and even death.
Other Names for Hemochromatosis
What Causes Hemochromatosis?
The two types of hemochromatosis are primary and secondary. Each type has a different cause.
Primary Hemochromatosis
Primary hemochromatosis is caused by a defect in the genes that control how much iron you absorb from food. This form of the disease sometimes is called hereditary or classical hemochromatosis. Primary hemochromatosis is more common than the secondary form of the disease.
The genes usually involved in primary hemochromatosis are called HFE genes. Faulty HFE genes cause the body to absorb too much iron. If you inherit two copies of the faulty HFE gene (one from each parent), you're at risk for iron overload and signs and symptoms of hemochromatosis.
If you inherit one faulty HFE gene and one normal HFE gene, you're a hemochromatosis ""carrier."" Carriers usually don't develop the disease. However, they can pass the faulty gene on to their children. Estimates suggest that about 1 in 10 people in the United States are hemochromatosis carriers.
If two parents are carriers of the faulty HFE gene, then each of their children has a 1 in 4 chance of inheriting two faulty HFE genes.
Although less common, other faulty genes also can cause hemochromatosis. Researchers continue to study what changes to normal genes may cause the disease.
Secondary Hemochromatosis
Secondary hemochromatosis usually is the result of another disease or condition that causes iron overload. Examples of such diseases and conditions include:
Other factors also can cause secondary hemochromatosis, including:
Who Is at Risk for Hemochromatosis?
Hemochromatosis is one of the most common genetic diseases in the United States. It's most common in Caucasians of Northern European descent. The disease is less common in African Americans, Hispanics, Asians, and American Indians.
Primary hemochromatosis is more common in men than in women. Also, older people are more likely to develop the disease than younger people. In fact, signs and symptoms usually don't occur in men until they're 40 to 60 years old.
In women, signs and symptoms usually don't occur until after the age of 50 (after menopause). Young children rarely develop hemochromatosis.
Inheriting two faulty HFE genes (one from each parent) is the major risk factor for hemochromatosis. However, many people who have two copies of the faulty gene don't develop signs or symptoms of the disease.
Alcoholism is another risk factor for hemochromatosis. A family history of certain diseases and conditions also puts you at higher risk for hemochromatosis. Examples of such diseases and conditions include heart attack, liver disease, diabetes, arthritis, and erectile dysfunction (impotence).
What Are the Signs and Symptoms of Hemochromatosis?
Hemochromatosis can affect many parts of the body and cause various signs and symptoms. Many of the signs and symptoms are similar to those of other diseases.
Signs and symptoms of hemochromatosis usually don't occur until middle age. Women are more likely to have general symptoms first, such as fatigue (tiredness). In men, complications such as diabetes or cirrhosis (scarring of the liver) often are the first signs of the disease.
Signs and symptoms also vary based on the severity of the disease. Common signs and symptoms of hemochromatosis include joint pain, fatigue, general weakness, weight loss, and stomach pain.
Not everyone who has hemochromatosis has signs or symptoms of the disease. Estimates of how many people develop signs and symptoms vary greatly. Some estimates suggest that as many as half of all people who have the disease don't have signs or symptoms.
Hemochromatosis Complications
If hemochromatosis isn't found and treated early, iron builds up in your body and can lead to:
Changes in
skin color that make the skin look gray or bronze
How Is Hemochromatosis Diagnosed?
Your doctor will diagnose hemochromatosis based on your medical and family histories, a physical exam, and the results from tests and procedures.
The disease sometimes is detected while checking for other diseases or conditions, such as arthritis, liver disease, diabetes, heart disease, or erectile dysfunction (impotence).
Specialists Involved
Family doctors and internal medicine specialists may diagnose and treat hemochromatosis. Other doctors also may be involved in diagnosing and treating the disease, including:
Hematologists (
blood disease specialists)
Endocrinologists (
gland system specialists)
Hepatologists (
liver specialists)
Medical and Family Histories
To learn about your medical and family histories, your doctor may ask:
Diagnostic Tests and Procedures
Your doctor may recommend one or more tests or procedures to diagnose hemochromatosis.
Blood Tests
In hemochromatosis, the amount of iron in your body may be too high, even though the level of iron in your blood is normal. Certain blood tests can help your doctor find out how much iron is in your body.
During these tests, a sample of blood is taken from your body. It's usually drawn from a vein in your arm using a needle. The procedure usually is quick and easy, although it may cause some short-term discomfort.
The blood tests you have may include transferrin saturation (TS), serum ferritin level, and liver function tests.
Transferrin is a protein that carries iron in the blood. The TS test shows how much iron the transferrin is carrying. This helps your doctor find out how much iron is in your body.
Your doctor may test your serum ferritin level if your TS level is high. A serum ferritin level test shows how much iron is stored in your body's organs. A buildup of iron may suggest hemochromatosis.
You may have liver function tests to check for damage to your liver. Liver damage may be a sign of hemochromatosis. If you have hemochromatosis, liver function tests may show the severity of the disease.
Blood tests alone can't diagnose hemochromatosis. Thus, your doctor may recommend other tests as well.
Liver Biopsy
During a liver biopsy, your doctor numbs an area near your liver and then removes a small sample of liver tissue using a needle. The tissue is then looked at under a microscope.
A liver biopsy can show how much iron is in your liver. This procedure also can help your doctor diagnose liver damage (for example, scarring and cancer). Liver biopsies are less common now than in the past.
Magnetic Resonance Imaging
Magnetic resonance imaging (MRI) is a safe test that uses radio waves, magnets, and a computer to create pictures of your organs. An MRI may be done to show the amount of iron in your liver.
Superconducting Quantum Interference Device
A superconducting quantum interference device (SQuID) is a machine that uses very sensitive magnets to measure the amount of iron in your liver. This machine is available at only a few medical centers.
How Is Hemochromatosis Treated?
Treatments for hemochromatosis include therapeutic phlebotomy (fleh-BOT-o-me), iron chelation (ke-LAY-shun) therapy, dietary changes, and treatment for complications.
The goals of treating hemochromatosis include:
Therapeutic Phlebotomy
Therapeutic phlebotomy is a procedure that removes blood (and iron) from your body. A needle is inserted into a vein, and your blood flows through an airtight tube into a sterile container or bag.
The process is similar to donating blood; it can be done at blood donation centers, hospital donation centers, or a doctor's office.
In the first stage of treatment, about 1 pint of blood is removed once or twice a week. After your iron levels return to normal, you may continue phlebotomy treatments. However, you may need them less often—typically every 2–4 months.
As long as treatment continues, which often is for the rest of your life, you'll need frequent blood tests to check your iron levels.
Iron Chelation Therapy
Iron chelation therapy uses medicine to remove excess iron from your body. This treatment is a good option for people who can't have routine blood removal.
The medicine used in iron chelation therapy is either injected or taken orally (by mouth). Injected iron chelation therapy is done at a doctor's office. Oral iron chelation therapy can be done at home.
Dietary Changes
Your doctor may suggest that you change your diet if you have hemochromatosis. You may be advised to:
Avoid taking
iron, including iron pills, iron
injections, or multivitamins that contain iron.
Limit your intake of
vitamin C. Vitamin C helps your body absorb
iron from food. Talk with your doctor about how much vitamin C is safe for you.
Avoid uncooked fish and shellfish. Some fish and shellfish contain
bacteria that can cause infections in people who have
chronic diseases, such as
hemochromatosis.
Limit alcohol intake. Drinking alcohol increases the risk of
liver disease. It also can make existing liver disease worse.
Treatment for Complications
Your doctor may prescribe other treatments as needed for complications such as liver disease, heart problems, or diabetes.
Living With Hemochromatosis
The outlook for people who have hemochromatosis largely depends on how much organ damage has already occurred at the time of diagnosis. Early diagnosis and treatment of the disorder are important.
Treatment may help prevent, delay, or sometimes reverse complications of the disorder. Treatment also may lead to higher energy levels and better quality of life. With early diagnosis and treatment, a normal lifespan is possible.
If organ damage has already occurred, treatment may prevent further damage and improve life expectancy. However, treatment may not be able to reverse existing damage.
If hemochromatosis isn't treated, it can lead to severe organ damage or even death.
What To Expect After Treatment
People have different responses to treatment. Some people who have frequent therapeutic phlebotomy may feel very tired. People who have advanced disease or who are getting intense treatment that weakens them may need help with daily tasks and activities.
At first, you may need to have therapeutic phlebotomy often. How long you'll need this treatment depends on how much extra iron is in your body.
After the initial treatment period, you may need ongoing treatment two to six times a year. This will help prevent the iron from building up again.
Ongoing Care
If you have hemochromatosis, getting ongoing care is important. Ongoing care may include:
If you need routine phlebotomy, you may have to change your usual work times to schedule your treatments. You also may have to change your work schedule to allow for periods of fatigue (tiredness) or recovery, especially if your treatment weakens you.
Emotional Issues and Support
Living with hemochromatosis may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with hemochromatosis. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
Screening Family Members for Hemochromatosis
Parents, grandparents, brothers and sisters, and children (blood relatives) of a person who has hemochromatosis may be at risk for the disease. Talk with your doctor to see whether your relatives should have their iron levels checked.
Your doctor may recommend genetic testing to show whether family members are at risk for the disease. If a relative already has been diagnosed with hemochromatosis, a genetic test can show whether he or she has the primary (inherited) form of the disease.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to hemochromatosis, talk with your doctor. You also can visit the following Web sites to learn more about cli","June 11, 2014.","{
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42,"2018-02-02 05:14:36","Hemolytic Anemia","
What Is Hemolytic Anemia?
Hemolytic anemia (HEE-moh-lit-ick uh-NEE-me-uh) is a condition in which red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over.
Red blood cells are disc-shaped and look like doughnuts without holes in the center. These cells carry oxygen to your body. They also remove carbon dioxide (a waste product) from your body.
Red blood cells are made in the bone marrow—a sponge-like tissue inside the bones. They live for about 120 days in the bloodstream and then die.
White blood cells and platelets (PLATE-lets) also are made in the bone marrow. White blood cells help fight infections. Platelets stick together to seal small cuts or breaks on blood vessel walls and stop bleeding.
When blood cells die, the body's bone marrow makes more blood cells to replace them. However, in hemolytic anemia, the bone marrow can't make red blood cells fast enough to meet the body's needs.
Hemolytic anemia can lead to many health problems, such as fatigue (tiredness), pain, irregular heartbeats called arrhythmias (ah-RITH-me-ahs), an enlarged heart, and heart failure.
Outlook
There are many types of hemolytic anemia. Treatment and outlook depend on what type you have and how severe it is. The condition can develop suddenly or slowly. Symptoms can range from mild to severe.
Hemolytic anemia often can be successfully treated or controlled. Mild hemolytic anemia may need no treatment at all. Severe hemolytic anemia requires prompt and proper treatment, or it may be fatal.
Inherited forms of hemolytic anemia are lifelong conditions that may require ongoing treatment. Acquired forms of hemolytic anemia may go away if the cause of the condition is found and corrected.
Types of Hemolytic Anemia
There are many types of hemolytic anemia. The condition can be inherited or acquired. ""Inherited"" means your parents passed the gene for the condition on to you. ""Acquired"" means you aren't born with the condition, but you develop it.
Inherited Hemolytic Anemias
With inherited hemolytic anemias, one or more of the genes that control red blood cell production are faulty. This can lead to problems with the hemoglobin, cell membrane, or enzymes that maintain healthy red blood cells.
The abnormal cells may be fragile and break down while moving through the bloodstream. If this happens, an organ called the spleen may remove the cell debris from the bloodstream.
Thalassemias
Thalassemias (thal-a-SE-me-ahs) are inherited blood disorders in which the body doesn't make enough of certain types of hemoglobin. This causes the body to make fewer healthy red blood cells than normal.
Thalassemias most often affect people of Southeast Asian, Indian, Chinese, Filipino, Mediterranean, or African origin or descent.
Hereditary Spherocytosis
In this condition, a defect in the surface membrane (the outer covering) of red blood cells causes them to have a sphere, or ball-like, shape. These blood cells have a lifespan that's shorter than normal.
Hereditary spherocytosis (SFER-o-si-to-sis) is the most common cause of hemolytic anemia among people of Northern European descent.
Hereditary Elliptocytosis (Ovalocytosis)
Like hereditary spherocytosis, this condition also involves a problem with the cell membrane. In this condition, the red blood cells are elliptic (oval) in shape. They aren't as flexible as normal red blood cells, and they have a shorter lifespan.
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
In G6PD deficiency, the red blood cells are missing an important enzyme called G6PD. G6PD is part of the normal chemistry inside red blood cells.
In G6PD deficiency, if red blood cells come into contact with certain substances in the bloodstream, the missing enzyme causes the cells to rupture (burst) and die.
Many factors can trigger the breakdown of the red blood cells. Examples include taking sulfa or antimalarial medicines; being exposed to naphthalene, a substance found in some moth balls; eating fava beans; or having an infection.
G6PD deficiency mostly affects males of African or Mediterranean descent. In the United States, the condition is more common among African Americans than Caucasians.
Pyruvate Kinase Deficiency
In this condition, the body is missing an enzyme called pyruvate (PI-ru-vate) kinase. Not having enough of this enzyme causes red blood cells to break down easily.
This disorder is more common among the Amish than other groups.
Acquired Hemolytic Anemias
With acquired hemolytic anemias, your red blood cells may be normal. However, some other disease or factor causes the body to destroy red blood cells and remove them from the bloodstream.
The destruction of the red blood cells occurs in the bloodstream or, more commonly, in the spleen.
Immune Hemolytic Anemia
In immune hemolytic anemia, your immune system destroys your red blood cells. The three main types of immune hemolytic anemia are autoimmune, alloimmune, and drug-induced.
Autoimmune hemolytic anemia (AIHA). In this condition, your immune system makes antibodies (proteins) that attack your red blood cells. Why this happens isn't known.
AIHA accounts for half of all cases of hemolytic anemia. AIHA may come on very quickly and become serious.
Having certain diseases or infections can raise your risk for AIHA. Examples include:
Epstein-Barr virus
Cytomegalovirus
HIV
AIHA also can develop after you have a blood and marrow stem cell transplant.
In some types of AIHA, the antibodies made by the body are called warm antibodies. This means they're active (that is, they destroy red blood cells) at warm temperatures, such as body temperature.
In other types of AIHA, the body makes cold-reactive antibodies. These antibodies are active at cold temperatures.
Cold-reactive antibodies can become active when parts of the body, such as the hands or feet, are exposed to temperatures lower than 32 to 50 degrees Fahrenheit (0 to 10 degrees Celsius).
Warm antibody AIHA is more common than cold antibody AIHA.
Alloimmune hemolytic anemia. This type of hemolytic anemia occurs if your body makes antibodies against red blood cells that you get from a blood transfusion. This can happen if the transfused blood is a different blood type than your blood.
This type of hemolytic anemia also can occur during pregnancy if a woman has Rh-negative blood and her baby has Rh-positive blood. ""Rh-negative"" and ""Rh-positive"" refer to whether your blood has Rh factor. Rh factor is a protein on red blood cells.
For more information, go to the Health Topics Rh Incompatibility article.
Drug-induced hemolytic anemia. Certain medicines can cause a reaction that develops into hemolytic anemia. Some medicines, such as penicillin, bind to red blood cell surfaces and can cause antibodies to develop.
Other medicines cause hemolytic anemia in other ways. Examples of these medicines include chemotherapy, acetaminophen, quinine and antimalarial medicines, anti-inflammatory medicines, and levodopa.
Mechanical Hemolytic Anemias
Physical damage to red blood cell membranes can cause them to break down faster than normal. Damage may be due to:
Preeclampsia (pre-e-KLAMP-se-ah) or eclampsia. Preeclampsia is
high blood pressure during
pregnancy. Eclampsia, which follows preeclampsia, is a serious condition that causes
seizures in pregnant women.
Blood cell damage also may occur in the limbs as a result of doing strenuous activities, such as taking part in marathons.
Other Causes of Damage to Red Blood Cells
Certain infections and substances also can damage red blood cells and lead to hemolytic anemia. Examples include malaria and blackwater fever, tick-borne diseases, snake venom, and toxic chemicals.
Other Names for Hemolytic Anemia
What Causes Hemolytic Anemia?
The immediate cause of hemolytic anemia is the early destruction of red blood cells. This means that red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over.
Many diseases, conditions, and factors can cause the body to destroy its red blood cells. These causes can be inherited or acquired. ""Inherited"" means your parents passed the gene for the condition on to you. ""Acquired"" means you aren't born with the condition, but you develop it.
Sometimes, the cause of hemolytic anemia isn't known.
For more information about the specific causes of hemolytic anemia, go to ""Types of Hemolytic Anemia.""
Acquired Hemolytic Anemias
In acquired hemolytic anemias, the body makes normal red blood cells. However, a disease, condition, or other factor destroys the cells. Examples of conditions that can destroy the red blood cells include:
Immune disorders
Infections
Hypersplenism (HI-per-
SPLEEN-izm; an enlarged spleen)
Who Is at Risk for Hemolytic Anemia?
Hemolytic anemia can affect people of all ages and races and both sexes. Some types of hemolytic anemia are more likely to occur in certain populations than others.
For example, glucose-6-phosphate dehydrogenase (G6PD) deficiency mostly affects males of African or Mediterranean descent. In the United States, the condition is more common among African Americans than Caucasians.
In the United States, sickle cell anemia mainly affects African Americans.
What Are the Signs and Symptoms of Hemolytic Anemia?
The signs and symptoms of hemolytic anemia will depend on the type and severity of the disease.
People who have mild hemolytic anemia often have no signs or symptoms. More severe hemolytic anemia may cause many signs and symptoms, and they may be serious.
Many of the signs and symptoms of hemolytic anemia apply to all types of anemia.
Signs and Symptoms of Hemolytic Anemia
Leg Ulcers and Pain
In people who have sickle cell anemia, the sickle-shaped cells can clog small blood vessels and block blood flow. This can cause leg sores and pain throughout the body.
A Severe Reaction to a Blood Transfusion
You may develop hemolytic anemia due to a blood transfusion. This can happen if the transfused blood is a different blood type than your blood.
Signs and symptoms of a severe reaction to a transfusion include fever, chills, low blood pressure, and shock. (Shock is a life-threatening condition that occurs if the body isn't getting enough blood flow.)
How Is Hemolytic Anemia Diagnosed?
Your doctor will diagnose hemolytic anemia based on your medical and family histories, a physical exam, and test results.
Specialists Involved
Primary care doctors, such as a family doctor or pediatrician, may help diagnose and treat hemolytic anemia. Your primary care doctor also may refer you to a hematologist. This is a doctor who specializes in diagnosing and treating blood diseases and disorders.
Doctors and clinics that specialize in treating inherited blood disorders, such as sickle cell anemia and thalassemias, also may be involved.
If your hemolytic anemia is inherited, you may want to consult a genetic counselor. A counselor can help you understand your risk of having a child who has the condition. He or she also can explain the choices that are available to you.
Medical and Family Histories
To find the cause and severity of hemolytic anemia, your doctor may ask detailed questions about your symptoms, personal medical history, and your family medical history.
He or she may ask whether:
You or anyone in your family has had problems with
anemia You've recently had any illnesses or medical conditions
You take any medicines, and which ones
You've been exposed to certain chemicals or substances
Physical Exam
Your doctor will do a physical exam to check for signs of hemolytic anemia. He or she will try to find out how severe the condition is and what's causing it.
The exam may include:
Diagnostic Tests and Procedures
Many tests are used to diagnose hemolytic anemia. These tests can help confirm a diagnosis, look for a cause, and find out how severe the condition is.
Complete Blood Count
Often, the first test used to diagnose anemia is a complete blood count (CBC). The CBC measures many parts of your blood.
This test checks your hemoglobin and hematocrit (hee-MAT-oh-crit) levels. Hemoglobin is an iron-rich protein in red blood cells that carries oxygen to the body. Hematocrit is a measure of how much space red blood cells take up in your blood. A low level of hemoglobin or hematocrit is a sign of anemia.
The normal range of these levels may vary in certain racial and ethnic populations. Your doctor can explain your test results to you.
The CBC also checks the number of red blood cells, white blood cells, and platelets in your blood. Abnormal results may be a sign of hemolytic anemia, a different blood disorder, an infection, or another condition.
Finally, the CBC looks at mean corpuscular (kor-PUS-kyu-lar) volume (MCV). MCV is a measure of the average size of your red blood cells. The results may be a clue as to the cause of your anemia.
Other Names for Hemophilia
Hemophilia B
Christmas disease
Factor IX deficiency
What Causes Hemophilia?
If you have inherited hemophilia, you're born with the disorder. It's caused by a defect in one of the genes that determine how the body makes blood clotting factor VIII or IX. These genes are located on the X chromosomes (KRO-muh-somz).
Chromosomes come in pairs. Females have two X chromosomes, while males have one X and one Y chromosome. Only the X chromosome carries the genes related to clotting factors.
A male who has a faulty hemophilia gene on his X chromosome will have hemophilia. A female must have the faulty gene on both of her X chromosomes to have hemophilia, which is very rare.
If a female has the faulty gene on only one of her X chromosomes, she is a ""hemophilia carrier.” Carriers don't have hemophilia, but they can pass the faulty gene to their children.
Below are two examples of how the hemophilia gene is inherited.
![]()
Inheritance Pattern for Hemophilia—Example 1. The image shows one example of how the hemophilia gene is inherited. In this example, the father doesn't have hemophilia (that is, he has two normal chromosomes—X and Y). The mother is a carrier (more...)
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Inheritance Pattern for Hemophilia—Example 1 Inheritance Pattern for Hemophilia—Example 2. The image shows one example of how the hemophilia gene is inherited. In this example, the father has hemophilia (that is, his X chromosome is faulty). (more...)
Females who are hemophilia carriers usually have enough clotting factors from their one normal X chromosome to prevent serious bleeding problems. However, up to 50 percent of carriers may have an increased risk of bleeding.
Very rarely, a girl is born with hemophilia. This can happen if her father has hemophilia and her mother is a carrier.
Some males who have the disorder are born to mothers who aren't carriers. In these cases, a mutation (random change) occurs in the gene as it is passed to the child.
What Are the Signs and Symptoms of Hemophilia?
The major signs and symptoms of hemophilia are excessive bleeding and easy bruising.
Excessive Bleeding
The extent of bleeding depends on how severe the hemophilia is.
Children who have mild hemophilia may not have signs unless they have excessive bleeding from a dental procedure, an accident, or surgery. Males who have severe hemophilia may bleed heavily after circumcision.
Bleeding can occur on the body's surface (external bleeding) or inside the body (internal bleeding).
Signs of external bleeding may include:
Signs of internal bleeding may include:
Bleeding in the Joints
Bleeding in the knees, elbows, or other joints is another common form of internal bleeding in people who have hemophilia. This bleeding can occur without obvious injury.
At first, the bleeding causes tightness in the joint with no real pain or any visible signs of bleeding. The joint then becomes swollen, hot to touch, and painful to bend.
Swelling continues as bleeding continues. Eventually, movement in the joint is temporarily lost. Pain can be severe. Joint bleeding that isn't treated quickly can damage the joint.
Bleeding in the Brain
Internal bleeding in the brain is a very serious complication of hemophilia. It can happen after a simple bump on the head or a more serious injury. The signs and symptoms of bleeding in the brain include:
How Is Hemophilia Diagnosed?
If you or your child appears to have a bleeding problem, your doctor will ask about your personal and family medical histories. This will reveal whether you or your family members, including women and girls, have bleeding problems. However, some people who have hemophilia have no recent family history of the disease.
You or your child also will likely have a physical exam and blood tests to diagnose hemophilia. Blood tests are used to find out:
How long it takes for your
blood to clot
The test results will show whether you have hemophilia, what type of hemophilia you have, and how severe it is.
Hemophilia A and B are classified as mild, moderate, or severe, depending on the amount of clotting factor VIII or IX in the blood.
The severity of symptoms can overlap between the categories. For example, some people who have mild hemophilia may have bleeding problems almost as often or as severe as some people who have moderate hemophilia.
Severe hemophilia can cause serious bleeding problems in babies. Thus, children who have severe hemophilia usually are diagnosed during the first year of life. People who have milder forms of hemophilia may not be diagnosed until they're adults.
The bleeding problems of hemophilia A and hemophilia B are the same. Only special blood tests can tell which type of the disorder you or your child has. Knowing which type is important because the treatments are different.
Pregnant women who are known hemophilia carriers can have the disorder diagnosed in their unborn babies as early as 12 weeks into their pregnancies.
Women who are hemophilia carriers also can have ""preimplantation diagnosis"" to have children who don't have hemophilia.
For this process, women have their eggs removed and fertilized by sperm in a laboratory. The embryos are then tested for hemophilia. Only embryos without the disorder are implanted in the womb.
How Is Hemophilia Treated?
Treatment With Replacement Therapy
The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor that's missing or low.
Clotting factor concentrates can be made from human blood. The blood is treated to prevent the spread of diseases, such as hepatitis. With the current methods of screening and treating donated blood, the risk of getting an infectious disease from human clotting factors is very small.
To further reduce the risk, you or your child can take clotting factor concentrates that aren't made from human blood. These are called recombinant clotting factors. Clotting factors are easy to store, mix, and use at home—it only takes about 15 minutes to receive the factor.
You may have replacement therapy on a regular basis to prevent bleeding. This is called preventive or prophylactic (PRO-fih-lac-tik) therapy. Or, you may only need replacement therapy to stop bleeding when it occurs. This use of the treatment, on an as-needed basis, is called demand therapy.
Demand therapy is less intensive and expensive than preventive therapy. However, there's a risk that bleeding will cause damage before you receive the demand therapy.
Complications of Replacement Therapy
Complications of replacement therapy include:
Antibodies to the clotting factor. Antibodies can destroy the clotting factor before it has a chance to work. This is a very serious problem. It prevents the main treatment for hemophilia (replacement therapy) from working.
These antibodies, also called inhibitors, develop in about 20–30 percent of people who have severe hemophilia A. Inhibitors develop in 2–5 percent of people who have hemophilia B.
When antibodies develop, doctors may use larger doses of clotting factor or try different clotting factor sources. Sometimes the antibodies go away.
Researchers are studying new ways to deal with antibodies to clotting factors.
Viruses from human clotting factors. Clotting factors made from human blood can carry the viruses that cause HIV/AIDS and hepatitis. However, the risk of getting an infectious disease from human clotting factors is very small due to:
Careful screening of
blood donors
Testing of donated
blood products
Treating donated
blood products with a detergent and heat to destroy
viruses
Damage to joints, muscles, and other parts of the body. Delays in treatment can cause damage such as:
Bleeding into a
joint. If this happens many times, it can lead to changes in the shape of the joint and impair the joint's function.
Swelling of the membrane around a
joint.
Pain, swelling, and redness of a
joint.
Pressure on a
joint from swelling, which can destroy the joint.
Home Treatment With Replacement Therapy
You can do both preventive (ongoing) and demand (as-needed) replacement therapy at home. Many people learn to do the infusions at home for their child or for themselves. Home treatment has several advantages:
Discuss options for home treatment with your doctor or your child's doctor. A doctor or other health care provider can teach you the steps and safety procedures for home treatment. Hemophilia treatment centers are another good resource for learning about home treatment (discussed in ""Living With Hemophilia”).
Doctors can surgically implant vein access devices to make it easier for you to access a vein for treatment with replacement therapy. These devices can be helpful if treatment occurs often. However, infections can be a problem with these devices. Your doctor can help you decide whether this type of device is right for you or your child.
Other Types of Treatment
Desmopressin
Desmopressin (DDAVP) is a man-made hormone used to treat people who have mild hemophilia A. DDAVP isn't used to treat hemophilia B or severe hemophilia A.
DDAVP stimulates the release of stored factor VIII and von Willebrand factor; it also increases the level of these proteins in your blood. Von Willebrand factor carries and binds factor VIII, which can then stay in the bloodstream longer.
DDAVP usually is given by injection or as nasal spray. Because the effect of this medicine wears off if it's used often, the medicine is given only in certain situations. For example, you may take this medicine prior to dental work or before playing certain sports to prevent or reduce bleeding.
Antifibrinolytic Medicines
Antifibrinolytic medicines (including tranexamic acid and epsilon aminocaproic acid) may be used with replacement therapy. They're usually given as a pill, and they help keep blood clots from breaking down.
These medicines most often are used before dental work or to treat bleeding from the mouth or nose or mild intestinal bleeding.
Treatment of a Specific Bleeding Site
Pain medicines, steroids, and physical therapy may be used to reduce pain and swelling in an affected joint. Talk with your doctor or pharmacist about which medicines are safe for you to take.
Which Treatment Is Best for You?
The type of treatment you or your child receives depends on several things, including how severe the hemophilia is, the activities you'll be doing, and the dental or medical procedures you'll be having.
Mild
hemophilia—Replacement
therapy usually isn't needed for mild hemophilia. Sometimes, though, DDAVP is given to raise the body's level of factor VIII.
Moderate
hemophilia—You may need replacement
therapy only when
bleeding occurs or to prevent bleeding that could occur when doing certain activities. Your doctor also may recommend DDAVP prior to having a procedure or doing an activity that increases the risk of bleeding.
Severe
hemophilia—You usually need replacement
therapy to prevent
bleeding that could damage your
joints,
muscles, or other parts of your body. Typically, replacement therapy is given at home two or three times a week. This preventive therapy usually is started in patients at a young age and may need to continue for life.
For both types of hemophilia, getting quick treatment for bleeding is important. Quick treatment can limit damage to your body. If you or your child has hemophilia, learn to recognize signs of bleeding.
Other family members also should learn to watch for signs of bleeding in a child who has hemophilia. Children sometimes ignore signs of bleeding because they want to avoid the discomfort of treatment.
Living With Hemophilia
If you or your child has hemophilia, you can take steps to prevent bleeding problems. Thanks to improvements in treatment, a child who has hemophilia today is likely to live a normal lifespan.
Hemophilia Treatment Centers
The Federal Government funds a nationwide network of hemophilia treatment centers (HTCs). These centers are an important resource for people who have hemophilia and their families.
The medical experts at HTCs provide treatment, education, and support. They can teach you or your family members how to do home treatments. Center staff also can provide your doctor with information.
People who get care at HTCs are less likely than those who get care elsewhere to have bleeding complications and hospitalizations. They're also more likely to have a better quality of life. This may be due to the centers' emphasis on bleeding prevention and the education and support provided to patients and their caregivers.
More than 100 federally funded HTCs are located throughout the United States. Many HTCs are located at major university medical and research centers. The hemophilia teams at these centers include:
Nurse coordinators
Pediatricians (doctors who treat children) and adult and pediatric hematologists (doctors who specialize in
blood disorders)
Social workers (who can help with financial issues, transportation, mental health, and other issues)
Dentists
To find an HTC located near you, go to the directory of HTCs on the Centers for Disease Control and Prevention's Web site. Many people who have hemophilia go to HTCs for annual checkups, even if it means traveling some distance to do so.
At an HTC, you or your child may be able to take part in clinical research and benefit from the latest hemophilia research findings. The HTC team also will work with your local health care providers to help meet your needs or your child's needs.
Ongoing Care
If you have hemophilia, you can take steps to avoid complications. For example:
It's a good idea to keep a record of all previous treatments. Be sure to take this information with you to medical appointments and to the hospital or emergency room.
If Your Child Is Diagnosed With Hemophilia
You may have emotional, financial, social, or other strains as you adjust to having a child who has hemophilia. Learn all you can about the disorder and get the support you need.
Talk with doctors and other health care providers about treatment, prevention of bleeding, and what to do during an emergency.
The care teams at HTCs can provide your child with treatment and help educate and support you. The social worker on the team can help with emotional issues, financial and transportation problems, and other concerns.
Seek the many resources available through the Web, books, and other materials, including those provided by national and local hemophilia organizations.
Look into support groups that offer a variety of activities for children who have hemophilia and for family members. Some groups offer summer camps for children who have hemophilia. Ask your doctor, nurse coordinator, or social worker about these groups and camps.
Challenges will occur as your child grows and becomes more active. In addition to treatment and regular health and dental care, your child needs information about hemophilia that he or she can understand.
Children who have hemophilia also need ongoing support, and they need to be reassured that the condition isn't their fault.
