Treatment

Even though von Willebrand disease is a lifelong condition with no cure, treatment can help prevent or stop bleeding episodes. Your treatment depends on:

  • The type and severity of your condition
  • How you've responded to previous therapy
  • Your other medications and conditions

Your doctor may suggest one or more of the following treatments to increase your von Willebrand factor, strengthen blood clots or, in women, control heavy menstrual bleeding:

  • Desmopressin. This medication is available as an injection (DDAVP) or nasal spray (Stimate). It's a synthetic hormone, similar to the natural hormone vasopressin. It controls bleeding by stimulating your body to release more von Willebrand factor already stored in the lining of your blood vessels. DDAVP is usually effective in people with type 1 and some subtypes of type 2 disease.

    Many doctors consider DDAVP the first treatment to use in the management of von Willebrand disease. Some women use the nasal spray (Stimate) at the beginning of their menstrual periods to control excessive bleeding. It can also be effective when used before a minor surgical procedure.

  • Replacement therapies. These include infusions of prepared doses of concentrated blood-clotting factors containing von Willebrand factor and factor VIII (Humate-P, others). These therapies can be useful in all disease types. Your doctor may recommend them if DDAVP isn't an option for you or was ineffective.

    Another replacement therapy approved by the FDA for treating adults 18 and older is a genetically engineered (recombinant) von Willebrand factor product (Vonvendi). Because recombinant factor is made without plasma, it may reduce the risk of a viral infection or allergic reaction.

  • Contraceptives. For women, these can be useful for controlling heavy bleeding during menstrual periods. The estrogen hormones present in birth control pills can boost levels of von Willebrand factor and factor VIII activity. This effect is likely available with birth control patches, though further study is needed to confirm it.
  • Clot-stabilizing medications. These anti-fibrinolytic medications — such as aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda, others) — can help stop bleeding by slowing the breakdown of blood clots. Doctors often prescribe these drugs before or after a surgical procedure or tooth extraction.
  • Drugs applied to cuts. A fibrin sealant (Tisseel VHSD) placed directly on a cut helps curtail bleeding. These substances are applied like a glue using syringes.

If your condition is mild, your doctor might recommend treatment only when you're undergoing surgery or dental work or when you've experienced trauma (in an automobile accident, for example).

Dec. 03, 2016
References
  1. The diagnosis, evaluation, and management of von Willebrand disease. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. Accessed Aug. 5, 2016.
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  3. Berntorp E. Von Willebrand disease. Pediatric Blood Cancer. 2013;60:s34.
  4. Rick ME. Clinical presentation and diagnosis of von Willebrand disease. http://www.uptodate.com/home. Accessed Aug. 5, 2016.
  5. Facts about von Willebrand disease. U.S. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/vwd/facts.html. Accessed Aug. 5, 2016.
  6. Rick ME. Treatment of von Willebrand disease. http://www.uptodate.com/home. Accessed Aug. 5, 2016.
  7. AskMayoExpert. Von Willebrand disease. Rochester, MN: Mayo Foundation for Medical Education and Research; 2015.
  8. Patient education: von Willebrand disease (The Basics). http://www.uptodate.com/home. Accessed Sept. 22, 2016.
  9. Hoots WK, et al. Clinical manifestations and diagnosis of hemophilia. http://www.uptodate.com/home. Accessed Sept. 23, 2016.
  10. How is von Willebrand disease treated? National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/health-topics/topics/vwd/treatment#. Accessed Nov. 30, 2016.
  11. Von Willebrand disease. National Hemophilia Foundation. https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease. Accessed Nov. 30, 2016.
  12. 12. Leebeek FWG, et al. Von Willebrand's disease. New England Journal of Medicine. 2016;375:2067.