Von Willebrand disease (VWD) is a bleeding disorder. It affects your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs. Rarely, the bleeding may even cause death.
In VWD, you either have low levels of a certain protein in your blood or the protein doesn't work well. The protein is called von Willebrand factor, and it helps your blood clot.
Normally, when one of your blood vessels is injured, you start to bleed. Small blood cell fragments called platelets (PLATE-lets) clump together to plug the hole in the blood vessel and stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot.
Von Willebrand factor also carries clotting factor VIII (8), another important protein that helps your blood clot. Factor VIII is the protein that's missing or doesn't work well in people who have hemophilia, another bleeding disorder.
VWD is more common and usually milder than hemophilia. In fact, VWD is the most common inherited bleeding disorder. It occurs in about 1 out of every 100 to
The three major types of VWD are called type 1, type 2, and type 3.
People who have type 1 VWD have low levels of von Willebrand factor and may have low levels of factor VIII. Type 1 is the mildest and most common form of VWD. About 3 out of 4 people who have VWD have type 1.
In type 2 VWD, the von Willebrand factor doesn't work well. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N. Different gene mutations (changes) cause each type, and each is treated differently. Thus, it's important to know the exact type of VWD that you have.
People who have type 3 VWD usually have no von Willebrand factor and low levels of factor VIII. Type 3 is the most serious form of VWD, but it's very rare.
Most people who have VWD have type 1, a mild form. This type usually doesn't cause life-threatening bleeding. You may need treatment only if you have surgery, tooth extraction, or trauma. Treatment includes medicines and medical therapies.
Some people who have severe forms of VWD need emergency treatment to stop bleeding before it becomes life threatening.
Early diagnosis is important. With the proper treatment plan, even people who have type 3 VWD can live normal, active lives.
Von Willebrand disease (VWD) is almost always inherited. "Inherited" means that the disorder is passed from parents to children though genes.
You can inherit type 1 or type 2 VWD if only one of your parents passes the gene on to you. You usually inherit type 3 VWD only if both of your parents pass the gene on to you. Your symptoms may be different from your parents' symptoms.
Some people have the genes for the disorder but don't have symptoms. However, they still can pass the genes on to their children.
Some people get VWD later in life as a result of other medical conditions. This type of VWD is called acquired von Willebrand syndrome.
The signs and symptoms of von Willebrand disease (VWD) depend on which type of the disorder you have. They also depend on how serious the disorder is. Many people have such mild symptoms that they don't know they have VWD.
If you have type 1 or type 2 VWD, you may have the following mild-to-moderate bleeding symptoms:
People who have type 3 VWD may have all of the symptoms listed above and severe bleeding episodes for no reason. These bleeding episodes can be fatal if not treated right away. People who have type 3 VWD also may have bleeding into soft tissues or joints, causing severe pain and swelling.
Heavy menstrual bleeding often is the main symptom of VWD in women. Doctors call this menorrhagia (men-o-RA-je-ah). They define it as:
However, just because a woman has heavy menstrual bleeding doesn't mean she has VWD.
Early diagnosis of von Willebrand disease (VWD) is important to make sure that you're treated and can live a normal, active life.
Sometimes VWD is hard to diagnose. People who have type 1 or type 2 VWD may not have major bleeding problems. Thus, they may not be diagnosed unless they have heavy bleeding after surgery or some other trauma.
On the other hand, type 3 VWD can cause major bleeding problems during infancy and childhood. So, children who have type 3 VWD usually are diagnosed during their first year of life.
To find out whether you have VWD, your doctor will review your medical history and the results from a physical exam and tests.
Your doctor will likely ask questions about your medical history and your family's medical history. He or she may ask about:
Your doctor will do a physical exam to look for unusual bruising or other signs of recent bleeding. He or she also will look for signs of liver disease or anemia (a low red blood cell count).
No single test can diagnose VWD. Your doctor may recommend one or more blood tests to diagnose the disorder. These tests may include:
You may have these tests more than once to confirm a diagnosis. Your doctor also may refer you to a hematologist to confirm the diagnosis and for followup care. A hematologist is a doctor who specializes in diagnosing and treating blood disorders.
