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von Willebrand Disease (vWD)

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Treatment of von Willebrand's Disease

Patients with vWD normally require no regular treatment, although they are always at increased risk for bleeding. For more serious bleeding, medications that can raise the von Willebrand factor (VWF) level and thereby limit bleeding are available. A paediatric or adult haematologist needs to be consulted.

The goal of therapy is to correct the defect in platelet adhesiveness and the defect in blood coagulation. These are achieved by raising the effective vWF level and factor VIII level respectively.

The drug desmopressin (1-deamine-8-D-arginine vasopressin [DDAVP]) has become a mainstay of therapy for most patients with mild von Willebrand disease. It is a synthetic analogue of antidiuretic hormone. It can be administered intravenously (into a vein), intranasally (through the nostrils), or subcutaneously (just under the skin). It is however better to do prior testing to see how the patient responds before instituting therapy.

Plasma products are used for patients with von Willebrand disease who do not respond to desmopressin, and for individuals with the rare types 2B or 3 von Willebrand disease. The product contains plasma-derived factor VIII (FVIII) concentrates that contain von Willebrand factor.

The adjunctive use of antifibrinolytic agents, such as -aminocaproic acid or tranexamic acid are helpful after DDAVP therapy for bleeds. Medications are also needed to control heavy menstrual bleeding in women.

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