Treatment for VWD depends on the diagnosis and severity. The mainstay of treatment is DDAVP (desmopressin acetate), the synthetic version of a natural hormone vasopressin,. It stimulates the release of VWF from cells, which also increases FVIII.DDAVP comes in two forms: injectable and nasal spray. Because DDAVP is an antidiuretic, causing the body to retain water, fluid restrictions are important so patients don’t develop hyponatremia, reduced sodium in the bloodstream.
There are a few clotting factor concentrates that are rich in VWF, and are recommended for patients with VWD. These therapies are given by intravenous infusion. In December 2015, the US Food and Drug Administration (FDA) approved Baxalta’s Vonvendi®, the first recombinant VWF product. Unlike other products, it contains VWF only, not VWF and factor VIII. It is approved to treat on-demand and for control of bleeding in adults 18 and older.
Aminocaproic acid and tranexamic acid are antifibrinolytics agents that prevent the breakdown of blood clots. These drugs are often recommended before dental procedures, to treat nose and mouth bleeds, and for menorrhagia. Antifibrinolytics are taken orally, as a tablet or liquid. MASAC recommends that a dose of clotting factor be taken first to form a clot, then aminocaproic acid, to preserve the clot and keep it from being prematurely broken down.