Overview of von Willebrand Disease Treatment

von Willebrand Disease (vWD) is a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein. VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which helps form a platelet plug during the clotting process. Treatment strategies for vWD focus on increasing the level of functional VWF in the blood and controlling bleeding episodes.

Replacement Therapy

The most commonly used treatment for vWD, particularly in severe cases, involves replacement therapy. Synthetic or plasma-derived von Willebrand factor concentrates are administered to replace the deficient or defective VWF. This can be done regularly as preventive therapy or on-demand in response to bleeding episodes.

Medications

Several medications are used to manage vWD. Desmopressin (DDAVP), a synthetic hormone, stimulates the release of VWF stored in the body and is used primarily for patients with Type 1 vWD. Antifibrinolytic drugs like tranexamic acid can also be prescribed to help stabilize clots and prevent their breakdown. Hormonal therapy, such as birth control pills, can be effective in reducing menstrual bleeding in women with vWD.

My uncle has von Willebrand Disease and it’s not been easy for him. For his treatment, he gets injections of a medicine that helps his blood clot better. This medicine is some type of concentrated clotting factor. He has these treatment sessions every couple of months or whenever he has a bad bleeding episode. Besides that, they’ve got him on some pills that he takes to help prevent bleeds. It’s a whole routine, but it helps him manage his condition and live a more normal life.