Young children who have hemophilia need extra protection from things in the home and elsewhere that could cause injuries and bleeding:
Protect toddlers with kneepads, elbow pads, and protective helmets. All children should wear safety helmets when riding tricycles or bicycles.
Be sure to use the safety belts and straps in highchairs, car seats, and strollers to protect your child from
falls.
Remove furniture with sharp corners or pad them while your child is a toddler.
Keep out of reach or locked away small and sharp objects and other items that could cause
bleeding or harm.
Check play equipment and outdoor play areas for possible hazards.
You also should learn how to examine your child for and recognize signs of bleeding. Learn to prepare for bleeding episodes when they occur. Keep a cold pack in the freezer ready to use as directed or to take along with you to treat bumps and bruises.
Popsicles work fine when there is minor bleeding in the mouth. You also might want to keep a bag ready to go with items you'll need if you must take your child to the emergency room or elsewhere.
Be sure that anyone who is responsible for your child knows that he or she has Go to:
What Is Cholesterol?
To understand high blood cholesterol (ko-LES-ter-ol), it helps to learn about cholesterol. Cholesterol is a waxy, fat-like substance that’s found in all cells of the body.
Your body needs some cholesterol to make hormones, vitamin D, and substances that help you digest foods. Your body makes all the cholesterol it needs. However, cholesterol also is found in some of the foods you eat.
Cholesterol travels through your bloodstream in small packages called lipoproteins (lip-o-PRO-teens). These packages are made of fat (lipid) on the inside and proteins on the outside.
Two kinds of lipoproteins carry cholesterol throughout your body: low-density lipoproteins (LDL) and high-density lipoproteins (HDL). Having healthy levels of both types of lipoproteins is important.
LDL cholesterol sometimes is called “bad” cholesterol. A high LDL level leads to a buildup of cholesterol in your arteries. (Arteries are blood vessels that carry blood from your heart to your body.)
HDL cholesterol sometimes is called “good” cholesterol. This is because it carries cholesterol from other parts of your body back to your liver. Your liver removes the cholesterol from your body.
What Is High Blood Cholesterol?
High blood cholesterol is a condition in which you have too much cholesterol in your blood. By itself, the condition usually has no signs or symptoms. Thus, many people don’t know that their cholesterol levels are too high.
People who have high blood cholesterol have a greater chance of getting coronary heart disease, also called coronary artery disease. (In this article, the term “heart disease” refers to coronary heart disease.)
The higher the level of LDL cholesterol in your blood, the GREATER your chance is of getting heart disease. The higher the level of HDL cholesterol in your blood, the LOWER your chance is of getting heart disease.
Coronary heart disease is a condition in which plaque (plak) builds up inside the coronary (heart) arteries. Plaque is made up of cholesterol, fat, calcium, and other substances found in the blood. When plaque builds up in the arteries, the condition is called atherosclerosis (ATH-er-o-skler-O-sis).
Atherosclerosis
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Figure A shows the location of the heart in the body. Figure B shows a normal coronary artery with normal blood flow. The inset image shows a cross-section of a normal coronary artery. Figure C shows a coronary artery narrowed by plaque. The buildup of (more...)
Over time, plaque hardens and narrows your coronary arteries. This limits the flow of oxygen-rich blood to the heart.
Eventually, an area of plaque can rupture (break open). This causes a blood clot to form on the surface of the plaque. If the clot becomes large enough, it can mostly or completely block blood flow through a coronary artery.
If the flow of oxygen-rich blood to your heart muscle is reduced or blocked, angina (an-JI-nuh or AN-juh-nuh) or a heart attack may occur.
Angina is chest pain or discomfort. It may feel like pressure or squeezing in your chest. The pain also may occur in your shoulders, arms, neck, jaw, or back. Angina pain may even feel like indigestion.
A heart attack occurs if the flow of oxygen-rich blood to a section of heart muscle is cut off. If blood flow isn’t restored quickly, the section of heart muscle begins to die. Without quick treatment, a heart attack can lead to serious problems or death.
Plaque also can build up in other arteries in your body, such as the arteries that bring oxygen-rich blood to your brain and limbs. This can lead to problems such as carotid artery disease, stroke, and peripheral arterial disease (P.A.D.).
Outlook
Lowering your cholesterol may slow, reduce, or even stop the buildup of plaque in your arteries. It also may reduce the risk of plaque rupturing and causing dangerous blood clots.
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The image focuses on high cholesterol in women and explains how high cholesterol increases the risk of developing heart disease. An estimated 1 in 2 women has high or borderline high cholesterol. The image also lists the ranges of total cholesterol numbers (more...)
Other Names for High Blood Cholesterol
What Causes High Blood Cholesterol?
Many factors can affect the cholesterol levels in your blood. You can control some factors, but not others.
Factors You Can Control
Diet
Cholesterol is found in foods that come from animal sources, such as egg yolks, meat, and cheese. Some foods have fats that raise your cholesterol level.
For example, saturated fat raises your low-density lipoprotein (LDL) cholesterol level more than anything else in your diet. Saturated fat is found in some meats, dairy products, chocolate, baked goods, and deep-fried and processed foods.
Trans fatty acids (trans fats) raise your LDL cholesterol and lower your high-density lipoprotein (HDL cholesterol. Trans fats are made when hydrogen is added to vegetable oil to harden it. Trans fats are found in some fried and processed foods.
Limiting foods with cholesterol, saturated fat, and trans fats can help you control your cholesterol levels.
Physical Activity and Weight
Lack of physical activity can lead to weight gain. Being overweight tends to raise your LDL level, lower your HDL level, and increase your total cholesterol level. (Total cholesterol is a measure of the total amount of cholesterol in your blood, including LDL and HDL.)
Routine physical activity can help you lose weight and lower your LDL cholesterol. Being physically active also can help you raise your HDL cholesterol level.
Factors You Can’t Control
Age and Sex
Starting at puberty, men often have lower levels of HDL cholesterol than women. As women and men age, their LDL cholesterol levels often rise. Before age 55, women usually have lower LDL cholesterol levels than men. However, after age 55, women can have higher LDL levels than men.
What Are the Signs and Symptoms of High Blood Cholesterol?
High blood cholesterol usually has no signs or symptoms. Thus, many people don't know that their cholesterol levels are too high.
If you're 20 years old or older, have your cholesterol levels checked at least once every 5 years. Talk with your doctor about how often you should be tested.
How Is High Blood Cholesterol Diagnosed?
Your doctor will diagnose high blood cholesterol by checking the cholesterol levels in your blood. A blood test called a lipoprotein panel can measure your cholesterol levels. Before the test, you’ll need to fast (not eat or drink anything but water) for 9 to 12 hours.
The lipoprotein panel will give your doctor information about your:
LDL cholesterol. LDL, or “bad,” cholesterol is the main source of cholesterol buildup and blockages in the
arteries.
Triglycerides (tri-GLIH-seh-rides). Triglycerides are a type of
fat found in your
blood. Some studies suggest that a high level of triglycerides in the blood may raise the risk of
coronary heart disease, especially in women.
If it’s not possible to have a lipoprotein panel, knowing your total cholesterol and HDL cholesterol can give you a general idea about your cholesterol levels.
Testing for total and HDL cholesterol does not require fasting. If your total cholesterol is 200 mg/dL or more, or if your HDL cholesterol is less than 40 mg/dL, your doctor will likely recommend that you have a lipoprotein panel. (Cholesterol is measured as milligrams (mg) of cholesterol per deciliter (dL) of blood.)
The tables below show total, LDL, and HDL cholesterol levels and their corresponding categories. See how your cholesterol numbers compare to the numbers in the tables below.
Triglycerides also can raise your risk for heart disease. If your triglyceride level is borderline high (150–199 mg/dL) or high (200 mg/dL or higher), you may need treatment.
Factors that can raise your triglyceride level include:
How Is High Blood Cholesterol Treated?
High blood cholesterol is treated with lifestyle changes and medicines. The main goal of treatment is to lower your low-density lipoprotein (LDL) cholesterol level enough to reduce your risk for coronary heart disease, heart attack, and other related health problems.
Your risk for heart disease and heart attack goes up as your LDL cholesterol level rises and your number of heart disease risk factors increases.
Some people are at high risk for heart attacks because they already have heart disease. Other people are at high risk for heart disease because they have diabetes or more than one heart disease risk factor.
Talk with your doctor about lowering your cholesterol and your risk for heart disease. Also, check the list to find out whether you have risk factors that affect your LDL cholesterol goal:
You can use the NHLBI 10-Year Risk Calculator to find your risk score. The score, given as a percentage, refers to your chance of having a heart attack in the next 10 years.
Based on your medical history, number of risk factors, and risk score, figure out your risk of getting heart disease or having a heart attack using the table below.
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| If You Have | You Are in Category | Your LDL Goal Is |
|---|
| Heart disease, diabetes, or a risk score higher than 20% | I. High risk* | Less than 100 mg/dL |
| Two or more risk factors and a risk score of 10–20% | II. Moderately high risk | Less than 130 mg/dL |
| Two or more risk factors and a risk score lower than 10% | III. Moderate risk | Less than 130 mg/dL |
| One or no risk factors | IV. Low to moderate risk | Less than 160 mg/dL |
After following the above steps, you should have an idea about your risk for heart disease and heart attack. The two main ways to lower your cholesterol (and, thus, your heart disease risk) include:
Medicines. If
cholesterol-lowering medicines are needed, they’re used with the TLC program to help lower your
LDL cholesterol level.
Your doctor will set your LDL goal. The higher your risk for heart disease, the lower he or she will set your LDL goal. Using the following guide, you and your doctor can create a plan for treating your high blood cholesterol.
Category I, high risk, your LDL goal is less than 100 mg/dL.*
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| Your LDL Level | Treatment |
|---|
| If your LDL level is 100 or higher | You will need to begin the TLC diet and take medicines as prescribed. |
| Even if your LDL level is below 100 | You should follow the TLC diet to keep your LDL level as low as possible. |
Category II, moderately high risk, your LDL goal is less than 130 mg/dL
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| Your LDL Level | Treatment |
|---|
| If your LDL level is 130 mg/dL or higher | You will need to begin the TLC diet. |
| If your LDL level is 130 mg/dL or higher after 3 months on the TLC diet | You may need medicines along with the TLC diet. |
| If your LDL level is less than 130 mg/dL | You will need to follow a heart healthy diet. |
Category III, moderate risk, your LDL goal is less than 130 mg/dL.
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| Your LDL Level | Treatment |
|---|
| If your LDL level is 130 mg/dL or higher | You will need to begin the TLC diet. |
| If your LDL level is 160 mg/dL or higher after 3 months on the TLC diet | You may need medicines along with the TLC diet. |
| If your LDL level is less than 130 mg/dL | You will need to follow a heart healthy diet. |
Category IV, low to moderate risk, your LDL goal is less than 160 mg/dL.
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| Your LDL Level | Treatment |
|---|
| If your LDL level is 160 mg/dL or higher | You will need to begin the TLC diet. |
| If your LDL level is 160 mg/dL or higher after 3 months on the TLC diet | You may need medicines along with the TLC diet. |
| If your LDL level is less than 160 mg/dL | You will need to follow a heart healthy diet. |
Lowering Cholesterol Using Therapeutic Lifestyle Changes
TLC is a set of lifestyle changes that can help you lower your LDL cholesterol. The main parts of the TLC program are a healthy diet, weight management, and physical activity.
The TLC Diet
With the TLC diet, less than 7 percent of your daily calories should come from saturated fat. This kind of fat is found in some meats, dairy products, chocolate, baked goods, and deep-fried and processed foods.
No more than 25 to 35 percent of your daily calories should come from all fats, including saturated, trans, monounsaturated, and polyunsaturated fats.
You also should have less than 200 mg a day of cholesterol. The amounts of cholesterol and the types of fat in prepared foods can be found on the foods' Nutrition Facts labels.
Foods high in soluble fiber also are part of the TLC diet. They help prevent the digestive tract from absorbing cholesterol. These foods include:
Whole-grain cereals such as oatmeal and oat bran
Fruits such as apples, bananas, oranges, pears, and prunes
Legumes such as
kidney beans, lentils, chick peas, black-eyed peas, and lima beans
A diet rich in fruits and vegetables can increase important cholesterol-lowering compounds in your diet. These compounds, called plant stanols or sterols, work like soluble fiber.
A healthy diet also includes some types of fish, such as salmon, tuna (canned or fresh), and mackerel. These fish are a good source of omega-3 fatty acids. These acids may help protect the heart from blood clots and inflammation and reduce the risk of heart attack. Try to have about two fish meals every week.
You also should try to limit the amount of sodium (salt) that you eat. This means choosing low-salt and ""no added salt"" foods and seasonings at the table or while cooking. The Nutrition Facts label on food packaging shows the amount of sodium in the item.
Try to limit drinks with alcohol. Too much alcohol will raise your blood pressure and triglyceride level. (Triglycerides are a type of fat found in the blood.) Alcohol also adds extra calories, which will cause weight gain.
Men should have no more than two drinks containing alcohol a day. Women should have no more than one drink containing alcohol a day. One drink is a glass of wine, beer, or a small amount of hard liquor.
For more information about TLC, go to the National Heart, Lung, and Blood Institute’s (NHLBI’s) ""Your Guide to Lowering Your Cholesterol With TLC.""
Cholesterol-Lowering Medicines
In addition to lifestyle changes, your doctor may prescribe medicines to help lower your cholesterol. Even with medicines, you should continue the TLC program.
Medicines can help control high blood cholesterol, but they don’t cure it. Thus, you must continue taking your medicine to keep your cholesterol level in the recommended range.
The five major types of cholesterol-lowering medicines are statins, bile acid sequestrants (seh-KWES-trants), nicotinic (nick-o-TIN-ick) acid, fibrates, and ezetimibe.
Statins work well at lowering
LDL cholesterol. These medicines are safe for most people. Rare side effects include
muscle and
liver problems.
Bile acid sequestrants also help lower
LDL cholesterol. These medicines usually aren’t prescribed as the only medicine to lower cholesterol. Sometimes they’re prescribed with statins.
While you’re being treated for high blood cholesterol, you’ll need ongoing care. Your doctor will want to make sure your cholesterol levels are controlled. He or she also will want to check for other health problems.
If needed, your doctor may prescribe medicines for other health problems. Take all medicines exactly as your doctor prescribes. The combination of medicines may lower your risk for heart disease and heart attack.
While trying to manage your Go to:
What Is High Blood Pressure?
High blood pressure (HBP) is a serious condition that can lead to coronary heart disease, heart failure, stroke, kidney failure, and other health problems.
""Blood pressure"" is the force of blood pushing against the walls of the arteries as the heart pumps blood. If this pressure rises and stays high over time, it can damage the body in many ways.
Overview
About 1 in 3 adults in the United States has HBP. The condition itself usually has no signs or symptoms. You can have it for years without knowing it. During this time, though, HBP can damage your heart, blood vessels, kidneys, and other parts of your body.
Knowing your blood pressure numbers is important, even when you're feeling fine. If your blood pressure is normal, you can work with your health care team to keep it that way. If your blood pressure is too high, treatment may help prevent damage to your body's organs.
Blood Pressure Numbers
Blood pressure is measured as systolic (sis-TOL-ik) and diastolic (di-ah-STOL-ik) pressures. ""Systolic"" refers to blood pressure when the heart beats while pumping blood. ""Diastolic"" refers to blood pressure when the heart is at rest between beats.
You most often will see blood pressure numbers written with the systolic number above or before the diastolic number, such as 120/80 mmHg. (The mmHg is millimeters of mercury—the units used to measure blood pressure.)
The table below shows normal blood pressure numbers for adults. It also shows which numbers put you at greater risk for health problems.
Categories for Blood Pressure Levels in Adults (measured in millimeters of mercury, or mmHg)
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| Category | Systolic (top number) | | Diastolic (bottom number) |
|---|
| Normal | Less than 120 | And | Less than 80 |
|---|
| Prehypertension | 120–139 | Or | 80–89 |
|---|
| High blood pressure | | | |
|---|
| Stage 1 | 140–159 | Or | 90–99 |
|---|
| Stage 2 | 160 or higher | Or | 100 or higher |
|---|
The ranges in the table apply to most adults (aged 18 and older) who don't have short-term serious illnesses.
Blood pressure doesn't stay the same all the time. It lowers as you sleep and rises when you wake up. Blood pressure also rises when you're excited, nervous, or active. If your numbers stay above normal most of the time, you're at risk for health problems. The risk grows as blood pressure numbers rise. ""Prehypertension"" means you may end up with HBP, unless you take steps to prevent it.
If you're being treated for HBP and have repeat readings in the normal range, your blood pressure is under control. However, you still have the condition. You should see your doctor and follow your treatment plan to keep your blood pressure under control.
Your systolic and diastolic numbers may not be in the same blood pressure category. In this case, the more severe category is the one you're in. For example, if your systolic number is 160 and your diastolic number is 80, you have stage 2 HBP. If your systolic number is 120 and your diastolic number is 95, you have stage 1 HBP.
If you have diabetes or chronic kidney disease, HBP is defined as 130/80 mmHg or higher. HBP numbers also differ for children and teens. (For more information, go to ""How Is High Blood Pressure Diagnosed?"")
Outlook
Blood pressure tends to rise with age. Following a healthy lifestyle helps some people delay or prevent this rise in blood pressure.
People who have HBP can take steps to control it and reduce their risk for related health problems. Key steps include following a healthy lifestyle, having ongoing medical care, and following your treatment plan.
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The image focuses on high blood pressure in women and explains how high blood pressure increases the risk of heart disease. An estimated 1 in 3 women has high blood pressure, and the condition is dangerous because it often causes no symptoms. The image (more...)
Other Names for High Blood Pressure
High blood pressure (HBP) also is called hypertension (HI-per-TEN-shun).
When HBP has no known cause, it might be called essential hypertension, primary hypertension, or idiopathic (id-ee-o-PATH-ick) hypertension.
When another condition causes HBP, it's sometimes called secondary hypertension.
Some people only have high systolic blood pressure. This condition is called isolated systolic hypertension (ISH). Many older adults have this condition. ISH can cause as much harm as HBP in which both numbers are too high.
What Causes High Blood Pressure?
Blood pressure tends to rise with age, unless you take steps to prevent or control it.
Some medical problems—such as chronic kidney disease, thyroid disease, and sleep apnea—may cause blood pressure to rise. Some medicines also may raise your blood pressure. Examples include asthma medicines (for example, corticosteroids) and cold-relief products.
Other medicines also can cause high blood pressure (HBP). If you have HBP, let your doctor know about all of the medicines you take, including over-the-counter products.
In some women, birth control pills, pregnancy, or hormone therapy (HT) may cause blood pressure to rise.
Women taking birth control pills usually have a small rise in both systolic and diastolic blood pressures. If you already have HBP and want to use birth control pills, make sure your doctor knows about your HBP. Talk with him or her about how often you should have your blood pressure checked and how to control it while taking the pill.
Taking HT to reduce the symptoms of menopause can cause a small rise in systolic blood pressure. If you already have HBP and want to start using HT, talk with your doctor about the risks and benefits. If you decide to take hormones, find out how to control your blood pressure and how often you should have it checked.
Children younger than 10 years old who have HBP often have another condition that's causing it (such as kidney disease). Treating the underlying condition may resolve the HBP.
The older a child is when HBP is diagnosed, the more likely he or she is to have essential hypertension. This means that doctors don't know what's causing the HBP.
Who Is at Risk for High Blood Pressure?
High blood pressure (HBP) is a common condition. In the United States, about 1 in 3 adults has HBP.
Certain traits, conditions, and habits can raise your risk for HBP. The major risk factors for HBP are described below.
Older Age
Blood pressure tends to rise with age. About 65 percent of Americans aged 60 or older have HBP.
Isolated systolic hypertension (ISH) is the most common form of HBP in older adults. ISH occurs when only systolic blood pressure (the top number) is high. About 2 out of 3 people over age 60 with HBP have ISH.
HBP doesn't have to be a routine part of aging. You can take steps to keep your blood pressure at a normal level. (For more information, go to ""How Is High Blood Pressure Treated?"")
Race/Ethnicity
HBP can affect anyone. However, it's more common in African American adults than in Caucasian or Hispanic American adults. In relation to these groups, African Americans:
HBP risks vary among different groups of Hispanic American adults. For instance, Puerto Rican American adults have higher rates of HBP-related death than all other Hispanic groups and Caucasians. However, Cuban Americans have lower rates of HBP-related death than Caucasians.
Overweight or Obesity
You're more likely to develop prehypertension or HBP if you're overweight or obese. The terms ""overweight"" and ""obesity"" refer to body weight that's greater than what is considered healthy for a certain height.
Gender
Men and women are equally likely to develop HBP during their lifetimes. However, before age 45, men are more likely to have HBP than women. After age 65, the condition is more likely to affect women than men.
Also, men younger than 55 are more likely to have uncontrolled HBP than women. However, after age 65, women are more likely to have uncontrolled HBP.
Unhealthy Lifestyle Habits
Many unhealthy lifestyle habits can raise your risk for HBP, including:
Other Risk Factors
A family history of HBP raises your risk for the condition. Long-lasting stress also can put you at risk for HBP.
You're also more likely to develop HBP if you have prehypertension. Prehypertension means that your blood pressure is in the 120–139/80–89 mmHg range.
Risk Factors for Children and Teens
Prehypertension and HBP are becoming more common in children and teens. This is due in part to a rise in overweight and obesity among children and teens.
African American and Mexican American youth are more likely to have HBP and prehypertension than Caucasian youth. Also, boys are at higher risk for HBP than girls.
Like adults, children and teens need to have routine blood pressure checks, especially if they're overweight.
What Are the Signs and Symptoms of High Blood Pressure?
High blood pressure (HBP) itself usually has no signs or symptoms. Rarely, headaches may occur.
You can have HBP for years without knowing it. During this time, the condition can damage your heart, blood vessels, kidneys, and other parts of your body.
Some people only learn that they have HBP after the damage has caused problems, such as coronary heart disease, stroke, or kidney failure.
Knowing your blood pressure numbers is important, even when you're feeling fine. If your blood pressure is normal, you can work with your health care team to keep it that way. If your blood pressure is too high, you can take steps to lower it. Lowering your blood pressure will help reduce your risk for related health problems.
Complications of High Blood Pressure
When blood pressure stays high over time, it can damage the body. HBP can cause:
Blood vessels in the
eyes to burst or bleed. This may lead to vision changes or blindness.
How Is High Blood Pressure Diagnosed?
High blood pressure (HBP) is diagnosed using a blood pressure test. This test will be done several times to make sure the results are correct. If your numbers are high, your doctor may have you return for repeat tests to check your blood pressure over time.
If your blood pressure is 140/90 mmHg or higher over time, your doctor will likely diagnose you with HBP. If you have diabetes or chronic kidney disease, a blood pressure of 130/80 mmHg or higher is considered HBP.
The ranges for HBP in children are different, as discussed below.
How Is Blood Pressure Tested?
A blood pressure test is easy and painless. This test is done at a doctor's office or clinic.
To prepare for the test:
Don't drink coffee or smoke cigarettes for 30 minutes prior to the test. These actions may cause a short-term rise in your
blood pressure.
Sit for 5 minutes before the test. Movement can cause short-term rises in
blood pressure.
To measure your blood pressure, your doctor or nurse will use some type of a gauge, a stethoscope (or electronic sensor), and a blood pressure cuff.
Most often, you will sit or lie down with the cuff around your arm as your doctor or nurse checks your blood pressure. If he or she doesn't tell you what your blood pressure numbers are, you should ask.
Diagnosing High Blood Pressure in Children and Teens
Doctors measure blood pressure in children and teens the same way they do in adults. Your child should have routine blood pressure checks starting at 3 years of age.
Blood pressure normally rises with age and body size. Newborn babies often have very low blood pressure numbers, while older teens have numbers similar to adults.
The ranges for normal blood pressure and HBP generally are lower for youth than for adults. To find out whether a child has HBP, a doctor will compare the child's blood pressure numbers to average numbers for his or her age, gender, and height.
For more information, go to the National Heart, Lung, and Blood Institute's ""A Pocket Guide to Blood Pressure Measurement in Children.""
What Does a Diagnosis of High Blood Pressure Mean?
If you're diagnosed with HBP, your doctor will prescribe treatment. Your blood pressure will be tested again to see how the treatment affects it.
Once your blood pressure is under control, you'll still need treatment. ""Under control"" means that your blood pressure numbers are in the normal range. Your doctor will likely recommend routine blood pressure tests. He or she can tell you how often you should be tested.
The sooner you find out about HBP and treat it, the better. Early treatment may help you avoid problems such as heart attack, stroke, and kidney failure.
How Is High Blood Pressure Treated?
High blood pressure (HBP) is treated with lifestyle changes and medicines.
Most people who have HBP will need lifelong treatment. Sticking to your treatment plan is important. It can help prevent or delay problems related to HBP and help you live and stay active longer.
For more tips on controlling your blood pressure, go to the National Heart, Lung, and Blood Institute's (NHLBI's) ""Your Guide to Lowering Blood Pressure.""
Lifestyle Changes
Healthy lifestyle habits can help you control HBP. These habits include:
If you combine healthy lifestyle habits, you can achieve even better results than taking single steps.
You may find it hard to make lifestyle changes. Start by making one healthy lifestyle change and then adopt others.
Some people can control their blood pressure with lifestyle changes alone, but many people can't. Keep in mind that the main goal is blood pressure control.
If your doctor prescribes medicines as a part of your treatment plan, keep up your healthy lifestyle habits. They will help you better control your blood pressure.
Following a Healthy Diet
Your doctor may recommend the DASH (Dietary Approaches to Stop Hypertension) eating plan if you have HBP. The DASH eating plan focuses on fruits, vegetables, whole grains, and other foods that are heart healthy and low in fat, cholesterol, and sodium (salt).
DASH also focuses on fat-free or low-fat dairy products, fish, poultry, and nuts. The DASH eating plan is reduced in red meats (including lean red meats), sweets, added sugars, and sugar-containing beverages. It's rich in nutrients, protein, and fiber.
To help control HBP, you should limit the amount of salt that you eat. This means choosing low-sodium and no added salt foods and seasonings at the table and while cooking. The Nutrition Facts label on food packaging shows the amount of sodium in an item. You should eat no more than about 1 teaspoon of salt a day.
Also, try to limit alcoholic drinks. Too much alcohol will raise your blood pressure. Men should have no more than two alcoholic drinks a day. Women should have no more than one alcoholic drink a day. One drink is a glass of wine, beer, or a small amount of hard liquor.
For more information, go to the NHLBI's ""Your Guide to Lowering Your Blood Pressure With DASH.""
Being Physically Active
Routine physical activity can lower HBP and reduce your risk for other health problems. Talk with your doctor before you start a new exercise plan. Ask him or her how much and what kinds of physical activity are safe for you.
People gain health benefits from as little as 60 minutes of moderate-intensity aerobic activity per week. The more active you are, the more you will benefit.
For more information about physical activity, go to the U.S. Department of Health and Human Services' ""2008 Physical Activity Guidelines for Americans,"" the Health Topics Physical Activity and Your Heart article, and the NHLBI's ""Your Guide to Physical Activity and Your Heart.""
Maintaining a Healthy Weight
Maintaining a healthy weight can help you control HBP and reduce your risk for other health problems.
If you're overweight or obese, aim to reduce your weight by 5 to 10 percent during your first year of treatment. This amount of weight loss can lower your risk for health problems related to HBP.
To lose weight, cut back your calorie intake and do more physical activity. Eat smaller portions and choose lower calorie foods. Don't feel that you have to finish the entrees served at restaurants. Many restaurant portions are oversized and have too many calories for the average person.
After your first year of treatment, you may have to continue to lose weight so you can lower your body mass index (BMI) to less than 25. BMI measures your weight in relation to your height and gives an estimate of your total body fat.
A BMI between 25 and 29.9 is considered overweight. A BMI of 30 or more is considered obese. A BMI of less than 25 is the goal for controlling blood pressure.
You can use the NHLBI's online BMI calculator to figure out your BMI, or your doctor can help you.
For more information about losing weight and keeping it off, go to the Health Topics Overweight and Obesity article.
Quit Smoking
If you smoke or use tobacco, quit. Smoking can damage your blood vessels and raise your risk for HBP. Smoking also can worsen health problems related to HBP.
Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article and the NHLBI's ""Your Guide to a Healthy Heart.""
Managing Stress
Learning how to manage stress, relax, and cope with problems can improve your emotional and physical health.
Physical activity helps some people cope with stress. Other people listen to music or focus on something calm or peaceful to reduce stress. Some people learn yoga, tai chi, or how to meditate.
Medicines
Today's blood pressure medicines can safely help most people control their blood pressure. These medicines are easy to take. The side effects, if any, tend to be minor.
If you have side effects from your medicines, talk with your doctor. He or she might adjust the doses or prescribe other medicines. You shouldn't decide on your own to stop taking your medicines.
Blood pressure medicines work in different ways to lower blood pressure. Some remove extra fluid and salt from the body to lower blood pressure. Others slow down the heartbeat or relax and widen blood vessels. Often, two or more medicines work better than one.
Diuretics
Diuretics sometimes are called water pills. They help your kidneys flush excess water and salt from your body. This reduces the amount of fluid in your blood, and your blood pressure goes down.
Diuretics often are used with other HBP medicines and sometimes combined into one pill.
Treatment for Children and Teens
If another condition is causing your child's HBP, treating it often resolves the HBP. When the cause of a child or teen's HBP isn't known, the first line of treatment is lifestyle changes (as it is for adults).
If lifestyle changes don't control blood pressure, children and teens also may need to take medicines. Most of the medicines listed above for adults have special doses for children.
How Can High Blood Pressure Be Prevented?
If You Have Normal Blood Pressure
If you don't have high blood pressure (HBP), you can take steps to prevent it. Healthy lifestyle habits can help you maintain normal blood pressure.
Many people who adopt these healthy lifestyle habits are able to prevent or delay HBP. The more lifestyle changes you make, the more likely you are to lower your blood pressure and avoid related health problems.
For more information about healthy lifestyle habits, go to ""How Is High Blood Pressure Treated?""
If You Have High Blood Pressure
If you have HBP, you can still take steps to prevent the long-term problems it can cause. Healthy lifestyle habits (listed above) and medicines can help you live a longer, more active life.
Follow the treatment plan your doctor prescribes to control your blood pressure. Treatment can help you prevent or delay coronary heart disease, stroke, kidney disease, and other health problems.
Children and Teens
A healthy lifestyle also can help prevent HBP in children and teens. Key steps include having a child:
Follow a healthy diet that focuses on plenty of fruits, vegetables, and, for children older than 4 years old, low-
fat dairy products. A healthy diet also is low in saturated and
trans fats and salt.
Be active for at least 1 to 2 hours per day. Limit screen time in
front of the TV or computer to 2 hours per day at most.
Maintain a healthy weight. If your child is
overweight, ask his or her doctor about how your child can safely lose weight.
Make these healthy habits part of a family health plan to help your child adopt and maintain a healthy lifestyle.
Living With High Blood Pressure
If you have high blood pressure (HBP), you'll need to treat and control it for life. This means making lifestyle changes, taking prescribed medicines, and getting ongoing medical care.
Treatment can help control blood pressure, but it will not cure HBP. If you stop treatment, your blood pressure and risk for related health problems will rise.
For a healthy future, follow your treatment plan closely. Work with your health care team for lifelong blood pressure control.
Medicines
Take all blood pressure medicines that your doctor prescribes. Know the names and doses of your medicines and how to take them. If you have questions about your medicines, talk with your doctor or pharmacist.
Make sure you refill your medicines before they run out. Take your medicines exactly as your doctor directs—don't skip days or cut pills in half.
If you're having side effects from your medicines, talk with your doctor. He or she may need to adjust the doses or prescribe other medicines. You shouldn't decide on your own to stop taking your medicines.
Ongoing Care
If you have HBP, have medical checkups or tests as your doctor advises. Your doctor may need to change or add medicines to your treatment plan over time. Routine checkups allow your doctor to change your treatment right away if your blood pressure goes up again.
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46,"2018-02-02 05:15:51","Holes in the Heart","
What Are Holes in the Heart?
