The Other Bleeding Disorder
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.
Why is such a common illness such a secret?
Perhaps because the disease is usually less severe than haemophilia, it has not generated the kind of publicity, educational efforts and support that other chronic illnesses have. In fact, it is often so mild, it is not diagnosed at all. Some people live with the disease for many years before getting an accurate diagnosis. For more severely affected patients, this lack of knowledge has caused real suffering.
Fortunately, substantial progress has been made in recent years. The von Willebrand gene has been identified and research is underway to develop gene therapy. As research continues, however, the challenge continues to educate healthcare professionals and the public about this disorder. That is the best way to assure those with von Willebrand that they will receive proper diagnoses and treatment.
The Basics of von Willebrand
Von Willebrand disease is not a type of haemophilia. Like haemophilia, von Willebrand disease does make it difficult to stop bleeding when an injury occurs. But the two disorders have different causes, different symptoms an many cases, different patterns of inheritance and different treatments.
Types of von Willebrand Disease
An Equal Opportunity Disorder
“Acquiring” von Willebrand Disease
A Difficult Diagnosis
Hemostasis / Platelets
Classification of von Willebrand Disease
How von Willebrand Disease is Inherited
Unlike haemophilia, von Willebrand disease is not gender-related. So the effect is the same regardless of whether the defective von Willebrand factor (vWF) is passed down from the mother or father. It also makes no difference whether the child is a boy or girl; the disease shows up in the same way.