von Willebrand Disease

von Willebrand disease is an inherited blood disorder that keeps your blood from clotting. It happens when you don’t have enough functioning von Willebrand factor (VWF). VWF is a protein that helps your blood clot.

If you have this condition, you may bleed more than usual. For example, you may get frequent nosebleeds. You may bleed for a long time after getting a minor cut. Severe forms of von Willebrand disease can cause bleeding in your joints or soft tissues, which can lead to severe pain and swelling. Some people develop anemia.

There’s no cure for von Willebrand disease. But your primary care provider or hematologist can help you manage it.

Types of this condition

There are three main types of von Willebrand disease:

• Type 1: This is the most common type, affecting 3 out of 4 people with the disease. It may cause mild symptoms or even no symptoms.
• Type 2: With this type, von Willebrand factor doesn’t work as it should. It leads to mild to moderate bleeding.
• Type 3: This is the rarest and most severe form of von Willebrand disease. It happens when you have low von Willebrand factor or even none. It can lead to serious bleeding issues.

Symptoms of von Willebrand disease

Many people with this condition have mild or no symptoms. If you have a more severe form of it, you may have the following symptoms:

• Bleeding from a cut or other injury that lasts longer than 10 minutes
• Blood in your poop or pee
• Easy bruising or bruises that appear large or raised
• Frequent nosebleeds
• Heavy bleeding after childbirth or miscarriage
• Heavy bleeding after surgery, including dental work
• Heavy period bleeding

von Willebrand disease causes

von Willebrand disease is a genetic disorder. It happens when you inherit abnormal genes that keep your body from making von Willebrand factor (VWF) like it should. Factors are proteins that help your blood to clot.

You have VWF in your plasma, platelets and walls of your blood vessels. Plasma is the liquid part of blood. Platelets are cells that help blood clot when blood vessels rupture from damage.

Normally, platelets stop bleeding by sticking to damaged blood vessels and helping to form blood clots. VWF helps platelets stick. It also prevents another protein that helps with clotting from breaking down too soon. This protein is called factor VIII. When you don’t have enough VWF, it takes longer for your body to form blood clots.

People may also develop von Willebrand disease as a complication of certain cancers, autoimmune disorders, and heart and blood vessel diseases. This is called acquired von Willebrand syndrome.

How is von Willebrand disease inherited?

Some people have von Willebrand disease because they inherited a faulty gene from either their biological mother or their biological father. This is called autosomal dominant inheritance.

Other genes that lead to von Willebrand’s disease are autosomal recessive. This means that a person has to inherit a faulty gene from both biological parents to have the condition.

How doctors diagnose this condition

Your healthcare provider will ask you to describe your symptoms. They’ll examine you for signs of bruising and bleeding. They’ll ask about medicines you take and if you have a family history of bleeding disorders.

Your provider may need to do several blood tests to diagnose von Willebrand disease, including:

• Complete blood count (CBC): This test measures how many blood cells you have. Low platelets or hemoglobin (a protein in red blood cells) may be a sign of this disease.
• Platelet aggregation tests: These tests measure how well your platelets stick together to make blood clots.
• Clotting tests: These tests measure how long it takes for your blood to clot. The clotting time helps providers see if there are issues with clotting proteins.
• von Willebrand factor tests: Certain tests check your levels of VWF and how it’s working.
• Factor VIII tests: These tests can show if there are issues with Factor VIII. This clotting protein needs von Willebrand factor to help form clots.

How do healthcare providers treat von Willebrand disease?

von Willebrand disease treatments include medications, like:

• Desmopressin: This hormone boosts the levels of von Willebrand factor in your bloodstream. It’s also called DDAVP. This is the most common treatment.
• von Willebrand factor infusions: You may need infusions to give you more von Willebrand factor. With severe disease, you may need regular infusions.
• Antifibrinolytics: These medicines keep blood clots from breaking down. Your provider may prescribe them to reduce bleeding related to surgery, including dental work.
• Birth control pills: This medication helps control heavy period bleeding. It contains estrogen that increases von Willebrand factor levels in your bloodstream.

When should I see my healthcare provider?

Schedule an appointment with your provider if you bleed or bruise easily. Let them know if you have heavy periods. Lots of conditions can cause you to bleed too much. But it’s important to get a proper diagnosis, so you know how to stay safe.

Go to the emergency room any time you have bleeding you can’t control.

What can I expect if I have this condition?

A hematologist can treat von Willebrand disease, but they can’t cure it. And it doesn’t go away on its own.

Most people have Type 1 or Type 2 von Willebrand disease. When this is the case, you may only need treatment if you’re injured or need surgery. If you have Type 3 von Willebrand disease, you may need ongoing medical treatment to reduce your risk of serious bleeding.

Is there anything I can do to feel better?

Having von Willebrand disease may mean you have to take extra steps to prevent bleeding. What you should and shouldn’t do depends on which type you have. Your provider can advise you. For example, you may need to avoid:

• Activities where you could get hurt, like contact sports
• Taking blood thinners, like aspirin or NSAIDs, unless your provider tells you it’s safe
• Taking nutritional supplements that may thin your blood, like vitamin E, fish oil or turmeric. Your healthcare provider can tell you which supplements are safe to take.

Let all your healthcare providers know if you have von Willebrand disease. That way, they can take extra care to manage bleeding after surgery or dental procedures. You may also need to wear a medical alert bracelet. This can help providers care for you during emergencies.

Is von Willebrand disease the same as hemophilia?

No. von Willebrand is similar to hemophilia. But it typically causes less severe symptoms. Both are inherited bleeding disorders. But the way you inherit them — and the genes involved — are different.