Medical Care
Evidence-based guidelines for the diagnosis and management of von Willebrand disease (VWD) have been established. [18, 19]
Minor bleeding problems in patients with von Willebrand disease, such as bruising or a brief nosebleed, may not require specific treatment. For more serious bleeding, medications that can raise the von Willebrand factor (VWF) level and, thereby, limit bleeding are available. The goal of therapy is to correct the defect in platelet adhesiveness (by raising the level of effective von Willebrand factor) and the defect in blood coagulation (by raising the factor VIII [FVIII] level). Desmopressin (1-deamine-8-D-arginine vasopressin [DDAVP]) has become a mainstay of therapy for most patients with mild von Willebrand disease. At appropriate doses, DDAVP causes a 2-fold to 5-fold increase in plasma von Willebrand factor and FVIII concentrations in individuals who are healthy and patients who are responsive. DDAVP can be used to treat bleeding complications or to prepare patients with von Willebrand disease for surgery.
In general, a patient's responsiveness to DDAVP prior to its use for these purposes can be determined. Once determined, such responsiveness is generally consistent in patients over time and within families. In patients with serious bleeding, prompt treatment is important in order to decrease the possibility of complications.
Remember that in type 2B von Willebrand disease, DDAVP may cause a paradoxical drop in the platelet count and should not be used in a therapeutic setting without prior testing to see how the patient responds. Some other forms of type 2 von Willebrand disease may not be responsive to DDAVP either.
Consultations
Consult a pediatric or adult hematologist.
Activity
No evidence suggests that extensive activity restrictions are necessary for most patients with mild type 1 von Willebrand disease. Patients with more severe forms of von Willebrand disease should follow guidelines developed for patients with severe hemophilia, including considering a prophylactic regimen in patients with severe type 3 who are active or have repeated bleeding.
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Structure and domains of von Willebrand factor.