Holes in the heart are simple congenital (kon-JEN-ih-tal) heart defects. Congenital heart defects are problems with the heart's structure that are present at birth. These defects change the normal flow of blood through the heart.
The heart has two sides, separated by an inner wall called the septum. With each heartbeat, the right side of the heart receives oxygen-poor blood from the body and pumps it to the lungs. The left side of the heart receives oxygen-rich blood from the lungs and pumps it to the body.
The septum prevents mixing of blood between the two sides of the heart. However, some babies are born with holes in the upper or lower septum.
A hole in the septum between the heart's two upper chambers is called an atrial septal defect (ASD). A hole in the septum between the heart's two lower chambers is called a ventricular septal defect (VSD).
ASDs and VSDs allow blood to pass from the left side of the heart to the right side. Thus, oxygen-rich blood mixes with oxygen-poor blood. As a result, some oxygen-rich blood is pumped to the lungs instead of the body.
Over the past few decades, the diagnosis and treatment of ASDs and VSDs have greatly improved. Children who have simple congenital heart defects can survive to adulthood. They can live normal, active lives because their heart defects close on their own or have been repaired.
How the Heart Works
To understand holes in the heart, it's helpful to know how a healthy heart works. Your child's heart is a muscle about the size of his or her fist. The heart works like a pump and beats 100,000 times a day.
The heart has two sides, separated by an inner wall called the septum. The right side of the heart pumps blood to the lungs to pick up oxygen. The left side of the heart receives the oxygen-rich blood from the lungs and pumps it to the body.
The heart has four chambers and four valves and is connected to various blood vessels. Veins are blood vessels that carry blood from the body to the heart. Arteries are blood vessels that carry blood away from the heart to the body.
The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body.
Heart Valves
Four valves control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart.
The tricuspid (tri-CUSS-
pid) valve is in the right side of the
heart, between the right atrium and the right
ventricle.
The mitral (MI-trul) valve is in the left side of the
heart, between the left atrium and the left
ventricle.
The aortic (ay-OR-tik) valve is in the left side of the
heart, between the left
ventricle and the entrance to the
aorta. This
artery carries
blood from the heart to the body.
Valves are like doors that open and close. They open to allow blood to flow through to the next chamber or to one of the arteries. Then they shut to keep blood from flowing backward.
When the heart's valves open and close, they make a ""lub-DUB"" sound that a doctor can hear using a stethoscope.
The first sound—the ""lub""—is made by the mitral and tricuspid valves closing at the beginning of systole (SIS-toe-lee). Systole is when the
ventricles contract, or squeeze, and pump
blood out of the
heart.
The second sound—the ""DUB""—is made by the aortic and
pulmonary valves closing at the beginning of diastole (di-AS-toe-lee). Diastole is when the
ventricles relax and fill with
blood pumped into them by the
atria.
Veins
The veins also are major blood vessels connected to your heart.
For more information about how a healthy heart works, go to the Health Topics How the Heart Works article. This article contains animations that show how your heart pumps blood and how your heart's electrical system works.
Types of Holes in the Heart
Atrial Septal Defect
An atrial septal defect (ASD) is a hole in the part of the septum that separates the atria. (The atria are the upper chambers of the heart.)
An ASD allows oxygen-rich blood to flow from the left atrium into the right atrium, instead of flowing into the left ventricle as it should. So, instead of going to the body, the oxygen-rich blood is pumped back to the lungs, where it has just been.
Figure A shows the structure and blood flow inside a normal heart. Figure B shows a heart with an atrial septal defect. The hole allows oxygen-rich blood from the left atrium to mix with oxygen-poor blood from the right atrium.
An ASD can be small, medium, or large. Small ASDs allow only a little blood to flow from one atrium to the other. Small ASDs don't affect how the heart works and don't need any special treatment. Many small ASDs close on their own as the heart grows during childhood.
Medium and large ASDs allow more blood to leak from one atrium to the other. They're less likely to close on their own.
Most children who have ASDs have no symptoms, even if they have large ASDs.
The three major types of ASDs are:
Secundum. This defect is in the middle of the
atrial septum and is the most common form of
ASD. About 8 out of every 10 babies born with ASDs have secundum defects. At least half of all secundum ASDs close on their own. However, this is less likely if the defect is large.
Primum. This defect is in the lower part of the
atrial septum. Primum defects often occur with
heart valve problems. These defects aren't very common, and they don't close on their own.
Sinus venosus. This defect is in the upper part of the
atrial septum. It's close to where a large
vein (the
superior vena cava) brings
oxygen-poor
blood from the upper body to the right atrium. Sinus venosus defects are rare, and they don't close on their own.
Atrial Septal Defect Complications
If an ASD isn't repaired, the extra blood flow to the right side of the heart and lungs may cause heart problems. Most of these problems don't occur until adulthood, often around age 30 or later.
Possible complications include:
Right heart failure. An
ASD causes the right side of the
heart to work harder because it has to pump extra
blood to the
lungs. Over time, the heart may become tired from this extra work and not pump well.
Stroke. Usually, the
lungs filter out small
blood clots that can form on the right side of the
heart. Sometimes, though, a blood clot can pass from the right atrium to the left atrium through an
ASD and be pumped out to the body. The clot can travel to an
artery in the
brain, block blood flow, and cause a
stroke.
These problems develop over many years and rarely occur in infants and children. They also are rare in adults because most ASDs close on their own or are repaired in early childhood.
Ventricular Septal Defect
A ventricular septal defect (VSD) is a hole in the part of the septum that separates the ventricles. (The ventricles are the lower chambers of the heart.)
A VSD allows oxygen-rich blood to flow from the left ventricle into the right ventricle, instead of flowing into the aorta as it should. So, instead of going to the body, the oxygen-rich blood is pumped back to the lungs, where it has just been.
Figure A shows the structure and blood flow inside a normal heart. Figure B shows two common locations for a ventricular septal defect. The defect allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood in the right ventricle.
An infant who is born with a VSD may have one or more holes in the wall that separates the two ventricles. The defect also may occur alone or with other congenital heart defects.
Doctors will classify a VSD based on the:
Size of the defect.
Location of the defect.
Number of defects.
Presence or absence of a ventricular septal
aneurysm—a thin flap of
tissue on the
septum. This tissue is harmless and can help a VSD close on its own.
VSDs can be small, medium, or large. Small VSDs don't cause problems and may close on their own. Small VSDs sometimes are called restrictive VSDs because they allow only a small amount of blood to flow between the ventricles. Small VSDs don't cause any symptoms.
Medium VSDs are less likely to close on their own. They may cause symptoms in infants and children. Surgery may be needed to close medium VSDs.
Large VSDs allow a lot of blood to flow from the left ventricle to the right ventricle. They're sometimes called nonrestrictive VSDs. Large VSDs likely won't close completely on their own, but they may get smaller over time.
Large VSDs often cause symptoms in infants and children. Surgery usually is needed to close large VSDs.
VSDs are found in different parts of the septum.
Membranous VSDs are located near the
heart valves. These VSDs can close at any time.
Muscular VSDs are found in the lower part of the
septum. They're surrounded by
muscle, and most close on their own during early childhood.
Inlet VSDs are located close to where
blood enters the
ventricles. They're less common than membranous and muscular VSDs.
Outlet VSDs are found in the part of the
ventricle where
blood leaves the
heart. These are the rarest type of VSD.
Ventricular Septal Defect Complications
Over time, if a VSD isn't repaired, it may cause heart problems. A medium or large VSD can cause:
Heart failure. Infants who have large VSDs may develop heart failure. This is because the left side of the heart pumps
blood into the right
ventricle in addition to its normal work of pumping blood to the body. The increased workload on the heart also increases the
heart rate and the body's demand for energy.
Growth failure, especially in infants. A baby may not be able to eat enough to keep up with his or her body's increased energy demands. As a result, the baby may lose weight or not grow and develop normally.
What Are the Signs and Symptoms of Holes in the Heart?
Atrial Septal Defect
Many babies who are born with atrial septal defects (ASDs) have no signs or symptoms. However, as they grow, these children may be small for their age.
When signs and symptoms do occur, a heart murmur is the most common. A heart murmur is an extra or unusual sound heard during a heartbeat.
Often, a heart murmur is the only sign of an ASD. However, not all murmurs are signs of congenital heart defects. Many healthy children have heart murmurs. Doctors can listen to heart murmurs and tell whether they're harmless or signs of heart problems.
If a large ASD isn't repaired, the extra blood flow to the right side of the heart can damage the heart and lungs and cause heart failure. This generally doesn't occur until adulthood. Signs and symptoms of heart failure include:
Ventricular Septal Defect
Babies born with ventricular septal defects (VSDs) usually have heart murmurs. Murmurs may be the first and only sign of a VSD. Heart murmurs often are present right after birth in many infants. However, the murmurs may not be heard until the babies are 6 to 8 weeks old.
Most newborns who have VSDs don't have heart-related symptoms. However, babies who have medium or large VSDs can develop heart failure. Signs and symptoms of heart failure usually occur during the baby's first 2 months of life.
The signs and symptoms of heart failure due to VSD are similar to those listed above for ASD, but they occur in infancy.
A major sign of heart failure in infancy is poor feeding and growth. VSD signs and symptoms are rare after infancy. This is because the defects either decrease in size on their own or they're repaired.
How Are Holes in the Heart Diagnosed?
Doctors usually diagnose holes in the heart based on a physical exam and the results from tests and procedures. The exam findings for an atrial septal defect (ASD) often aren't obvious. Thus, the diagnosis sometimes isn't made until later in childhood or even in adulthood.
Ventricular septal defects (VSDs) cause a very distinct heart murmur. Because of this, a diagnosis usually is made in infancy.
Specialists Involved
Doctors who specialize in diagnosing and treating heart problems are called cardiologists. Pediatric cardiologists take care of babies and children who have heart problems. Cardiac surgeons repair heart defects using surgery.
Diagnostic Tests and Procedures
Your child's doctor may recommend several tests to diagnose an ASD or VSD. These tests also will help the doctor figure out the location and size of the defect.
Echocardiography
Echocardiography (echo) is a painless test that uses sound waves to create a moving picture of the heart. The sound waves (called ultrasound) bounce off the structures of the heart. A computer converts the sound waves into pictures on a screen.
Echo allows the doctor to clearly see any problem with the way the heart is formed or the way it's working.
Echo is an important test for both diagnosing a hole in the heart and following the problem over time. Echo can show problems with the heart's structure and how the heart is reacting to the problems. This test will help your child's cardiologist decide whether and when treatment is needed.
EKG (Electrocardiogram)
An EKG is a simple, painless test that records the heart's electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). It also records the strength and timing of electrical signals as they pass through the heart.
An EKG can detect whether one of the heart's chambers is enlarged, which can help diagnose a heart problem.
Pulse Oximetry
Pulse oximetry shows the level of oxygen in the blood. A small sensor is attached to a finger or ear. The sensor uses light to estimate how much oxygen is in the blood.
How Are Holes in the Heart Treated?
Many holes in the heart don't need treatment, but some do. Those that do often are repaired during infancy or early childhood. Sometimes adults are treated for holes in the heart if problems develop.
The treatment your child receives depends on the type, location, and size of the hole. Other factors include your child's age, size, and general health.
Treating an Atrial Septal Defect
If a child has an atrial septal defect (ASD), routine checkups are done to see whether it closes on its own. About half of all ASDs close on their own over time, and about 20 percent close within the first year of life.
Your child's doctor will let you know how often your child should have checkups. For an ASD, frequent checkups aren't needed.
If an ASD requires treatment, catheter or surgical procedures are used to close the hole. Doctors often decide to close ASDs in children who still have medium- or large-sized holes by the time they're 2 to 5 years old.
Catheter Procedure
Until the early 1990s, surgery was the usual method for closing all ASDs. Now, thanks to medical advances, doctors can use catheter procedures to close secundum ASDs. These are the most common type of ASD.
Before a catheter procedure, your child is given medicine so he or she will sleep and not feel any pain. Then, the doctor inserts a catheter (a thin, flexible tube) into a vein in the groin (upper thigh). He or she threads the tube to the Go to:
What Is Hypersensitivity Pneumonitis?
Hypersensitivity pneumonitis (noo-mo-NI-tis), or HP, is a disease in which the lungs become inflamed from breathing in foreign substances, such as molds, dusts, and chemicals. These substances also are known as antigens (AN-tih-jens).
People are exposed to antigens at home, while at work, and in other settings. However, most people who breathe in these substances don't develop HP.
Overview
To understand HP, it helps to understand how the lungs work. When you breathe, air passes through your nose and mouth into your windpipe. The air then travels to your lungs' air sacs. These sacs are called alveoli (al-VEE-uhl-eye).
Small blood vessels called capillaries run through the walls of the air sacs. When air reaches the air sacs, the oxygen in the air passes through the air sac walls into the blood in the capillaries. The capillaries connect to a network of arteries and veins that move blood through your body.
In HP, the air sacs become inflamed and may fill with fluid. This makes it harder for oxygen to pass through the air sacs and into the bloodstream.
The two main types of HP are acute (short-term) and chronic (ongoing). Both types can develop as a result of repeatedly breathing in an antigen.
Over time, your lungs can become sensitive to that antigen. If this happens, they'll become inflamed, which can lead to symptoms and may even cause long-term lung damage.
With acute HP, symptoms usually occur within 2–9 hours of exposure to an antigen you're sensitive to. Acute HP can cause chills, body aches, coughing, and chest tightness. After hours or days of no contact with the antigen, symptoms usually go away.
If acute HP isn't found and treated early, chronic HP may develop. Symptoms of chronic HP occur slowly, over months. Chronic HP can cause a worsening cough, shortness of breath with physical activity, fatigue (tiredness), and weight loss. Severe HP may cause clubbing (a widening and rounding of the tips of the fingers or toes).
With chronic HP, symptoms may continue and/or worsen, even after avoiding the antigen. Sometimes, chronic HP can cause long-term lung damage, such as pulmonary fibrosis (PULL-mun-ary fi-BRO-sis). This is a condition in which tissue deep in your lungs becomes scarred over time.
Outlook
Avoiding or reducing your contact with antigens can help prevent and treat HP. For example, cleaning heating and ventilation filters can help reduce your contact with mold. Wetting compost prior to handling it can reduce contact with harmful dust.
If HP is caught early, avoiding the antigen that caused it may be the only treatment you need. If you have chronic HP, your doctor may prescribe medicines to reduce lung inflammation.
Researchers continue to study why some people develop HP after being exposed to antigens, while others don't. They're also looking for better ways to quickly pinpoint which antigens are causing HP in people who are believed to have the disease.
Other Names for Hypersensitivity Pneumonitis
What Causes Hypersensitivity Pneumonitis?
Repeatedly breathing in foreign substances can cause hypersensitivity pneumonitis (HP). Examples of these substances include molds, dusts, and chemicals. (Mold often is the cause of HP.) These substances also are known as antigens.
Over time, your lungs can become sensitive to antigens. If this happens, your lungs will become inflamed, which can lead to symptoms and may even cause long-term lung damage.
Antigens may be found in the home, workplace, or in other settings. Antigens can come from many sources, such as:
Bird droppings
Humidifiers, heating systems, and hot tubs
Liquid chemicals used in the landscaping and florist industries
Moldy hay, straw, and grain
Chemicals released during the production of plastics and electronics, and chemicals released during painting
Mold released during lumber milling, construction, and wood stripping
Who Is At Risk for Hypersensitivity Pneumonitis?
People who repeatedly breathe in foreign substances are at risk for hypersensitivity pneumonitis (HP). These substances, which also are known as antigens, include molds, dusts, and chemicals. However, most people who breathe in these substances don't develop HP.
People at increased risk include:
Farm and dairy cattle workers
People who use hot tubs often
People who are exposed to
molds or dusts from humidifiers, heating systems, or wet carpeting
Bird fanciers (people who keep pet birds) and poultry handlers
Florists and landscapers, especially those who use liquid chemicals on lawns and gardens
People who work in grain and flour processing and loading
Lumber milling, construction, wood stripping, and paper and wallboard workers
People who make plastics or electronics, and those who paint or work with other chemicals
What Are the Signs and Symptoms of Hypersensitivity Pneumonitis?
Signs and symptoms of hypersensitivity pneumonitis (HP) depend on whether the disease is acute (short-term) or chronic (ongoing).
Acute Hypersensitivity Pneumonitis
With acute HP, symptoms usually occur within 2–9 hours of exposure to an antigen you're sensitive to. (An antigen is a substance that your body reacts against, such as molds, dusts, and chemicals.)
Acute HP can cause chills, body aches, coughing, and chest tightness. After hours or days of no contact with the antigen, symptoms usually go away.
Chronic Hypersensitivity Pneumonitis
If acute HP isn't found and treated early, chronic HP may develop. With chronic HP, symptoms occur slowly, over months. Chronic HP can cause a worsening cough, shortness of breath with physical activity, fatigue (tiredness), and weight loss.
Some symptoms may continue and/or worsen, even after avoiding the antigen. Chronic HP can cause long-term lung damage, such as pulmonary fibrosis. This is a condition in which tissue deep in your lungs becomes scarred over time.
Clubbing also may occur if HP is severe. Clubbing is the widening and rounding of the tips of the fingers or toes. A low level of oxygen in the blood causes this condition.
How Is Hypersensitivity Pneumonitis Diagnosed?
To diagnose hypersensitivity pneumonitis (HP), your doctor must pinpoint the antigen that's causing the disease and its source. (An antigen is a substance that your body reacts against, such as molds, dusts, and chemicals.)
Your doctor will ask you detailed questions about:
Your current and past jobs
Your hobbies and leisure activities
The types of places where you spend time
Your exposure to damp and moldy places
Your doctor also will do a physical exam and look at test results to diagnose HP.
Physical Exam
During the physical exam, your doctor will ask about your signs and symptoms, such as coughing and weight loss. Your doctor also will look for signs of HP. For example, he or she will listen to your lungs with a stethoscope for abnormal breathing sounds. HP can cause a crackling sound when you breathe.
Your doctor also may look for signs of pulmonary fibrosis, a possible complication of chronic (ongoing) HP. Pulmonary fibrosis is a condition in which tissue deep in your lungs becomes scarred over time.
Your doctor also may check for clubbing. Clubbing is the widening and rounding of the tips of the fingers or toes. A low level of oxygen in the blood causes this condition.
Diagnostic Tests and Procedures
To help diagnose HP, your doctor may recommend or more of the following tests or procedures.
Lung Function Tests
Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs can deliver oxygen to your blood. One of these tests is spirometry (spi-ROM-eh-tre).
During this test, a technician will ask you to take a deep breath. Then, you'll blow as hard as you can into a tube connected to a small machine. The machine is called a spirometer. The machine measures how much air you breathe out. It also measures how fast you can blow air out.
Pulse Oximetry
This test measures the amount of oxygen in your blood. A small sensor is attached to your finger or ear. The sensor uses light to estimate how much oxygen is in your blood.
Challenge Test
During this test, you're re-exposed to the suspected antigen. Then, you'll be watched for signs and symptoms of HP.
Bronchoscopy
For bronchoscopy (bron-KOS-ko-pee), your doctor passes a thin, flexible tube through your nose (or sometimes your mouth), down your throat, and into your airways. At the tip of the tube are a light and mini-camera. This allows your doctor to see your windpipe and airways.
Your doctor may insert forceps (a device used to grab or hold things) through the tube to collect a tissue sample. You'll be given medicine to make you relaxed and sleepy during the procedure.
Bronchoalveolar Lavage
During bronchoscopy, your doctor may inject a small amount of salt water (saline) through the tube into your lungs. This method is called bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH).
This fluid washes the lungs and helps bring up cells from the airways and the area around the air sacs. Your doctor will look at these cells under a microscope.
Surgical Lung Biopsy
To confirm a diagnosis of HP, your doctor may do a surgical lung biopsy. Your doctor can use a biopsy to rule out other causes of symptoms and check the condition of your lungs.
For a surgical lung biopsy, your doctor takes samples of lung tissue from several places in your lungs. He or she then looks at them under a microscope. Your doctor may use one of the following methods to get lung tissue samples.
Video-assisted thoracoscopy (thor-ah-KOS-ko-pee). For this procedure, your doctor inserts a small, lighted tube with a camera (endoscope) into your chest through small cuts between your ribs.
The endoscope provides a video image of your lungs and allows your doctor to collect tissue samples. This procedure is done in a hospital. You'll be given medicine to help you sleep through the procedure.
Thoracotomy (thor-ah-KOT-o-me). For this procedure, your doctor removes a few small pieces of lung tissue through a cut in the chest wall between your ribs. Thoracotomy is done in a hospital. You'll be given medicine to help you sleep through the procedure.
How Is Hypersensitivity Pneumonitis Treated?
The best way to treat hypersensitivity pneumonitis (HP) is to avoid the antigen that caused it. (An antigen is a substance that your body reacts against, such as molds, dusts, and chemicals.)
In acute (short-term) HP, symptoms usually go away once you're no longer in contact with the antigen. In chronic (ongoing) HP, you may need medicines to relieve your symptoms.
People who have chronic HP may develop pulmonary fibrosis. This is a condition in which tissue deep in your lungs becomes scarred over time. People who have this condition may need further treatment, such as oxygen therapy and pulmonary rehabilitation (rehab).
Avoiding Antigens
Once the antigen that caused the HP and its source are found, you can take steps to avoid it. If HP is caught early, avoiding the antigen may be the only treatment you need.
Avoiding an antigen may be easier at home than at work. For example, if your pet bird, moldy carpet, or hot tub is the source of the antigen, you can remove it from your home. If your heating system is the source of the antigen, you can have your system properly serviced.
However, if the antigen is at work, you may need to talk with your supervisor about your condition and ways to protect yourself. For example, masks or personal respirators may help protect you from antigens in the air. (A personal respirator is a device that helps filter the air you breathe in.)
Some people who have HP may need to move to a different home or change jobs to avoid antigens. After hurricanes, for example, some people have to move from their homes to avoid molds that could harm their lungs. However, moving and changing jobs sometimes isn't possible.
Medicines and Other Treatments
If you have chronic HP, your doctor may prescribe medicines called corticosteroids. These medicines reduce lung inflammation. Prednisone is an example of a corticosteroid.
Long-term use of prednisone, especially at high doses, can cause serious side effects. Thus, if your doctor prescribes this medicine, he or she may reduce the dose over time.
Examples of side effects from corticosteroids are increased risk of infections, high blood pressure, high blood sugar, and osteoporosis (thinning of the skin and bones).
People who develop pulmonary fibrosis may need medicines, oxygen therapy, and/or pulmonary rehab. Pulmonary fibrosis is a condition in which tissue deep in your lungs becomes scarred over time.
If you smoke, try to quit. Smoking can make HP symptoms worse and lead to other lung diseases. Talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke.
Living With Hypersensitivity Pneumonitis
If you've had hypersensitivity pneumonitis (HP), avoiding the antigen that caused it is important. (An antigen is a substance that your body reacts against, such as molds, dusts, and chemicals.)
If HP is caught early, your symptoms will likely go away if you avoid contact with the antigen. Continued contact with the antigen can make your symptoms worse and may lead to long-term lung damage.
To avoid the antigen, you may need to find other hobbies, change jobs, move, or use protective gear (like a mask) at work.
If you smoke, try to quit. Smoking can make HP symptoms worse and lead to other lung diseases. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke. Ask family members, friends, and coworkers not to smoke in front of you or in your home, car, or workplace.
If you've developed pulmonary fibrosis from the HP, you'll need further ongoing care. Pulmonary fibrosis is a condition in which tissue deep in your lungs becomes scarred over time.
If you have this condition, you may need medicines, oxygen therapy, and/or pulmonary rehabilitation. Follow your treatment plan as your doctor advises.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to hypersensitivity pneumonitis, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
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48,"2018-02-02 05:16:30",Hypotension,"
What Is Hypotension?
Hypotension (HI-po-TEN-shun) is abnormally low blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood.
Blood pressure is measured as systolic (sis-TOL-ik) and diastolic (di-a-STOL-ik) pressures. ""Systolic"" refers to blood pressure when the heart beats while pumping blood. ""Diastolic"" refers to blood pressure when the heart is at rest between beats.
You most often will see blood pressure numbers written with the systolic number above or before the diastolic number, such as 120/80 mmHg. (The mmHg is millimeters of mercury—the units used to measure blood pressure.)
Normal blood pressure in adults is lower than 120/80 mmHg. Hypotension is blood pressure that's lower than 90/60 mmHg.
Overview
Blood pressure doesn't stay the same all the time. It lowers as you sleep and rises when you wake up. Blood pressure also rises when you're excited, nervous, or active.
Your body is very sensitive to changes in blood pressure. For example, if you stand up quickly, your blood pressure may drop for a short time. Your body adjusts your blood pressure to make sure enough blood and oxygen are flowing to your brain, kidneys, and other vital organs.
Most forms of hypotension happen because your body can't bring blood pressure back to normal or can't do it fast enough.
Some people have low blood pressure all the time. They have no signs or symptoms, and their low blood pressure is normal for them.
In other people, certain conditions or factors cause abnormally low blood pressure. As a result, less blood and oxygen flow to the body's organs.
For the most part, hypotension is a medical concern only if it causes signs or symptoms or is linked to a serious condition, such as heart disease. Signs and symptoms of hypotension may include dizziness, fainting, cold and sweaty skin, fatigue (tiredness), blurred vision, or nausea (feeling sick to your stomach).
In extreme cases, hypotension can lead to shock.
Outlook
In a healthy person, low blood pressure without signs or symptoms usually isn't a problem and needs no treatment. If it causes signs or symptoms, your doctor will try to find and treat the condition that's causing it.
Hypotension can be dangerous. It can make you fall because of dizziness or fainting. Shock, a severe form of hypotension, is a condition that's often fatal if not treated right away. With prompt and proper treatment, shock can be successfully treated.
Types of Hypotension
There are several types of hypotension. People who always have low blood pressure have chronic asymptomatic hypotension. They usually have no signs or symptoms and need no treatment. Their low blood pressure is normal for them.
Other types of hypotension occur if blood pressure suddenly drops too low. The signs and symptoms range from mild to severe.
The three main types of this kind of hypotension are orthostatic (OR-tho-STAT-ik) hypotension, neurally mediated hypotension, and severe hypotension linked to shock.
Orthostatic Hypotension
This type of hypotension occurs when standing up from a sitting or lying down position. You may feel dizzy or light-headed, or you may even faint.
Orthostatic hypotension occurs if your body isn't able to adjust blood pressure and blood flow fast enough for the change in position. The drop in blood pressure usually lasts only for a few seconds or minutes after you stand up. You may need to sit or lie down for a short time while your blood pressure returns to normal.
Orthostatic hypotension can occur in all age groups. However, it's more common in older adults, especially those who are frail or in poor health. This type of hypotension can be a symptom of another medical condition. Thus, treatment often focuses on treating underlying conditions.
Some people have orthostatic hypotension, but also have high blood pressure when lying down.
A form of orthostatic hypotension called postprandial hypotension is a sudden drop in blood pressure after a meal. This type of hypotension mostly affects older adults. People who have high blood pressure or a central nervous system disorder, such as Parkinson's disease, also are at increased risk for postprandial hypotension.
Neurally Mediated Hypotension
With neurally mediated hypotension (NMH), blood pressure drops after you've been standing for a long time. You may feel dizzy, faint, or sick to the stomach as a result. NMH also can occur as the result of an unpleasant, upsetting, or scary situation.
NMH affects children and young adults more often than people in other age groups. Children often outgrow NMH.
Severe Hypotension Linked to Shock
Shock is a life-threatening condition in which blood pressure drops so low that the brain, kidneys, and other vital organs can't get enough blood to work well. Blood pressure drops much lower in shock than in other types of hypotension.
Many factors can cause shock. Examples include major blood loss, certain severe infections, severe burns and allergic reactions, and poisoning. Shock can be fatal if it's not treated right away.
Other Names for Hypotension
What Causes Hypotension?
Conditions or factors that disrupt the body's ability to control blood pressure cause hypotension. The different types of hypotension have different causes.
Orthostatic Hypotension
Orthostatic hypotension has many causes. Sometimes two or more factors combine to cause this type of low blood pressure.
Dehydration (de-hi-DRA-shun) is the most common cause of orthostatic hypotension. Dehydration occurs if the body loses more water than it takes in.
You may become dehydrated if you don't drink enough fluids or if you sweat a lot during physical activity. Fever, vomiting, and severe diarrhea also can cause dehydration.
Orthostatic hypotension also may occur during pregnancy, but it usually goes away after birth.
Because an older body doesn't manage changes in blood pressure as well as a younger body, getting older also can lead to this type of hypotension.
Postprandial hypotension (a type of orthostatic hypotension) mostly affects older adults. Postprandial hypotension is a sudden drop in blood pressure after a meal.
Certain medical conditions can raise your risk of orthostatic hypotension, including:
Some medicines for high blood pressure and heart disease can raise your risk of orthostatic hypotension. These medicines include:
Medicines for conditions such as anxiety, depression, erectile dysfunction, and central nervous system disorders also can increase your risk of orthostatic hypotension.
Other substances, when taken with high blood pressure medicines, also can lead to orthostatic hypotension. These substances include alcohol, barbiturates, and some prescription and over-the-counter medicines.
Finally, other factors or conditions that can trigger orthostatic hypotension include being out in the heat or being immobile for a long time. ""Immobile"" means you can't move around very much.
Neurally Mediated Hypotension
Neurally mediated hypotension (NMH) occurs when the brain and heart don't communicate with each other properly.
For example, when you stand for a long time, blood begins to pool in your legs. This causes your blood pressure to drop. In NMH, the body mistakenly tells the brain that blood pressure is high. In response, the brain slows the heart rate. This makes blood pressure drop even more, causing dizziness and other symptoms.
Severe Hypotension Linked to Shock
Many factors and conditions can cause severe hypotension linked to shock. Some of these factors also can cause orthostatic hypotension. In shock, though, blood pressure drops very low and doesn't return to normal on its own.
Shock is an emergency and must be treated right away. If a person has signs or symptoms of shock, call 9–1–1.
Some severe infections can cause shock. This is known as septic shock. It can occur if bacteria enter the bloodstream. The bacteria release a toxin (poison) that leads to a dangerous drop in blood pressure.
A severe loss of blood or fluids from the body also can cause shock. This is known as hypovolemic (HI-po-vo-LE-mik) shock. Hypovolemic shock can happen as a result of:
Major external
bleeding (for example, from a severe cut or injury)
Major loss of body fluids from severe
burns
A major decrease in the heart's ability to pump blood also can cause shock. This is known as cardiogenic (KAR-de-o-JEN-ik) shock.
A heart attack, pulmonary embolism, or an ongoing arrhythmia (ah-RITH-me-ah) that disrupts heart function can cause this type of shock.
A sudden and extreme relaxation of the arteries linked to a drop in blood pressure also can cause shock. This is known as vasodilatory (VA-so-DI-la-tory) shock. It can occur due to:
Who Is At Risk for Hypotension?
Hypotension can affect people of all ages. However, people in certain age groups are more likely to have certain types of hypotension.
Older adults are more likely to have orthostatic and postprandial hypotension. Children and young adults are more likely to have neurally mediated hypotension.
People who take certain medicines—such as diuretics (""water pills"") or other high blood pressure medicines—are at increased risk for hypotension. Certain conditions also increase the risk for hypotension. Examples include central nervous system disorders (such as Parkinson's disease) and some heart conditions.
Other risk factors for hypotension include being immobile (not being able to move around very much) for long periods, being out in the heat for a long time, and pregnancy. Hypotension during pregnancy is normal and usually goes away after birth.
What Are the Signs and Symptoms of Hypotension?
Orthostatic Hypotension and Neurally Mediated Hypotension
The signs and symptoms of orthostatic hypotension and neurally mediated hypotension (NMH) are similar. They include:
Blurry vision
Confusion
Weakness
Orthostatic hypotension may happen within a few seconds or minutes of standing up after you've been sitting or lying down.
You may feel that you're going to faint, or you may actually faint. These signs and symptoms go away if you sit or lie down for a few minutes until your blood pressure adjusts to normal.
The signs and symptoms of NMH occur after standing for a long time or in response to an unpleasant, upsetting, or scary situation. The drop in blood pressure with NMH doesn't last long and often goes away after sitting down.
