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von Willebrand Disease

von Willebrand Disease is the most common of all inherited bleeding disorders. It is caused by two problems: a deficiency in Factor VIII and von Willebrand factor, another clotting protein.

What is von Willebrand disease?

von Willebrand Factor is a glue-like "adhesive" protein that carries and protects Factor VIII in the bloodstream. It also helps platelets stick to the blood vessel walls at the injury site. When there is not enough von Willebrand factor or when von Willebrand factor does not work correctly, a person has von Willebrand Disease.

This disease is characterized by prolonged bleeding after surgery or trauma. In severe cases, the prolonged bleeding may occur without a known injury. Von Willebrand Disease is diagnosed with a detailed patient and family history and blood tests. These tests are sensitive to monthly hormonal changes, stress, medications and exercise; therefore, sometimes the tests must be repeated for accuracy.

Treatment of von Willebrand disease

There are effective treatments for bleeding related to von Willebrand Disease. The type of treatment depends on the individual’s type and severity of von Willebrand disease and the specific bleeding problems. A variety of medications can be given to stop an active bleed or to prevent bleeding from happening during surgery or other invasive procedures.  For more severe von Willebrand’s Disease, there are preparations of von Willebrand factor that are given intravenously. Milder forms of the disease often respond to DDAVP (desmopressin acetate) which can be given either intravenously or as a nose spray. Birth control pills are usually the best treatment of choice for bleeding related to heavy menstrual periods. An oral medication called aminocaproic acid is often used in conjunction with the above medications to help stop or prevent bleeding. This medication is especially helpful in controlling bleeding in the mouth, often associated with dental work.

VCU Health System | Central Virginia Center for Coagulation Disorders
P.O. Box 980461 | Richmond, VA | 23298-0461
phone: (804) 827-3306 | toll free: (866) 288-2516 | fax: (804) 692-0291

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last updated: 02/17/2014
Central Virginia Center for Coagulation Disorders