von Willebrand Disease

About Bleeding Disorders

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Understanding von Willebrand Disease (VWD)

von Willebrand disease (VWD) stands as the most prevalent form of bleeding disorder worldwide. Individuals with VWD encounter difficulties with a crucial protein within their blood known as von Willebrand factor (VWF), which plays a pivotal role in regulating bleeding. When a blood vessel sustains injury, leading to bleeding, VWF aids in the aggregation of blood platelets, facilitating the formation of a clot to staunch the bleeding. However, those with VWD either lack a sufficient amount of VWF or possess VWF that fails to function properly. Consequently, their blood takes a longer time to clot, prolonging the duration of bleeding episodes.

Research indicates that up to 1% of the global population suffers from VWD. However, due to the mildness of symptoms experienced by many individuals, a significant portion of those affected may remain undiagnosed. Studies suggest that as many as 9 out of 10 people with VWD have yet to receive a formal diagnosis. Efforts aimed at raising awareness and improving access to diagnostic testing are crucial in ensuring timely detection and appropriate management of VWD. By addressing the underdiagnosis of VWD, individuals affected by the condition can receive the necessary support and interventions to effectively manage their symptoms and enhance their quality of life.

Types of VWD

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. Symptoms are usually very mild. Still, it is possible for someone with Type 1 VWD to have serious bleeding.
  • Type 2 VWD involves a defect in the VWF structure. The VWF protein does not work properly, causing lower than normal VWF activity. There are different Type 2 VWD defects. Symptoms are usually moderate.
  • Type 3 VWD is usually the most serious form. People with Type 3 VWD have very little or no VWF. Symptoms are more severe. People with Type 3 VWD can have bled into muscles and joints, sometimes without injury.
Von Willebrand Disease (VWD) is the most common type of inherited bleeding disorder characterized by a deficiency or dysfunction of a protein called von Willebrand factor (VWF) in the blood. VWF plays a crucial role in blood clotting by facilitating the adhesion of platelets to damaged blood vessel walls and promoting clot formation to stop bleeding.
Diagnosis of von Willebrand Disease typically involves a combination of medical history assessment, physical examination, laboratory tests to measure VWF levels and activity, platelet function tests, and genetic testing to identify specific gene mutations associated with the disorder.
Yes, von Willebrand Disease is treatable, and management strategies aim to prevent or reduce bleeding episodes and improve quality of life. Treatment may include desmopressin (DDAVP) therapy to increase VWF release, replacement therapy with VWF-containing products, and medications to promote clot formation.