Severe Hypotension Linked to Shock
In shock, not enough blood and oxygen flow to the body's major organs, including the brain. The early signs and symptoms of reduced blood flow to the brain include light-headedness, sleepiness, and confusion.
In the earliest stages of shock, it may be hard to detect any signs or symptoms. In older people, the first symptom may only be confusion.
Over time, as shock worsens, a person won't be able to sit up without passing out. If the shock continues, the person will lose consciousness. Shock often is fatal if not treated right away.
Other signs and symptoms of shock vary, depending on what's causing the shock. When low blood volume (from major blood loss, for example) or poor pumping action in the heart (from heart failure, for example) causes shock:
When extreme relaxation of blood vessels causes shock (such as in vasodilatory shock), a person feels warm and flushed at first. Later, the skin becomes cold and sweaty, and the person feels very sleepy.
Shock is an emergency and must be treated right away. If a person has signs or symptoms of shock, call 9–1–1.
How Is Hypotension Diagnosed?
Hypotension is diagnosed based on your medical history, a physical exam, and test results. Your doctor will want to know:
Whether an underlying condition is causing the
hypotension
Diagnostic Tests
Shock is a life-threatening condition that requires emergency treatment. For other types of hypotension, your doctor may recommend tests to find out how your blood pressure responds in certain situations.
The test results will help your doctor understand why you're fainting or having other symptoms.
EKG (Electrocardiogram)
An EKG is a simple test that detects and records your heart's electrical activity. It shows how fast your heart is beating and whether its rhythm is steady or irregular. An EKG also shows the strength and timing of electrical signals as they pass through each part of your heart.
Holter and Event Monitors
Holter and event monitors are medical devices that record your heart's electrical activity. These monitors are similar to an EKG. However, a standard EKG only records your heartbeat for a few seconds. It won't detect heart rhythm problems that don't occur during the test.
Holter and event monitors are small, portable devices. You can wear one while you do your normal daily activities. This allows the monitor to record your heart for longer periods than a standard EKG.
Stress Test
Some heart problems are easier to diagnose when your heart is working hard and beating fast. During stress testing, you exercise (or are given medicine if you're unable to exercise) to make your heart work hard and beat fast while heart tests are done.
These tests may include nuclear heart scanning, echo, and positron emission tomography (PET) scanning of the heart.
Valsalva Maneuver
This is a simple test for the part of your nervous system that controls functions such as your heartbeat and the narrowing and widening of your blood vessels. If something goes wrong with this part of the nervous system, blood pressure problems may occur.
During this test, you take a deep breath and then force the air out through your lips. You will do this several times. Your heart rate and blood pressure will be checked during the test.
Tilt Table Test
This test is used if you have fainting spells for no known reason. For the test, you lie on a table that moves from a lying down to an upright position. Your doctor checks your reaction to the change in position.
Doctors use a tilt table test to diagnose orthostatic hypotension and neurally mediated hypotension (NMH). People who have NMH usually faint during this test. The test can help your doctor find any underlying brain or nerve condition.
How Is Hypotension Treated?
Treatment depends on the type of hypotension you have and the severity of your signs and symptoms. The goal of treatment is to bring blood pressure back to normal to relieve signs and symptoms. Another goal is to manage any underlying condition causing the hypotension.
Your response to treatment depends on your age, overall health, and strength. It also depends on how easily you can stop, start, or change medicines.
In a healthy person, low blood pressure without signs or symptoms usually isn't a problem and needs no treatment.
If you have signs or symptoms of hypotension, you should sit or lie down right away. Put your feet above the level of your heart. If your signs or symptoms don't go away quickly, you should seek medical care.
Orthostatic Hypotension
Many treatments are available for orthostatic hypotension. If you have this condition, your doctor may advise making lifestyle changes, such as:
Drinking plenty of fluids, such as water or sports drinks that contain
nutrients like sodium and
potassium.
Drinking little or no alcohol.
Standing up slowly.
Not crossing your legs while sitting.
Slowly increasing the amount of time you sit up if you've been immobile for a long time because of a medical condition. The term ""immobile"" refers to not being able to move around very much.
Talk with your doctor about using compression stockings. These stockings apply pressure to your lower legs. The pressure helps move blood throughout your body.
If medicine is causing your low blood pressure, your doctor may change the medicine or adjust the dose you take.
Several medicines are used to treat orthostatic hypotension. These medicines, which raise blood pressure, include fludrocortisone and midodrine.
Neurally Mediated Hypotension
If you have neurally mediated hypotension (NMH), you may need to make lifestyle changes. These may include:
If medicine is causing your hypotension, your doctor may change the medicine or adjust the dose you take. He or she also may prescribe medicine to treat NMH.
Children who have NHM often outgrow it.
Severe Hypotension Linked to Shock
Shock is a life-threatening emergency. People who have shock need prompt treatment from medical personnel. If a person has signs or symptoms of shock, call 9–1–1 right away.
The goals of treating shock are to:
Blood or special fluids are put into the bloodstream to restore blood flow to the organs. Medicines can help raise blood pressure or make the heartbeat stronger. Depending on the cause of the shock, other treatments—such as antibiotics or surgery—may be needed.
Living With Hypotension
Doctors can successfully treat hypotension. Many people who had the condition and were successfully treated live normal, healthy lives.
If you have hypotension, you can take steps to prevent or limit symptoms, such as dizzy spells and fainting.
If you have orthostatic hypotension, get up slowly after sitting or lying down, or move your legs before changing your position. Eat small, low-carbohydrate meals if you have postprandial hypotension (a form of orthostatic hypotension).
If you have neurally mediated hypotension, try not to stand for long periods. If you do have to stand for a long time, move around and wear compression stockings. These stockings apply pressure to your lower legs. The pressure helps move blood throughout your body.
Drink plenty of fluids, such as water or sports drinks that contain nutrients like sodium and potassium. Also, try to avoid unpleasant, upsetting, or scary situations. Learn to recognize symptoms and take action to raise your blood pressure. Children who have NMH often outgrow it.
Other lifestyle changes also can help you control hypotension. For more information, talk with your doctor and go to ""How Is Hypotension Treated?""
Ask your doctor about learning how to measure your own blood pressure. This will help you find out what a normal blood pressure reading is for you. Keeping a record of blood pressure readings done by health providers also can help you learn more about your blood pressure.
Severe hypotension linked to shock is an emergency. Shock can lead to death if it's not treated right away. If you see someone having signs or symptoms of shock, call 9–1–1.
Signs and symptoms of shock include light-headedness, sleepiness, and confusion. Over time, as shock worsens, a person won't be able to sit up without passing out. If the shock continues, the person can lose consciousness.
Other signs and symptoms of shock ","June 11, 2014.","{
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49,"2018-02-02 05:16:48","Idiopathic Pulmonary Fibrosis","
What Is Idiopathic Pulmonary Fibrosis?
Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis.
As the lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (For more information, go to the ""How the Lungs Work"" section of this article.)
Sometimes doctors can find out what's causing fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF).
IPF is a serious disease that usually affects middle-aged and older adults. IPF varies from person to person. In some people, fibrosis happens quickly. In others, the process is much slower. In some people, the disease stays the same for years.
IPF has no cure yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes of death include pulmonary hypertension (HI-per-TEN-shun), heart failure, pulmonary embolism (EM-bo-lizm), pneumonia (nu-MO-ne-ah), and lung cancer.
Genetics may play a role in causing IPF. If more than one member of your family has IPF, the disease is called familial IPF.
Research has helped doctors learn more about IPF. As a result, they can more quickly diagnose the disease now than in the past. Also, researchers are studying several medicines that may slow the progress of IPF. These efforts may improve the lifespan and quality of life for people who have the disease.
How the Lungs Work
To understand idiopathic pulmonary fibrosis (IPF), it helps to understand how the lungs work. The air that you breathe in through your nose or mouth travels down through your trachea (windpipe) into two tubes in your lungs called bronchial (BRONG-ke-al) tubes or airways.
The airways are shaped like an upside-down tree with many branches. The windpipe is the trunk. It splits into two bronchial tubes, or bronchi. Thinner tubes called bronchioles branch out from the bronchi.
The bronchioles end in tiny air sacs called alveoli (al-VEE-uhl-eye). These air sacs have very thin walls, and small blood vessels called capillaries run through them. There are about 300 million alveoli in a normal lung.
When the air that you've just breathed in reaches these air sacs, the oxygen in the air passes through the air sac walls into the blood in the capillaries. At the same time, carbon dioxide (a waste gas) moves from the capillaries into the air sacs. This process is called gas exchange.
The oxygen-rich blood in the capillaries then flows into larger veins, which carry it to the heart. Your heart pumps the oxygen-rich blood to all your body's organs. These organs can't function without an ongoing supply of oxygen.
The animation below shows how the lungs work. Click the ""start"" button to play the animation. Written and spoken explanations are provided with each frame. Use the buttons in the lower right corner to pause, restart, or replay the animation, or use the scroll bar below the buttons to move through the frames.
![]()
The animation shows how the lungs inhale oxygen and transfer it to the blood. It also shows how carbon dioxide (a waste product) is removed from the blood and exhaled.
In IPF, scarring begins in the air sac walls and the spaces around them. The scarring makes the walls of the air sacs thicker. This makes it harder for oxygen to pass through the air sac walls into the bloodstream.
![]()
Idiopathic Pulmonary Fibrosis. Figure A shows the location of the lungs and airways in the body. The inset image shows a detailed view of the lung's airways and air sacs in cross-section. Figure B shows fibrosis (scarring) in the lungs. The inset image (more...)
For more information about lung function, go to the Health Topics How the Lungs Work article.
Other Names for Idiopathic Pulmonary Fibrosis
What Causes Idiopathic Pulmonary Fibrosis?
Sometimes doctors can find out what is causing pulmonary fibrosis (lung scarring). For example, exposure to environmental pollutants and certain medicines can cause the disease.
Environmental pollutants include inorganic dust (silica and hard metal dusts) and organic dust (bacteria and animal proteins).
Medicines that are known to cause pulmonary fibrosis in some people include nitrofurantoin (an antibiotic), amiodarone (a heart medicine), methotrexate and bleomycin (both chemotherapy medicines), and many other medicines.
In most cases, however, the cause of lung scarring isn’t known. These cases are called idiopathic pulmonary fibrosis (IPF). With IPF, doctors think that something inside or outside of the lungs attacks them again and again over time.
These attacks injure the lungs and scar the tissue inside and between the air sacs. This makes it harder for oxygen to pass through the air sac walls into the bloodstream.
The following factors may increase your risk of IPF:
Genetics also may play a role in causing IPF. Some families have at least two members who have IPF.
Researchers have found that 9 out of 10 people who have IPF also have gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat.
Some people who have GERD may regularly breathe in tiny drops of acid from their stomachs. The acid can injure their lungs and lead to IPF. More research is needed to confirm this theory.
What Are the Signs and Symptoms of Idiopathic Pulmonary Fibrosis?
The signs and symptoms of idiopathic pulmonary fibrosis (IPF) develop over time. They may not even begin to appear until the disease has done serious damage to your lungs. Once they occur, they're likely to get worse over time.
The most common signs and symptoms are:
Shortness of breath. This usually is the main symptom of IPF. At first, you may be short of breath only during
exercise. Over time, you'll likely feel breathless even at rest.
A dry, hacking
cough that doesn't get better. Over time, you may have repeated bouts of
coughing that you can't control.
Other signs and symptoms that you may develop over time include:
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Clubbing. The illustration shows clubbing of the fingertips associated with idiopathic pulmonary fibrosis.
IPF may lead to other medical problems, including a collapsed lung, lung infections, blood clots in the lungs, and lung cancer.
As the disease worsens, you may develop other potentially life-threatening conditions, including respiratory failure, pulmonary hypertension, and heart failure.
How Is Idiopathic Pulmonary Fibrosis Diagnosed?
Idiopathic pulmonary fibrosis (IPF) causes the same kind of scarring and symptoms as some other lung diseases. This makes it hard to diagnose.
Seeking medical help as soon as you have symptoms is important. If possible, seek care from a pulmonologist. This is a doctor who specializes in diagnosing and treating lung problems.
Your doctor will diagnose IPF based on your medical history, a physical exam, and test results. Tests can help rule out other causes of your symptoms and show how badly your lungs are damaged.
Medical History
Your doctor may ask about:
Your age
Your history of smoking
Things in the air at your job or elsewhere that could irritate your
lungsYour hobbies
Your history of legal and illegal drug use
Other medical conditions that you have
How long you've had symptoms
Diagnostic Tests
No single test can diagnose IPF. Your doctor may recommend several of the following tests.
Chest X Ray
A chest x ray is a painless test that creates a picture of the structures in your chest, such as your heart and lungs. This test can show shadows that suggest scar tissue. However, many people who have IPF have normal chest x rays at the time they're diagnosed.
High-Resolution Computed Tomography
A high-resolution computed tomography scan, or HRCT scan, is an x ray that provides sharper and more detailed pictures than a standard chest x ray.
HRCT can show scar tissue and how much lung damage you have. This test can help your doctor spot IPF at an early stage or rule it out. HRCT also can help your doctor decide how likely you are to respond to treatment.
Lung Function Tests
Your doctor may suggest a breathing test called spirometry (spi-ROM-eh-tree) to find out how much lung damage you have. This test measures how much air you can blow out of your lungs after taking a deep breath. Spirometry also measures how fast you can breathe the air out.
If you have a lot of lung scarring, you won't be able to breathe out a normal amount of air.
Pulse Oximetry
For this test, your doctor attaches a small sensor to your finger or ear. The sensor uses light to estimate how much oxygen is in your blood.
Arterial Blood Gas Test
For this test, your doctor takes a blood sample from an artery, usually in your wrist. The sample is sent to a laboratory, where its oxygen and carbon dioxide levels are measured.
This test is more accurate than pulse oximetry. The blood sample also can be tested to see whether an infection is causing your symptoms.
Skin Test for Tuberculosis
For this test, your doctor injects a substance under the top layer of skin on one of your arms. This substance reacts to tuberculosis (TB). If you have a positive reaction, a small hard lump will develop at the injection site 48 to 72 hours after the test. This test is done to rule out TB.
Lung Biopsy
For a lung biopsy, your doctor will take samples of lung tissue from several places in your lungs. The samples are examined under a microscope. A lung biopsy is the best way for your doctor to diagnose IPF.
This procedure can help your doctor rule out other conditions, such as sarcoidosis (sar-koy-DO-sis), cancer, or infection. Lung biopsy also can show your doctor how far your disease has advanced.
Doctors use several procedures to get lung tissue samples.
Video-assisted thoracoscopy (thor-ah-KOS-ko-pee). This is the most common procedure used to get lung tissue samples. Your doctor inserts a small tube with an attached light and camera into your chest through small cuts between your ribs. The tube is called an endoscope.
The endoscope provides a video image of the lungs and allows your doctor to collect tissue samples. This procedure must be done in a hospital. You'll be given medicine to make you sleep during the procedure.
Bronchoscopy (bron-KOS-ko-pee). For a bronchoscopy, your doctor passes a thin, flexible tube through your nose or mouth, down your throat, and into your airways. At the tube's tip are a light and mini-camera. They allow your doctor to see your windpipe and airways.
Your doctor then inserts a forceps through the tube to collect tissue samples. You'll be given medicine to help you relax during the procedure.
Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH). During bronchoscopy, your doctor may inject a small amount of salt water (saline) through the tube into your lungs. This fluid washes the lungs and helps bring up cells from the area around the air sacs. These cells are examined under a microscope.
Thoracotomy (thor-ah-KOT-o-me). For this procedure, your doctor removes a few small pieces of lung tissue through a cut in the chest wall between your ribs. Thoracotomy is done in a hospital. You'll be given medicine to make you sleep during the procedure.
How Is Idiopathic Pulmonary Fibrosis Treated?
Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation (PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF).
Medicines
Currently, no medicines are proven to slow the progression of IPF.
Prednisone, azathioprine (A-zah-THI-o-preen), and N-acetylcysteine (a-SEH-til-SIS-tee-in) have been used to treat IPF, either alone or in combination. However, experts have not found enough evidence to support their use.
Prednisone
Prednisone is an anti-inflammatory medicine. You usually take it by mouth every day. However, your doctor may give it to you through a needle or tube inserted into a vein in your arm for several days. After that, you usually take it by mouth.
Because prednisone can cause serious side effects, your doctor may prescribe it for 3 to 6 months or less at first. Then, if it works for you, your doctor may reduce the dose over time and keep you on it longer.
Azathioprine
Azathioprine suppresses your immune system. You usually take it by mouth every day. Because it can cause serious side effects, your doctor may prescribe it with prednisone for only 3 to 6 months.
If you don't have serious side effects and the medicines seem to help you, your doctor may keep you on them longer.
N-acetylcysteine
N-acetylcysteine is an antioxidant that may help prevent lung damage. You usually take it by mouth several times a day.
A common treatment for IPF is a combination of prednisone, azathioprine, and N-acetylcysteine. However, this treatment was recently found harmful in a study funded by the National Heart, Lung, and Blood Institute (NHLBI).
If you have IPF and take this combination of medicines, talk with your doctor. Do not stop taking the medicines on your own.
The NHLBI currently supports research to compare N-acetylcysteine treatment with placebo treatment (sugar pills) in patients who have IPF.
New Medicines Being Studied
Researchers, like those in the Idiopathic Pulmonary Fibrosis Network, are studying new treatments for IPF. With the support and guidance of the NHLBI, these researchers continue to look for new IPF treatments and therapies.
Some of these researchers are studying medicines that may reduce inflammation and prevent or reduce scarring caused by IPF.
If you're interested in joining a research study, talk with your doctor. For more information about ongoing research, go to the ""Clinical Trials"" section of this article.
Other Treatments
Other treatments that may help people who have IPF include the following:
Flu and
pneumonia vaccines may help prevent infections and keep you healthy.
Oxygen Therapy
If the amount of oxygen in your blood gets low, you may need oxygen therapy. Oxygen therapy can help reduce shortness of breath and allow you to be more active.
Oxygen usually is given through nasal prongs or a mask. At first, you may need it only during exercise and sleep. As your disease worsens, you may need it all the time.
For more information, go to the Health Topics Oxygen Therapy article.
Pulmonary Rehabilitation
PR is now a standard treatment for people who have chronic (ongoing) lung disease. PR is a broad program that helps improve the well-being of people who have breathing problems.
The program usually involves treatment by a team of specialists in a special clinic. The goal is to teach you how to manage your condition and function at your best.
PR doesn't replace medical therapy. Instead, it's used with medical therapy and may include:
For more information, go to the Health Topics Pulmonary Rehabilitation article.
Lung Transplant
Your doctor may recommend a lung transplant if your condition is quickly worsening or very severe. A lung transplant can improve your quality of life and help you live longer.
Some medical centers will consider patients older than 65 for lung transplants if they have no other serious medical problems.
The major complications of a lung transplant are rejection and infection. (""Rejection"" refers to your body creating proteins that attack the new organ.) You will have to take medicines for the rest of your life to reduce the risk of rejection.
Because the supply of donor lungs is limited, talk with your doctor about a lung transplant as soon as possible.
For more information, go to the Health Topics Lung Transplant article.
Living With Idiopathic Pulmonary Fibrosis
No cure is available for idiopathic pulmonary fibrosis (IPF) yet. Your symptoms may get worse over time. As your symptoms worsen, you may not be able to do many of the things that you did before you had IPF.
However, lifestyle changes and ongoing care can help you manage the disease.
Lifestyle Changes
If you're still smoking, the most important thing you can do is quit. Talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke. Ask family members and friends not to smoke in front of you or in your home, car, or workplace.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article and the National Heart, Lung, and Blood Institute's (NHLBI's) ""Your Guide to a Healthy Heart."" Although these resources focus on heart health, they include general tips on how to quit smoking.
Staying active can help with both your physical and mental health. Physical activity can help you maintain your strength and lung function and reduce stress. Try moderate exercise, such as walking or riding a stationary bike. Ask your doctor about using oxygen while exercising.
As your condition advances, use a wheelchair or motorized scooter, or stay busy with activities that aren't physical in nature.
You also should follow a healthy diet. A healthy diet includes a variety of fruits and vegetables. It also includes whole grains, fat-free or low-fat dairy products, and protein foods, such as lean meats, poultry without skin, seafood, processed soy products, nuts, seeds, beans, and peas.
A healthy diet is low in sodium (salt), added sugars, solid fats, and refined grains. Solid fats are saturated fat and trans fatty acids. Refined grains come from processing whole grains, which results in a loss of nutrients (such as dietary fiber).
Eating smaller, more frequent meals may relieve stomach fullness, which can make it hard to breathe. If you need help with your diet, ask your doctor to arrange for a dietitian to work with you.
For more information about following a healthy diet, go to the NHLBI's ""Your Guide to Lowering Your Blood Pressure With DASH"" and the U.S. Department of Agriculture's ChooseMyPlate.gov Web site. Both resources provide general information about healthy eating.
Getting plenty of rest can increase your energy and help you deal with the stress of living with a serious condition like IPF.
Try to maintain a positive attitude; relaxation techniques may help you do this. These techniques also may help you avoid excessive oxygen intake caused by tension or overworked muscles.
Avoid situations that can make your symptoms worse. For example, avoid traveling by air or living at or traveling to high altitudes where the air is thin and the amount of oxygen in the air is low.
Ongoing Care
If you have IPF, you will need ongoing medical care. If possible, seek treatment from a doctor who specializes in IPF. These specialists often are located at major medical centers.
Treatment may relieve your symptoms and even slow or stop the fibrosis (scarring). Follow your treatment plan as your doctor advises. For example:
As your condition worsens, you may need oxygen therapy full time. Some people who have IPF carry portable oxygen when they go out.
Emotional Issues and Support
Living with IPF may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with IPF. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. For example, this research has uncovered some of the causes of chronic lung diseases, as well as ways to prevent and treat these diseases.
Many more questions remain about lung diseases, including idiopathic pulmonary fibrosis (IPF). The NHLBI continues to support research aimed at learning more about these diseases. For example, NHLBI-supported research on IPF includes studies that explore:
What Is Immune Thrombocytopenia?
Immune thrombocytopenia (THROM-bo-si-toe-PE-ne-ah), or ITP, is a bleeding disorder. In ITP, the blood doesn't clot as it should. This is due to a low number of blood cell fragments called platelets (PLATE-lets) or thrombocytes (THROM-bo-sites).
Platelets are made in your bone marrow along with other kinds of blood cells. They stick together (clot) to seal small cuts or breaks on blood vessel walls and stop bleeding.
Overview
Without enough platelets, bleeding can occur inside the body (internal bleeding) or underneath or from the skin (external bleeding).
People who have ITP often have purple bruises called purpura (PURR-purr-ah). These bruises appear on the skin or mucous membranes (for example, in the mouth). Bleeding from small blood vessels under the skin causes purpura.
People who have ITP also may have bleeding that causes tiny red or purple dots on the skin. These pinpoint-sized dots are called petechiae (peh-TEE-kee-ay). Petechiae may look like a rash.
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Purpura and Petechiae. The photograph shows purpura (bruises) and petechiae (red and purple dots) on the skin. Bleeding under the skin causes the purple, brown, and red color of the purpura and petechiae.
People who have ITP also may have nosebleeds, bleeding from the gums during dental work, or other bleeding that's hard to stop. Women who have ITP may have menstrual bleeding that's heavier than normal.
A lot of bleeding can cause hematomas (he-mah-TO-mas). A hematoma is a collection of clotted or partially clotted blood under the skin. It looks or feels like a lump.
Bleeding in the brain as a result of ITP is very rare, but can be life threatening if it occurs.
In most cases, an autoimmune response is thought to cause ITP. Normally, your immune system helps your body fight off infections and diseases. But if you have ITP, your immune system attacks and destroys its own platelets. The reason why this happens isn't known.
ITP can't be passed from one person to another.
Types of Immune Thrombocytopenia
The two types of ITP are acute (temporary or short-term) and chronic (long-lasting).
Acute ITP generally lasts less than 6 months. It mainly occurs in children—both boys and girls—and is the most common type of ITP. Acute ITP often occurs after a viral infection.
Chronic ITP lasts 6 months or longer and mostly affects adults. However, some teenagers and children do get this type of ITP. Chronic ITP affects women two to three times more often than men.
Treatment depends on the severity of bleeding and the platelet count. In mild cases, treatment may not be needed.
Outlook
For most children and adults, ITP isn't a serious or life-threatening condition.
Acute ITP in children often goes away on its own within a few weeks or months and doesn't return. In 80 percent of children who have ITP, the platelet count returns to normal within 6 to 12 months. Treatment may not be needed.
For a small number of children, ITP doesn't go away on its own and may require further medical or surgical treatment.
Chronic ITP varies from person to person and can last for many years. Even people who have severe forms of chronic ITP can live for decades. Most people who have chronic ITP can stop treatment at some point and maintain a safe platelet count.
Other Names for Immune Thrombocytopenia
What Causes Immune Thrombocytopenia?
In most cases, an autoimmune response is thought to cause immune thrombocytopenia (ITP).
Normally, your immune system helps your body fight off infections and diseases. In ITP, however, your immune system attacks and destroys your body's platelets by mistake. Why this happens isn't known.
In some people, ITP may be linked to viral or bacterial infections, such as HIV, hepatitis C, or H. pylori.
Children who have acute (short-term) ITP often have had recent viral infections. These infections may ""trigger"" or set off the immune reaction that leads to ITP.
Who Is at Risk for Immune Thrombocytopenia?
Immune thrombocytopenia (ITP) is a fairly common blood disorder. Both children and adults can develop ITP.
Children usually have the acute (short-term) type of ITP. Acute ITP often develops after a viral infection.
Adults tend to have the chronic (long-lasting) type of ITP. Women are two to three times more likely than men to develop chronic ITP.
The number of cases of ITP is rising because routine blood tests that can detect a low platelet count are being done more often.
ITP can't be passed from one person to another.
What Are the Signs and Symptoms of Immune Thrombocytopenia?
Immune thrombocytopenia (ITP) may not cause any signs or symptoms. However, ITP can cause bleeding inside the body (internal bleeding) or underneath or from the skin (external bleeding). Signs of bleeding may include:
Pinpoint red spots on the
skin called
petechiae. These spots often are found in groups and may look like a
rash.
Bleeding under the skin causes petechiae.
A collection of clotted or partially clotted
blood under the
skin that looks or feels like a lump. This is called a hematoma.
Nosebleeds or
bleeding from the
gums (for example, during dental work).
Any kind of bleeding that's hard to stop could be a sign of ITP. This includes menstrual bleeding that's heavier than normal. Bleeding in the brain is rare, and its symptoms may vary.
A low platelet count doesn't directly cause pain, problems concentrating, or other symptoms. However, a low platelet count might be associated with fatigue (tiredness).
How Is Immune Thrombocytopenia Diagnosed?
Your doctor will diagnose immune thrombocytopenia (ITP) based on your medical history, a physical exam, and test results.
Your doctor will want to make sure that your low platelet count isn't due to another condition (such as an infection) or medicines you're taking (such as chemotherapy medicines or aspirin).
Medical History
Your doctor may ask about:
Your signs and symptoms of
bleeding and any other signs or symptoms you're having
Whether you have illnesses that could lower your
platelet count or cause
bleedingMedicines or any over-the-counter supplements or remedies you take that could cause
bleeding or lower your
platelet count
Physical Exam
During a physical exam, your doctor will look for signs of bleeding and infection. For example, your doctor may look for purplish areas on the skin or mucous membranes and pinpoint red spots on the skin. These are signs of bleeding under the skin.
How Is Immune Thrombocytopenia Treated?
Treatment for immune thrombocytopenia (ITP) is based on how much and how often you're bleeding and your platelet count.
Adults who have mild ITP may not need any treatment, other than watching their symptoms and platelet counts. Adults who have ITP with very low platelet counts or bleeding problems often are treated.
The acute (short-term) type of ITP that occurs in children often goes away within a few weeks or months. Children who have bleeding symptoms, other than merely bruising (purpura), usually are treated.
Children who have mild ITP may not need treatment other than monitoring and followup to make sure their platelet counts return to normal.
Medicines
Medicines often are used as the first course of treatment for both children and adults.
Corticosteroids (cor-ti-co-STEER-roids), such as prednisone, are commonly used to treat ITP. These medicines, called steroids for short, help increase your platelet count. However, steroids have many side effects. Some people relapse (get worse) when treatment ends.
The steroids used to treat ITP are different from the illegal steroids that some athletes take to enhance performance. Corticosteroids aren't habit-forming, even if you take them for many years.
Other medicines also are used to raise the platelet count. Some are given through a needle inserted into a vein. These medicines include rituximab, immune globulin, and anti-Rh (D) immunoglobulin.
Medicines also may be used with a procedure to remove the spleen called splenectomy (splee-NECK-tuh-mee).
If medicines or splenectomy don't help, two newer medicines—eltrombopag and romiplostim—can be used to treat ITP.
Removal of the Spleen (Splenectomy)
If needed, doctors can surgically remove the spleen. This organ is located in the upper left abdomen. The spleen is about the size of a golf ball in children and a baseball in adults.
The spleen makes antibodies (proteins) that help fight infections. In ITP, these antibodies destroy platelets by mistake.
If ITP hasn't responded to medicines, removing the spleen will reduce the destruction of platelets. However, it also may raise your risk for infections. Before you have the surgery, your doctor may give you vaccines to help prevent infections.
If your spleen is removed, your doctor will explain what steps you can take to help avoid infections and what symptoms to watch for.
Other Treatments
Treating Infections
Some infections can briefly lower your platelet count. Treating the infection may help increase your platelet count and reduce bleeding problems.
Stopping Medicines
Some medicines can lower your platelet count or cause bleeding. Stopping the medicine can sometimes help raise your platelet count or prevent bleeding.
For example, aspirin and ibuprofen are common medicines that increase the risk of bleeding. If you have ITP, your doctor may suggest that you avoid these medicines.
How Can Immune Thrombocytopenia Be Prevented?
You can't prevent immune thrombocytopenia (ITP), but you can prevent its complications.
Talk with your doctor about which medicines are safe for you. Your doctor may advise you to avoid medicines that can affect your
platelets and increase your risk of
bleeding. Examples of such medicines include
aspirin and
ibuprofen.
Protect yourself from injuries that can cause bruising or
bleeding.
Seek
treatment right away if you develop any infections. Report any symptoms of
infection, such as a
fever, to your doctor. This is very important for people who have ITP and have had their
spleens removed.
Living With Immune Thrombocytopenia
If you have immune thrombocytopenia (ITP), you can take steps to prevent complications. Lifestyle changes and ongoing care can help you manage the condition.
Lifestyle Changes
Try to avoid injuries, especially head injuries, that can cause bleeding in the brain. For example, don't take part in contact sports, such as boxing, football, or karate. Other sports, such as skiing or horseback riding, also put you at risk for injuries that can cause bleeding.
Some safe activities are swimming, biking (with a helmet), and walking. Ask your doctor about physical activities that are safe for you.
Take precautions such as regular use of seatbelts and wearing gloves while working with knives and other tools.
If your child has ITP, ask his or her doctor whether you need to restrict your child's activities.
Ongoing Care
You may want to find a doctor who is familiar with treating people who have ITP. For example, hematologists are doctors who specialize in diagnosing and treating blood disorders. Discuss with your doctor how to manage ITP and when to seek medical care.
Talk with your doctor before taking prescription medicines or over-the-counter medicines, supplements, vitamins, or herbal remedies. Some medicines and supplements can affect platelets and increase your chance of bleeding. Common examples are aspirin or ibuprofen. Your doctor may advise you to avoid these medicines.
Watch for symptoms of infection, such as a fever, and report them to your doctor promptly. If you've had your spleen removed, you may be more likely to become ill from certain infections.
Immune Thrombocytopenia in Pregnancy
In women who are pregnant and have ITP, the ITP usually doesn't affect the baby. However, some babies may be born with or develop low platelet counts soon after birth.
The babies' platelet counts almost always return to normal without any treatment. Treatment can speed up recovery in the babies whose platelet counts are very low.
Treatment for ITP during pregnancy depends on a woman's platelet count. If treatment is needed, the doctor will take a close look at the possible effects of the treatment on the unborn baby.
