Von Willebrand Disease (VWD)

Living with a lifelong medical condition can be difficult. If you or a loved one has been diagnosed with von Willebrand disease (VWD), you may have many questions or concerns.

At Banner Health, our dedicated team is here to provide the support, care and information you need. Read on to learn more about VWD and how to live a healthy, fulfilling life. 

What is von Willebrand disease?

Von Willebrand disease (VWD) is a blood disorder that keeps your blood from clotting. Blood clotting is important because it stops bleeding when you get a cut or injury. 

If you have VWD, there is a problem with a protein in your blood called von Willebrand factor (VWF). With VWD, you either don’t have enough protein or it doesn’t work the way it should. This means you might bleed more than usual.

Causes of von Willebrand disease

VWD is the most common inherited bleeding disorder worldwide. This means it is passed down from parents to their children through their genes. If one or both of your parents have VWD, there’s a chance you might have it, too. 

VWD affects males and females equally. However, due to the bleeding problems of monthly periods, females are more often diagnosed. 

In some rare cases, VWD can develop later in life due to other health conditions including autoimmune disorders, heart and blood vessel diseases or some cancers. 

Types of von Willebrand disease

The severity of VWD depends on the type and level of VWF in the blood. The bleeding associated with VWD is usually different than other bleeding disorders, such as hemophilia. 

There are three main types of VWD, ranging from mild to severe:

• Type 1 VWD: The most common and mildest form. People with Type 1 have lower-than-normal levels of a protein that helps blood clot. They usually experience minor bleeding problems.
• Type 2 VWD: This type involves having the blood-clotting protein, but it doesn’t work correctly. Bleeding issues are generally more severe than in Type 1. Type 2 VWD can be further broken down into four subtypes: 2A, 2B, 2M and 2N.
• Type 3 VWD: The rarest and most severe form. People with Type 3 have very little or none of the clotting protein, leading to severe bleeding problems. 

Symptoms of von Willebrand disease

The symptoms of VWD can vary depending on the type and severity. Common signs include:

• Frequent nosebleeds
• Easy bruising
• Bleeding from gums
• Prolonged bleeding from cuts, injuries or after surgery
• Blood in your pee or stool (poop)

Females and people assigned female at birth (AFAB) may have:

• Heavy or long menstrual periods
• The need to change a pad or tampon frequently
• Heavy bleeding during labor and delivery
• Bleeding between periods (metrorrhagia)
• Symptoms of anemia (iron deficiency), like tiredness, fatigue and shortness of breath

In severe cases, bleeding can happen in joints or muscles, which is more serious and painful.

Diagnosing von Willebrand disease

If you have symptoms of VWD, your health care provider will ask about your health and your family’s health history. They may refer you to a blood disorder specialist (hematologist).

To check for the disease, your provider will likely recommend some of the following blood tests:

• von Willebrand factor antigen test: This blood test measures the levels of VWF in your blood.
• von Willebrand factor activity test: This blood test measures how well VWF works in your blood to help clotting.
• Factor VIII clotting activity test: This test measures the levels of factor VIII, another protein that works with VWF to help blood clot. 
• von Willebrand multimers test: This looks at the structure and size distribution of VWF in your blood.
• Gp1bM testing: This test is an alternative to von Willebrand factor activity and is more accurate for diagnosing VWD and following levels.
• Blood type: Levels of von Willebrand factor can change depending on blood type.

Your provider may need to do several blood tests to confirm you have VWD. That’s because results can change due to stress, exercise, pregnancy, infection and medication.

If you are diagnosed with VWD, your provider might recommend other family members also undergo testing to see if it runs in your family.

Treating von Willebrand disease

While there is no cure for VWD, treatments can help manage the symptoms and prevent complications. Your health care provider might recommend one or more of the following:

• Medication: Medicines can help improve clotting. Some can be taken by mouth, while others might be given as injections or nasal sprays. Examples include: 
• Desmopressin: A synthetic hormone that causes an increase in VWF and factor VIII
• Antifibrinolytics: To help prevent the breakdown of clots
• Hormone therapy: For women who have heavy menstrual bleeding due to VWD, birth control pills may help reduce bleeding and regulate periods.
• Clotting factor concentrates: These intravenous (IV) infusions add more clotting proteins to your blood. They can treat or prevent bleeding episodes, especially before surgery or dental procedures.
• Fibrin glue: A special medical gel that can be placed on a cut or wound to stop bleeding.
• Lifestyle changes: Be careful to avoid injuries, use soft-bristled toothbrushes and other simple steps to reduce risks.

For severe cases, you may need medications and supportive therapies to help increase factor levels. For minor cases, you may not need treatment at all.

Living with von Willebrand disease

Most people with VWD can live normal, healthy lives with the proper care and precautions. Here are some tips:

• Regular check-ups: Stay in close contact with your health care provider. Regular check-ups are important to check your condition and adjust treatment as needed.
• Educate others: Make sure your family, friends and coworkers understand your condition. This helps them recognize signs of bleeding and know how to respond in emergencies.
• Avoid high-risk activities: Minimize activities that could lead to injury or bleeding, such as contact sports. Use caution with sharp objects, like scissors or knives and take steps to prevent falls.
• Dental and medical procedures: Before any procedures, let your providers know about your VWD. They may need to take special precautions or adjust treatments to prevent heavy bleeding.
• Avoid aspirin: You should avoid aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs). These pain medications can interfere with blood clotting and increase the risk of bleeding. Talk to your provider about what over-the-counter (OTC) medications are safe to take.
• Healthy lifestyle: Maintain a balanced diet with iron-rich foods and exercise regularly as recommended by your health care provider. Avoid smoking and drinking too much, as these can affect blood clotting. 
• Wear a medical ID bracelet: Wear a medical alert identity necklace or bracelet, in case of emergencies.
• Monitor symptoms: Pay attention to how you feel. Record any bleeding episodes, noting how often, how long and how severe they are. This information is helpful for your provider. 
• Family planning: Since VWD is a genetic condition that can be passed on from parent to child, consider genetic counseling before planning a family. 

If you experience severe or uncontrolled bleeding that doesn’t stop with the usual measures, call 911 or go to the nearest emergency department. It’s important to plan for emergencies and know where to go for specialized care.

Contact us

If you or a loved one has been diagnosed with von Willebrand disease or another blood disorder, Banner Health is here to support you. Our team of specialized providers, including hematologists and other medical professionals, can provide the care and guidance you need to manage your condition and live a full life.