Von Willebrand disease is an inherited condition in which one of the clotting factors needed in order for the blood to clot normally — von Willebrand factor — is either deficient or abnormal. This clotting problem causes abnormal bleeding.

Von Willebrand disease is common, occurring in at least one in 1000 of the population, possibly much higher given that there might be a significant number of people who remain undiagnosed. This is because many people with this condition have no symptoms and may only become aware of the problem after surgery or a major accident.

Symptoms of von Willebrand disease

Von Willebrand disease causes problems with bleeding, including:

  • frequent nosebleeds and gum bleeds;
  • easy bruising;
  • heavy periods;
  • prolonged bleeding after minor cuts and scrapes; and
  • heavy bleeding after dental work.

Occasionally, someone with von Willebrand disease will have a severe haemorrhage after surgery or trauma.

Von Willebrand disease has a wide range of severity and it is quite rare for people to be seriously affected. There can even be marked differences in severity within the same family.

Types of von Willebrand disease

Von Willebrand disease is divided into three types:

  • Type 1 is where there are low levels of von Willebrand factor but the factor itself functions normally. This is the most common type, making up almost 75 per cent of cases of von Willebrand disease. It is usually not severe.
  • Type 2 is where there is an irregularity with the von Willebrand factor affecting its ability to function.
  • Type 3 occurs where there is total or near total absence of von Willebrand factor. This is the most severe and the rarest form of the disease.

Von Willebrand disease is an inherited condition. Someone with the disease has a 50 per cent chance of passing it on to their child. Men and women are affected equally, and the diagnosis can be confirmed with blood tests taken by your doctor.

Treatment

In general, no treatment is required for von Willebrand disease. However, those affected should notify their health professional before having surgery or a dental procedure in which excessive bleeding might be a problem.

In more severe cases of von Willebrand disease, treatment may be required to stimulate the production of von Willebrand factor and to treat or prevent the bleeding. Concentrated clotting factors, including von Willebrand factor, are also sometimes used (given through a drip into a vein).

The oral contraceptive pill (the pill) can be taken by women with von Willebrand disease to help control heavy periods. The oestrogen in the pill can help to increase levels of von Willebrand factor and reduce blood loss.

Another option to help control heavy periods is a levonorgestrel intrauterine device. This is a contraceptive device that is placed in the uterus and releases synthetic progesterone.

Precautions

Pregnancy and childbirth does not usually present a risk for a woman with this disease because, in most women, the levels of von Willebrand factor rise during pregnancy. However, it is still advisable for a woman to notify those supervising her antenatal care of the fact she has the disease.

People with von Willebrand disease should consult their doctor before taking non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or aspirin, because these medicines can affect blood clotting.

Last Reviewed: 28/02/2013

myDr


References

1. Royal Children’s Hospital Melbourne. Clinical Practice Guidelines: von Willebrand disease (updated 1 Sep 2012). http://www.rch.org.au/clinicalguide/guideline_index/Von_Willebrand_Disease_vWD/ (accessed Mar 2013).
2. Haemophilia Foundation Australia. Von Willebrand disorder: Frequently asked questions and facts (updated 5 November 2012). http://www.haemophilia.org.au/bleedingdisorders/cid/30/parent/0/pid/1/t/bleedingdisorders/title/von-willebrand-disorder (accessed Mar 2013).
3. National Heart, Lung and Blood Institute. Von Willebrand disease (updated 1 Jun 2011). http://www.nhlbi.nih.gov/health/health-topics/topics/vwd/ (accessed Mar 2013).
4. Haemophilia Foundation Australia. Guide for people living with von Willebrand disorder (June 2010). http://www.haemophilia.org.au/documents/item/1301 (accessed Mar 2013).