Women who have mild cases of ITP usually can go through pregnancy without treatment. Pregnant women who have very low platelet counts or a lot of bleeding are more likely to have heavy bleeding during delivery or afterward. To prevent heavy bleeding, these women usually are treated.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to immune thrombocytopenia, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
What Is Insomnia?
Insomnia (in-SOM-ne-ah) is a common sleep disorder. People who have insomnia have trouble falling asleep, staying asleep, or both. As a result, they may get too little sleep or have poor-quality sleep. They may not feel refreshed when they wake up.
Overview
Insomnia can be acute (short-term) or chronic (ongoing). Acute insomnia is common and often is brought on by situations such as stress at work, family pressures, or a traumatic event. Acute insomnia lasts for days or weeks.
Chronic insomnia lasts for a month or longer. Most cases of chronic insomnia are secondary, which means they are the symptom or side effect of some other problem. Certain medical conditions, medicines, sleep disorders, and substances can cause secondary insomnia.
In contrast, primary insomnia isn't due to medical problems, medicines, or other substances. It is its own distinct disorder, and its cause isn’t well understood. Many life changes can trigger primary insomnia, including long-lasting stress and emotional upset.
Insomnia can cause daytime sleepiness and a lack of energy. It also can make you feel anxious, depressed, or irritable. You may have trouble focusing on tasks, paying attention, learning, and remembering. These problems can prevent you from doing your best at work or school.
Insomnia also can cause other serious problems. For example, you may feel drowsy while driving, which could lead to an accident.
Outlook
Treating the underlying cause of secondary insomnia may resolve or improve the sleep problem, especially if you can correct the problem soon after it starts. For example, if caffeine is causing your insomnia, stopping or limiting your intake of the substance might make the insomnia go away.
Lifestyle changes, including better sleep habits, often help relieve acute insomnia. For chronic insomnia, your doctor may recommend medicines or cognitive-behavioral therapy.
What Causes Insomnia?
Secondary Insomnia
Secondary insomnia is the symptom or side effect of another problem. This type of insomnia often is a symptom of an emotional, neurological, or other medical or sleep disorder.
Emotional disorders that can cause insomnia include depression, anxiety, and posttraumatic stress disorder. Alzheimer's disease and Parkinson's disease are examples of neurological disorders that can cause insomnia.
Many other disorders or factors also can cause insomnia, such as:
Gastrointestinal disorders, such as
heartburn
Secondary insomnia also can be a side effect of some medicines. For example, certain asthma medicines, such as theophylline, and some allergy and cold medicines can cause insomnia. Beta blockers also can cause the condition. These medicines are used to treat heart conditions.
Commonly used substances also can cause insomnia. Examples include caffeine and other stimulants, tobacco and other nicotine products, and alcohol and other sedatives.
Primary Insomnia
Primary insomnia isn't a symptom or side effect of another medical condition. It is its own distinct disorder, and its cause isn’t well understood. Primary insomnia usually lasts for at least 1 month.
Many life changes can trigger primary insomnia. It may be due to major or long-lasting stress or emotional upset. Travel or other factors, such as work schedules that disrupt your sleep routine, also may trigger primary insomnia.
Even if these issues are resolved, the insomnia may not go away. Trouble sleeping can persist because of habits formed to deal with the lack of sleep. These habits might include taking naps, worrying about sleep, and going to bed early.
Researchers continue to try to find out whether some people are born with an increased risk for primary insomnia.
Who Is at Risk for Insomnia?
Insomnia is a common disorder. It affects women more often than men. The disorder can occur at any age. However, older adults are more likely to have insomnia than younger people.
People who might be at increased risk for insomnia include those who:
Are
depressed or have other emotional distress, such as divorce or death of a spouse.
Have lower incomes.
Work at night or have frequent major shifts in their work hours.
Travel long distances with time changes.
Have an inactive lifestyle.
Young and middle-aged African Americans also might be at increased risk for insomnia. Research shows that, compared with Caucasian Americans, it takes African Americans longer to fall asleep. They also have lighter sleep, don't sleep as well, and take more naps. Sleep-related breathing problems also are more common among African Americans.
What Are the Signs and Symptoms of Insomnia?
The main symptom of insomnia is trouble falling or staying asleep, which leads to lack of sleep. If you have insomnia, you may:
The lack of sleep can cause other symptoms. You may wake up feeling tired or not well-rested, and you may feel tired during the day. You also may have trouble focusing on tasks. Insomnia can cause you to feel anxious, depressed, or irritable.
Insomnia also can affect your daily activities and cause serious problems. For example, you may feel drowsy while driving. Driver sleepiness (not related to alcohol) is responsible for almost 20 percent of all serious car crash injuries. Research also shows that insomnia raises older women’s risk of falling.
If insomnia is affecting your daily activities, talk with your doctor. Treatment may help you avoid symptoms and problems related to the disorder. Also, poor sleep may be a sign of other health problems. Finding and treating those problems could improve your overall health and sleep.
How Is Insomnia Diagnosed?
Your doctor will likely diagnose insomnia based on your medical and sleep histories and a physical exam. He or she also may recommend a sleep study. For example, you may have a sleep study if the cause of your insomnia is unclear.
Medical History
To find out what's causing your insomnia, your doctor may ask whether you:
Have any new or ongoing health problems
Have painful injuries or health conditions, such as
arthritisTake any medicines, either over-the-counter or prescription
Are coping with highly stressful life events, such as divorce or death
Your doctor also may ask questions about your work and leisure habits. For example, he or she may ask about your work and exercise routines; your use of caffeine, tobacco, and alcohol; and your long-distance travel history. Your answers can give clues about what's causing your insomnia.
Your doctor also may ask whether you have any new or ongoing work or personal problems or other stresses in your life. Also, he or she may ask whether you have other family members who have sleep problems.
Sleep History
To get a better sense of your sleep problem, your doctor will ask you for details about your sleep habits. Before your visit, think about how to describe your problems, including:
How often you have trouble sleeping and how long you've had the problem
When you go to bed and get up on workdays and days off
How long it takes you to
fall asleep, how often you wake up at night, and how long it takes to fall back asleep
Whether you snore loudly and often or wake up gasping or feeling out of breath
How refreshed you feel when you wake up, and how tired you feel during the day
How often you doze off or have trouble staying awake during routine tasks, especially driving
To find out what's causing or worsening your insomnia, your doctor also may ask you:
Whether you worry about
falling asleep, staying asleep, or getting enough
sleepWhat you eat or drink, and whether you take medicines before going to bed
What routine you follow before going to bed
What the noise level, lighting, and temperature are like where you
sleepWhat distractions, such as a TV or computer, are in your bedroom
To help your doctor, consider keeping a sleep diary for 1 or 2 weeks. Write down when you go to sleep, wake up, and take naps. (For example, you might note: Went to bed at 10 a.m.; woke up at 3 a.m. and couldn't fall back asleep; napped after work for 2 hours.)
Also write down how much you sleep each night, as well as how sleepy you feel throughout the day.
You can find a sample sleep diary in the National Heart, Lung, and Blood Institute's ""Your Guide to Healthy Sleep.""
Physical Exam
Your doctor will do a physical exam to rule out other medical problems that might cause insomnia. You also may need blood tests to check for thyroid problems or other conditions that can cause sleep problems.
How Is Insomnia Treated?
Lifestyle changes often can help relieve acute (short-term) insomnia. These changes might make it easier to fall asleep and stay asleep.
A type of counseling called cognitive-behavioral therapy (CBT) can help relieve the anxiety linked to chronic (ongoing) insomnia. Anxiety tends to prolong insomnia.
Several medicines also can help relieve insomnia and re-establish a regular sleep schedule. However, if your insomnia is the symptom or side effect of another problem, it's important to treat the underlying cause (if possible).
Lifestyle Changes
If you have insomnia, avoid substances that make it worse, such as:
Caffeine,
tobacco, and other stimulants. The effects of these substances can last as long as 8 hours.
Certain over-the-counter and prescription medicines that can disrupt
sleep (for example, some cold and
allergy medicines). Talk with your doctor about which medicines won't disrupt your sleep.
Alcohol. An alcoholic drink before bedtime might make it easier for you to
fall asleep. However, alcohol triggers
sleep that tends to be lighter than normal. This makes it more likely that you will wake up during the night.
Try to adopt bedtime habits that make it easier to fall asleep and stay asleep. Follow a routine that helps you wind down and relax before bed. For example, read a book, listen to soothing music, or take a hot bath.
Try to schedule your daily exercise at least 5 to 6 hours before going to bed. Don't eat heavy meals or drink a lot before bedtime.
Make your bedroom sleep-friendly. Avoid bright lighting while winding down. Try to limit possible distractions, such as a TV, computer, or pet. Make sure the temperature of your bedroom is cool and comfortable. Your bedroom also should be dark and quiet.
Go to sleep around the same time each night and wake up around the same time each morning, even on weekends. If you can, avoid night shifts, alternating schedules, or other things that may disrupt your sleep schedule.
Cognitive-Behavioral Therapy
CBT for insomnia targets the thoughts and actions that can disrupt sleep. This therapy encourages good sleep habits and uses several methods to relieve sleep anxiety.
For example, relaxation techniques and biofeedback are used to reduce anxiety. These strategies help you better control your breathing, heart rate, muscles, and mood.
CBT also aims to replace sleep anxiety with more positive thinking that links being in bed with being asleep. This method also teaches you what to do if you're unable to fall asleep within a reasonable time.
CBT also may involve talking with a therapist one-on-one or in group sessions to help you consider your thoughts and feelings about sleep. This method may encourage you to describe thoughts racing through your mind in terms of how they look, feel, and sound. The goal is for your mind to settle down and stop racing.
CBT also focuses on limiting the time you spend in bed while awake. This method involves setting a sleep schedule. At first, you will limit your total time in bed to the typical short length of time you're usually asleep.
This schedule might make you even more tired because some of the allotted time in bed will be taken up by problems falling asleep. However, the resulting tiredness is intended to help you get to sleep more quickly. Over time, the length of time spent in bed is increased until you get a full night of sleep.
For success with CBT, you may need to see a therapist who is skilled in this approach weekly over 2 to 3 months. CBT works as well as prescription medicine for many people who have chronic insomnia. It also may provide better long-term relief than medicine alone.
For people who have insomnia and major depressive disorder, CBT combined with antidepression medicines has shown promise in relieving both conditions.
Medicines
Prescription Medicines
Many prescription medicines are used to treat insomnia. Some are meant for short-term use, while others are meant for longer use.
Talk to your doctor about the benefits and side effects of insomnia medicines. For example, insomnia medicines can help you fall asleep, but you may feel groggy in the morning after taking them.
Rare side effects of these medicines include sleep eating, sleep walking, or driving while asleep. If you have side effects from an insomnia medicine, or if it doesn't work well, tell your doctor. He or she might prescribe a different medicine.
Some insomnia medicines can be habit forming. Ask your doctor about the benefits and risks of insomnia medicines.
Over-the-Counter Products
Some over-the-counter (OTC) products claim to treat insomnia. These products include melatonin, L-tryptophan supplements, and valerian teas or extracts.
The Food and Drug Administration doesn't regulate “natural” products and some food supplements. Thus, the dose and purity of these substances can vary. How well these products work and how safe they are isn't well understood.
Some OTC products that contain antihistamines are sold as sleep aids. Although these products might make you sleepy, talk to your doctor before taking them.
Antihistamines pose risks for some people. Also, these products may not offer the best treatment for your insomnia. Your doctor can advise you whether these products will benefit you.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. For example, this research has uncovered some of the causes of sleep disorders and ways to diagnose and treat these conditions.
The NHLBI continues to support research aimed at learning more about sleep disorders, including insomnia. For example, the NHLBI currently is involved in research comparing two nondrug, self-help treatments for insomnia.
The NHLBI also supports research that explores the factors that affect sleep, how a lack of sleep increases certain health risks, and new ways to diagnose and treat sleep disorders.
Much of this research depends on the willingness of volunteers to take part in clinical trials. Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions.
For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they’re widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don’t directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You’ll learn about treatments and tests you may receive, and the benefits and risks they may pose. You’ll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you’ll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to insomnia, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI’s Children and Clinical Studies Web page.
What Is Iron-Deficiency Anemia?
Iron-deficiency anemia is a common, easily treated condition that occurs if you don't have enough iron in your body. Low iron levels usually are due to blood loss, poor diet, or an inability to absorb enough iron from food.
Overview
Iron-deficiency anemia is a common type of anemia. The term ""anemia"" usually refers to a condition in which your blood has a lower than normal number of red blood cells. Red blood cells carry oxygen and remove carbon dioxide (a waste product) from your body.
Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE-muh-glow-bin). Hemoglobin is an iron-rich protein that carries oxygen from the lungs to the rest of the body.
Iron-deficiency anemia usually develops over time if your body doesn't have enough iron to build healthy red blood cells. Without enough iron, your body starts using the iron it has stored. Soon, the stored iron gets used up.
After the stored iron is gone, your body makes fewer red blood cells. The red blood cells it does make have less hemoglobin than normal.
Iron-deficiency anemia can cause fatigue (tiredness), shortness of breath, chest pain, and other symptoms. Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in children, and other complications.
Infants and young children and women are the two groups at highest risk for iron-deficiency anemia.
What Causes Iron-Deficiency Anemia?
Not having enough iron in your body causes iron-deficiency anemia. Lack of iron usually is due to blood loss, poor diet, or an inability to absorb enough iron from food.
Blood Loss
When you lose blood, you lose iron. If you don't have enough iron stored in your body to make up for the lost iron, you'll develop iron-deficiency anemia.
In women, long or heavy menstrual periods or bleeding fibroids in the uterus may cause low iron levels. Blood loss that occurs during childbirth is another cause of low iron levels in women.
Internal bleeding (bleeding inside the body) also may lead to iron-deficiency anemia. This type of blood loss isn't always obvious, and it may occur slowly. Some causes of internal bleeding are:
Regular use of
aspirin or other pain medicines, such as nonsteroidal anti-inflammatory drugs (for example,
ibuprofen and
naproxen)
Blood loss from severe injuries, surgery, or frequent blood drawings also can cause iron-deficiency anemia.
Poor Diet
The best sources of iron are meat, poultry, fish, and iron-fortified foods (foods that have iron added). If you don't eat these foods regularly, or if you don't take an iron supplement, you're more likely to develop iron-deficiency anemia.
Vegetarian diets can provide enough iron if you eat the right foods. For example, good nonmeat sources of iron include iron-fortified breads and cereals, beans, tofu, dried fruits, and spinach and other dark green leafy vegetables.
During some stages of life, such as pregnancy and childhood, it may be hard to get enough iron in your diet. This is because your need for iron increases during these times of growth and development.
Inability To Absorb Enough Iron
Even if you have enough iron in your diet, your body may not be able to absorb it. This can happen if you have intestinal surgery (such as gastric bypass) or a disease of the intestine (such as Crohn's disease or celiac disease).
Prescription medicines that reduce acid in the stomach also can interfere with iron absorption.
Who Is at Risk for Iron-Deficiency Anemia?
Infants and Young Children
Infants and young children need a lot of iron to grow and develop. The iron that full-term infants have stored in their bodies is used up in the first 4 to 6 months of life.
Premature and low-birth-weight babies (weighing less than 5.5 pounds) are at even greater risk for iron-deficiency anemia. These babies don't have as much iron stored in their bodies as larger, full-term infants.
Iron-fortified baby food or iron supplements, when used properly, can help prevent iron-deficiency anemia in infants and young children. Talk with your child's doctor about your child's diet.
Young children who drink a lot of cow's milk may be at risk for iron-deficiency anemia. Milk is low in iron, and too much milk may take the place of iron-rich foods in the diet. Too much milk also may prevent children's bodies from absorbing iron from other foods.
Children who have lead in their blood also may be at risk for iron-deficiency anemia. Lead can interfere with the body's ability to make hemoglobin. Lead may get into the body from breathing in lead dust, eating lead in paint or soil, or drinking water that contains lead.
Teens
Teens are at risk for iron-deficiency anemia if they're underweight or have chronic (ongoing) illnesses. Teenage girls who have heavy periods also are at increased risk for the condition.
Women
Women of childbearing age are at higher risk for iron-deficiency anemia because of blood loss during their monthly periods. About 1 in 5 women of childbearing age has iron-deficiency anemia.
Pregnant women also are at higher risk for the condition because they need twice as much iron as usual. The extra iron is needed for increased blood volume and for the fetus' growth.
About half of all pregnant women develop iron-deficiency anemia. The condition can increase a pregnant woman's risk for a premature or low-birth-weight baby.
Adults Who Have Internal Bleeding
Adults who have internal bleeding, such as intestinal bleeding, can develop iron-deficiency anemia due to blood loss. Certain conditions, such as colon cancer and bleeding ulcers, can cause blood loss. Some medicines, such as aspirin, also can cause internal bleeding.
What Are the Signs and Symptoms of Iron-Deficiency Anemia?
The signs and symptoms of iron-deficiency anemia depend on its severity. Mild to moderate iron-deficiency anemia may have no signs or symptoms.
When signs and symptoms do occur, they can range from mild to severe. Many of the signs and symptoms of iron-deficiency anemia apply to all types of anemia.
Signs and Symptoms of Anemia
The most common symptom of all types of anemia is fatigue (tiredness). Fatigue occurs because your body doesn't have enough red blood cells to carry oxygen to its many parts.
Also, the red blood cells your body makes have less hemoglobin than normal. Hemoglobin is an iron-rich protein in red blood cells. It helps red blood cells carry oxygen from the lungs to the rest of the body.
Anemia also can cause shortness of breath, dizziness, headache, coldness in your hands and feet, pale skin, chest pain, weakness, and fatigue (tiredness).
If you don't have enough hemoglobin-carrying red blood cells, your heart has to work harder to move oxygen-rich blood through your body. This can lead to irregular heartbeats called arrhythmias (ah-RITH-me-ahs), a heart murmur, an enlarged heart, or even heart failure.
In infants and young children, signs of anemia include poor appetite, slowed growth and development, and behavioral problems.
Signs and Symptoms of Iron Deficiency
Signs and symptoms of iron deficiency may include brittle nails, swelling or soreness of the tongue, cracks in the sides of the mouth, an enlarged spleen, and frequent infections.
People who have iron-deficiency anemia may have an unusual craving for nonfood items, such as ice, dirt, paint, or starch. This craving is called pica (PI-ka or PE-ka).
Some people who have iron-deficiency anemia develop restless legs syndrome (RLS). RLS is a disorder that causes a strong urge to move the legs. This urge to move often occurs with strange and unpleasant feelings in the legs. People who have RLS often have a hard time sleeping.
Iron-deficiency anemia can put children at greater risk for lead poisoning and infections.
Some signs and symptoms of iron-deficiency anemia are related to the condition's causes. For example, a sign of intestinal bleeding is bright red blood in the stools or black, tarry-looking stools.
Very heavy menstrual bleeding, long periods, or other vaginal bleeding may suggest that a woman is at risk for iron-deficiency anemia.
How Is Iron-Deficiency Anemia Diagnosed?
Your doctor will diagnose iron-deficiency anemia based on your medical history, a physical exam, and the results from tests and procedures.
Once your doctor knows the cause and severity of the condition, he or she can create a treatment plan for you.
Mild to moderate iron-deficiency anemia may have no signs or symptoms. Thus, you may not know you have it unless your doctor discovers it from a screening test or while checking for other problems.
Specialists Involved
Primary care doctors often diagnose and treat iron-deficiency anemia. These doctors include pediatricians, family doctors, gynecologists/obstetricians, and internal medicine specialists.
A hematologist (a blood disease specialist), a gastroenterologist (a digestive system specialist), and other specialists also may help treat iron-deficiency anemia.
Medical History
Your doctor will ask about your signs and symptoms and any past problems you've had with anemia or low iron. He or she also may ask about your diet and whether you're taking any medicines.
If you're a woman, your doctor may ask whether you might be pregnant.
Physical Exam
Your doctor will do a physical exam to look for signs of iron-deficiency anemia. He or she may:
Look at your
skin,
gums, and
nail beds to see whether they're pale
Listen to your
lungs for rapid or uneven breathing
Do a pelvic and rectal exam to check for internal
bleeding
Diagnostic Tests and Procedures
Many tests and procedures are used to diagnose iron-deficiency anemia. They can help confirm a diagnosis, look for a cause, and find out how severe the condition is.
Complete Blood Count
Often, the first test used to diagnose anemia is a complete blood count (CBC). The CBC measures many parts of your blood.
This test checks your hemoglobin and hematocrit (hee-MAT-oh-crit) levels. Hemoglobin is an iron-rich protein in red blood cells that carries oxygen to the body. Hematocrit is a measure of how much space red blood cells take up in your blood. A low level of hemoglobin or hematocrit is a sign of anemia.
The normal range of these levels varies in certain racial and ethnic populations. Your doctor can explain your test results to you.
The CBC also checks the number of red blood cells, white blood cells, and platelets in your blood. Abnormal results may be a sign of infection, a blood disorder, or another condition.
Finally, the CBC looks at mean corpuscular (kor-PUS-kyu-lar) volume (MCV). MCV is a measure of the average size of your red blood cells. The results may be a clue as to the cause of your anemia. In iron-deficiency anemia, for example, red blood cells usually are smaller than normal.
Other Blood Tests
If the CBC results confirm you have anemia, you may need other blood tests to find out what's causing the condition, how severe it is, and the best way to treat it.
Reticulocyte count. This test measures the number of reticulocytes (re-TIK-u-lo-sites) in your blood. Reticulocytes are young, immature red blood cells. Over time, reticulocytes become mature red blood cells that carry oxygen throughout your body.
A reticulocyte count shows whether your bone marrow is making red blood cells at the correct rate.
Peripheral smear. For this test, a sample of your blood is examined under a microscope. If you have iron-deficiency anemia, your red blood cells will look smaller and paler than normal.
Tests to measure iron levels. These tests can show how much iron has been used from your body's stored iron. Tests to measure iron levels include:
Serum
iron. This test measures the amount of iron in your
blood. The level of iron in your blood may be normal even if the total amount of iron in your body is low. For this reason, other iron tests also are done.
Serum ferritin. Ferritin is a
protein that helps store
iron in your body. A measure of this protein helps your doctor find out how much of your body's stored iron has been used.
Transferrin level, or total
iron-binding capacity. Transferrin is a
protein that carries iron in your
blood. Total iron-binding capacity measures how much of the transferrin in your blood isn't carrying iron. If you have iron-deficiency
anemia, you'll have a high level of transferrin that has no iron.
Other tests. Your doctor also may recommend tests to check your hormone levels, especially your thyroid hormone. You also may have a blood test for a chemical called erythrocyte protoporphyrin. This chemical is a building block for hemoglobin.
Children also may be tested for the level of lead in their blood. Lead can make it hard for the body to produce hemoglobin.
Tests and Procedures for Gastrointestinal Blood Loss
To check whether internal bleeding is causing your iron-deficiency anemia, your doctor may suggest a fecal occult blood test. This test looks for blood in the stools and can detect bleeding in the intestines.
If the test finds blood, you may have other tests and procedures to find the exact spot of the bleeding. These tests and procedures may look for bleeding in the stomach, upper intestines, colon, or pelvic organs.
How Is Iron-Deficiency Anemia Treated?
Treatment for iron-deficiency anemia will depend on its cause and severity. Treatments may include dietary changes and supplements, medicines, and surgery.
Severe iron-deficiency anemia may require a blood transfusion, iron injections, or intravenous (IV) iron therapy. Treatment may need to be done in a hospital.
The goals of treating iron-deficiency anemia are to treat its underlying cause and restore normal levels of red blood cells, hemoglobin, and iron.
Dietary Changes and Supplements
Iron
You may need iron supplements to build up your iron levels as quickly as possible. Iron supplements can correct low iron levels within months. Supplements come in pill form or in drops for children.
Large amounts of iron can be harmful, so take iron supplements only as your doctor prescribes. Keep iron supplements out of reach from children. This will prevent them from taking an overdose of iron.
Iron supplements can cause side effects, such as dark stools, stomach irritation, and heartburn. Iron also can cause constipation, so your doctor may suggest that you use a stool softener.
Your doctor may advise you to eat more foods that are rich in iron. The best source of iron is red meat, especially beef and liver. Chicken, turkey, pork, fish, and shellfish also are good sources of iron.
The body tends to absorb iron from meat better than iron from nonmeat foods. However, some nonmeat foods also can help you raise your iron levels. Examples of nonmeat foods that are good sources of iron include:
Iron-fortified breads and cereals
Peas; lentils; white, red, and baked beans; soybeans; and chickpeas
Tofu
Dried fruits, such as prunes, raisins, and apricots
Spinach and other dark green leafy vegetables
Prune juice
The Nutrition Facts labels on packaged foods will show how much iron the items contain. The amount is given as a percentage of the total amount of iron you need every day.
Vitamin C
Vitamin C helps the body absorb iron. Good sources of vitamin C are vegetables and fruits, especially citrus fruits. Citrus fruits include oranges, grapefruits, tangerines, and similar fruits. Fresh and frozen fruits, vegetables, and juices usually have more vitamin C than canned ones.
If you're taking medicines, ask your doctor or pharmacist whether you can eat grapefruit or drink grapefruit juice. Grapefruit can affect the strength of a few medicines and how well they work.
Other fruits rich in vitamin C include kiwi fruit, strawberries, and cantaloupes.
Vegetables rich in vitamin C include broccoli, peppers, Brussels sprouts, tomatoes, cabbage, potatoes, and leafy green vegetables like turnip greens and spinach.
Treatment To Stop Bleeding
If blood loss is causing iron-deficiency anemia, treatment will depend on the cause of the bleeding. For example, if you have a bleeding ulcer, your doctor may prescribe antibiotics and other medicines to treat the ulcer.
If a polyp or cancerous tumor in your intestine is causing bleeding, you may need surgery to remove the growth.
If you have heavy menstrual flow, your doctor may prescribe birth control pills to help reduce your monthly blood flow. In some cases, surgery may be advised.
Treatments for Severe Iron-Deficiency Anemia
Iron Therapy
If you have severe anemia, your doctor may recommend iron therapy. For this treatment, iron is injected into a muscle or an IV line in one of your blood vessels.
IV iron therapy presents some safety concerns. It must be done in a hospital or clinic by experienced staff. Iron therapy usually is given to people who need iron long-term but can't take iron supplements by mouth. This therapy also is given to people who need immediate treatment for iron-deficiency anemia.
How Can Iron-Deficiency Anemia Be Prevented?
Eating a well-balanced diet that includes iron-rich foods may help you prevent iron-deficiency anemia.
Taking iron supplements also may lower your risk for the condition if you're not able to get enough iron from food. Large amounts of iron can be harmful, so take iron supplements only as your doctor prescribes.
For more information about diet and supplements, go to ""How Is Iron-Deficiency Anemia Treated?""
Infants and young children and women are the two groups at highest risk for iron-deficiency anemia. Special measures can help prevent the condition in these groups.
Infants and Young Children
A baby's diet can affect his or her risk for iron-deficiency anemia. For example, cow's milk is low in iron. For this and other reasons, cow's milk isn't recommended for babies in their first year. After the first year, you may need to limit the amount of cow's milk your baby drinks.
Also, babies need more iron as they grow and begin to eat solid foods. Talk with your child's doctor about a healthy diet and food choices that will help your child get enough iron.
Your child's doctor may recommend Go to:
What Is Kawasaki Disease?
Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease. It's a form of a condition called vasculitis (vas-kyu-LI-tis). This condition involves inflammation of the blood vessels.
In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries.
Sometimes Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, some children who have Kawasaki disease may develop serious heart problems.
Overview
The cause of Kawasaki disease isn't known. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been found, and the role of genetics isn't known.
The disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it to another child.
Kawasaki disease affects children of all races and ages and both genders. It occurs most often in children of Asian and Pacific Island descent. The disease is more likely to affect boys than girls. Most cases occur in children younger than 5 years old.
One of the main symptoms of Kawasaki disease is a fever that lasts longer than 5 days. The fever remains high even after treatment with standard childhood fever medicines.
Children who have the disease also may have red eyes, red lips, and redness on the palms of their hands and soles of their feet. These are all signs of inflamed blood vessels.
Early treatment helps reduce the risk of Kawasaki disease affecting the coronary arteries and causing serious problems.
Outlook
Kawasaki disease can't be prevented. However, most children who have the disease usually recover within weeks of getting symptoms. Further problems are rare.
The disease affects some children's coronary arteries, which can cause serious problems. These children need long-term care and treatment.
Researchers continue to look for the cause of Kawasaki disease and better ways to diagnose and treat it. They also hope to learn more about long-term health risks, if any, for people who have had the disease.
Other Names for Kawasaki Disease
What Causes Kawasaki Disease?
The cause of Kawasaki disease isn't known. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been found, and the role of genetics isn't known.
Kawasaki disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it to another child.
Who Is at Risk for Kawasaki Disease?
Kawasaki disease affects children of all races and ages and both genders. It occurs most often in children of Asian and Pacific Island descent.
The disease is more likely to affect boys than girls. Most cases occur in children younger than 5 years old. Kawasaki disease is rare in children older than 8.
What Are the Signs and Symptoms of Kawasaki Disease?
Major Signs and Symptoms
One of the main symptoms during the early part of Kawasaki disease, called the acute phase, is fever. The fever lasts longer than 5 days. It remains high even after treatment with standard childhood fever medicines.
Other classic signs of the disease are:
A
rash on the mid-section of the body and in the genital area
Red, swollen
palms of the
hands and soles of the feet
Other Signs and Symptoms
During the acute phase, your child also may be irritable and have a sore throat, joint pain, diarrhea, vomiting, and stomach pain.
Within 2 to 3 weeks of the start of symptoms, the skin on your child's fingers and toes may peel, sometimes in large sheets.
How Is Kawasaki Disease Diagnosed?
Kawasaki disease is diagnosed based on your child's signs and symptoms and the results from tests and procedures.
Specialists Involved
Pediatricians often are the first to suspect a child has Kawasaki disease. Pediatricians are doctors who specialize in treating children.
If the disease has affected your child's coronary (heart) arteries, a pediatric cardiologist will confirm the diagnosis and give ongoing treatment. Pediatric cardiologists treat children who have heart problems.
Other specialists also may be involved in treating children who have Kawasaki disease.
Signs and Symptoms
The doctor will check your child for the classic signs and symptoms of Kawasaki disease.
The doctor will rule out other diseases that cause similar symptoms. These diseases include Rocky Mountain spotted fever, scarlet fever, and juvenile rheumatoid arthritis.
Generally, your child will be diagnosed with Kawasaki disease if he or she has a fever that lasts longer than 5 days plus four other classic signs or symptoms of the disease.
However, not all children have classic signs and symptoms of Kawasaki disease. Tests and procedures can help confirm whether a child has the disease.
Tests and Procedures
Echocardiography
If the doctor thinks that your child has Kawasaki disease, he or she will likely recommend echocardiography (EK-o-kar-de-OG-ra-fee), or echo. This painless test uses sound waves to create pictures of the heart and coronary arteries.
Echo also can help show the disease's effects over time, if any, on your child's coronary arteries. Often, the disease's effects on the coronary arteries don't show up until the second or third week after the first symptoms appear. Thus, this test is done regularly after the diagnosis.
Some children who have Kawasaki disease don't have the classic signs and symptoms of the acute phase. Doctors may not diagnose these children until 2 to 3 weeks after the onset of the disease. This is when another common sign of Kawasaki disease occurs—peeling of the skin on the fingers and toes.
If your child is diagnosed at this point, he or she will likely need echo right away to see whether the disease has affected the coronary arteries.
Other Diagnostic Tests
Doctors also use other tests to help diagnose Kawasaki disease, such as:
How Is Kawasaki Disease Treated?
Medicines are the main treatment for Kawasaki disease. Rarely, children whose coronary (heart) arteries are affected may need medical procedures or surgery.
The goals of treatment include:
Initial Treatment
Kawasaki disease can cause serious health problems. Thus, your child will likely be treated in a hospital, at least for the early part of treatment.
The standard treatment during the disease's acute phase is high-dose aspirin and immune globulin. Immune globulin is a medicine that's injected into a vein.
