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What is von Willebrand Disease (VWD)

Von Willebrand Disease (VWD) is a type of bleeding disorder where the von Willebrand factor (VWF), which helps control bleeding, is either at a very low level or does not work properly.

There are three main types of VWD. Within each type, the disorder can be mild, moderate or severe. Bleeding symptoms can be quite variable within each type depending in part on the VWF activity. It is important to know which type of VWD a person has, because treatment is different for each type.

  • Type 1 VWD is the most common form. People with Type 1 VWD have lower than normal levels of VWF.
  • Type 2 VWD involves a defect in the VWF structure. The VWF protein does not work properly, causing lower than normal VWF activity.
  • Type 3 VWD means that individuals affected by this condition have very little or no VWF, which leads to severe symptoms.

Despite VWD being a more common bleeding disorder, it is still difficult for some patients to get proper diagnosis due to the lack of laboratory equipment and expertise. Also it should be noted that people affected with VWD can either have a mild or severe form. People with mild forms of VWD may need only some specialised care during surgery, dental care or child birth, while people with severe VWD will need to have access to more regular treatment. Despite this fact, it is not uncommon in some European countries for people with mild VWD to not want to be officially diagnosed in order not to increase their health insurance premiums. One of the most common symptoms in VWD in women is heavy and prolonged periods, which besides causing iron deficiency may also be life-impairing and is comparable to a haemophiliac bleed.

Once a person with VWD is diagnosed, they have access to several types of treatments such as:

  • Desmopressin
  • Factor concentrates
  • Tranexamic acid
  • Fibrin glue
  • Hormone treatments

The types of treatment will depend on each individual.
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vwdHow do you get VWD?

VWD is usually inherited. It is passed down through the genes from either parent to a child of either sex. However, sometimes VWD occurs due to a spontaneous change in the VWD gene before the baby is born. The figure below shows the inheritance of VWD.

VWD Types 1 and 2 are usually inherited in what is known as a ‘dominant’ pattern. This means that a parent who has VWD has a one in two (50 per cent) chance of passing a VWD gene on to each of his or her children.

Type 3 VWD, however, is usually inherited in a ‘recessive’ pattern. This type occurs when the child inherits the gene from both parents. Even if both parents have mild symptoms or no symptoms at all, their child may be severely affected.
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Resources

You can find more information about VWD via the following websites and documents:

Adapted from the World Federation of Hemophilia website

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Image courtesy of EHC | EHC - European Haemophilia Consortium