Therapeutic Management
The primary therapy for hemophilia is replacement of the missing clotting factor. The products available are factor VIII concentrate from pooled plasma or a genetically engineered recombinant, to be reconstituted with sterile water immediately before use, and DDAVP (1-deamino-8-D-arginine vasopressin), a synthetic form of vasopressin that increases plasma factor VIII and vWF levels and is the treatment of choice in mild hemophilia and vWD if the child shows an appropriate response. DDAVP is not effective in the treatment of severe hemophilia A, severe vWD, or any form of hemophilia B. Vigorous therapy is instituted to prevent chronic crippling effects from joint bleeding.