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von Willebrand disease

Description

von Willebrand disease (VWD) is a disorder that is caused by a problem with one of the proteins in the blood (von Willebrand factor or vWF). People with VWD either don’t have enough vWF or what they have doesn’t work properly.

vWF plays 2 important roles in blood clotting. It makes platelets stick to the wall of an injured blood vessel and to each other. Without it, a platelet plug cannot be made. vWF is also a called a carrier protein because it carries one of the clotting factors, factor VIII (8), with it. This means that it helps make sure there is enough factor VIII (8) in the blood and that it gets to where it’s needed. Without vWF, factor VIII (8) will be broken down in the bloodstream and there may not be enough of it to stop bleeding.

There are several different classifications of VWD.

Classifications of VWD
Type 1
  • Most common and mildest form
  • A person with Type 1 VWD has lower-than-normal levels of vWF and may also have low levels of factor VIII, which is another type of blood-clotting protein
Type 2
  • Body makes normal amounts of vWF but it does not work the way it should
  • Type 2 VWD is further broken down into 4 subtypes—2A, 2B, 2M, and 2N—depending on the specific problem with the person’s vWF.
  • Each type is treated differently
Subtype 2A
  • vWF levels are reduced, as is the ability of platelets to clump together
Subtype 2B
  • Although the factor itself is defective, the ability of platelets to clump together is actually increased
Subtype 2M
  • vWF levels are decreased and the interaction of vWF with platelets or connecting tissue is reduced
Subtype 2N
  • Binding of vWF to factor VIII (8) is markedly decreased
Type 3
  • Most severe form of VWD; a person has very little or no vWF and low levels of factor VIII (8)

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Diagnosis

Some people may have VWD for years and never know it. That’s because they don’t have symptoms or their symptoms are so mild they’re easily overlooked.

The signs and symptoms of VWD are similar in both men and women. Bleeding in persons with VWD usually involves the mucous membranes of the body. Many people may not be diagnosed because their symptoms are subtle, ignored, or believed to be caused by something else. Some of these symptoms include frequent nosebleed, easy bruising, heavy menstrual bleeding (called menorrhagia) and prolonged bleeding after surgery, childbirth or dental work.

A combination of blood tests is needed to diagnose VWD. In fact, these tests may need to be repeated several times before a definitive diagnosis can be made, because the amount of clotting factor in the blood can vary over time (for example, during pregnancy or if the person has an infection), which can affect the test results.

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Treatment

Some treatments are given by injection, nasal spray, or pills. The treatment for VWD depends on the type and severity of the bleeding disorder. For minor bleeds, treatment might not be needed.

The most commonly used treatments are:

  • Desmopressin acetate (DDAVP) injection: this medicine is injected into a vein or just under the skin to treat milder forms of VWD (mainly Type 1). DDAVP is the synthetic form of a naturally occurring hormone. It works by making the body release more vWF into the blood from storage sites along the blood vessel walls. This helps the body form a stable, firm clot and increase the level of factor VIII (8) in the blood
  • Desmopressin acetate (DDAVP) nasal spray: this spray is the nasal form of the injection. This high-dose nasal spray is used to treat milder forms of VWD. It works by boosting the levels of vWF and factor VIII (8) in the blood
  • Factor replacement therapy: the plasma-derived clotting factor products used to treat VWD are rich in vWF and factor VIII (8). They are used to treat more severe forms of VWD or milder forms of VWD in people who do not respond well to DDAVP given either by nasal spray or injection. These clotting factor products are injected into a vein in the arm to replace the missing factor in the blood
  • Antifibrinolytic drugs: these drugs help slow or prevent the breakdown of blood clots. They are either injected or taken orally
  • Birth control pills (called oral contraceptives): the hormones in birth control pills can increase the levels of vWF and factor VIII (8) in the blood and reduce menstrual blood loss. A health care provider can prescribe these pills for heavy menstrual bleeding (called menorrhagia)

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NEW! von Willebrand Disease: Piecing together the VWD Puzzle and Youth Activity Book

NHF is thrilled to announce the launch of an educational booklet called “von Willebrand Disease; Piecing together the VWD Puzzle” and a Youth Activity Book to help individuals and families as they learn more about living with VWD. 

von Willebrand Disease: Piecing together the VWD Puzzle

VWD Youth Activity Book