Von Willebrand Disease

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The blood disorders team at UT Southwestern Medical Center provides comprehensive, specialized care for people with von Willebrand disease, which is the most common bleeding disorder in the United States.

UT Southwestern is home to the only adult-focused hemophilia treatment center in Texas, providing access to a multidisciplinary team of physicians, nurses, physical therapists, social workers, and other providers who have expertise in diagnosing and treating bleeding disorders, including von Willebrand disease.

Comprehensive, State-of-the-Art Care for Von Willebrand Disease

Von Willebrand disease, a bleeding disorder that slows down the blood clotting process, affects up to 1% of people in the United States. Many people with this condition bleed for a long time due to injury, surgery, dental care, menstruation, or childbirth.

Although von Willebrand disease has similarities to hemophilia, it tends to be milder. As with hemophilia, most cases of von Willebrand disease are inherited (present at birth), and in rare cases it is acquired. Unlike hemophilia, von Willebrand disease can affect both males and females.

The multidisciplinary North Texas Comprehensive Hemophilia Treatment Center at UT Southwestern provides comprehensive, state-of-the-art care and education for people with bleeding disorders, including von Willebrand disease.

In partnership with other hemophilia treatment centers as well as the pharmaceutical industry, we participate in research to advance knowledge of and care for bleeding disorders.

Types of Von Willebrand Disease

Types of von Willebrand disease include:

  • Type 1 von Willebrand disease, which is by far the most common type and is characterized by low levels of a clotting protein called von Willebrand factor (VWF); symptoms, if any, tend to be mild
  • Type 2 von Willebrand disease, which has four subtypes (2A, 2B, 2M, and 2N) and occurs when VWF doesn’t function properly; symptoms tend to be mild to moderate
  • Type 3 von Willebrand disease, which is the least common type and characterized by very low levels or absence of VWF and, in some cases, low levels of clotting factor VIII (FVIII); symptoms tend to be severe
  • Acquired von Willebrand disease, which may develop due to an underlying medical condition or after taking certain medications

Causes of Von Willebrand Disease

Von Willebrand disease occurs when there’s a lack of a clotting protein called von Willebrand factor (VWF) or when VWF is not working properly, making it difficult for the blood to clot.

Having a family history of von Willebrand disease is the primary risk factor. Most of the time, the bleeding disorder is inherited and caused by a mutation of the VWF gene, which aids the production of VWF.

Most people with Type 1 and Type 2 von Willebrand disease inherit a mutated gene from one parent. People with Type 3, as well as some people with Type 1 and Type 2, inherit a mutated gene from each parent. People can be carriers of von Willebrand disease without having symptoms.

In rare cases, von Willebrand disease can be acquired, which happens when the patient’s immune system attacks the clotting factor, VWF. This might occur due to an autoimmune condition such as lupus or other medical conditions. It may also develop after taking certain medications. People with acquired von Willebrand disease will not pass the condition to their children.

In some instances, von Willebrand disease occurs because of a spontaneous mutation – there is no prior family history of the disease, but it can be passed on to their children.

Von Willebrand disease affects males and females. It may be more obvious in females, who may bleed heavily during their periods and after childbirth.

Symptoms of Von Willebrand Disease

Symptoms, which vary depending on the type and severity of the condition, include:

  • Frequent, long-lasting nosebleeds
  • Bleeding for more than 10 minutes after sustaining a cut or injury
  • Frequent, easy bruising (bruises are often lumpy)
  • Bleeding heavily after surgery or dental work
  • Heavy menstrual bleeding
  • Bleeding heavily following childbirth or miscarriage
  • Bloody urine or stool

Signs of heavy menstrual bleeding include:

  • Having to change a pad or tampon at least every hour
  • Periods lasting longer than a week
  • Blood clots larger than an inch in diameter
  • Signs of anemia, such as fatigue and shortness of breath

People with severe von Willebrand disease may also have spontaneous bleeding episodes (bleeding for no apparent reason) and bleeding in the muscles and joints, resulting in pain and swelling.

People with mild cases of von Willebrand disease may have no symptoms. Some people don’t realize they have it until they bleed at length following a surgical or dental procedure.

It’s important to tell a doctor about any symptoms and to seek care if the bleeding does not stop.

Diagnosing Von Willebrand Disease

At UT Southwestern, our doctors check for von Willebrand disease by examining the patient, reviewing the patient’s medical and family history, and ordering a series of blood tests.

These blood tests help determine:

  • How much VWF and FVIII is in the blood
  • How well those clotting factors are functioning
  • How well platelets are functioning
  • The specific type of von Willebrand disease

Some blood tests may need to be done more than once because factors such as exercise, stress, pregnancy, and birth control pills can make VWF levels fluctuate.

Von Willebrand Disease Treatment

Treatments for von Willebrand disease focus on preventing or stopping bleeding events.

Our doctors will determine the appropriate course of treatment, which depends on the type and severity of von Willebrand disease, and provide education to help people stay healthy and avoid complications such as anemia, swelling and pain in the joints, and life-threatening bleeding.

The most common treatment is desmopressin, a synthetic hormone that helps increase VWF and FVIII levels when injected or sprayed in the nose. It’s typically used to treat mild cases. Response to desmopressin varies from person to person; ideally, response to desmopressin should be determined before it is needed.

Treatment options also include:

  • Replacement therapies, which involve injecting clotting factors made from donated human plasma or produced using DNA technology (known as recombinant clotting factors) into the patient’s vein; replacement therapy is typically used for more severe cases, as well as for mild cases that can’t be successfully treated with desmopressin
  • Birth control pills or an IUD containing progestin, which may help alleviate heavy menstrual bleeding
  • Anti-fibrinolytics, which slow the rate at which clots break down; these may be helpful before surgery or dental work or for alleviating heavy menstrual bleeding
  • Fibrin sealants, which can help heal wounds

People with mild cases may need treatment only after an injury or before surgery or dental work.

People with von Willebrand disease who are considering becoming pregnant might want to speak with their doctor about how to reduce risk of complications.

In addition to treatment, it may be helpful to:

  • Discuss pain relief options with a doctor; it might make sense to take acetaminophen instead of blood-thinning drugs such as aspirin, ibuprofen, and naproxen sodium
  • Alert all care providers, including doctors and dentists, about a diagnosis or family history of von Willebrand disease
  • Wear a medical ID bracelet and carry a medical alert card
  • Exercise (while avoiding contact sports)

Support Services for Von Willebrand Disease

At UT Southwestern, we deliver comprehensive care that extends outside our clinic. We can help you coordinate with social workers and financial counselors. When needed, we can refer you to experts in physical therapy, oral surgery, orthopaedic surgery, infectious diseases, and hepatology.

Research Studies for Von Willebrand Disease

Our team is active in research to improve understanding and treatment of von Willebrand disease.

We are currently participating in the Von Willebrand Factor in Pregnancy (VIP) Study. This study involves pregnant women with von Willebrand disease (VWD) who by the third trimester do not have von Willebrand factor (VWF) or factor VIII (FVIII) levels greater than 50-100%.