Most children who receive these treatments improve greatly within 24 hours. For a small number of children, fever remains. These children may need a second round of immune globulin.
At the start of treatment, your child will receive high doses of aspirin. As soon as his or her fever goes away, a low dose of aspirin is given. The low dose helps prevent blood clots, which can form in the inflamed small arteries.
Most children treated for Kawasaki disease fully recover from the acute phase and don't need any further treatment. They should, however, follow a healthy diet and adopt healthy lifestyle habits. Taking these steps can help lower the risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease.)
Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines from working well.
Long-Term Care and Treatment
If Kawasaki disease has affected your child's coronary arteries, he or she will need ongoing care and treatment.
It's best if a pediatric cardiologist provides this care to reduce the risk of severe heart problems. A pediatric cardiologist is a doctor who specializes in treating children who have heart problems.
Medicines and Tests
When Kawasaki disease affects the coronary arteries, they may expand and twist. If this happens, your child's doctor may prescribe blood-thinning medicines (for example, warfarin). These medicines help prevent blood clots from forming in the affected coronary arteries.
Blood-thinning medicines usually are stopped after the coronary arteries heal. Healing may occur about 18 months after the acute phase of the disease.
In a small number of children, the coronary arteries don't heal. These children likely will need routine tests, such as:
Medical Procedures and Surgery
Rarely, a child who has Kawasaki disease may need cardiac catheterization (KATH-eh-ter-ih-ZA-shun). Doctors use this procedure to diagnose and treat some heart conditions.
A flexible tube called a catheter is put into a blood vessel in the arm, groin (upper thigh), or neck and threaded to the heart. Through the catheter, doctors can perform tests and treatments on the heart.
Very rarely, a child may need to have other procedures or surgery if inflammation narrows his or her coronary arteries and blocks blood flow to the heart. Coronary angioplasty (AN-jee-oh-plas-tee), stent placement, or coronary artery bypass grafting (CABG) may be used.
Coronary angioplasty restores blood flow through narrowed or blocked coronary arteries. A thin tube with a balloon on the end is inserted into a blood vessel in the arm or groin. The tube is threaded to the narrowed or blocked coronary artery. Then, the balloon is inflated to widen the artery and restore blood flow.
A stent (small mesh tube) may be placed in the coronary artery during angioplasty. This device helps support the narrowed or weakened artery. A stent can improve blood flow and prevent the artery from bursting.
Rarely, a child may need to have CABG. This surgery is used to treat blocked coronary arteries. During CABG, a healthy artery or vein from another part of the body is connected, or grafted, to the blocked coronary artery.
The grafted artery or vein bypasses (that is, goes around) the blocked part of the coronary artery. This improves blood flow to the heart.
How Can Kawasaki Disease Be Prevented?
Kawasaki disease can't be prevented. However, most children who have the disease recover—usually within weeks of getting signs and symptoms. Further problems are rare.
Living With Kawasaki Disease
Most children who have Kawasaki disease recover—usually within weeks of getting symptoms. Further problems are rare. Early treatment reduces the risk of serious problems.
Researchers continue to look for the cause of Kawasaki disease and better ways to diagnose and treat it. They also hope to learn more about long-term health risks, if any, for people who have had the disease.
What To Expect After Treatment
Most children who are treated for Kawasaki disease fully recover from the acute phase. They don't need further treatment.
They should, however, follow a healthy diet and adopt healthy lifestyle habits. Taking these steps can help lower their risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease).
Children treated with immune globulin should wait 11 months before having measles and chicken pox vaccines. Immune globulin can prevent these vaccines from working well.
Ongoing Health Care Needs
If Kawasaki disease has affected your child's coronary arteries, he or she will need ongoing care and treatment. It's best if a pediatric cardiologist provides this care to reduce the risk of severe heart problems. A pediatric cardiologist is a doctor who specializes in treating children who have heart problems.
Support Groups
Joining a support group may help you adjust to caring for a child who has Kawasaki disease. You can see how other parents have coped with the disease. Ask your child's doctor about local support groups or check with an area medical center.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, your child can gain access to new treatments before they're widely available. Your child also will have the support of a team of health care providers, who will likely monitor his or her health closely. Even if your child doesn't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
Children (aged 18 and younger) get special protection as research subjects. Almost always, parents must give legal consent for their child to take part in a clinical trial.
When researchers think that a trial's potential risks are greater than minimal, both parents must give permission for their child to enroll. Also, children aged 7 and older often must agree (assent) to take part in clinical trials.
If you agree to have your child take part in a clinical trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw your child from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to Kawasaki disease, talk with your child's doctor. For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
What Is LAM?
LAM, or lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), is a rare lung disease that mostly affects women of childbearing age.
In LAM, abnormal, muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys.
Over time, these LAM cells can destroy the normal lung tissue. As a result, air can’t move freely in and out of the lungs. In some cases, this means the lungs can’t supply the body’s other organs with enough oxygen.
Overview
There are two forms of LAM. Sporadic LAM occurs for unknown reasons. LAM also can occur in women who have a rare disease called tuberous sclerosis complex (TSC). Women who have TSC often have a milder form of LAM.
About 50 percent of women who have LAM develop pneumothorax (noo-mo-THOR-aks), or collapsed lung. In this condition, air leaks out of the lung and into the space between the lung and chest wall (the pleural space).
A collapsed lung can cause pain and shortness of breath. Sometimes one lung will collapse over and over again. Pneumothorax is a serious condition. It usually requires treatment and might be life threatening.
Many women who have LAM get tumors called angiomyolipomas (AN-je-o-my-o-li-PO-mas), or AMLs, in their kidneys. Women who have LAM also may develop:
Outlook
LAM has no cure, and the disease tends to worsen over time. How quickly the disease worsens varies from woman to woman. LAM may lead to death from respiratory failure. Lung transplant is a treatment option for women whose lungs have been damaged by LAM.
Not long ago, doctors thought women who had LAM wouldn't live more than 8–10 years following diagnosis. They now know that some women may survive longer (as long as 20 years following diagnosis, although this is rare).
Doctors have learned a lot about LAM in recent years. They're now able to diagnose the condition earlier. Support services also are now available to help improve the quality of life for women who have LAM.
Researchers continue to explore and test new treatments for LAM.
What Causes LAM?
The cause of LAM and why it mainly affects women isn’t known. Recent studies show that sporadic LAM has some of the same traits as another rare disease called tuberous sclerosis complex (TSC). This information has provided some valuable clues about what causes LAM.
The common features of sporadic LAM and TSC are:
Kidney growths. People who have TSC get growths in their
kidneys. These growths are the same as the
angiomyolipomas that many women who have LAM get in their kidneys.
Lung
cysts. Some women who have TSC get cysts in their
lungs. These cysts are the same as the ones that women who have sporadic LAM get in their lungs. When a woman who has TSC gets cysts in her lungs, the
lung disease is called TSC-associated LAM or TSC–LAM.
TSC is a genetic disease. A defect in one of two genes causes the disease. These genes are called TSC1 and TSC2. They normally make proteins that control cell growth and movement in the body. In people who have TSC, the genes are faulty. The proteins that the genes make can’t control cell growth and movement.
Women who have LAM also have abnormal TSC1 and TSC2 genes. Researchers have found that these genes play a role in causing LAM. This finding is leading to new treatments for LAM.
Because LAM affects women, the hormone estrogen also may play a role in causing the disease.
Who Is at Risk for LAM?
LAM is a rare disease that mostly affects women of childbearing age. Many women who develop LAM are between the ages of 20 and 40 when they begin to have symptoms. LAM can occur in older women as well, although this is less common.
Some women might have LAM and not know it. Many of LAM’s signs and symptoms are the same as those of other diseases, such as asthma, emphysema (em-fih-SE-ma), and bronchitis (brong-KI-tis).
LAM affects about 3 out of every 10 women who have tuberous sclerosis complex (TSC). Some of these women may have mild cases of LAM that don’t cause symptoms. Not everyone who has TSC and LAM has lung symptoms.
In rare cases, LAM has been reported in men.
What Are the Signs and Symptoms of LAM?
The uncontrolled growth of LAM cells and their effect on nearby body tissues causes the signs and symptoms of LAM. The most common signs and symptoms are:
Shortness of breath, especially during activity. At first, shortness of breath may occur only during high-energy activities. Over time, you may have trouble breathing during simple activities, such as dressing and showering.
Chest pain or aches. This pain might be worse when you breathe in.
Frequent
cough. This may occur with bloody
phlegm (a sticky fluid).
Wheezing (a whistling sound when you breathe).
Other signs and symptoms of LAM include:
Other diseases also can cause many of these signs and symptoms. If you’re having any of these problems, see your doctor. He or she can help find the cause of your symptoms.
How Is LAM Diagnosed?
Methods for diagnosing LAM have improved. It's now possible for doctors to diagnose the disease at an early stage.
LAM is diagnosed based on your signs and symptoms and the results from tests and procedures. If you have LAM, you may need to see a pulmonologist. This is a doctor who specializes in lung diseases and conditions.
Signs and Symptoms
Your doctor will ask about your signs and symptoms related to LAM. He or she may ask how long you’ve had symptoms, and whether they’ve become worse over time.
Many of LAM’s signs and symptoms are the same as those of other diseases, such as asthma, emphysema, and bronchitis. Your doctor will want to rule out those conditions before making a final diagnosis.
Diagnostic Tests and Procedures
Your doctor may recommend tests to show how well your lungs are working and what your lung tissue looks like.
These tests can show whether your lungs are delivering enough oxygen to your blood. You also may have tests to check for complications of LAM.
Tests for Lung Function
Lung function tests. For lung function tests, you breathe through a mouthpiece into a machine called a spirometer (spi-ROM-eh-ter). The spirometer measures the amount of air you breathe in and out.
Other lung function tests can show about how much air your lungs can hold and how well your lungs deliver oxygen to your blood.
Blood tests. Your doctor may take a blood sample from a vein in your arm to look at your blood cells and blood chemistry.
Pulse oximetry. For this test, a small sensor is attached to your finger or ear. The sensor uses light to estimate how much oxygen is in your blood.
Tests To Check for Complications or Detect LAM Cells
Chest x ray. A chest x ray creates a picture of the structures in your chest, such as your heart and lungs. The test can show a collapsed lung or fluid in your chest. In the early stages of LAM, your chest x rays may look normal. As the disease gets worse, the x rays may show cysts in your lungs.
High-resolution CT (HRCT) scan. The most useful imaging test for diagnosing LAM is an HRCT scan. This test creates a computer-generated picture of your lungs. The picture shows more detail than the pictures from a chest x ray.
An HRCT scan can show cysts, shadows of cell clusters, excess fluid, a collapsed lung, and enlarged lymph nodes. The test also can show how much normal lung tissue has been replaced by the LAM cysts.
HRCT scans of your abdomen and pelvis can show whether you have growths in your kidneys, other abdominal organs, or lymph nodes.
Procedures To Look for LAM Cells
The results from the above tests—along with information about your signs, symptoms, and medical history—might be enough for your doctor to diagnose LAM.
However, if your doctor needs more information, the most useful method involves looking at samples of your lung tissue for LAM cells.
You may want to see a doctor who specializes in LAM for this test. Several procedures can be used to get a sample of lung tissue.
Video-assisted thoracoscopy (tho-rah-KOS-ko-pe). In this procedure, also called VAT, your doctor inserts a small, lighted tube into little cuts made in your chest wall. This lets him or her look inside your chest and snip out a few small pieces of lung tissue.
VAT is done in a hospital. The procedure isn’t major surgery, but it does require general anesthesia (that is, you’re given medicine to make you sleep during the procedure).
Open lung biopsy. In this procedure, your doctor removes a few small pieces of lung tissue through a cut made in your chest wall between your ribs. An open lung biopsy is done in a hospital. You’ll be given medicine to make you sleep during the procedure.
Open lung biopsies are rarely done anymore because the recovery time is much longer than the recovery time from VAT.
Transbronchial biopsy. In this procedure, your doctor inserts a long, narrow, flexible, lighted tube down your windpipe and into your lungs. He or she then snips out bits of lung tissue using a tiny device.
This procedure usually is done in a hospital. Your mouth and throat are numbed to prevent pain.
The amount of tissue that your doctor removes is very small, so this test doesn’t always provide enough information.
Other biopsies. Your doctor also can diagnose LAM using the results from other tissue biopsies, such as biopsies of lymph nodes or lymphatic tumors called lymphangiomyomas.
Other Tests
If you’re diagnosed with sporadic LAM, your doctor may advise you to have a computed tomography (CT) scan or magnetic resonance imaging (MRI) scan of your head. These tests can help screen for underlying tuberous sclerosis complex (TSC).
CT and MRI scans will reveal TSC in only a small number of people who are initially diagnosed with sporadic LAM.
Researchers are exploring other tests that may help diagnose LAM. These tests include blood tests for the LAM cells or a blood vessel growth factor called VEGF-D.
How Is LAM Treated?
Currently, no treatment is available to stop the growth of the cysts and cell clusters that occur in LAM. Most treatments for LAM are aimed at easing symptoms and preventing complications.
The main treatments are:
Medicines to improve air flow in the
lungs and reduce wheezing
Procedures to remove fluid from the
chest or
abdomen and stop it from building up again
Medicines
Medicines That Help You Breathe Better
If you’re having trouble breathing, your doctor may prescribe bronchodilators. These medicines relax the muscles around the airways. This helps the airways open up, making it easier for you to breathe.
Lung function tests can sometimes show whether these medicines are likely to help you.
Medicines That Prevent Bone Loss
Women who have LAM are at risk for a bone-weakening condition called osteoporosis (OS-te-o-po-RO-sis). This is in part because many LAM therapies block the estrogen action needed to keep bones strong.
To prevent osteoporosis, your doctor may measure your bone density. If you have lost bone density, your doctor may prescribe medicines to prevent bone loss. He or she also may prescribe calcium and vitamin D supplements.
Rapamycin (Sirolimus)
Sirolimus was originally developed to prevent the immune system from rejecting kidney transplants. However, studies have shown that the medicine helps regulate the abnormal growth and movement of LAM cells.
Research suggests that sirolimus may shrink tumors in the kidneys of women who have LAM.
A recent study funded in part by the National Institutes of Health showed that sirolimus also helps stabilize lung function, reduce symptoms, and improve quality of life for people who have LAM.
Sirolimus does have side effects, some of which can be serious. If you have LAM, talk with your doctor about the benefits and risks of this medicine, and whether it’s an option for you.
Oxygen Therapy
If the level of oxygen in your blood is low, your doctor may suggest oxygen therapy. Oxygen usually is given through nasal prongs or a mask. At first, you may need oxygen only while exercising. It also may help to use it while sleeping. Over time, you may need full-time oxygen therapy.
A standard exercise stress test or a 6-minute walk test can show whether you need oxygen while exercising. A 6-minute walk test measures the distance you can walk in 6 minutes. An exercise stress test measures how well your lungs and heart work while you walk on a treadmill or pedal a stationary bike.
You also may need a blood test to show your blood oxygen level and how much oxygen you need.
For more information, go to the Health Topics Oxygen Therapy article.
Procedures To Remove Air or Fluid From the Chest or Abdomen
Several procedures can remove excess air or fluid from your chest and abdomen. These procedures also help prevent air or fluid from building up again.
Removing fluid from your chest or abdomen may help relieve discomfort and shortness of breath. The procedure to remove fluid from the chest is called thoracentesis (THOR-ah-sen-TE-sis). The procedure to remove fluid from the abdomen is called paracentesis (PAR-ah-sen-TE-sis).
Your doctor often can remove the fluid with a needle and syringe. If large amounts of fluid build up in your chest, your doctor may have to insert a tube into your chest to remove the fluid.
Removing air from your chest may relieve shortness of breath and chest pain caused by a collapsed lung. Your doctor usually can remove the air with a tube. The tube is inserted into your chest between your side ribs. Often, the tube is attached to a suction device. If this procedure doesn't work, or if your lungs collapse often, you may need surgery.
If fluid and air often leak into your chest, your doctor may inject a chemical at the site of the leakage. The chemical fuses your lung and chest wall together. This removes the space for leakage.
Your doctor may do this procedure at your bedside in the hospital. You will be given medicine to prevent pain. The procedure also can be done in an operating room using video-assisted thoracoscopy. In this case, you will be given medicine to make you sleep during the procedure.
Procedures To Remove or Shrink Angiomyolipomas
AMLs often don't cause symptoms, but sometimes they can cause ongoing pain or bleeding. If this happens, you may need surgery to remove some of the tumors.
If bleeding isn't too severe, a radiologist often can block the blood vessels feeding the AMLs. This may cause them to shrink.
Lung Transplant
Lung transplants can improve lung function and quality of life in patients who have advanced LAM.
However, lung transplants have a high risk of complications, including infections and rejection of the transplanted lung by the body.
Studies suggest that more than three-quarters of women with LAM who receive a lung transplant survive for at least 3 years.
In a few cases, doctors have found LAM cells in the newly transplanted lungs and other parts of the body. However, the LAM cells don't seem to stop the transplanted lung from working.
For more information, go to the Health Topics Lung Transplant article.
Hormone Therapy
Estrogen is thought to play a role in causing LAM. Thus, your doctor may want to treat you with hormone therapy that limits the effects of estrogen on your body. Hormone therapy is given in pill form or as injections.
Some doctors also suggest surgery to remove the ovaries. This causes menopause and greatly reduces estrogen levels in the body.
Unfortunately, at this time, no clear evidence shows that this type of treatment works for women who have LAM.
Living With LAM
In the early stages of LAM, you usually can do your normal daily activities. These may include attending school, going to work, and doing common physical activities such as walking up stairs.
In the later stages of LAM, you may find it harder to be active. You also may need oxygen therapy full time.
Ongoing Care
Getting ongoing medical care is important. You may need to see a pulmonologist. This is a doctor who specializes in treating lung diseases and conditions.
Take steps to care for your lungs. For example, talk with your doctor about getting a yearly flu shot and pneumonia vaccine.
Lifestyle Changes
If you have LAM, taking good care of your health is important. Follow a healthy eating plan, be as physically active as you can, and get plenty of rest.
If you smoke, quit. Talk to your doctor about programs and products that can help you quit smoking.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking. Ask your family members and friends to support you in your efforts to quit.
For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article and the National Heart, Lung, and Blood Institute's (NHLBI’s) ""Your Guide to a Healthy Heart."" Although these resources focus on heart health, they include basic information about how to quit smoking.
Also, check with your doctor before traveling by air or traveling to areas where medical attention isn't readily available. Also, talk to your doctor before traveling to places where the amount of oxygen in the air is low.
Pregnancy and Birth Control
If your lung function is normal, pregnancy might be an option. However, hormones during pregnancy can worsen LAM. Thus, you should discuss a possible pregnancy with both a pulmonologist who specializes in LAM and your obstetrician.
Most doctors don't recommend birth control pills containing estrogen to women who have LAM. Talk to your doctor about birth control options.
Emotional Issues and Support
Living with LAM may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you’re very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with LAM. Information about patient support groups is available from the NHLBI Health Information Center at 301–592–8573 or the National Institutes of Health/NHLBI Pulmonary Vascular Medicine Branch at 1–877–NIH–LUNG (1–877–644–5864), extension 3.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. For example, this research has uncovered some of the causes of chronic lung diseases, as well as ways to prevent and treat these diseases.
Many more questions remain about chronic lung diseases, including LAM. The NHLBI continues to support research aimed at learning more about these diseases. For example, NHLBI-supported research on LAM includes studies that explore:
The disease processes involved in LAM, and how to develop better
treatments based on these processes
Much of the NHLBI’s research depends on the willingness of volunteers to take part in clinical trials. Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions.
For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you may gain access to new treatments before they’re widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don’t directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You’ll learn about treatments and tests you may receive, and the benefits and risks they may pose. You’ll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you’ll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to LAM, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
What Is Long QT Syndrome?
Long QT syndrome (LQTS) is a disorder of the heart's electrical activity. It can cause sudden, uncontrollable, dangerous arrhythmias (ah-RITH-me-ahs) in response to exercise or stress. Arrhythmias are problems with the rate or rhythm of the heartbeat.
People who have LQTS also can have arrhythmias for no known reason. However, not everyone who has LQTS has dangerous heart rhythms. When they do occur, though, they can be fatal.
What Does ""Long QT"" Mean?
The term ""long QT"" refers to an abnormal pattern seen on an EKG (electrocardiogram). An EKG is a test that detects and records the heart's electrical activity.
With each heartbeat, an electrical signal spreads from the top of your heart to the bottom. As it travels, the signal causes the heart to contract and pump blood. An EKG records electrical signals as they move through your heart.
Data from the EKG are mapped on a graph so your doctor can study your heart's electrical activity. Each heartbeat is mapped as five distinct electrical waves: P, Q, R, S, and T.
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EKG. The picture shows the standard setup for an EKG. Figure A shows the data from the EKG, which are mapped on a graph. In figure B, a patient lies in bed with EKG electrodes attached to his chest, upper arms, and legs. A nurse oversees the painless procedure. (more...)
The electrical activity that occurs between the Q and T waves is called the QT interval. This interval shows electrical activity in the heart's lower chambers, the ventricles (VEN-trih-kuls).
The timing of the heart's electrical activity is complex, and the body carefully controls it. Normally the QT interval is about a third of each heartbeat cycle. However, in people who have LQTS, the QT interval lasts longer than normal.
A long QT interval can upset the careful timing of the heartbeat and trigger dangerous heart rhythms.
For more information about the heart's electrical system, go to the Health Topics How the Heart Works article.
Overview
On the surface of each heart muscle cell are tiny pores called ion channels. Ion channels open and close to let electrically charged sodium, calcium, and potassium atoms (ions) flow into and out of each cell. This generates the heart's electrical activity.
In people who have LQTS, the ion channels may not work well, or there may be too few of them. This may disrupt electrical activity in the heart's ventricles and cause dangerous arrhythmias.
LQTS often is inherited, which means you're born with the condition and have it your whole life. There are seven known types of inherited LQTS. The most common ones are LQTS 1, 2, and 3.
In LQTS 1, emotional stress or exercise (especially swimming) can trigger arrhythmias. In LQTS 2, extreme emotions, such as surprise, can trigger arrhythmias. In LQTS 3, a slow heart rate during sleep can trigger arrhythmias.
You also can acquire LQTS. This means you aren't born with the disorder, but you develop it during your lifetime. Some medicines and conditions can cause acquired LQTS. (For more information, go to ""What Causes Long QT Syndrome?"")
Outlook
More than half of the people who have untreated, inherited types of LQTS die within 10 years. However, lifestyle changes and medicines can help people who have LQTS prevent complications and live longer.
Some of these lifestyle changes and treatments include:
If you have LQTS, talk with your doctor about which lifestyle changes and treatments are best for you.
Other Names for Long QT Syndrome
What Causes Long QT Syndrome?
Long QT syndrome (LQTS) can be inherited or acquired. ""Inherited"" means you're born with the condition and have it your whole life. Inherited conditions are passed from parents to children through genes. ""Acquired"" means you aren't born with the condition, but you develop it during your lifetime.
Inherited Long QT Syndrome
Faulty genes cause inherited LQTS. These genes control the production of certain types of ion channels in your heart. Faulty genes may cause the body to make too few ion channels, ion channels that don't work well, or both.
There are seven known types of inherited LQTS (types 1 though 7). The most common types are LQTS 1, 2, and 3.
Some types of LQTS involve faulty or lacking potassium ion or sodium ion channels.
If you have LQTS 1 or LQTS 2, the flow of potassium ions through the ion channels in your heart cells isn't normal. This may cause problems when you exercise or when you have strong emotions.
You may develop a rapid, uncontrollable heart rhythm that prevents your heart from pumping blood. This type of heart rhythm can be fatal if it's not quickly brought under control.
If you have LQTS 3, the flow of sodium ions through ion channels in your heart cells isn't normal. This can trigger a rapid, uncontrollable heart rhythm that can be fatal. In LQTS 3, problems usually occur when your heart beats slower than normal, such as during sleep.
Acquired Long QT Syndrome
Some medicines and conditions can cause acquired LQTS.
Medication-Induced Long QT Syndrome
More than 50 medicines have been found to cause LQTS. Some common medicines that may cause the disorder include:
Some people who have medication-induced LQTS also may have an inherited form of the disorder. They may not have symptoms unless they take medicines that lengthen the QT interval or lower potassium levels in the blood. When LQTS doesn't cause symptoms, it's called silent LQTS.
Who Is at Risk for Long QT Syndrome?
Long QT syndrome (LQTS) is a rare disorder. Experts think that about 1 in 7,000 people has LQTS. But no one knows for sure, because LQTS often goes undiagnosed.
LQTS causes about 3,000 to 4,000 sudden deaths in children and young adults each year in the United States. Unexplained sudden deaths in children are rare. When they do occur, LQTS often is the cause.
Inherited LQTS usually is first detected during childhood or young adulthood. Half of all people who have LQTS have their first abnormal heart rhythm by the time they're 12 years old, and 90 percent by the time they're 40 years old. The condition rarely is diagnosed after age 40.
In boys who have LQTS, the QT interval (which can be seen on an EKG test) often returns toward normal after puberty. If this happens, the risk of LQTS symptoms and complications goes down.
LQTS is more common in women than men. Women who have LQTS are more likely to faint or die suddenly from the disorder during menstruation and shortly after giving birth.
Children who are born deaf also are at increased risk for LQTS. This is because the same genetic problem that affects hearing also affects the function of ion channels in the heart.
Major Risk Factors
You're at risk of having LQTS if anyone in your family has ever had it. Unexplained fainting or seizures, drowning or near drowning, and unexplained sudden death are all possible signs of LQTS.
You're also at risk for LQTS if you take medicines that make the QT interval longer. Your doctor can tell you whether your prescription or over-the-counter medicines might do this.
You also may develop LQTS if you have excessive vomiting or diarrhea or other conditions that cause low blood levels of potassium or sodium. These conditions include the eating disorders anorexia nervosa and bulimia, as well as some thyroid disorders.
What Are the Signs and Symptoms of Long QT Syndrome?
Major Signs and Symptoms
If you have long QT syndrome (LQTS), you can have sudden and dangerous arrhythmias (abnormal heart rhythms). Signs and symptoms of LQTS-related arrhythmias often first occur during childhood and include:
Unexplained
fainting. This happens because the
heart isn't pumping enough
blood to the
brain. Fainting may occur during physical or emotional
stress. Fluttering feelings in the
chest may occur before fainting.
Unexplained drowning or near drowning. This may be due to
fainting while swimming.
Unexplained
sudden cardiac arrest (SCA) or death. SCA is a condition in which the
heart suddenly stops beating for no obvious reason. People who have SCA die within minutes unless they receive
treatment. In about 1 out of 10 people who have LQTS, SCA or sudden death is the first sign of the disorder.
Other Signs and Symptoms
Often, people who have LQTS 3 develop an abnormal heart rhythm during sleep. This may cause noisy gasping while sleeping.
Silent Long QT Syndrome
Sometimes long QT syndrome doesn't cause any signs or symptoms. This is called silent LQTS. For this reason, doctors often advise family members of people who have LQTS to be tested for the disorder, even if they have no symptoms.
Medical and genetic tests may reveal whether these family members have LQTS and what type of the condition they have.
How Is Long QT Syndrome Diagnosed?
Cardiologists diagnose and treat long QT syndrome (LQTS). Cardiologists are doctors who specialize in diagnosing and treating heart diseases and conditions. To diagnose LQTS, your cardiologist will consider your:
EKG (Electrocardiogram)
An EKG is a simple test that detects and records the heart's electrical activity. This test may show a long QT interval and other signs that suggest LQTS. Often, doctors first discover a long QT interval when an EKG is done for another suspected heart problem.
Not all people who have LQTS will always have a long QT interval on an EKG. The QT interval may change from time to time; it may be long sometimes and normal at other times. Thus, your doctor may want you to have several EKG tests over a period of days or weeks. Or, your doctor may have you wear a device called a Holter monitor.
A Holter monitor records the heart's electrical activity for a full 24- or 48-hour period. It can detect heart problems that occur for only a few minutes out of the day.
You wear small patches called electrodes on your chest. Wires connect the patches to a small, portable recorder. You can clip the recorder to a belt, keep it in a pocket, or hang it around your neck.
While you wear the monitor, you do your usual daily activities. You also keep a notebook, noting any symptoms you have and the time they occur. You then return both the recorder and the notebook to your doctor to read the results. Your doctor can see how your heart was beating at the time you had symptoms.
Some people have a long QT interval only while they exercise. For this reason, your doctor may recommend that you have a stress test.
During a stress test, you exercise to make your heart work hard and beat fast. An EKG is done while you exercise. If you can't exercise, you may be given medicine to increase your heart rate.
Medical History and Physical Exam
Your doctor will ask whether you've had any symptoms of an abnormal heart rhythm. Symptoms may include:
A fluttering feeling in your
chest, which is the result of your
heart beating too fast
Loud gasping during
sleep
Your doctor may ask what over-the-counter, prescription, or other drugs you take. He or she also may want to know whether anyone in your family has been diagnosed with or has had signs of LQTS. Signs of LQTS include unexplained fainting, drowning, sudden cardiac arrest, or sudden death.
Your doctor will check you for signs of conditions that may lower blood levels of potassium or sodium. These conditions include the eating disorders anorexia nervosa and bulimia, excessive vomiting or diarrhea, and certain thyroid disorders.
Genetic Tests
Genetic blood tests can detect some forms of inherited LQTS. If your doctor thinks that you have LQTS, he or she may suggest genetic testing. Genetic blood tests usually are suggested for family members of people who have LQTS as well.
However, genetic tests don't always detect LQTS. So, even if you have the disorder, the tests may not show it.
Also, some people who test positive for LQTS don't have any signs or symptoms of the disorder. These people may have silent LQTS. Less than 10 percent of these people will faint or suddenly die from an abnormal heart rhythm.
Even if you have silent LQTS, you may be at increased risk of having an abnormal heart rhythm while taking medicines that affect potassium ion channels or blood levels of potassium.
Types of Inherited Long QT Syndrome
If you have inherited LQTS, it may be helpful to know which type you have. This will help you and your doctor plan your treatment and decide which lifestyle changes you should make.
To find out what type of LQTS you have, your doctor will consider:
How Is Long QT Syndrome Treated?
The goal of treating long QT syndrome (LQTS) is to prevent life-threatening, abnormal heart rhythms and fainting spells.
Treatment isn't a cure for the disorder and may not restore a normal QT interval on an EKG (electrocardiogram). However, treatment greatly improves the chances of survival.
Specific Types of Treatment
Your doctor will recommend the best treatment for you based on:
People who have LQTS without symptoms may be advised to:
Make lifestyle changes that reduce the risk of
fainting or SCA. Lifestyle changes may include avoiding certain sports and strenuous
exercise, such as swimming, which can cause abnormal
heart rhythms.
Take medicines such as beta-
blockers, which reduce the risk of symptoms by slowing the
heart rate.
The type of medicine you take will depend on the type of LQTS you have. For example, doctors usually will prescribe sodium channel blocker medicines only for people who have LQTS 3.
If your doctor thinks you're at increased risk for LQTS complications, he or she may suggest more aggressive treatments (in addition to medicines and lifestyle changes). These treatments may include:
People at increased risk are those who have fainted or who have had dangerous heart rhythms from their LQTS.
Lifestyle Changes
If possible, try to avoid things that can trigger abnormal heart rhythms. For example, people who have LQTS should avoid medicines that lengthen the QT interval or lower potassium blood levels. (For more information, go to ""What Causes Long QT Syndrome?"")
Many people who have LQTS also benefit from adding more potassium to their diets. Check with your doctor about eating more potassium-rich foods (such as bananas) or taking potassium supplements daily.
Medicines
Beta blockers are medicines that prevent the heart from beating faster in response to physical or emotional stress. Most people who have LQTS are treated with beta blockers.
Doctors may suggest that people who have LQTS 3 take sodium channel blockers, such as mexiletine. These medicines make sodium ion channels less active.
Medical Devices
Pacemakers and ICDs are small devices that help control abnormal heart rhythms. Both devices use electrical currents to prompt the heart to beat normally. Surgeons implant pacemakers and ICDs in the chest or belly with a minor procedure.
