Definition

von Willebrand disease (vWD) is a genetic blood disorder. It decreases the amount or the effectiveness of von Willebrand factor. This factor is a sticky protein. It helps to create a plug at the site of an injury to stop bleeding. Low levels or ineffective von Willebrand factor can cause bleeding problems.

There are 3 major types of vWD:

• Type 1—most mild and most common form
• Type 2—less common than type 1
• Type 3—most serious form and very rare

Causes

vWD is caused by a genetic defect. The specific defect varies by the type of vWD, for example:

• Type 1—results in low levels of von Willebrand factor
• Type 2—results in von Willebrand factor that does not work well
• Type 3—results in no von Willebrand factor

This genetic defect is most often inherited from the parent. A child can develop type 1 or type 2 vWD if just one parent has the defect. For type 3, both parents must have the defective gene. Some people may have the gene for vWD without having symptoms. They can still pass this gene to their offspring.

vWD can also develop from other medical conditions or medications. This is called acquired von Willebrand syndrome.

Risk Factors

Having family members with vWD increases your risk of getting this disease.

Symptoms

Many people with the vWD gene have mild symptoms or none at all. It may only be noticed after an injury or medical procedure.

Symptoms usually begin in childhood and waver throughout life. The severity will vary from person to person. Common symptoms include:

• Easy bruising
• Frequent or prolonged nosebleeds
• Prolonged bleeding from the gums and minor cuts
• Heavy or prolonged bleeding during menstrual periods
• Bloody urine
• Prolonged bleeding after injury, childbirth, surgery, or invasive dental procedures

Type 3 vWD can also result in severe bleeding events with no apparent cause. This type can also cause bleeding into the joints. This can cause pain and swelling in joints.

Diagnosis

You will be asked about your symptoms and medical history. You will be specifically asked about any bleeding events. A physical exam will be done. vWD may be suspected based on your bleeding history.

Blood tests will help to confirm the diagnosis and determine which type of vWD you have. These tests will look at different factors related to blood clotting. They will also measure the amount of von Willebrand factor in your blood.

Treatment

Many people with vWD do not need treatment. If you do, your treatment will depend on the type and severity of your vWD. For most, treatment is only necessary if you are having a surgical or dental procedure.

Treatment may include:

• Desmopressin nasal spray or injection—raises the level of vWF in the blood; may be used to control bleeding in mild cases of type 1
• IV infusions of von Willebrand factors—to control your bleeding
• Birth control pills—to control heavy menstrual periods in women with type 1 vWD
• Antifibrinolytic medicine—for bleeding in the nose or mouth; keeps a clot from being dissolved before the bleeding has stopped
• Recombinant Factor VIIIa—if antibodies to von Willebrand Factor are developed

Special steps will also be taken during childbirth in women with vWD. You will be monitored throughout your pregnancy and receive treatment as needed.

Prevention

There are no guidelines for preventing vWD.

If you have a family history of vWD, genetic counseling may be helpful. Your counselor can determine the risk of vWD to your child.

National Hemophilia Foundation http://www.hemophilia.org

Canadian Hemophilia Society http://www.hemophilia.ca

What is von Willebrand disease? National Heart Lung and Blood Institute website. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/vwd/
Updated June 1, 2011. Accessed June 30, 2015.

von Willebrand’s disease: what you need to know about this inherited disorder. Am J Nurs. February 2000.