Von Willebrand Disorder

Von Willebrand disorder

Von Willebrand disorder (VWD), also known as von Willebrand disease, is the most common inherited bleeding disorder. It is caused by a deficiency of von Willebrand factor (VWF), a type of protein that helps your blood clot. In WVD, either the level of VWF is low or the VWF does not work very well, or both.

Normally, when a blood vessel is injured, you start to bleed. Small blood cells called platelets clump together to plug the hole in the blood vessel and stop the bleeding. VWF acts like glue to help the platelets stick together and form a blood clot for most people. When you have VWD, the glue does not stick the platelets together, so clots to not form as easily.

VWF is also responsible for carrying the blood-clotting factor called factor VIII (eight), another essential protein that helps your blood clot. People with VWD also have low factor VIII levels and may share some bleeding symptoms with people who have haemophilia.

Symptoms include easy bruising, frequent or long-lasting nosebleeds and gum bleeds. Girls and women may experience very heavy menstrual bleeding.

VWD affects men and women equally and is caused by a mutation (change) on the gene responsible for producing VWF. In most cases this mutation is passed from parent to child, but the way this happens depends on the type of VWD. The type of VWD people are born with mostly depends on whether they inherit copies of this faulty gene from one or both parents.

Treatment depends on the severity and subtype of the condition and includes intravenous factor replacement therapy, Desmopressin (DDAVP), a synthetic drug modelled on a hormone found normally in the body, and tranexamic acid, an anti-fibrinolytic agent.

More information from www.haemophilia.org.uk.