A Guide to von Willebrand Disease

Medically Reviewed By Julie Scott, DNP, ANP-BC, AOCNP
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Von Willebrand disease (VWD) is a condition that prevents blood from clotting properly. It is the most common inherited bleeding disorder. There is no cure for VWD, but it is possible to manage the condition. The main symptom of VWD is bleeding that occurs more easily or is difficult to stop. This can include frequent nosebleeds, easy bruising, or heavy menstrual periods.

The Centers for Disease Control and Prevention (CDC) explains that VWD is most often an inherited disorder. This means that one or both parents can pass it to their child. However, in rare cases, the condition can develop due to a spontaneous gene mutation or underlying condition.

This article will discuss VWD types, symptoms, and causes. It will also discuss treatment and self-care tips for VWD.

What is von Willebrand disease?

Young female with bloody nose
Kayla Johnson/Stocksy United

VWD is a bleeding disorder in which blood does not clot as expected. It is the most common inherited bleeding disorder.

Healthy blood contains proteins that help the blood to clot. One of these proteins is the von Willebrand factor (VWF). People with VWD either have low levels of this protein in their blood, or it does not work the way it should.

According to the CDC, about 3.2 million people in the United States live with VWD. This makes it the most common bleeding disorder.

Is von Willebrand disease the same as hemophilia?

VWD and hemophilia are both types of bleeding disorders in which the blood does not clot properly. However, they are not the same.

People with hemophilia have low levels of the blood proteins factor VIII or factor IX. Symptoms can include bleeding in the joints, mouth, and brain.

The mutation that causes hemophilia occurs on the X chromosome. Because males have only one X chromosome, they need only one mutation to cause hemophilia. Females need two. For this reason, hemophilia is more common in males.

VWD however, is seen equally in males and females. This is because the mutation that causes VWD occurs in chromosome 12, which is present in both sexes.Learn more about hemophilia symptoms, prevalence, and treatments.

What are the types of von Willebrand disease?

There are three main types of hereditary VWD, and one type that develops later in life. The National Hemophilia Foundation classifies these types as follows.

TypePercent of VWD casesDescription
type 160–80%People with type 1 VWD have 20–50% of the normal levels of VWF protein and experience mild symptoms.
type 215–20%People with this form have normal levels of VWF in their blood, but it does not function properly. Symptoms range from mild to moderate. There are four subtypes of type 2 VWD.
type 2AVWF proteins are the incorrect size and cannot assist with platelet attachments. These attachments are necessary for clots to form.
type 2BVWF connects to platelets when no injury is present. This makes the body discard these platelets and reduces the amount of both platelets and VWF in the blood.
type 2MVWF cannot attach to platelets properly. This prevents the platelets from forming a clot during  injury.
type 2NVWF attaches to platelets normally, but cannot connect to the VII protein correctly. The body discards this protein as a result.
type 3
5–10%
People with type 3 VWD have extremely low levels of VWF or no VWF in their blood. They are likely to experience severe and prolonged bleeding that occurs suddenly.
acquired VWDAcquired VWD occurs in adults. This form is a complication from an autoimmune disease, heart disease, or cancer. Medications can also trigger acquired VWD.

What are the symptoms of von Willebrand disease?

The symptoms of VWD and their severity can vary depending on the type of the condition. However, these are the major signs.

Long periods of bleeding after a health event

If you have VWD, you may experience prolonged bleeding after an injury. Even a small cut or scrape could lead to bleeding that lasts for more than 10 minutes.

Dental work, surgery, and childbirth can lead to excessive bleeding. You may also notice blood in your stool or urine.

People with type 3 VWD may experience internal organ and joint bleeding.

Easy and severe bruising

VWD causes the skin to bruise more easily. These bruises may occur even from a light tap.

Bruises in people with VWD can be larger than a quarter in diameter and have a raised appearance. These bruises can occur 1–4 times a month.

Frequent and hard-to-stop nosebleeds

While nosebleeds are generally common, people with VWD experience more frequent and more severe nosebleeds.

Severe nosebleeds may last longer than 10 minutes and require care to stop the bleeding. People with VWD experience nosebleeds at least five times a year, according to the CDC. These episodes tend to happen without warning or an apparent trigger.

Heavy menstrual bleeding

VWD can cause heavy bleeding during menstrual periods, according to the Office on Women’s Health.

