Von Willebrand Disease

Overview

What is von Willebrand disease?

Von Willebrand disease (VWD) is a bleeding disorder in which your blood has trouble clotting. It is inherited, meaning it is passed down from parents to their children.

People with von Willebrand disease sometimes experience heavier-than-normal bleeding from injury, surgery, and, in women, menstrual flow and childbirth. This bleeding can cause health problems including pain and anemia (low number of red blood cells). In rare cases, the bleeding can lead to death.

How common is von Willebrand disease?

Von Willebrand disease is the most common type of bleeding disorder in the U.S. It is similar to another blood disorder called hemophilia, though it is usually less severe than hemophilia. Von Willebrand disease affects 1% of the U.S. population. It occurs in both males and females.

Symptoms and Causes

What causes von Willebrand disease?

Problems with a protein in the blood cause von Willebrand disease. This protein, known as von Willebrand factor (VWF), helps the blood clot.

In some cases, the blood has too little VWF. In others, the protein has a defect. In both of these scenarios the ability of the blood to clot properly is impaired. A variety of gene mutations cause either a reduced production of VWF or production of an abnormally functioning VWF. Although the cause of these mutations is unknown, the abnormal gene is passed from one generation to another.

What are the symptoms of von Willebrand disease?

Many people with von Willebrand disease have mild cases and no symptoms. Symptoms in more severe cases include:

  • Heavy bleeding after injury or surgery
  • Easy bruising
  • Frequent nosebleeds
  • Heavy menstrual bleeding in women
  • Long-lasting bleeding of the gums after dental work
  • Blood in the stool or urine

Diagnosis and Tests

How do doctors diagnose von Willebrand disease?

To diagnose von Willebrand disease, your doctor will ask about your family history to see if any relatives have bleeding disorders. Blood tests confirm a diagnosis of von Willebrand disease. One test looks at the level and activity of von Willebrand factor (VWF) in the blood. Your doctor may also test your blood to measure clotting times and platelet function.

It is common for people to take the same blood test multiple times to confirm von Willebrand disease. Doctors often perform repeat tests because levels of VWF in the blood can change depending on different factors, including hormone levels.

What are the types of von Willebrand disease?

Doctors categorize von Willebrand disease into 3 main types. Sophisticated laboratory testing is sometimes needed to determine the type of VWF defect you have which then allows classification of the type of VWD:

  • Type 1: People with this type have low levels of VWF in their blood. It is the most common type of von Willebrand disease. Most people with Type 1 experience mild bleeding symptoms or none at all.
  • Type 2: In this type, the VWF has a defect that makes it unable to work properly. Bleeding symptoms can be mild or moderate.
  • Type 3: The most severe and rarest type of von Willebrand disease. People with this type typically have no VWF or very low amounts of VWF. Symptoms can be serious, but your doctor can help manage them.

Management and Treatment

What are the common treatments for von Willebrand disease?

Treatment for von Willebrand disease varies depending on the condition’s severity. All treatment aims to prevent episodes of excessive bleeding. Doctors use several approaches to manage the condition. They include:

  • Desmopressin: Doctors prescribe this hormone to boost levels of von Willebrand factor (VWF) in the blood. It is injected into a vein or inhaled through the nose. This therapy is the most common treatment for von Willebrand disease.
  • VWF infusions: Depending on the type of von Willebrand disease, infusions of VWF are necessary for bleeding episodes, and more often for surgical procedures. Some patients with severe forms of von Willebrand disease receive regular VWF infusions or prophylactic therapy to maintain normal levels of the VWF protein in their blood.
  • Antifibrinolytics: These drugs prevent blood clots from breaking down. Doctors sometimes prescribe them before dental work and for women with heavy menstrual bleeding.
  • Birth control pills: The estrogen content in these pills will cause an increase in the level of VWF in the blood, and thus provide some protection in women with heavy menstrual bleeding. Estrogen therapy is not appropriate for men with von Willebrand disease.

What are common complications or side effects of von Willebrand disease?

Women are more likely than men to experience complications from von Willebrand disease. Abnormal bleeding can cause problems in menstrual cycles and after childbirth.

People with severe von Willebrand disease (Type 3) may experience severe pain and swelling if bleeding occurs in their joints or soft tissues. In extreme cases, without treatment, excessive bleeding can lead to death.

Prevention

Can von Willebrand disease be prevented?

Because von Willebrand disease is usually inherited, it is not preventable.

Outlook / Prognosis

What is the outlook for people with von Willebrand disease?

Von Willebrand disease is a lifelong condition. For most people with the disease it doesn’t interfere with living active lives. Usually, it only needs to be addressed after a severe injury or if you need surgery.

Receiving treatment when necessary and visiting your doctor regularly are the best ways to make sure von Willebrand disease does not interfere with enjoying a healthy life.

Living With

When should I call a doctor?

Contact your doctor if you have bleeding that lasts a long time or is difficult to stop. Women with von Willebrand disease should speak to their doctor when considering pregnancy. Your doctor can recommend precautions to lower your risk of complications.

Last reviewed by a Cleveland Clinic medical professional on 02/14/2018.

References

  • National Hemophilia Foundation. . Accessed 2/14/2018. Von Willebrand Disease (https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease)
  • Genetics Home Reference. . Accessed 2/14/2018. Von Willebrand Disease (https://ghr.nlm.nih.gov/condition/von-willebrand-disease)
  • National Heart, Lung, and Blood Institute. . Accessed 2/14/2018. Von Willebrand Disease (https://www.nhlbi.nih.gov/health-topics/von-willebrand-disease)
  • Genetic and Rare Diseases Information Center. . Accessed 2/14/2018.Von Willebrand disease (https://rarediseases.info.nih.gov/diseases/7867/von-willebrand-disease)
  • World Federation of Hemophilia. Accessed 2/14/2018. What is von Willebrand disease (VWD)? (https://www.wfh.org/en/page.aspx?pid=673)
  • National Organization for Rare Diseases. . Accessed 2/14/2018. Von Willebrand Disease (https://rarediseases.org/rare-diseases/von-willebrand-disease/)

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