Von Willebrand's Disease: Treatments

There are a range of treatment choices, depending on whether the illness is mild or severe. Many people with von Willebrand disease never even need treatment.

Stimate, desmopressin acetate (DDAVP), a drug sprayed into the nose, is the treatment of choice for mild von Willebrand disease. Bleeding is usually controlled in individuals with mild von Willebrand disease by using this nasal spray to boost their own factor VIII and von Willebrand levels. DDAVP may be given to increase the amount of the von Willebrand factor long enough for surgery or dental procedures to be performed. DDAVP is a synthetic product that carries no risk of infectious disease.

For excessive bleeding, infusions of a viral inactivated factor VIII product rich in von Willebrand factor such as Humate-P, Alphanate, and Koate DVI may be required. If trauma occurs or surgery is scheduled, desmopressin acetate can be given to raise the levels of von Willebrand factor, which will decrease the tendency towards bleeding. Fresh plasma may also be used to decrease bleeding but the risk of viral infection can be high.

Children with von Willebrand disease should avoid unnecessary trauma, and those with severe disease should avoid certain contact sports. During menstruation, adolescent females may want to take extra precautions to avoid accidents. During bleeding episodes, elevate and apply cold compresses and gentle pressure to the area. During nosebleeds, apply pressure over the bridge of the nose, and have the child lean forward to prevent blood running down the back of the throat and being swallowed.

Aspirin and many of the drugs used for arthritis can aggravate bleeding because they interfere with platelet function. People who have von Willebrand's disease can take acetaminophen for pain relief because it doesn't inhibit platelet function.

The National Hemophilia Foundation's Medical and Scientific Advisory Council (MASAC) made recommendations for treatment of von Willebrand disease in November of 1999. They include:

  • Stimate, desmopressin acetate (DDAVP), a nasal spray or injection.
  • For patients who have become unresponsive to DDAVP or need surgical procedures, viral-inactivated factor VIII preparations rich in von Willebrand factor such as Alphanate, Humate-P and Koate DVI are recommended.
  • Cryoprecipitate is not recommended except in life threatening emergencies because of the risk of HIV and hepatitis infection. Despite greatly improved screening and purification for viral inactivation in blood products, cryoprecipitate can still be infectious.

Hemophilia Foundation of Southern California
6720 Melrose Avenue,
Hollywood, CA 90038
Toll free: 800.371.4123
Telephone: 323.525.0440
Fax: 323.525.0445

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