Von Willebrand Disease: What to Know About This Bleeding Disorder

Cuts and bruises are common, and most people don’t give them a second thought. The body’s ability to repair and heal is pretty amazing. A complex network of cells and proteins work together to stop bleeding — a process called “clotting.” 

But what happens if part of the network doesn’t work quite right? Bleeding can last longer than normal, or it can occur more easily. Bleeding disorders are what happens when clotting is abnormal.

Von Willebrand disease (vWD) is the most common type of bleeding disorder. About 1% of people have vWD, and it equally affects men and women. Most people with vWD are born with it, but you can also get it later in life. Many cases are mild and may not even need treatment. But many treatment options exist for more severe forms.

What causes von Willebrand disease?

To understand vWD, let’s first talk about how clotting works. When bleeding starts, many different proteins and cells spring into action to stop it. 

You might think of clotting like plugging a hole in a leaky bucket. You’ll need something to plug the hole and some sort of glue to make sure the plug stays in place and forms a good seal. Platelets are blood cells that form a clot and plug the hole. Von Willebrand factor (vWF) is the protein that acts as glue, helping platelets stick to the hole and stay in place. VWD is caused by problems with vWF (the glue). 

So what causes problems with vWF in the first place? Causes fall into three buckets:

1. Inherited (genetic): Most people with vWD are born with a genetic defect in vWF.

2. Spontaneous genetic mutation: Genetic conditions are typically passed down through your parents’ genes, but sometimes genes can change (mutate) during conception. So it’s possible to have genetic vWD without a family history of it. This is called a “spontaneous genetic mutation.”

3. Acquired: Sometimes vWD can occur later in life. Other medical conditions and some medications can cause problems with vWF, resulting in acquired vWD.

The cause of vWD isn’t quite as important as the way in which vWF is affected. There are many ways vWF can work incorrectly, and there are several different types of vWD (more on this below). The type of vWD determines the severity of symptoms.

What are the symptoms of von Willebrand disease?

Symptoms of vWD include:

• Frequent, severe nosebleeds: These nosebleeds typically last longer than 10 minutes and occur more than 5 times a year. They often need some kind of intervention to stop them, like packing the nose with gauze for many hours (as opposed to just pinching the bridge of the nose).

• Easy bruising: Bruising can seem to come out of nowhere, without prior trauma.

• Heavy, prolonged periods: Menstrual cycles last longer than normal with very heavy flow, requiring frequent pad or tampon changes.

• Prolonged bleeding: This can happen after injury, dental procedures, surgery, or childbirth.

In severe cases of vWD (like type 3 vWD), bleeding may occur inside of some organs. For example:

• Bleeding in the stomach or bowels can cause bloody stools or dark black, tar-colored stools. 

• Bleeding in the bladder can cause red, pink, or brown-colored urine.

• Bleeding in the joints can cause rapid onset of severe joint pain and swelling.

How do you diagnose von Willebrand disease?

Healthcare providers might suspect vWD when a person has the bleeding symptoms described above or a family history of a bleeding disorder. Blood tests to check levels of vWF and some other clotting proteins confirm the diagnosis.

Types of von Willebrand disease

VWD comes in a few different types, and the severity of symptoms depends on which type you have. These include:

• Type 1: This accounts for 70% to 80% of cases. The cause is low levels of vWF (not enough glue). Symptoms are mild.

• Type 2A, 2B, 2M, or 2N: These make up 10% to 20% of cases. The cause is vWF that doesn’t work properly — the glue isn’t sticky enough. Symptoms can be mild or moderate.

• Type 3: Less than 5% of cases fall into this type. It’s caused by a complete lack of vWF (no glue at all). This is the most severe form of vWD.

How do you treat von Willebrand disease?

Treatment depends on how severe the bleeding symptoms are. Some people don’t need any treatment. Others need treatment, and there are a lot of options, including:

• Desmopressin nasal spray and injections: These help the body make more vWF.

• Hormonal birth control: This helps control heavy menstrual bleeding.

You may need advanced therapies if these options don’t help or there’s severe bleeding. These are typically infused directly into the vein. Examples include:

• Medications that replace vWF, like Vonvendi

• Medications that prevent clots from breaking down, like aminocaproic acid (Amicar) 

People with vWD may also need to avoid certain medications that increase bleeding risk, like nonsteroidal anti-inflammatory drugs (NSAIDs) or clopidogrel. It’s also a good idea to avoid contact sports that increase the risk of injury, like kickboxing and football. Talk to your healthcare provider about medications and activities that might be best to avoid.

How serious is von Willebrand disease?

It depends. Some people with vWD may never even know that they have it because their symptoms are so mild. On the other hand, people with type 3 vWD can experience life-threatening bleeding. 

No matter which type you have, treatment options exist. The National Hemophilia Foundation also has excellent resources for people with bleeding disorders.

Why trust our experts?

Written by:

Samantha C. Shapiro, MD

Samantha Shapiro, MD, is a board-certified rheumatologist and internist with expertise in autoimmune and inflammatory conditions. She founded the division of rheumatology at Dell Medical School at The University of Texas at Austin.

Edited by:

Mandy Armitage, MD

Mandy Armitage, MD, has combined her interests in clinical medicine with her passion for education and content development for many years. She served as medical director for the health technology companies HealthLoop (now Get Well) and Doximity.

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