Treatment for von Willebrand disease (VWD) is based on the type of VWD you have and how severe it is. Most cases of VWD are mild, and you may need treatment only if you have surgery, tooth extraction, or an accident.
Medicines are used to:
One treatment for VWD is a man-made hormone called desmopressin. You usually take this hormone by injection or nasal spray. It makes your body release more von Willebrand factor and factor VIII into your bloodstream. Desmopressin works for most people who have type 1 VWD and for some people who have type 2 VWD.
Another type of treatment is von Willebrand factor replacement therapy. This involves an infusion of concentrated von Willebrand factor and factor VIII into a vein in your arm. This treatment may be used if you:
Antifibrinolytic (AN-te-fi-BRIN-o-LIT-ik) medicines also are used to treat VWD. These medicines help prevent the breakdown of blood clots. They're mostly used to stop bleeding after minor surgery, tooth extraction, or an injury. These medicines may be used alone or with desmopressin and replacement therapy.
Fibrin glue is medicine that's placed directly on a wound to stop bleeding.
Treatments for women who have VWD with heavy menstrual bleeding include:
For some women who are done having children or don't want children, endometrial ablation (EN-do-ME-tre-al ab-LA-shun) is done. This procedure destroys the lining of the uterus. It has been shown to reduce menstrual blood loss in women who have VWD.
If you need a hysterectomy (HIS-ter-EK-to-me; surgical removal of the uterus) for another reason, this procedure will stop menstrual bleeding and possibly improve your quality of life. However, hysterectomy has its own risk of bleeding complications.
If you have von Willebrand disease (VWD), you can take steps to prevent bleeding and stay healthy.
For example, avoid over-the-counter medicines that can affect blood clotting, such as aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs). Always check with your doctor before taking any medicines.
Tell your doctor, dentist, and pharmacist that you have VWD. Your dentist can ask your doctor whether you need medicine before dental work to reduce bleeding.
You also may want to tell other people about your condition, like your employee health nurse, gym trainer, and sports coach. Making them aware will allow them to act quickly if you have an injury.
Consider wearing a medical ID bracelet or necklace if you have a serious form of VWD (for example, type 3). In case of a serious accident or injury, the health care team treating you will know that you have VWD.
Be physically active and maintain a healthy weight. Physical activity helps keep muscles flexible. It also helps prevent damage to muscles and joints. Always stretch before exercising.
Some safe physical activities are swimming, biking, and walking. Football, hockey, wrestling, and lifting heavy weights are not safe activities if you have bleeding problems. Always check with your doctor before starting any exercise program.
Your parents, brothers and sisters, and children also may have VWD. Talk with them about your diagnosis and suggest that they get tested too.
Pregnancy can be a challenge for women who have VWD. Blood levels of von Willebrand factor and factor VIII tend to increase during pregnancy. However, women who have VWD can have bleeding problems during delivery. They also are likely to have heavy bleeding for an extended time after delivery.
You can take steps to lower the risk of complications during pregnancy. If possible, talk with a hematologist and an obstetrician who specializes in high-risk pregnancies before you become pregnant.
A hematologist is a doctor who specializes in diagnosing and treating blood disorders. An obstetrician is a doctor who provides treatment and care for pregnant women.
Consider using a medical center that specializes in high-risk obstetrics and has a hematologist on staff for prenatal care and delivery.
Before you have any invasive procedure, such as amniocentesis (AM-ne-o-sen-TE-sis), discuss with your doctor whether you need to take steps to prevent serious blood loss.
During your third trimester, you should have blood tests to measure von Willebrand factor and factor VIII to help plan for delivery.
You also should meet with an anesthesiologist to review your choices for anesthesia (AN-es-THE-ze-ah) and to discuss taking medicine to reduce your bleeding risk. The term "anesthesia" refers to a loss of feeling and awareness. Some types of anesthesia temporarily put you to sleep, while others only numb certain areas of your body.
With these steps for safety, most women who have VWD can have successful pregnancies.
If your child has VWD that's severe enough to cause bleeding, anyone who cares for him or her should be told about the condition.
For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities should know, especially if your child has severe VWD. This information will help them handle the situation if your child has an injury.
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 von Willebrand disease, 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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