von Willebrand Disease Treatment

Treatment for von Willebrand disease (VWD) is based on your type of VWD and the severity of your symptoms. The aim of therapy is to correct the clotting problem. This is usually accomplished by raising the levels of von Willebrand factor (VWF) and another protein called factor VIII in the bloodstream. Most cases of VWD are mild, so you might need treatment only if you have surgery, tooth extraction, or an accident.1

If you need treatment for VWD, there are several options available:

  • Desmopressin acetate injections1,2

    Desmopressin is a synthetic copy of a natural hormone called vasopressin (VAY-so-press-in), which helps the body release stored VWF into the bloodstream and increases levels of factor VIII.

    It is usually given to patients as an infusion (injection into a vein).

  • Desmopressin acetate nasal spray (high concentration)

    Desmopressin may also be prescribed as a high-concentrate nasal spray.2 Because it is sprayed into the nose (not injected), it can be easy to use. (Note: Only the high-concentration type is used to treat people with VWD.3,4)

  • Factor replacement therapies1,2,5

    Factor replacement therapies are medicines that replace missing VWF. There are 2 kinds of replacement therapy: factor concentrates containing VWF and cryoprecipitate. Both of these therapies are made from plasma and are given by infusion. VWF concentrates are strongly preferred for safety reasons because certain viruses are inactivated during the manufacturing process. This is not the case with cryoprecipitate. According to the NHF MASAC recommendations, cryoprecipitate should not be used except in life- or limb-threatening situations when factor concentrates containing VWF are not available.

  • Local clotting agents

    Local clotting agents (antifibrinolytics) help protect clots and hold them in place, and are often used in combination with other medicines. Clotting agents can be used to stop bleeding in the mouth, and genitourinary and gastrointestinal tracts. They can also be used to help control heavy bleeding during the menstrual period. Clotting agents can be given intravenously or by mouth (via tablet, capsule, syrup, or mouthwash).2

  • Birth control pills

    Birth control pills (oral contraceptives) can increase VWF levels, although the way they do this is not well understood. These medicines can be helpful for women with heavy menstrual bleeding.2

References

  1. What is von Willebrand disease? National Institutes of Health; National Heart, Lung, and Blood Institute website. http://www.nhlbi.nih.gov/health/health-topics/topics/vwd. Accessed October 21, 2014.
  2. Management of von Willebrand disease. In: The Diagnosis, Evaluation and Management of von Willebrand Disease. Washington, DC: National Institutes of Health; National Heart, Lung, and Blood Institute; 2007. NIH Publication No. 08-5832. https://www.nhlbi.nih.gov/health-pro/guidelines/current/von-willebrand-guidelines/full-report/4-management-of-vwd.htm. Accessed October 23, 2014.
  3. FDA Orange Book [database online]. Silver Spring, MD: Food and Drug Administration. http://www.accessdata.fda.gov/scripts/cder/ob/docs/tempai.cfm. Updated December 16, 2014. Accessed December 17, 2014.
  4. Drug Encyclopedia [database online]. Kaiser Permanente website. https://healthy.kaiserpermanente.org/health/care/consumer/health-wellness/drugs-and-natural-medicines/drug-encyclopedia. Accessed December 17, 2014.
  5. MASAC recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders (revised September 2014.) MASAC Document 230. National Hemophilia Foundation website. http://www.hemophilia.org/sites/default/files/document/files/230Text2014-09.pdf.
Coagulation