"Top of the page Von Willebrand's Disease Topic OverviewWhat 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, so 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 by helping the platelets stick together. The disease is mild in most people. It can stay the same or get better or worse as you get older. What are the types of von Willebrand's disease? There are three major types of the disease. They range from mild to severe. In type 1, you are missing some von Willebrand factor. This can cause mild to moderate bleeding episodes. About 3 out of 4 people who have von Willebrand's disease have type 1.footnote 1 You may not know you have the disease, and you 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.
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 injury or during surgery.
What causes von Willebrand's disease? Von Willebrand's disease usually is passed down through families (inherited). If you have the disease, your doctor may suggest that your family members get tested for it too. It's possible to get acquired von Willebrand's disease later in life. This rare form of the disease isn't inherited. Instead, it seems to be caused by certain diseases or certain medicines. 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.
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.
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 who have 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 may suggest: Blood tests that measure von Willebrand factor activity levels or von Willebrand factor antigen.
Genetic testing that shows if you have a defect in your von Willebrand factor and what type of von Willebrand's disease you have.
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, ibuprofen (Advil or Motrin, for example), and naproxen (Aleve).
Take medicine to prevent heavy bleeding if you have an injury, are going to have surgery, or are about to give birth.
Avoid medicines (called blood thinners) that prevent blood clots.
If you have severe von Willebrand's disease, your treatment may include: Desmopressin medicine (DDAVP, Stimate) which helps your body release more of the von Willebrand clotting factor into your blood.
Replacement therapy, which replaces the clotting factor in your blood.
Antifibrinolytic medicines, to help stop blood clots from breaking down.
Birth control pills, or an intrauterine device (IUD) that contains hormones, to help 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. Avoid NSAIDs and blood thinners. 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. 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 may be at risk for dangerous bleeding. Most large hospitals have a "bleeding disorders" resource center. Learn the phone number to the center closest to you, and carry it with you.Related InformationBruises and Blood Spots Under the Skin Abnormal Vaginal Bleeding Hemophilia NosebleedsReferencesCitations National Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease. (NIH Publication No. 08â€“5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd.Other Works Consulted American College of Obstetricians and Gynecologists (2009). Von Willebrand disease in women. ACOG Committee Opinion No. 451. Obstetrics and Gynecology, 114(6): 1439â€“1443. Friedman KD, Rodgers GM (2009). Inherited coagulation disorders. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 12th ed., vol. 2, pp. 1379â€“1424. Philadelphia: Lippincott Williams and Wilkins. James AH, et al. (2009). Von Willebrand disease and other bleeding disorders in women: Consensus on diagnosis and management from an international expert panel. American Journal of Obstetrics and Gynecology, 201(12): e1â€“e8. National Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease. (NIH Publication No. 08â€“5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd. Nichols WL (2016). Von Willebrand disease and hemorrhagic abnormalities of platelet and vascular function. In L Goldman, A Shafer, eds., Goldman-Cecil Medicine, 24th ed., vol. 1, pp. 1167â€“1172. Philadelphia: Saunders.CreditsByHealthwise StaffPrimary Medical Reviewer E. Gregory Thompson, MD - Internal Medicine Adam Husney, MD - Family Medicine Martin J. Gabica, MD - Family Medicine Specialist Medical Reviewer Brian Leber, MDCM, FRCPC - Hematology Current as ofOctober 9, 2017 Top of PageNext Section:
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E. Gregory Thompson, MD - Internal Medicine & Adam Husney, MD - Family Medicine & Martin J. Gabica, MD - Family Medicine & Brian Leber, MDCM, FRCPC - HematologyNational Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease. (NIH Publication No. 08-5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd."@en