Von Willebrand Disease

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Von Willebrand Disease


Von WIllebrand Disease is a common inherited blood disorder that can cause heavy bleeding. People suffering with VWD either have a low level of a substance known as the Von WIllebrand Factor in their blood, or this substance doesn’t work very well.

The Von WIllebrand Factor helps the blood cells stick together i.e. clot when a person bleeds and if there isn’t enough of it or if it doesn’t work properly, it takes a longer time for the bleeding to stop.


The most common cause of VWD is an inherited abnormal gene that controls the Von WIllebrand Factor. Low levels of this protein or abnormal working of this protein makes the platelets not stick together properly and the result is interference with the clotting process that may lead to uncontrolled bleeding.

It is rare that the condition develops later in life in people who didn’t inherit an abnormal gene. This is known as Acquired VonWillebrand Disease, and it’s probably caused by another medical condition.


There are several types of VWD:

  • TYPE 1: This is the most common form of VWD with low Von Willebrand factor. In some people, levels of factor VIII are also low. The sign and symptoms of this type are usually mild.
  • TYPE 2: In this type, the Von WIllebrand Factor doesn’t function properly. This type has many subtypes and the signs & symptoms are more significant.
  • TYPE 3: This is a rare type where the Von WIllebrand Factor is absent and levels of factor VIII are low. The signs & symptom may be severe, such as bleeding into the joints and muscles.
  • ACQUIRED VWD: This type isn’t inherited and develops later in life.

The symptoms of VWD may start at any age and can range from very mild and barely noticeable to frequent and severe. The main symptoms are:

  • Easy bruising or getting large bruises
  • Frequent nosebleeds (or long-lasting)
  • Bleeding gums
  • Heavy bleeding from cuts
  • Heavy periods in women and bleeding during or after labour
  • Heavy or long-lasting bleeding after a tooth removal or a surgery

In certain cases, there is also a small risk of bleeding in the gut and painful bleeding into joints and muscles.


As the signs and symptoms are mild, the condition is difficult to diagnose. If your doctor sees any indication of a bleeding disorder, he/ she may refer you to a blood disorders specialist known as a Haematologist.

To diagnose the condition, your doctor is likely to recommend the following blood tests:

  • Von Willebrand Factor Antigen – this test determines the level of Von WIllebrand Factor in the blood by measuring a particular protein.
  • Ristocetin Cofactor Activity – this test measures how well the Von Willebrand Factor works in the clotting process. Ristocetin, an antibiotic is used in the lab testing.
  • Factor VII Clotting Activity – This test is done to determine abnormally low levels and activity of factor VIII.
  • Von WIllebrand Factor Multimers – This test evaluates the specific structure of the Von WIllebrand Factor in the blood, its protein complexes known as multimers and how its molecules break down. Doctors use this information to determine the type of VWD.

VWD is a lifelong condition with no cure, however, treatment can help prevent or stop bleeding episodes and it depends on three things:

  • The type and severity of the condition
  • Previous response to therapy, and
  • Other medications and conditions.

To increase the Von Willebrand Factor, strengthen blood clots, or control heavy menstrual bleeding, your doctor may suggest one or more of the following treatments:

  • DESMOPRESSIN – this medication is available as an injection (DDAVP) or a nasal spray (Stimate). It controls the bleeding by stimulating the body to release more Von Willebrand Factor already stored in the lining of the blood vessels. This treatment is usually effective in people with Type 1 and some subtypes of Type 2 disease.
  • REPLACEMENT THERAPIES – the therapies include infusions of prepared doses of concentrated blood-clotting factors containing Von WIllebrand Factor and factor VIII.
  • CONTRACEPTIVES – for women, these help in controlling heavy bleeding during menstrual periods.

If your condition is mild, your doctor, might recommend treatment only when you are undergoing surgery or dental work or when you’ve experienced trauma.