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In Brief: Your Guide to von Willebrand Disease
If you often have large, lumpy bruises; frequent or difficult-to-stop nosebleeds; bleed a lot after a fairly minor cut; or are a woman who has very heavy or long menstrual periods, you should ask your doctor about an inherited bleeding disorder called von Willebrand disease (VWD). Named for Dr. Erik von Willebrand, a Finnish doctor who first described the condition in 1926, VWD affects your bloods ability to clot and can lead to heavy, hard-to-stop bleeding after an injury. The bleeding from VWD can lead to damage of your internal organs or even be life threatening, but this is rare.
In VWD, you either have low levels of a certain protein in your blood or the protein doesnt work the way it should. The protein is called von Willebrand factor (VWF). It is made in the walls of your blood vessels and released into your blood.
Normally, when one of your blood vessels is injured, you start to bleed. As soon as this happens, small cells in your blood that are called platelets 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 with it clotting factor VIII (8), another important protein that helps your blood clot. Factor VIII is the protein that is inactive or missing in hemophilia, another clotting disorder.
Von Willebrand disease, like hemophilia, is an inherited bleeding disorder, but VWD is more common and usually milder. In fact, VWD is the most common of all the inherited bleeding disorders. It occurs in about 1 out of every 100 to 1,000 people. It also affects both males and females, while hemophilia mainly affects males.
There are three major types of von Willebrand disease.
Von Willebrand disease cannot be cured, but it can be treated. Early diagnosis is important, and with the right treatment plan, even people with type 3 VWD can be helped to live active lives.
What Causes von Willebrand disease?
Von Willebrand disease is almost always inherited. Your parents pass the gene for the disease on to you. You can develop types 1 or 2 VWD when only one of your parents carries the gene for it. 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.
Some people develop a form of the disease later in life as a result of other medical conditions. This is called Acquired von Willebrand Syndrome (AVWS).
What Are the Signs and Symptoms of von Willebrand Disease?
The signs and symptoms of VWD depend on the type and severity of the disease. Many people have such mild symptoms that they may not know they have the disorder. Some people have the gene for the disease but dont have any symptoms.
If you have type 1 or type 2 VWD, you may have the following mild to moderate symptoms:
*Heavy menstrual bleeding is the most common symptom in women. If it isnt treated, it can lead to iron deficiency and anemia. (Not all heavy menstrual bleeding is due to VWD.)
If you have type 3 VWD, you may have any or all of the symptoms listed above, as well as the following:
How is von Willebrand Disease Diagnosed?
Von Willebrand disease is sometimes difficult to diagnose. People with type 1 or type 2 VWD may not have major bleeding problems; as a result, they may not be diagnosed until 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. As a result, children with type 3 VWD are usually diagnosed during their first year of life.
To find out if you have VWD, your doctor will take a complete medical history and do a physical exam. For the history, he or she will likely want to know about your personal and family historyin particular:
The doctor will also do a physical examination to look for:
No single test exists for diagnosing VWD.
As a result, your doctor will order a combination of blood tests to diagnose the disease. These tests may include:
Your doctor may order these tests more than once to confirm the diagnosis. He or she may also refer you to a hematologist (a doctor who specializes in treating blood diseases) to confirm the diagnosis and for follow-up care.
Early diagnosis is important to make sure you are treated effectively and can live a normal, active life.
How is von Willebrand Disease Treated?
Your doctor will decide what treatment you need, based on the type of VWD you have and how severe it is. Most cases of von Willebrand disease are mild, and you may need treatment only if you have surgery, tooth extraction, or an accident.
Treatments for von Willebrand disease include medicines to:
Specific treatments include:
Tips for Living with von Willebrand Disease
Its important that you try to prevent bleeding and stay healthy. You should:
Frequently Asked Questions
No, von Willebrand disease is a lifelong disorder. However, most people have a mild form that causes little or no change in their lives. Even those with type 3 von Willebrand disease can live normal, active lives once they are treated.
People like your doctor, dentist, employee health nurse, gym trainer, and sports coach should be aware of your condition. If you have a severe form of VWD (for example, type 3 VWD), consider wearing a medical ID bracelet or necklace that states you have von Willebrand disease. In case of an accident or emergency, this will be very helpful to the health care team treating you.
Since your parents, brothers and sisters, and children may also have von Willebrand disease, you should consider telling them about your diagnosis and suggesting they consider getting tested.
Anyone who is responsible for your child should know about his or her condition. For example, his or her teacher, school nurse, daycare provider, coach, or any after-school program leader should know, particularly if your child has one of the more severe forms of VWD (for example, type 3 VWD).
Most people with von Willebrand disease have a mild form of the disease, which usually does not cause bleeding that is life threatening. But any bleeding that cant be controlled can be life threatening. Some people with severe forms of von Willebrand disease need to seek emergency treatment to stop bleeding before it becomes life threatening.
No. Some people carry the genes for the disease but do not have symptoms. They still can pass the disease on to their children.
The first step is a thorough history and physical examination. If you or anyone in your family has a history of bleeding, your doctor can order a number of blood tests to find out whether you have the disease. These tests will check how quickly your blood clots and the levels of clotting factors in your blood. Your doctor can use these test results to diagnose your exact type of VWD.
For Women Only
Heavy menstrual bleeding is often the main symptom of von Willebrand disease for women. Doctors call this menorrhagia. They define it as:
Of course, heavy menstrual bleeding can also be a sign of a gynecological disorder, so its important to have a complete gynecological exam before you seek testing for VWD.
If you have VWD and heavy menstrual bleeding, there are treatments to help you. They include:
For some women who no longer want children, endometrial ablation is performed. This procedure, which destroys the lining of your uterus, has been shown to reduce menstrual blood loss in women with VWD.
If you need a hysterectomy, or surgical removal of the uterus, for another reason, it will do away with menstrual bleeding altogether and possibly improve your quality of life. However, hysterectomy carries its own risk of bleeding complications.
Pregnancy can be a challenge for women with VWD. Although blood levels of VWF and factor VIII tend to increase during pregnancy, women with VWD can have bleeding complications during delivery. They also are likely to have heavy bleeding for an extended period after delivery.
However, there are things you can do to minimize the chances of complications in pregnancy:
With these precautions, most women with VWD can have successful pregnancies.
For More Information
The NHLBI Health Information Center is a service of the National Heart, Lung, and Blood Institute (NHLBI) of the National Instititues of Health. The NHLBI Health Information Center provides information to health professionals, patients, and the public about the treatment, diagnosis and prevention of heart, lung, and blood diseases and sleep disorders. For more information, contact:
NHLBI Health Information Center
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