If you have VWD and you menstruate, you may experience symptoms such as:

  • heavy flow that soaks through one tampon or pad every 1–2 hours
  • passing of blood clots larger in size than a strawberry or grape
  • bleeding that lasts more than 7 days

Learn more about other conditions that may cause heavy periods.

What causes von Willebrand disease?

Types 1–3 of VWD are caused by genetic mutations on chromosome 12. These mutations can lead to a deficiency of VWF proteins or prevent them from working properly.

Parents with hereditary types of VWD can pass the mutations that cause them to their children. A child can inherit the condition from one or both parents.

Type O blood and VWF

According to a 2020 study, people with type O blood may have lower levels of VWF than those with other types. This is due to differences in the structure of type O blood.

People with type O blood may have decreased VWF even if they do not have the genetic mutation associated with VWD. For this reason, they may not receive a diagnosis of VWD. However, because they may still experience bleeding, a doctor will monitor symptoms and recommend treatment when necessary.

Other causes

The following conditions and medications can trigger acquired VWD:

How do doctors diagnose von Willebrand disease?

To diagnose VWD, a doctor will start by asking about your personal and family health history. They may ask if you have bleeding tendencies or if anyone in your family has a diagnosed bleeding disorder. They may also ask about any medications you are taking.

If your doctor suspects VWD, they may order blood tests to examine the number of clotting proteins in your blood. These tests will help them determine the type of VWD present.

What are the treatments for von Willebrand disease?

Treatment for VWD varies depending on the type. Treatment options include:

  • Desmopressin acetate: This medication helps increase levels of VWF in the blood for people with type 1 VWD. It is available as an injection or a nasal spray.
  • Factor replacement therapy: This treatment injects lab-produced VWF directly into the bloodstream. Doctors may recommend this treatment in more severe cases of VWD or for people who do not respond to desmopressin acetate.
  • Antifibrinolytic drugs: These medications work by preventing or slowing the breakdown of clots when they form.
  • Oral contraceptives: For people who menstruate, birth control pills can increase VWF levels in the blood and help ease heavy periods.

Talk with your doctor about your treatment options. They will be able to recommend the best treatments for your specific condition.

Genetic counseling for prospective parents

People with a family history of VWD who want to become parents may choose to undergo genetic counseling before conceiving.

Genetic counseling helps you understand the risk of passing VWD to your child.

Your obstetrician can also discuss how VWD might affect your labor and delivery. They can recommend steps you can take to help manage your risk.

What are self-care tips for living with von Willebrand disease?

If you or your child has VWD, the following steps can help you manage the condition:

  • Take your prescribed medications on time.
  • Notify any new medical professionals you contact of the condition.
  • Wear a medical bracelet.
  • Avoid pain relievers that cause blood thinning, such as aspirin or ibuprofen.
  • Always carry gauze to use in case of a bleeding episode.

Q:

Can you play sports with von Willebrand’s disease?

Anonymous

A:

Usually, contact sports are not recommended for someone living with VWD. For these individuals, the bleeding that could result would be more severe than if they did not have VWD. Injuries that result in serious bleeding could potentially be life threatening.

If someone is living with a mild form of VWD, some sports may be safe while wearing the correct safety equipment. If your child is living with VWD, talk with their hematologist about the risks of playing sports.

Julie Scott, DNP, ANP-BC, AOCNP Answers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.

Other frequently asked questions

These are some other frequently asked questions about VWD. Julie Scott, DNP, ANP-BC, AOCNP, has reviewed the answers.

How serious is von Willebrand disease?

Type 1 VWD is the most common version of this condition and the least severe. Uncontrollable bleeding associated with more severe forms of VWD can be life threatening.

Can you get the COVID-19 vaccine with von Willebrand disease?

Talk with your hematologist before receiving the COVID-19 vaccine if you have VWD or any other bleeding disorder.

What is the life expectancy for von Willebrand disease?

VWD does not impact life expectancy. With careful management, people with this condition can live healthy, long lives.

Summary

Von Willebrand disease (VWD) is the most common bleeding disorder. It affects your blood’s ability to clot. Symptoms of VWD can include frequent nosebleeds, easy bruising, and heavy menstrual periods.

VWD is usually inherited. You can also acquire it from a complication of a medication or another condition.

Treatment for VWD involves managing the condition with medications and lifestyle changes. Taking the steps can help people with VWD lead a healthy life.

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Medical Reviewer: Julie Scott, DNP, ANP-BC, AOCNP
Last Review Date: 2022 Sep 28
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