The use of these devices is similar in children and adults. However, because children are still growing, other issues may arise. For example, as children grow, they may need to have their devices replaced.
Surgery
People who are at high risk of death from LQTS sometimes are treated with surgery. During surgery, the nerves that prompt the heart to beat faster in response to physical or emotional stress are cut.
This type of surgery keeps the heart beating at a steady pace and lowers the risk of dangerous heart rhythms in response to stress or exercise.
Living With Long QT Syndrome
Long QT syndrome (LQTS) usually is a lifelong condition. The risk of having an abnormal heart rhythm that leads to fainting or sudden cardiac arrest may lessen as you age. However, the risk never completely goes away.
You'll need to take certain steps for the rest of your life to prevent abnormal heart rhythms. You can:
Avoid things that trigger abnormal
heart rhythms
Let others know you might
faint or your
heart might stop beating, and tell them what steps they can take
Have a plan in place for how to handle abnormal
heart rhythms
If an abnormal heart rhythm does occur, you'll need to seek treatment right away.
Avoid Triggers
If exercise triggers an abnormal heart rhythm, your doctor may tell you to avoid any strenuous exercise, especially swimming. Ask your doctor what types and amounts of exercise are safe for you.
If you have a pacemaker or implantable cardioverter defibrillator, avoid contact sports that may dislodge these devices. You may want to exercise in public or with a friend who can help you if you faint.
Avoid medicines that can trigger an abnormal heart rhythm. This includes some medicines used to treat allergies, infections, high blood pressure, high blood cholesterol, depression, and arrhythmias. Talk with your doctor before taking any prescription, over-the-counter, or other medicines or drugs.
Seek medical care right away for conditions that lower the sodium or potassium level in your blood. These conditions include the eating disorders anorexia nervosa and bulimia, excessive vomiting or diarrhea, and certain thyroid disorders.
If you have LQTS 2, try to avoid unexpected noises, such as loud or jarring alarm clock buzzers and telephone ringers.
Inform Others
You may want to wear a medical ID necklace or bracelet that states that you have LQTS. This will help alert medical personnel and others about your condition if you have an emergency.
Let your roommates, coworkers, or other people with whom you have regular contact know that you have a condition that might cause you to faint or go into cardiac arrest. Tell them to call 9–1–1 right away if you faint.
Consider asking a family member and/or coworker to learn cardiopulmonary resuscitation (CPR) in case your heart stops beating.
You also may want to keep an automated external defibrillator (AED) with you at home or at work. This device uses electric shocks to restore a normal heart rhythm.
Someone at your home and/or workplace should be trained on how to use the AED, just in case your heart stops beating. If a trained person isn't available, an untrained person also can use the AED to help save your life.
If you have LQTS 3 and you sleep alone, you may want to have an intercom in your bedroom that's connected to someone else's bedroom. This will let others detect the noisy gasping that often occurs if you have an abnormal heart rhythm while lying down.
Ongoing Health Care Needs
You should see your cardiologist (heart specialist) regularly. He or she will adjust your treatment as needed. For example, if you still faint often while using less aggressive treatments, your doctor may suggest other treatment options.
Emotional Issues and Support
Living with LQTS may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with LQTS. You can see how other people have coped with the condition. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
Some people learn they have LQTS because they're tested after a family member dies suddenly from LQTS. Grief counseling may help you cope if this has happened to you. Talk with your doctor about finding a grief counselor.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to long QT syndrome, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
What Is Lymphocytopenia?
Lymphocytopenia (LIM-fo-si-to-PE-ne-ah) is a disorder in which your blood doesn’t have enough white blood cells called lymphocytes (LIM-fo-sites).
These cells are made in the bone marrow along with other kinds of blood cells. Lymphocytes help protect your body from infection. Low numbers of lymphocytes can raise your risk of infection.
Lymphocytopenia also is called lymphopenia.
Overview
About 20 to 40 percent of all white blood cells are lymphocytes. A normal lymphocyte count for adults usually is between 1,000 and 4,800 lymphocytes per microliter of blood. For children, a normal lymphocyte count usually is between 3,000 and 9,500 lymphocytes per microliter of blood.
The term ""lymphocytopenia"" refers to a count of less than 1,000 lymphocytes per microliter of blood in adults, or less than 3,000 lymphocytes per microliter of blood in children.
The three types of lymphocytes are B lymphocytes, T lymphocytes, and natural killer cells. All of these cells help protect the body from infection. Most people who have lymphocytopenia have low numbers of T lymphocytes. Sometimes they also have low numbers of the other types of lymphocytes.
Certain factors can cause a low lymphocyte count, such as:
Many diseases, conditions, and factors can cause the above problems that lead to lymphocytopenia. These causes can be acquired or inherited.
""Acquired"" means you aren't born with the condition, but you develop it. One of the most common acquired causes of lymphocytopenia is AIDS.
""Inherited"" means your parents passed the gene for the condition on to you. Inherited causes include DiGeorge anomaly, Wiskott-Aldrich syndrome, severe combined immunodeficiency syndrome, and ataxia-telangiectasia. These inherited conditions are rare.
Outlook
Lymphocytopenia can range from mild to severe. The condition alone may not cause any signs, symptoms, or serious problems.
How long lymphocytopenia lasts depends on its cause. The treatment for this condition depends on its cause and severity. Mild lymphocytopenia may not require treatment. If an underlying condition is successfully treated, lymphocytopenia will likely improve.
If lymphocytopenia causes serious infections, you may need medicines or other treatments.
What Causes Lymphocytopenia?
In general, lymphocytopenia (a low lymphocyte count) occurs because:
The body makes enough
lymphocytes, but they’re destroyed.
A combination of these factors also may cause a low lymphocyte count.
Many diseases, conditions, and factors can lead to a low lymphocyte count. These conditions can be acquired or inherited. ""Acquired"" means you aren't born with the condition, but you develop it. ""Inherited"" means your parents passed the gene for the condition on to you.
Exactly how each disease, condition, or factor affects your lymphocyte count isn't known. Some people have low lymphocyte counts with no underlying cause.
Acquired Causes
Many acquired diseases, conditions, and factors can cause lymphocytopenia. Examples include:
Inherited Causes
Certain inherited diseases and conditions can lead to lymphocytopenia. Examples include DiGeorge anomaly, Wiskott-Aldrich syndrome, severe combined immunodeficiency syndrome, and ataxia-telangiectasia. These inherited conditions are rare.
What Are the Signs and Symptoms of Lymphocytopenia?
A low lymphocyte count alone may not cause any signs or symptoms. The condition usually is found when a person is tested for other diseases or conditions, such as AIDS.
If you have unusual infections, repeat infections, and/or infections that won't go away, your doctor may suspect that you have lymphocytopenia. Fever is the most common symptom of infection.
How Is Lymphocytopenia Diagnosed?
Your doctor will diagnose lymphocytopenia based on your medical history, a physical exam, and test results.
A low lymphocyte count alone may not cause any signs or symptoms. Thus, the condition often is diagnosed during testing for other diseases or conditions.
Specialists Involved
Your primary care doctor may notice that you have unusual infections, repeat infections, and/or infections that won't go away. These infections may be signs of lymphocytopenia. Your primary care doctor may refer you to an infectious disease specialist to find out what's causing the infections.
You also may see a hematologist (blood disease specialist) or an immunologist (immune disorders specialist). Blood diseases and immune disorders can cause lymphocytopenia.
Medical History
To assess your risk for a low lymphocyte count, your doctor may ask:
About your risk for AIDS, including questions about
blood transfusions, sexual partners,
intravenous (IV) drug use, and exposure to infectious
blood or bodily fluids at work
Whether you've ever been diagnosed with a
blood disease or immune disorder, or whether you have a
family history of such illnesses
Physical Exam
Your doctor will do a physical exam to look for signs of infection, such as fever. He or she may check your abdomen for signs of an enlarged spleen and your neck for signs of enlarged lymph nodes.
Your doctor also will look for signs and symptoms of diseases and conditions that can affect your lymphocyte count, such as AIDS and blood cancers.
Diagnostic Tests
Your doctor may recommend one or more of the following tests to help diagnose a low lymphocyte count.
Tests for Underlying Conditions
Many diseases and conditions can cause lymphocytopenia. Your doctor will want to find the cause of the disorder. You may be tested for HIV/AIDS, tuberculosis, blood diseases, and immune disorders.
Tests for these underlying conditions might include blood tests, bone marrow tests, and lymph node tests.
Lymph nodes are part of the immune system. They're found in many places in your body. During a physical exam, your doctor may find that certain lymph nodes are swollen. In lymphocytopenia, the lymph nodes may hold on to too many lymphocytes instead of releasing them into the bloodstream.
To test a lymph node, you may need to have it removed. Removing a lymph node involves minor surgery.
How Is Lymphocytopenia Treated?
If you have mild lymphocytopenia with no underlying cause, you may not need treatment. The disorder may improve on its own.
If you have unusual infections, repeat infections, and/or infections that won't go away due to lymphocytopenia, you'll need treatment for the infections.
If you have a disease or condition that's causing lymphocytopenia, your doctor will prescribe treatment for that illness. Treating the underlying problem will help treat the lymphocytopenia.
Treatment for Underlying Diseases or Conditions
Many diseases and conditions can cause lymphocytopenia. Examples include infectious diseases, such as AIDS; blood diseases, such as aplastic anemia; and inherited diseases, such as Wiskott-Aldrich syndrome.
Your treatment will depend on your underlying disease or condition.
Emerging Treatments
Researchers are looking at ways to increase lymphocyte production in people who have lymphocytopenia with serious underlying conditions.
For example, some studies are looking into blood and marrow stem cell transplants. This procedure may help treat or cure some of the conditions that can cause a low lymphocyte count.
Other studies are looking at medicines and other substances that can help the body make more lymphocytes.
How Can Lymphocytopenia Be Prevented?
You can't prevent lymphocytopenia that's caused by an inherited condition. However, you can take steps to control lymphocytopenia. Follow your treatment plan and take all medicines as your doctor advises.
Early diagnosis also can help control lymphocytopenia. In the United States, newborns are routinely screened for an immune condition that can lead to lymphocytopenia. This allows doctors to diagnose the disorder before serious problems develop.
You may be able to lower your risk for acquired conditions that cause lymphocytopenia. For more information about acquired causes of the disorder, go to ""What Causes Lymphocytopenia?""
Living With Lymphocytopenia
If you have mild lymphocytopenia with no underlying cause, you may not need treatment. The disorder may improve on its own.
If an underlying condition is causing your lymphocytopenia, you'll need treatment for that condition. You'll also need treatment for infections if your body is unable to fight them because of lymphocytopenia.
Treating and Preventing Infections
The main risk of lymphocytopenia is getting unusual infections, repeat infections, and/or infections that won't go away. If you have the disorder, you may get treatments to prevent infections or to treat infections you already have.
You also can take other steps to prevent infections. For example:
Know the signs of an infection, such as a fever. Call your doctor right away if you think you have an infection.
Physical Activity
Talk with your doctor about what types and amounts of physical activity are safe for you. You may want to avoid activities that could result in injuries or increase your risk of infections.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they’re widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don’t directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You’ll learn about treatments and tests you may receive, and the benefits and risks they may pose. You’ll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you’ll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to lymphocytopenia, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI’s Children and Clinical Studies Web page.
What Is Marfan Syndrome?
Marfan syndrome is a condition in which your body's connective tissue is abnormal. Connective tissue helps support all parts of your body. It also helps control how your body grows and develops.
Marfan syndrome most often affects the connective tissue of the heart and blood vessels, eyes, bones, lungs, and covering of the spinal cord. Because the condition affects many parts of the body, it can cause many complications. Sometimes the complications are life threatening.
Overview
Marfan syndrome is a genetic disorder. A mutation, or change, in the gene that controls how the body makes fibrillin causes Marfan syndrome. Fibrillin is a protein that plays a major role in your body's connective tissue.
Most people who have Marfan syndrome inherit it from their parents. If you have Marfan syndrome, you have a 50 percent chance of passing the altered gene to each of your children.
In about 1 in 4 cases, the mutation that causes Marfan syndrome is not inherited. Thus, the affected person is the first in his or her family to have the condition.
Marfan syndrome often affects the long bones of the body. This can lead to signs, or traits, such as:
A tall, thin build.
A
spine that curves to one side. This condition is called
scoliosis (sko-le-O-sis).
A
chest that sinks in or sticks out. These conditions are called pectus excavatum (eks-ka-VA-tum) and pectus carinatum (ka-ri-NA-tum), respectively.
Teeth that are too crowded.
Flat feet.
Marfan syndrome traits vary from person to person, even in the same family. Some people who have the condition have many traits, while others have few.
The most serious complications of Marfan syndrome involve the heart and blood vessels. Marfan syndrome can affect the aorta, the main blood vessel that supplies oxygen-rich blood to the body. In Marfan syndrome, the aorta can stretch and grow weak. This condition is called aortic dilation (di-LA-shun) or aortic aneurysm (AN-u-rism).
If the aorta stretches and grows weak, it may tear or burst and leak blood. This condition is called aortic dissection. It's very serious and can lead to severe heart problems or even death.
Marfan syndrome has no cure, but treatments can help delay or prevent complications. Treatments include medicines, surgery, and other therapies. Limiting certain activities, or changing how you do them, may help reduce the risks to the aorta, eyes, and joints.
The type of treatment you receive depends on how the condition is affecting your body.
Outlook
About 1 out of every 5,000 people in the United States has Marfan syndrome. Men, women, children, and people of all races can have the condition.
Advances have been made in the early diagnosis and treatment of Marfan syndrome. It's now possible for people who have the condition to live longer and enjoy a good quality of life. Many people who have Marfan syndrome and are properly diagnosed and treated may live an average lifespan.
Researchers continue to study the condition and look for better treatments.
What Causes Marfan Syndrome?
Marfan syndrome is a genetic disorder. A mutation, or change, in the gene that controls how the body makes fibrillin causes Marfan syndrome. Fibrillin is a protein that plays a major role in your body's connective tissue.
Most people who have Marfan syndrome inherit it from their parents. If you have the condition, you have a 50 percent chance of passing the altered gene to each of your children.
Sometimes Marfan syndrome isn't inherited. The mutation in the fibrillin gene occurs in the egg or sperm cells. If a child is conceived, the altered gene may be passed on to the child. The risk of that child's brothers or sisters having Marfan syndrome is low.
Who Is at Risk for Marfan Syndrome?
People at highest risk for Marfan syndrome are those who have a family history of the condition. If you have Marfan syndrome, you have a 50 percent chance of passing the altered gene to each of your children.
Marfan syndrome affects about 1 out of every 5,000 people in the United States. Men, women, and children, and people of all races, can have the condition.
What Are the Signs and Symptoms of Marfan Syndrome?
Marfan syndrome can affect many parts of the body. As a result, the signs and symptoms of the disorder vary from person to person, even in the same family.
Marfan complications also vary, depending on how the condition affects your body. Marfan syndrome most often affects the connective tissue of the heart, eyes, bones, lungs, and covering of the spinal cord. This can cause many complications, some of which are life threatening.
Marfan Traits
Marfan syndrome often affects the long bones of the body. This can lead to signs, or traits, such as:
A tall, thin build.
A
chest that sinks in or sticks out. These conditions are called pectus excavatum and pectus carinatum, respectively.
Teeth that are too crowded.
Flat feet.
Stretch marks on the skin also are a common trait in people who have Marfan syndrome. Stretch marks usually appear on the lower back, buttocks, shoulders, breasts, thighs, and abdomen.
Not everyone who has these traits has Marfan syndrome. Some of these traits also are signs of other connective tissue disorders.
Complications of Marfan Syndrome
Heart and Blood Vessel Complications
The most serious complications of Marfan syndrome involve the heart and blood vessels.
Marfan syndrome can affect the aorta, the main blood vessel that supplies oxygen-rich blood to the body. In Marfan syndrome, the aorta can stretch and grow weak. This condition is called aortic dilation or aortic aneurysm.
If the aorta stretches and grows weak, it may tear and leak blood. This condition, called aortic dissection, can lead to severe heart problems or even death.
Aortic dissection can cause severe pain in either the front or back of the chest or abdomen. The pain can travel upward or downward. If you have symptoms of aortic dissection, call 9–1–1.
Marfan syndrome also can cause problems with the heart's mitral (MI-trul) valve. This valve controls blood flow between the upper and lower chambers on the left side of the heart. Marfan syndrome can lead to mitral valve prolapse (MVP).
MVP is a condition in which the flaps of the mitral valve are floppy and don't close tightly. MVP can cause shortness of breath, palpitations (pal-pi-TA-shuns), chest pain, and other symptoms.
If you have MVP, your doctor may hear a heart murmur if he or she listens to your heart with a stethoscope. A heart murmur is an extra or unusual sound heard during the heartbeat.
Eye Complications
Marfan syndrome can cause many eye problems. A common problem in Marfan syndrome is a dislocated lens in one or both of the eyes. In this condition, the lens (the part of the eye that helps focus light) shifts up, down, or to the side. This can affect your eyesight. A dislocated lens often is the first sign that someone has Marfan syndrome.
Other eye complications of Marfan syndrome include nearsightedness, early glaucoma (high pressure in the fluid in the eyes), and early cataracts (clouding of an eye's lens). A detached retina also can occur.
Lung Complications
Marfan syndrome can cause sudden pneumothorax (noo-mo-THOR-aks), or collapsed lung. In this condition, air or gas builds up in the space between the lungs and chest wall. If enough air or gas builds up, a lung can collapse.
The most common symptoms of a collapsed lung are sudden pain in one side of the lung and shortness of breath.
Conditions such as scoliosis (a curved spine) and pectus excavatum (a chest that sinks in) can prevent the lungs from expanding fully. This can cause breathing problems. Marfan syndrome also can cause changes in the lung tissue, and it can lead to early emphysema (em-fi-SE-ma).
Marfan syndrome also has been linked to sleep apnea. In people who have Marfan syndrome, the shape of the face, oral cavity, or teeth may increase the risk of sleep apnea. Sleep apnea causes one or more pauses in breathing or shallow breaths while you sleep.
Breathing pauses can last from a few seconds to minutes. They often occur 5 to 30 times or more an hour. Typically, normal breathing then starts again, sometimes with a loud snort or choking sound.
How Is Marfan Syndrome Diagnosed?
Your doctor will diagnose Marfan syndrome based on your medical and family histories, a physical exam, and test results. He or she also will consult a set of guidelines called Ghent criteria, which are used to diagnose Marfan syndrome.
Marfan syndrome can be hard to diagnose. This is because its signs, or traits, are the same as or similar to the signs of other connective tissue disorders.
If you're diagnosed with Marfan syndrome, all of your first-degree relatives (for example, parents, siblings, and children) also should be checked for the disorder. This is because, even in families, the outward traits of Marfan syndrome may vary quite a bit.
Specialists Involved
Your family doctor or another type of doctor, such as an orthopedist (bone specialist), may notice certain traits that suggest Marfan syndrome.
If so, your doctor will likely refer you to a geneticist or cardiologist. A geneticist is hereditary disease expert. A cardiologist is a heart specialist. These two types of specialists often have the most experience working with people who have Marfan syndrome.
A geneticist will ask for medical information about you and your family. He or she will examine you and perhaps other members of your family. The geneticist also will coordinate your visits with other doctors, including a cardiologist, an ophthalmologist (eye specialist), and an orthopedist.
After reviewing the medical findings, the geneticist will determine whether you have Marfan syndrome.
Medical and Family Histories
Your doctor will ask about your medical history and your family's medical history. For example, your doctor may ask whether:
Any of your family members have
Marfan syndrome, have died from
heart problems, or have died suddenly.
Physical Exam
During the physical exam, your doctor will look for Marfan syndrome traits. For example, he or she may check the curve of your spine and the shape of your feet. Your doctor also will listen to your heart and lungs with a stethoscope.
Diagnostic Tests
Your doctor may recommend one or more of the following tests to help diagnose Marfan syndrome.
Ghent Criteria
Because no single test can diagnose Marfan syndrome, doctors use a set of guidelines called Ghent criteria to help diagnose the condition. The Ghent criteria are divided into major criteria and minor criteria. Sometimes genetic testing is part of this evaluation.
Major criteria include traits that are common in people who have Marfan syndrome. Minor criteria include traits that are common in many people. Doctors use a scoring system based on the number and type of Ghent criteria present to diagnose Marfan syndrome.
Talk with your doctor about which traits you have and your likelihood of having Marfan syndrome.
How Is Marfan Syndrome Treated?
Marfan syndrome has no cure. However, treatments can help delay or prevent complications, especially when started early.
Marfan syndrome can affect many parts of your body, including your heart, bones and joints, eyes, nervous system, and lungs. The type of treatment you receive will depend on your signs and symptoms.
Heart Treatments
Aortic dilation, or aortic aneurysm, is the most common and serious heart problem linked to Marfan syndrome. In this condition, the aorta—the main artery that carries oxygen-rich blood to your body—stretches and grows weak.
Medicines are used to try to slow the rate of aortic dilation. Surgery is used to replace the dilated segment of aorta before it tears.
If you have Marfan syndrome, you'll need routine care and tests to check your heart valves and aorta.
Surgery
If your aorta stretches, it's more likely to tear (a condition called aortic dissection). To prevent this, your doctor may recommend surgery to repair or replace part of your aorta.
Surgery may involve:
A composite valve graft. For this
surgery, part of the
aorta and the
aortic valve are removed. The aorta is replaced with a man-made tube called a graft. A man-made valve replaces the original valve.
Aortic valve-sparing
surgery. If your aortic valve is working well, your doctor may recommend valve-sparing surgery. For this surgery, your doctor replaces the enlarged part of your
aorta with a graft. Your aortic valve is left in place.
After aortic surgery, you may need medicines or followup tests. For example, after a composite valve graft, your doctor will prescribe medicines called anticoagulants, or ""blood thinners.""
Blood thinners help prevent blood clots from forming on your man-made aortic valve. You'll need to take these medicines for the rest of your life. If you've had valve-sparing surgery, you'll only need to take blood thinners for a short time, as your doctor prescribes.
If you've had a composite valve graft, you're at increased risk for endocarditis (EN-do-kar-DI-tis). This is an infection of the inner lining of your heart chambers and valves. Your doctor may recommend that you take antibiotics before certain medical or dental procedures that increase your risk of endocarditis.
Your doctor also may advise you to continue taking beta blockers or other medicines after either type of aortic surgery.
After surgery, you may have routine cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT) scans to check your aorta.
Cardiac MRI is a painless test that uses radio waves and magnets to created detailed pictures of your organs and tissues. Cardiac CT is a painless test that uses an x-ray machine to take clear, detailed pictures of your heart.
Bone and Joint Treatments
If you have scoliosis (a curved spine), your doctor may suggest a brace or other device to prevent the condition from getting worse. Severe cases of scoliosis may require surgery.
Some people who have Marfan syndrome need surgery to repair a chest that sinks in or sticks out. This surgery is done to prevent the chest from pressing on the lungs and heart.
Nervous System Treatments
Marfan syndrome can lead to dural ectasia. In this condition, a substance called the dura (which covers the fluid around your brain and spinal cord) stretches and grows weak. This can cause the bones of the spine to wear away. Dural ectasia usually is treated with pain medicines.
Living With Marfan Syndrome
Advances have been made in the early diagnosis and treatment of Marfan syndrome. It's now possible for people who have Marfan syndrome to live longer and enjoy a good quality of life. Many people who have Marfan syndrome and are properly diagnosed and treated may live an average lifespan.
If you have Marfan syndrome, talk with your doctor about ongoing care and what types of physical activity are safe for you. If you're thinking about becoming pregnant, discuss the possible risks with your doctor.
Ongoing Care
If you have Marfan syndrome, you'll need regular checkups with the doctor who is coordinating your care and other medical experts, including:
Annual checkups with an orthopedist (
bone specialist) to look for changes in your
spine and
breastbone.
Regular
eye exams with an
ophthalmologist (eye specialist) to find and treat eye problems early.
Let your doctors and dentists know if you've had a composite valve graft. If you've had this type of surgery, you're at increased risk for endocarditis. This is an infection of the inner lining of your heart chambers and valves.
Your health care providers, including dentists, may give you antibiotics before certain medical or dental procedures that could raise your risk of endocarditis.
Take all of your medicines as your doctor prescribes.
Emergencies
If you have Marfan syndrome, you're at risk for aortic dissection. This is a condition in which the aorta tears and leaks blood. Aortic dissection is a life-threatening condition.
The main symptom of aortic dissection is severe pain in either the front or back of your Go to:
What Is Metabolic Syndrome?
Metabolic (met-ah-BOL-ik) syndrome is the name for a group of risk factors that raises your risk for heart disease and other health problems, such as diabetes and stroke.
The term ""metabolic"" refers to the biochemical processes involved in the body's normal functioning. Risk factors are traits, conditions, or habits that increase your chance of developing a disease.
In this article, ""heart disease"" refers to coronary heart disease (CHD). CHD is a condition in which a waxy substance called plaque (plak) builds up inside the coronary (heart) arteries.
Plaque hardens and narrows the arteries, reducing blood flow to your heart muscle. This can lead to chest pain, a heart attack, heart damage, or even death.
Metabolic Risk Factors
The five conditions described below are metabolic risk factors. You can have any one of these risk factors by itself, but they tend to occur together. You must have at least three metabolic risk factors to be diagnosed with metabolic syndrome.
A large waistline. This also is called abdominal
obesity or ""having an apple shape."" Excess
fat in the
stomach area is a greater risk factor for
heart disease than excess fat in other parts of the body, such as on the hips.
A low
HDL cholesterol level (or you're on medicine to treat low HDL cholesterol). HDL sometimes is called ""good"" cholesterol. This is because it helps remove cholesterol from your
arteries. A low HDL cholesterol level raises your risk for
heart disease.
Outlook
Metabolic syndrome is becoming more common due to a rise in obesity rates among adults. In the future, metabolic syndrome may overtake smoking as the leading risk factor for heart disease.
It is possible to prevent or delay metabolic syndrome, mainly with lifestyle changes. A healthy lifestyle is a lifelong commitment. Successfully controlling metabolic syndrome requires long-term effort and teamwork with your health care providers.
Other Names for Metabolic Syndrome
Who Is at Risk for Metabolic Syndrome?
People at greatest risk for metabolic syndrome have these underlying causes:
Abdominal
obesity (a large waistline)
An inactive lifestyle
Some people are at risk for metabolic syndrome because they take medicines that cause weight gain or changes in blood pressure, blood cholesterol, and blood sugar levels.
These medicines most often are used to treat inflammation, allergies, HIV, and depression and other types of mental illness.
Populations Affected
Metabolic syndrome is more common in African American women and Mexican American women than in men of the same racial groups. The condition affects White women and men about equally.
Some racial and ethnic groups in the United States are at higher risk for metabolic syndrome than others. Mexican Americans have the highest rate of metabolic syndrome, followed by Whites and African Americans.
Worldwide, certain ethnic groups, such as South Asians, are at increased risk for metabolic syndrome.
Other groups at increased risk for metabolic syndrome include:
People who have a sibling or parent who has
diabetesPeople who have a personal history of
diabetes
Heart Disease Risk
Metabolic syndrome increases your risk for heart disease. Heart disease risk can be divided into short-term risk and long-term risk.
""Short-term risk"" refers to the risk of having a heart attack or dying from heart disease in the next 10 years. ""Long-term risk"" refers to the risk of developing heart disease over your lifetime.
Other risk factors, besides metabolic syndrome, also increase your risk for heart disease. For example, a high LDL cholesterol level and smoking are major risk factors for heart disease. For details about all of the risk factors for heart disease, go to the Health Topics Coronary Heart Disease Risk Factors article.
Even if you don't have metabolic syndrome, you should find out your short-term risk for heart disease. The National Cholesterol Education Program (NCEP) divides short-term heart disease risk into four categories, as shown below. Your risk category depends on which risk factors you have and how many you have.
Your risk factors are used to calculate your 10-year risk of developing heart disease. The NCEP has an online calculator that you can use to determine your 10-year heart disease risk score.
High risk: You're in this category if you already have
heart disease or
diabetes, or if your 10-year risk score is more than 20 percent.
Moderately high risk: You're in this category if you have two or more risk factors and your 10-year risk score is 10–20 percent.
Moderate risk: You're in this category if you have two or more risk factors and your 10-year risk score is less than 10 percent.
Lower risk: You're in this category if you have zero or one risk factor.
Even if your 10-year risk score isn't high, metabolic syndrome will increase your risk for heart disease over time. Thus, metabolic syndrome should be treated (mainly with lifestyle changes).
What Are the Signs and Symptoms of Metabolic Syndrome?
Metabolic syndrome is a group of risk factors that raises your risk for heart disease and other health problems, such as diabetes and stroke. These risk factors can increase your risk for health problems even if they're only moderately raised (borderline-high risk factors).
Most of the metabolic risk factors have no signs or symptoms, although a large waistline is a visible sign.
Some people may have symptoms of high blood sugar if diabetes—especially type 2 diabetes—is present. Symptoms of high blood sugar often include increased thirst; increased urination, especially at night; fatigue (tiredness); and blurred vision.
High blood pressure usually has no signs or symptoms. However, some people in the early stages of high blood pressure may have dull headaches, dizzy spells, or more nosebleeds than usual.
How Is Metabolic Syndrome Diagnosed?
Your doctor will diagnose metabolic syndrome based on the results of a physical exam and blood tests. You must have at least three of the five metabolic risk factors to be diagnosed with metabolic syndrome.
Metabolic Risk Factors
A Large Waistline
Having a large waistline means that you carry excess weight around your waist (abdominal obesity). This is also called having an ""apple-shaped"" figure. Your doctor will measure your waist to find out whether you have a large waistline.
A waist measurement of 35 inches or more for women or 40 inches or more for men is a metabolic risk factor. A large waistline means you're at increased risk for heart disease and other health problems.
A High Triglyceride Level
Triglycerides are a type of fat found in the blood. A triglyceride level of 150 mg/dL or higher (or being on medicine to treat high triglycerides) is a metabolic risk factor. (The mg/dL is milligrams per deciliter—the units used to measure triglycerides, cholesterol, and blood sugar.)
A Low HDL Cholesterol Level
HDL cholesterol sometimes is called ""good"" cholesterol. This is because it helps remove cholesterol from your arteries.
An HDL cholesterol level of less than 50 mg/dL for women and less than 40 mg/dL for men (or being on medicine to treat low HDL cholesterol) is a metabolic risk factor.
High Fasting Blood Sugar
A normal fasting blood sugar level is less than 100 mg/dL. A fasting blood sugar level between 100–125 mg/dL is considered prediabetes. A fasting blood sugar level of 126 mg/dL or higher is considered diabetes.
A fasting blood sugar level of 100 mg/dL or higher (or being on medicine to treat high blood sugar) is a metabolic risk factor.
About 85 percent of people who have type 2 diabetes—the most common type of diabetes—also have metabolic syndrome. These people have a much higher risk for heart disease than the 15 percent of people who have type 2 diabetes without metabolic syndrome.
How Is Metabolic Syndrome Treated?
Healthy lifestyle changes are the first line of treatment for metabolic syndrome. Lifestyle changes include losing weight, being physically active, following a heart healthy diet, and quitting smoking.
If lifestyle changes aren't enough, your doctor may prescribe medicines. Medicines are used to treat and control risk factors such as high blood pressure, high triglycerides, low HDL cholesterol, and high blood sugar.
Blood-thinning medicines, such as aspirin, also may be used to reduce the risk of blood clots. Excessive blood clotting is a condition that often occurs with metabolic syndrome.
Lifestyle Changes
Losing Weight
If you have metabolic syndrome and are overweight or obese, your doctor will likely recommend weight loss. He or she can help you create a weight-loss plan and goals.
The long-range target is to lower your body mass index (BMI) to less than 25. BMI measures your weight in relation to your height and gives an estimate of your total body fat.
A BMI between 25 and 29.9 is considered overweight. A BMI of 30 or more is considered obese. A BMI of less than 25 is the goal for prevention and treatment of metabolic syndrome.
You can calculate your BMI using the National Heart, Lung, and Blood Institute's (NHLBI's) online calculator, or your health care provider can calculate your BMI.
For more information about losing weight or maintaining your weight, go to the Health Topics Overweight and Obesity article.
