It may surprise some in the Haemophilia community to learn than von Willebrand’s disease is believed to be the most common inherited bleeding disorder in humans, estimated to occur in up to 3% of the population. It was first described in 1926 by Erik von Willebrand, a Finnish physician, who reported a new type of bleeding disorder among the inhabitants of some islands between Sweden and Finland. Von Willebrand observed that these patients had an abnormality in their blood platelet function. Years later it was found that some people with von Willebrand’s disease also have a low level of factor VIII.
von Willebrand Disease (vWD) is the most common inherited bleeding disorder. It occurs when the body has too little of a protein called von Willebrand factor (VWF) or when the protein does not work properly.
VWF plays an important role in blood clotting because it helps platelets stick to damaged blood vessels and carries clotting factor VIII in the bloodstream.
When VWF is missing or dysfunctional, the body cannot form clots effectively.
There are three main types of vWD:
Type 1
The most common and mildest form. People have lower-than-normal levels of VWF.
Type 2
VWF is present but does not function properly. This type has several subtypes depending on how the protein is affected.
Type 3
The rarest and most severe form. People have very little or no VWF, leading to significant bleeding problems.
Many people with vWD have mild symptoms and may not realize they have the condition.
Common symptoms include:
Frequent nosebleeds
Easy bruising
Prolonged bleeding from cuts
Bleeding after surgery or dental procedures
Heavy menstrual bleeding in women
In severe cases, bleeding can also occur in joints and muscles.
Diagnosing vWD can be challenging because symptoms may be mild and laboratory results may vary.
Diagnosis usually includes:
Medical and family history review
Bleeding assessment tools
Blood tests measuring von Willebrand factor levels and clotting activity
Treatment depends on the type and severity of vWD.
Common treatment options include:
Desmopressin (DDAVP) – a medication that helps the body release stored VWF
VWF replacement therapy – infusion of VWF concentrates
Antifibrinolytic medicines such as tranexamic acid to stabilize clots
These treatments help control bleeding and allow patients to safely undergo procedures such as surgery or dental work.
Living with vWD
Most people with vWD live normal lives with appropriate care. Education, access to treatment, and support from specialized haemophilia treatment centres are key to managing the condition effectively.