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What is VWD?

  • Von Willebrand Disease (VWD) is the most common genetic bleeding disorder worldwide, affecting both women and men equally, and is caused by low levels of Von Willebrand Factor (VWF) (quantitative defect) or VWF that doesn’t work properly (qualitative defect) .
  • VWF is a glycoprotein in the blood that is crucial for blood clotting and helping the body to stop bleeding (haemostasis). It’s primary function is to 1) help platelets stick to one another and the damaged blood vessel wall following injury, enabling a blood clot to form, and 2) to carry factor VIII (FVIII) in the blood, which is also important in the formation of blood clots.
  • When levels of VWF are low, absent or defective, it results in VWD, the symptoms of which include prolonged bleeding (takes longer for bleeding to stop) and easy bruising.
  • Nosebleeds, gum bleeds, post-surgery bleeding and heavy periods (heavy menstrual bleeding) are amongst some of the most common symptoms. More severe symptoms include internal joint bleeds and gastrointestinal (GI) bleeds.
  • There are 3 types of genetic VWD: type 1, type 2 and type 3, and symptoms can vary depending on type, VWF levels, and other genetic factors.

Type 1

  • Caused by reduced levels of VWF in the blood (quantitative defect)
  • Most common type of VWD
  • Symptoms can vary from asymptomatic/mild to severe despite it being regarded as the most mild type
  • Autosomal dominant inheritance (50% chance of passing it on from 1 affected parent to any children)

Type 2

  • Caused by VWF that does not work properly (qualitative defect)
  • There are 4 subtypes: 2A, 2B, 2M, 2N
  • Treatment options can vary depending on subtype e.g. DDAVP is contraindicated in type 2B due to the risk of thrombocytopenia
  • Autosomal dominant inheritance (50% chance of passing it on from 1 affected parent to any children)

Type 3

  • Caused by very low/no VWF (quantitative defect)
  • Rarest type of VWD
  • Symptoms likely to be severe due to very low/no VWF (e.g. joint bleeds, gastrointestinal (GI) bleeds)
  • Autosomal recessive inheritance (if both parents are VWD carriers – have a faulty VWF gene – there’s a 25% chance of any children having type 3)