Von Willebrand's Disease

Topic Overview

What is von Willebrand's disease?

Von Willebrand's disease is a bleeding disorder. When you have this disease, it takes longer for your blood to form clots and you bleed for a longer time than other people.

Normally, when a person begins to bleed, small blood cells called platelets go to the site of the bleeding and clump together to help stop the bleeding. If you have von Willebrand's disease, your blood doesn't clot well because you don't have a certain protein in your blood or you have low levels of it. This protein is called the von Willebrand factor. It helps your blood to clot.

There are three major types of the disease.

• In type 1, you are missing some von Willebrand factor. This can cause mild to moderate bleeding episodes, depending on how much von Willebrand factor is missing. About 3 out of 4 people who have von Willebrand's disease have type 1.¹ You may not know you have the disease and may not need treatment.
• In type 2, you have the von Willebrand factor, but it doesn't work as it should. This usually causes mild bleeding episodes, but it can cause moderate bleeding episodes. Type 2 is further divided into classes.
• In type 3, you don't have the von Willebrand factor or you have a very small amount. This type can lead to serious bleeding episodes, but it is very rare. People who have type 3 disease can develop anemia and can have dangerous bleeding after an accident or during surgery.

Von Willebrand's disease can stay the same or get better or worse as you get older.

Tell all your doctors and other health professionals, such as your dentist, that you have this disease. Doctors need to know about it before you have any procedures, because you are at risk for dangerous bleeding.

What causes von Willebrand's disease?

Von Willebrand's disease usually is passed down through families (inherited). It is the most common bleeding disorder present at birth (congenital), but most cases are mild. Men and women are equally likely to have von Willebrand's disease.

What are the symptoms?

Bleeding a lot is the main symptom of von Willebrand's disease. How severe the bleeding is will be different for each person.

Symptoms of mild von Willebrand's disease include:

• Frequent nosebleeds.
• Some bleeding from the gums.
• Heavy menstrual periods in women (menorrhagia).
• Bruises that appear for no reason.
• Heavy bleeding after injury or surgery.

Symptoms of more severe von Willebrand's disease include those listed above and:

• Blood in the urine.
• Bruising easily.
• Black, tarry, or bloody stools.
• Bleeding into the joints, which causes stiffness, pain, and swelling. This symptom is rare.

How is von Willebrand's disease diagnosed?

Von Willebrand's disease can be hard to diagnose. Some people with mild von Willebrand's disease bleed about the same amount as other people do. You may not notice any symptoms until you bleed a lot after an injury, dental procedure, or surgery.

Your doctor will ask you how often and how much you bleed. If your doctor thinks you may have a problem with clotting, he or she will suggest:

• Blood tests that measure bleeding time, von Willebrand factor activity levels, or von Willebrand factor antigen.
• Genetic testing that shows if you have a defect in your von Willebrand factor.

How is it treated?

Treatment depends on the type of von Willebrand's disease you have, how much you bleed, and your risk for heavy bleeding.

If you have a mild type of von Willebrand's disease, you may need to:

• Avoid nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen (Advil or Motrin).
• Take medicine to prevent heavy bleeding if you have an injury, are going to have surgery, or are about to give birth.
• Avoid blood thinners, such as warfarin and heparin, and antiplatelet medicines like clopidogrel.

If you have severe von Willebrand's disease, you will need to avoid NSAIDs and blood thinners. Treatment may include:

• Desmopressin medicine, which helps control bleeding episodes.
• Replacement therapy, which replaces the clotting factor in your blood.
• Antifibrinolytic medicines, to help stop blood clots from breaking down.
• Hormone therapy for women, to control heavy menstrual periods.
• Fibrin glue or thrombin powder, which you place on a wound to help control bleeding.

If you have severe von Willebrand's disease, you most likely will need to take extra care to treat and prevent bleeding episodes. Try to stay at a healthy weight and keep active. This helps prevent bleeding into muscles and joints. Avoid sports or activities where injury and bleeding are likely, such as football and hockey.

Frequently Asked Questions

Learning about von Willebrand's disease:	What is von Willebrand's disease? What causes von Willebrand's disease? What are the symptoms?
Being diagnosed:	How is von Willebrand's disease diagnosed?
Getting treatment:	How is von Willebrand's disease treated? Are there steps I can take at home to treat von Willebrand's disease?

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Author:	Robin Parks, MS	Last Updated: November 24, 2008
Medical Review:	Anne C. Poinier, MD - Internal Medicine Joseph O'Donnell, MD - Hematology/Oncology
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