Von Willebrand Disease

Also known as: VMD

What is Von Willebrand disease?

VMD is a bleeding disorder. Children with Von Willebrand disease (VWD) have a defect or are missing a blood clotting protein called the Von Willebrand factor. This blood component acts like glue to help blood platelets stick together to form a blood clot at the site of an injured blood vessel. This stops the bleeding.

What causes Von Willebrand disease?

VWD is caused by either a decreased quality, abnormal function or absence (types 1, 2 and 3) of a protein that binds platelets (the blood cells that form a plug at the site of a damaged blood vessel) to the damaged part of a blood vessel, and also enhances the role of a blood clotting factor (factor V111); part of the bloods’ mechanism to stop bleeding. It affects boys and girls equally and is the most common inherited bleeding disorder in the United States. Occasionally the disease can be acquired as a complication from other medical conditions.

What are the symptoms of Von Willebrand disease?

Some children have no symptoms, others have excessive bleeding; bruising, gum and nose bleeds, heavy bleeding after accidents or surgical procedures and in girls heavy periods or bleeding after childbirth. 

What are Von Willebrand disease care options?

Children may not need treatment unless severe bleeding occurs. If needed, treatment is usually undertaken with a hormone called DDAVP which stimulates blood clotting. As DDAVP doesn't last more than 24 hours it's usually only given for severe bleeding, when bleeding is anticipated (e.g. surgery) or following trauma.

Other medications may be used to help stabilize blood clots; clotting factors may be administered and blood transfusions given in the face of severe bleeding. 

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: October 21, 2019 01:01 PM

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