Following a Heart Healthy Diet
A heart healthy diet is an important part of a healthy lifestyle. A healthy diet includes a variety of vegetables and fruits. These foods can be fresh, canned, frozen, or dried. A good rule is to try to fill half of your plate with vegetables and fruits.
A healthy diet also includes whole grains, fat-free or low-fat dairy products, and protein foods, such as lean meats, poultry without skin, seafood, processed soy products, nuts, seeds, beans, and peas.
Choose and prepare foods with little sodium (salt). Too much salt can raise your risk for high blood pressure. Studies show that following the Dietary Approaches to Stop Hypertension (DASH) eating plan can lower blood pressure.
Try to avoid foods and drinks that are high in added sugars. For example, drink water instead of sugary drinks, such as soda.
Also, try to limit the amount of solid fats and refined grains that you eat. Solid fats are saturated fat and trans fatty acids. Refined grains come from processing whole grains, which results in a loss of nutrients (such as dietary fiber).
If you drink alcohol, do so in moderation. Too much alcohol can raise your blood pressure and triglyceride level. Alcohol also adds extra calories, which can cause weight gain.
Aim for a healthy weight by staying within your daily calorie needs. Balance the calories you take in from food and drinks with the calories you use while doing physical activity.
For more information about following a healthy diet, go to the National Heart, Lung, and Blood Institute's ""Your Guide to Lowering Your Blood Pressure With DASH"" and the U.S. Department of Agriculture's ChooseMyPlate.gov Web site. Both resources provide general information about healthy eating.
Being Physically Active
Physical activity can help keep your heart and lungs healthy. Many Americans are not active enough. The good news is that even modest amounts of physical activity are good for your health. The more active you are, the more you'll benefit.
Before starting any kind of exercise program or new physical activity, talk with your doctor about the types and amounts of physical activity that are safe for you.
The four main types of physical activity are aerobic, muscle-strengthening, bone strengthening, and stretching.
You can do physical activity with light, moderate, or vigorous intensity. The level of intensity depends on how hard you have to work to do the activity. People who have metabolic syndrome usually are urged to keep up a moderate level of activity.
For more information about physical activity, go to the U.S. Department of Health and Human Services' ""2008 Physical Activity Guidelines for Americans,"" the Health Topics Physical Activity and Your Heart article, and the NHLBI's ""Your Guide to Physical Activity and Your Heart.""
Smoking
If you smoke, quit. Smoking can raise your risk for heart disease and heart attack and worsen other heart disease risk factors. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke.
If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.
For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article and the NHLBI's ""Your Guide to a Healthy Heart.""
How Can Metabolic Syndrome Be Prevented?
Making healthy lifestyle choices is the best way to prevent metabolic syndrome. One important lifestyle choice is to maintain a healthy weight. Other than weighing yourself on a scale, you can find out if you're at a healthy weight using your waist measurement and body mass index (BMI).
A waist measurement indicates your abdominal fat, which is linked to your risk for heart disease and other diseases. To measure your waist, stand and place a tape measure around your middle, just above your hipbones. Measure your waist just after you breathe out. Make sure the tape is snug but doesn't squeeze the flesh.
A waist measurement of less than 35 inches for women and less than 40 inches for men is the goal for preventing metabolic syndrome; it's also the goal when treating metabolic syndrome.
BMI measures your weight in relation to your height and gives an estimate of your total body fat. A BMI between 25 and 29.9 is considered overweight. A BMI of 30 or more is considered obese. A BMI of less than 25 is the goal for preventing metabolic syndrome; it's also the goal when treating metabolic syndrome.
You can figure out your BMI using the National Heart, Lung, and Blood Institute's (NHLBI's) online calculator, or your doctor can help you.
To maintain a healthy weight, follow a heart healthy diet and try not to overeat. A healthy diet includes a variety of vegetables and fruits. It also includes whole grains, fat-free or low-fat dairy products, and protein foods, such as lean meats, poultry without skin, seafood, processed soy products, nuts, seeds, beans, and peas.
A healthy diet is low in sodium (salt), added sugars, solid fats, and refined grains. Solid fats are saturated fat and trans fatty acids. Refined grains come from processing whole grains, which results in a loss of nutrients (such as dietary fiber).
For more information about following a healthy diet, go to the NHLBI's ""Your Guide to Lowering Your Blood Pressure With DASH"" and the U.S. Department of Agriculture's ChooseMyPlate.gov Web site. Both resources provide general information about healthy eating.
Being physically active also can help you maintain a healthy weight. Before starting any kind of exercise program or new physical activity, talk with your doctor about the types and amounts of physical activity that are safe for you.
For more information about physical activity, go to the U.S. Department of Health and Human Services' ""2008 Physical Activity Guidelines for Americans,"" the Health Topics Physical Activity and Your Heart article, and the NHLBI's ""Your Guide to Physical Activity and Your Heart.""
Make sure to schedule routine doctor visits to keep track of your cholesterol, blood pressure, and blood sugar levels. A blood test called a lipoprotein panel will show your levels of total cholesterol, LDL cholesterol, HDL cholesterol, and triglycerides.
Living With Metabolic Syndrome
Metabolic syndrome is a lifelong condition. However, lifestyle changes can help you control your risk factors and reduce your risk for heart disease and diabetes.
If you already have heart disease and/or diabetes, lifestyle changes can help you prevent or delay related problems. Examples of these problems include heart attack, stroke, and diabetes-related complications (for example, damage to your eyes, nerves, kidneys, feet, and legs).
Lifestyle changes may include losing weight, following a heart healthy diet, being physically active, and quitting smoking.
If lifestyle changes aren't enough, your doctor may recommend medicines. Take all of your medicines as your doctor prescribes. For more information about lifestyle changes and medicines, go to ""How Is Metabolic Syndrome Treated?""
Make realistic short- and long-term goals for yourself when you begin to make healthy lifestyle changes. Work closely with your doctor and seek regular medical care.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. For example, this research has uncovered some of the causes of various diseases and conditions, as well as ways to prevent or treat them.
The NHLBI continues to support research aimed at learning more about various diseases and conditions, including metabolic syndrome. For example, NHLBI-supported research includes studies that explore:
The effects of two diets on the
treatment of adolescents who have
heart disease risk factors
Much of this research depends on the willingness of volunteers to take part in clinical trials. Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions.
For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to metabolic syndrome, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
What Is Mitral Valve Prolapse?
Mitral (MI-tral) valve prolapse (MVP) is a condition in which the heart's mitral valve doesn't work well. The flaps of the valve are ""floppy"" and don't close tightly. These flaps normally help seal or open the valve.
Much of the time, MVP doesn't cause any problems. Rarely, blood can leak the wrong way through the floppy valve. This can lead to palpitations, shortness of breath, chest pain, and other symptoms. (Palpitations are feelings that your heart is skipping a beat, fluttering, or beating too hard or too fast.)
Normal Mitral Valve
The mitral valve controls blood flow between the upper and lower chambers of the left side of the heart. The upper chamber is called the left atrium (AY-tree-um). The lower chamber is called the left ventricle (VEN-trih-kul).
The mitral valve allows blood to flow from the left atrium into the left ventricle, but not back the other way. The heart also has a right atrium and ventricle, separated by the tricuspid (tri-CUSS-pid) valve.
With each heartbeat, the atria contract and push blood into the ventricles. The flaps of the mitral and tricuspid valves open to let blood through. Then, the ventricles contract to pump the blood out of the heart.
When the ventricles contract, the flaps of the mitral and tricuspid valves close. They form a tight seal that prevents blood from flowing back into the atria.
For more information, go to the Health Topics How the Heart Works article. This article contains animations that show how your heart pumps blood and how your heart's electrical system works.
Mitral Valve Prolapse
In MVP, when the left ventricle contracts, one or both flaps of the mitral valve flop or bulge back (prolapse) into the left atrium. This can prevent the valve from forming a tight seal.
As a result, blood may leak from the ventricle back into the atrium. The backflow of blood is called regurgitation (re-GUR-jih-TA-shun).
MVP doesn't always cause backflow. In fact, most people who have MVP don't have backflow and never have any related symptoms or problems. Their mitral valves still can form a tight seal.
When backflow does occur, it can cause shortness of breath, irregular heartbeats called arrhythmias (ah-RITH-me-ahs), or chest pain.
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Mitral Valve Prolapse. Figure A shows a normal mitral valve. The valve separates the left atrium from the left ventricle. Figure B shows a heart with mitral valve prolapse. Figure C shows a closeup view of mitral valve prolapse. Figure D shows a mitral (more...)
Backflow can get worse over time. It can change the heart's size and raise pressure in the left atrium and lungs. Backflow also raises the risk of heart valve infections.
Medicines can treat troublesome MVP symptoms and help prevent complications. Some people will need surgery to repair or replace their mitral valves.
MVP once was thought to affect as much as 5 to 15 percent of the population. Researchers now believe that many people who were diagnosed with MVP in the past didn't actually have a faulty mitral valve.
They may have had a slight bulging of the valve flaps due to other conditions, such as dehydration (lack of fluid in the body) or a small heart. However, their valves were normal, and little or no backflow of blood occurred.
Diagnosing MVP is more precise now because of a test called echocardiography (EK-o-kar-de-OG-ra-fee). This test allows doctors to easily see MVP and detect troublesome backflow.
As a result, researchers now think that less than 3 percent of the population actually has MVP. They believe an even smaller percentage has serious complications from the condition.
Outlook
Most people who have MVP have no symptoms or medical problems and don't need treatment. They're able to lead normal, active lives; they may not even know they have the condition.
A small number of people who have MVP may need medicine to relieve their symptoms. Very few people who have MVP need heart valve surgery to repair their mitral valves.
Rarely, MVP can cause problems such as arrhythmias (irregular heartbeats) or infective endocarditis (EN-do-kar-DI-tis). Endocarditis is an infection of the inner lining of the heart chambers and valves. Bacteria that enter the bloodstream can cause the infection.
Other Names for Mitral Valve Prolapse
Barlow's syndrome
Floppy valve syndrome
What Causes Mitral Valve Prolapse?
The exact cause of mitral valve prolapse (MVP) isn't known. Most people who have the condition are born with it. MVP tends to run in families. Also, it's more common in people who are born with connective tissue disorders, such as Marfan syndrome.
In people who have MVP, the mitral valve may be abnormal in the following ways:
These problems can keep the valve from making a tight seal. Some people's valves are abnormal in more than one way.
Who Is at Risk for Mitral Valve Prolapse?
Mitral valve prolapse (MVP) affects people of all ages and both sexes. MVP that causes complications or severe symptoms most often occurs in men older than 50.
Certain conditions increase the risk of MVP, including:
Scoliosis (SKO-le-O-sis) and other skeletal problems
What Are the Signs and Symptoms of Mitral Valve Prolapse?
Most people who have mitral valve prolapse (MVP) aren't affected by the condition. They don't have any symptoms or major mitral valve backflow.
When MVP does cause signs and symptoms, they may include:
Palpitations (feelings that your
heart is skipping a beat, fluttering, or beating too hard or too fast)
Shortness of breath
MVP symptoms can vary from one person to another. They tend to be mild but can worsen over time, mainly when complications occur.
Mitral Valve Prolapse Complications
MVP complications are rare. When present, they're most often caused by the backflow of blood through the mitral valve.
Mitral valve backflow is most common among men and people who have high blood pressure. People who have severe backflow may need valve surgery to prevent complications.
Mitral valve backflow causes blood to flow from the left ventricle back into the left atrium. Blood can even back up from the atrium into the lungs, causing shortness of breath.
The backflow of blood strains the muscles of both the atrium and the ventricle. Over time, the strain can lead to arrhythmias. Backflow also increases the risk of infective endocarditis (IE). IE is an infection of the inner lining of your heart chambers and valves.
Arrhythmias
Arrhythmias are problems with the rate or rhythm of the heartbeat. The most common types of arrhythmias are harmless. Other arrhythmias can be serious or even life threatening, such as ventricular arrhythmias.
If the heart rate is too slow, too fast, or irregular, the heart may not be able to pump enough blood to the body. Lack of blood flow can damage the brain, heart, and other organs.
One troublesome arrhythmia that MVP can cause is atrial fibrillation (AF). In AF, the walls of the atria quiver instead of beating normally. As a result, the atria aren't able to pump blood into the ventricles the way they should.
AF is bothersome but rarely life threatening, unless the atria contract very fast or blood clots form in the atria. Blood clots can occur because some blood ""pools"" in the atria instead of flowing into the ventricles. If a blood clot breaks off and travels through the bloodstream, it can reach the brain and cause a stroke.
How Is Mitral Valve Prolapse Diagnosed?
Mitral valve prolapse (MVP) most often is detected during a routine physical exam. During the exam, your doctor will listen to your heart with a stethoscope.
Stretched valve flaps can make a clicking sound as they shut. If the mitral valve is leaking blood back into the left atrium, your doctor may heart a heart murmur or whooshing sound.
However, these abnormal heart sounds may come and go. Your doctor may not hear them at the time of an exam, even if you have MVP. Thus, you also may have tests and procedures to diagnose MVP.
Diagnostic Tests and Procedures
Echocardiography
Echocardiography (echo) is the most useful test for diagnosing MVP. This painless test uses sound waves to create a moving picture of your heart.
Echo shows the size and shape of your heart and how well your heart chambers and valves are working. The test also can show areas of heart muscle that aren't contracting normally because of poor blood flow or injury to the heart muscle.
Echo can show prolapse of the mitral valve flaps and backflow of blood through the leaky valve.
There are several types of echo, including stress echo. Stress echo is done before and after a stress test. During a stress test, you exercise or take medicine (given by your doctor) to make your heart work hard and beat fast.
You may have stress echo to find out whether you have decreased blood flow to your heart (a sign of coronary heart disease).
Echo also can be done by placing a tiny probe in your esophagus to get a closer look at the mitral valve. The esophagus is the passage leading from your mouth to your stomach.
The probe uses sound waves to create pictures of your heart. This form of echo is called transesophageal (tranz-ih-sof-uh-JEE-ul) echocardiography, or TEE.
Doppler Ultrasound
A Doppler ultrasound is part of an echo test. A Doppler ultrasound shows the speed and direction of blood flow through the mitral valve.
Other Tests
Other tests that can help diagnose MVP include:
A
chest x ray. This test is used to look for fluid in your
lungs or to show whether your
heart is enlarged.
An
EKG (
electrocardiogram). An EKG is a simple test that records your
heart's electrical activity. An EKG can show how fast your heart is beating and whether its rhythm is steady or irregular. This test also records the strength and timing of electrical signals as they pass through your heart.
How Is Mitral Valve Prolapse Treated?
Most people who have mitral valve prolapse (MVP) don't need treatment because they don't have symptoms and complications.
Even people who do have symptoms may not need treatment. The presence of symptoms doesn't always mean that the backflow of blood through the valve is significant.
People who have MVP and troublesome mitral valve backflow usually need treatment. MVP is treated with medicines, surgery, or both.
The goals of treating MVP include:
Relieving symptoms
Correcting the underlying
mitral valve problem, if necessary
Medicines
Medicines called beta blockers may be used to treat palpitations and chest discomfort in people who have little or no mitral valve backflow.
If you have significant backflow and symptoms, your doctor may prescribe:
Digoxin to strengthen your heartbeat.
Surgery
Surgery is done only if the mitral valve is very abnormal and blood is flowing back into the atrium. The main goal of surgery is to improve symptoms and reduce the risk of heart failure.
The timing of the surgery is important. If it's done too early and your leaking valve is working fairly well, you may be put at needless risk from surgery. If it's done too late, you may have heart damage that can't be fixed.
Surgical Approaches
Traditionally, heart surgeons repair or replace a mitral valve by making an incision (cut) in the breastbone and exposing the heart.
A small but growing number of surgeons are using another approach that involves one or more small cuts through the side of the chest wall. This results in less cutting, reduced blood loss, and a shorter hospital stay. However, not all hospitals offer this method.
Valve Repair and Valve Replacement
In mitral valve surgery, the valve is repaired or replaced. Valve repair is preferred when possible. Repair is less likely than replacement to weaken the heart. Repair also lowers the risk of infection and decreases the need for lifelong use of blood-thinning medicines.
If repair isn't an option, the valve can be replaced. Mechanical and biological valves are used as replacement valves.
Mechanical valves are man-made and can last a lifetime. People who have mechanical valves must take blood-thinning medicines for the rest of their lives.
Biological valves are taken from cows or pigs or made from human tissue. Many people who have biological valves don't need to take blood-thinning medicines for the rest of their lives. The major drawback of biological valves is that they weaken over time and often last only about 10 years.
After surgery, you'll likely stay in the hospital's intensive care unit for 2 to 3 days. Overall, most people who have mitral valve surgery spend about 1 to 2 weeks in the hospital. Complete recovery takes a few weeks to several months, depending on your health before surgery.
If you've had valve repair or replacement, you may need antibiotics before dental work and surgery. These procedures can allow bacteria to enter your bloodstream. Antibiotics can help prevent IE, a serious heart valve infection. Discuss with your doctor whether you need to take antibiotics before such procedures.
Experimental Approaches
Researchers are testing whether they can repair leaky mitral valves using a catheter (tube) inserted through a large blood vessel.
This approach is less invasive and can prevent a patient from having open-heart surgery. However, only a few medical centers are doing this type of procedure. Large studies haven't yet shown that this new approach is better than traditional approaches.
How Can Mitral Valve Prolapse Be Prevented?
You can't prevent mitral valve prolapse (MVP). Most people who have the condition are born with it.
Complications from MVP, such as arrhythmias (irregular heartbeats) and infective endocarditis (IE), are rare. IE is an infection of the inner lining of your heart chambers and valves.
People at high risk for IE may be given antibiotics before some types of surgery and dental work. Antibiotics can help prevent IE. Your doctor will tell you whether you need this type of treatment.
People at high risk for IE may include those who've had valve repair or replacement or who have some types of underlying heart disease.
Living With Mitral Valve Prolapse
Most people who have mitral valve prolapse (MVP) have no symptoms or related problems, need no treatment, and are able to lead normal, active lives. If symptoms and complications do occur, you often can control them with medicine.
Some people may need heart valve surgery to relieve their symptoms and prevent complications. Rarely, MVP can cause arrhythmias (irregular heartbeats) and other problems.
Ongoing Care
If you have MVP, having ongoing care is important. Ask your doctor how often you should schedule followup visits. See your doctor if your symptoms worsen.
The backflow of blood that may occur with MVP increases your risk of infective endocarditis (IE). IE is an infection of the inner lining of your heart chambers and valves.
You can take steps to try to prevent IE. Tell your doctors and dentists that you have MVP. Floss and brush your teeth regularly. Gum infections and tooth decay can cause IE. Call your doctor if you have any signs of infection, such as sore throat, general body aches, or fever.
Take all of your medicines as your doctor prescribes, including blood-thinning and high blood pressure medicines.
Make healthy lifestyle choices. For example, avoid smoking and taking birth control pills; both can raise your risk for blood clots.
If you smoke, talk with your doctor about programs and products that can help you quit. Also, try to avoid secondhand smoke. For more information about how to quit smoking, go to the Health Topics Smoking and Your Heart article.
Ask your doctor how much and what types of physical activity are safe for you. Also, ask your doctor whether you need to make any changes to your diet.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to mitral valve prolapse, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.
What Is Narcolepsy?
Narcolepsy (NAR-ko-lep-se) is a disorder that causes periods of extreme daytime sleepiness. The disorder also may cause muscle weakness.
Most people who have narcolepsy have trouble sleeping at night. Some people who have the disorder fall asleep suddenly, even if they're in the middle of talking, eating, or another activity.
Narcolepsy also can cause:
Cataplexy (KAT-ah-plek-se). This condition causes a sudden loss of
muscle tone while you're awake. Muscle weakness can affect certain parts of your body or your whole body. For example, if cataplexy affects your
hand, you may drop what you're holding. Strong emotions often trigger this weakness. It may last seconds or minutes.
Sleep paralysis (pah-RAL-ih-sis). This condition prevents you from moving or speaking while waking up and sometimes while
falling asleep. Sleep paralysis usually goes away within a few minutes.
Overview
The two main phases of sleep are nonrapid eye movement (NREM) and rapid eye movement (REM). Most people are in the NREM phase when they first fall asleep. After about 90 minutes of sleep, most people go from NREM to REM sleep.
Dreaming occurs during the REM phase of sleep. During REM, your muscles normally become limp. This prevents you from acting out your dreams. (For more information about sleep cycles, go to the National Heart, Lung, and Blood Institute's ""Your Guide to Healthy Sleep."")
People who have narcolepsy often fall into REM sleep quickly and wake up directly from it. As a result, they may have vivid dreams while falling asleep and waking up.
Hypocretin (hi-po-KREET-in), a chemical in the brain, helps promote wakefulness. Most people who have narcolepsy have low levels of this chemical. What causes these low levels isn't well understood.
Researchers think that certain factors may work together to cause a lack of hypocretin. These factors may include heredity, infections, brain injuries, and autoimmune disorders. (Autoimmune disorders occur if the body's immune system mistakenly attacks the body's cells and tissues.)
Outlook
Narcolepsy symptoms usually begin during the teen or young adult years. People who have narcolepsy may find it hard to function at school, work, home, and in social situations because of extreme tiredness.
Narcolepsy has no cure, but medicines, lifestyle changes, and other therapies can improve symptoms. Research is ongoing on the causes of narcolepsy and new ways to treat it.
What Causes Narcolepsy?
Most people who have narcolepsy have low levels of hypocretin. This is a chemical in the brain that helps promote wakefulness. What causes low hypocretin levels isn't well understood.
Researchers think that certain factors may work together to cause a lack of hypocretin. These factors may include:
Heredity. Some people may inherit a
gene that affects hypocretin. Up to 10 percent of people who have
narcolepsy report having a relative who has the same symptoms.
Infections.
Low levels of
histamine, a substance in the
blood that promotes wakefulness.
Some research suggests that environmental toxins may play a role in triggering narcolepsy. Toxins may include heavy metals, pesticides and weed killers, and secondhand smoke.
Heredity alone doesn't cause narcolepsy. You also must have at least one other factor, such as one of those listed above, to develop narcolepsy.
Who Is At Risk for Narcolepsy?
Narcolepsy affects men and women. Symptoms usually begin during the teen or young adult years. The disorder also can develop later in life or in children, but it's rare before age 5.
Researchers think that certain factors may work together to cause narcolepsy. If these factors affect you, you may be at higher risk for the disorder. (For more information, go to ""What Causes Narcolepsy?"")
What Are the Signs and Symptoms of Narcolepsy?
The four major signs and symptoms of narcolepsy are extreme daytime sleepiness, cataplexy (muscle weakness) while awake, and hallucinations and sleep paralysis during sleep.
If you have narcolepsy, you may have one or more of these symptoms. They can range from mild to severe. Less than one-third of people who have narcolepsy have all four symptoms.
Extreme Daytime Sleepiness
All people who have narcolepsy have extreme daytime sleepiness. This often is the most obvious symptom of the disorder.
During the day, you may have few or many periods of sleepiness. Each period usually lasts 30 minutes or less. Strong emotions—such as anger, fear, laughter, or excitement—can trigger this sleepiness.
People who have daytime sleepiness often complain of:
Mental cloudiness or ""fog""
Memory problems or problems focusing
Lack of energy or extreme exhaustion
Some people who have narcolepsy have episodes in which they fall asleep suddenly. This is more likely to happen when they're not active—for example, while reading, watching TV, or sitting in a meeting.
However, sleep episodes also may occur in the middle of talking, eating, or another activity. Cataplexy also may occur at the same time.
Cataplexy
This condition causes loss of muscle tone while you're awake. Muscle weakness affects part or all of your body.
Cataplexy may make your head nod or make it hard for you to speak. Muscle weakness also may make your knees weak or cause you to drop things you're holding. Some people lose all muscle control and fall.
Strong emotions—such as anger, surprise, fear, or laughter—often trigger cataplexy. It usually lasts a few seconds or minutes. During this time, you're usually awake.
Cataplexy may occur weeks to years after you first start to have extreme daytime sleepiness.
Hallucinations
If you have narcolepsy, you may have vivid dreams while falling asleep, waking up, or dozing. These dreams can feel very real. You may feel like you can see, hear, smell, and taste things.
Sleep Paralysis
This condition prevents you from moving or speaking while falling asleep or waking up. However, you're fully conscious (aware) during this time. Sleep paralysis usually lasts just a few seconds or minutes, but it can be scary.
Other Symptoms
Most people who have narcolepsy don't sleep well at night. They may have trouble falling and staying asleep. Vivid, scary dreams may disturb sleep. Not sleeping well at night worsens daytime sleepiness.
Rarely, people who fall asleep in the middle of an activity, such as eating, may continue that activity for a few seconds or minutes. This is called automatic behavior.
During automatic behavior, you're not aware of your actions, so you don't do them well. For example, if you're writing before falling asleep, you may scribble rather than form words. If you're driving, you may get lost or have an accident. Most people who have this symptom don't remember what happened while it was going on.
Children who have narcolepsy often have trouble studying, focusing, and remembering things. Also, they may seem hyperactive. Some children who have narcolepsy speed up their activities rather than slow them down.
Children who have narcolepsy may have severe sleepiness. They may fall asleep while talking or eating, or during sporting events and social activities.
How Is Narcolepsy Diagnosed?
It can take as long as 10 to 15 years after the first symptoms appear before narcolepsy is recognized and diagnosed. This is because narcolepsy is fairly rare. Also, many narcolepsy symptoms are like symptoms of other illnesses, such as infections, depression, and sleep disorders.
Narcolepsy sometimes is mistaken for learning problems, seizure disorders, or laziness, especially in school-aged children and teens. When narcolepsy symptoms are mild, the disorder is even harder to diagnose.
Your doctor will diagnose narcolepsy based on your signs and symptoms, your medical and family histories, a physical exam, and test results.
Signs and Symptoms
Tell your doctor about any signs and symptoms of narcolepsy that you have. This is important because your doctor may not ask about them during a routine checkup.
Your doctor will want to know when you first had signs and symptoms and whether they bother your sleep or daily routine. He or she also will want to know about your sleep habits and how you feel and act during the day.
To help answer these questions, you may want to keep a sleep diary for a few weeks. Keep a daily record of how easy it is to fall and stay asleep, how much sleep you get at night, and how alert you feel during the day.
For a sample sleep diary, go to the National Heart, Lung, and Blood Institute's ""Your Guide to Healthy Sleep.""
Medical and Family Histories
Your doctor may ask whether:
You're affected by certain factors that can lead to
narcolepsy. Examples of these factors include infections,
brain injuries, and
autoimmune disorders. Some research suggests that environmental
toxins may play a role in triggering narcolepsy.
You take medicines and which ones you take. Some medicines can cause daytime sleepiness. Thus, your symptoms may be due to medicine, not
narcolepsy.
You have relatives who have
narcolepsy or who have signs or symptoms of the disorder.
Physical Exam
Your doctor will check you to see whether another condition is causing your symptoms. For example, infections, certain thyroid diseases, drug and alcohol use, and other medical or sleep disorders may cause symptoms similar to those of narcolepsy.
Diagnostic Tests
Sleep Studies
If your doctor thinks you have narcolepsy, he or she will likely suggest that you see a sleep specialist. This specialist may advise you to have sleep studies to find out more about your condition.
Sleep studies usually are done at a sleep center. Doctors use the results from two tests to diagnose narcolepsy. These tests are a polysomnogram (PSG) and a multiple sleep latency test (MSLT).
Polysomnogram. You usually stay overnight at a sleep center for a PSG. The test records brain activity, eye movements, heart rate, and blood pressure. A PSG can help find out whether you:
Multiple sleep latency test. This daytime sleep study measures how sleepy you are. It's often done the day after a PSG. During the test, you're asked to nap for 20 minutes every 2 hours throughout the day. (You will nap a total of four or five times.)
A technician checks your brain activity during this time. He or she notes how quickly you fall asleep and how long it takes you to reach various stages of sleep.
An MSLT finds out how quickly you fall asleep during the day (after a full night's sleep). It also shows whether you go into REM sleep soon after falling asleep.
Other Tests
Hypocretin test. This test measures the level of hypocretin in the fluid that surrounds your spinal cord. Most people who have narcolepsy have low levels of hypocretin. Hypocretin is a chemical that helps promote wakefulness.
To get a sample of spinal cord fluid, a spinal tap (also called a lumbar puncture) is done. For this procedure, your doctor inserts a needle into your lower back area and then withdraws a sample of your spinal fluid.
How Is Narcolepsy Treated?
Narcolepsy has no cure. However, medicines, lifestyle changes, and other therapies can relieve many of its symptoms. Treatment for narcolepsy is based on the type of symptoms you have and how severe they are.
Not all medicines and lifestyle changes work for everyone. It may take weeks to months for you and your doctor to find the best treatment.
Medicines
You may need one or more medicines to treat narcolepsy symptoms. These may include:
Some prescription and over-the-counter medicines can interfere with your sleep. Ask your doctor about these medicines and how to avoid them, if possible. For example, your doctor may advise you to avoid antihistamines. These medicines suppress the action of histamine, a substance in the blood that promotes wakefulness.
If you take regular naps when you feel sleepy, you may need less medicine to stay awake.
Lifestyle Changes
Lifestyle changes also may help relieve some narcolepsy symptoms. You can take steps to make it easier to fall asleep at night and stay asleep.
Certain activities, foods, and drinks before bedtime can keep you awake. Try to follow these guidelines:
For more tips on sleeping better, go to the National Heart, Lung, and Blood Institute's ""Your Guide to Healthy Sleep.""
Other Therapies
Light therapy may help you keep a regular sleep and wake schedule. For this type of therapy, you sit in front of a light box, which has special lights, for 10 to 30 minutes. This therapy can help you feel less sleepy in the morning.
Living With Narcolepsy
Living with narcolepsy can be hard. It can affect your ability to drive, work, go to school, and have relationships. Besides taking medicine, you can do many things to live a safe and fulfilling life.
Driving
Driving can be dangerous for people who have narcolepsy. Ask your doctor whether you can drive safely. To help make it safer for you to drive:
Take naps before driving. This helps some people who have
periods of extreme daytime sleepiness.
Stop often during long drives. Stretch and walk around during the stops.
Try to have family, friends, or coworkers in the car to keep you aware and engaged, or get rides from them.
Working
People who have narcolepsy can work in almost any type of job, but some jobs may be better than others.
For example, a job with a flexible work schedule can make it easier to take naps when needed. A job in which you interact with your coworkers can help keep you awake. Jobs that don't require you to drive or are closer to home also may better suit your needs.
Certain laws may apply to workers who have medical conditions, such as narcolepsy. These laws include the:
Americans with Disabilities Act (ADA). This law requires employers to reasonably accommodate the needs of their workers who have disabilities. This includes people who have
narcolepsy. For example, employers may allow workers to take short naps during the workday or adjust work schedules to avoid sleepy
periods.
Family and Medical Leave Act. This law requires employers who have 50 or more employees to provide unpaid leave to employees who have an illness, such as
narcolepsy. This law also gives leave to family members who need time to care for a close relative who has a serious illness.
Social Security Disability Insurance or Supplemental Security Income programs. These programs may offer financial help if you can't work because of your
narcolepsy.
Emotional Issues and Support
Living with narcolepsy may cause fear, anxiety, depression, and stress. Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with narcolepsy. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
Narcolepsy in Special Groups
School-Aged Children
Children who have narcolepsy may have trouble studying, focusing, and remembering things. To help your child in school:
Talk with your child's teachers and school officials about your child's
narcolepsy and the best ways to meet his or her needs. For example, your child may need to take naps or walks during the day or tape the teacher's lessons.
Talk with the school nurse about your child's
narcolepsy and medicines. Together you can work out a place to keep the medicines and a schedule for taking them at school.
Pregnant Women
If you're pregnant or planning a pregnancy, ask your doctor whether you should continue taking your narcolepsy medicines. Certain medicines may interfere with your pregnancy.
Clinical Trials
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. Often, these advances depend on the willingness of volunteers to take part in clinical trials.
Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions. For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to narcolepsy, talk with your doctor. You also can visit the following Web sites to learn more about clinical research and to search for clinical trials:
For more information about clinical trials for children, visit the NHLBI's Children and Clinical Studies Web page.