What is Von Willebrand Disease (VWD)?

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What is Von Willebrand Disease?

Von Willebrand Disease (VWD) is the world’s most common inherited bleeding disorder. It happens when von Willebrand factor — a protein needed for healthy blood clotting — is missing, too low, or not working properly. With the right information, care, and community, people with VWD can live full and active lives.

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Understanding VWD starts with clear information, supportive care, and connection with people who understand.
0.6–1.3% Estimated global population affected Including all forms of VWD
88M Estimated worldwide cases VWD may affect millions globally
75–80% Type 1 cases The mildest and most common form
Often missed Up to 9 in 10 people with VWD May not know they have it
What is VWD?

A clear introduction to the condition

Von Willebrand Disease is caused by a deficiency or defect in von Willebrand factor (VWF), which helps blood clot properly. Without enough working VWF, bleeding can last longer than normal. VWD affects people of all backgrounds and all genders, although women often experience more noticeable symptoms because of menstruation, pregnancy, and childbirth.

Key facts

Why VWD is often missed

  • Many people have very mild symptoms.
  • Bleeding may be mistaken for normal variation.
  • Awareness can still be limited among healthcare professionals.
  • Testing can be complex and may need specialist interpretation.
  • Symptoms sometimes only become obvious after surgery, injury, dental work, or childbirth.
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Understanding VWF

What does von Willebrand factor do?

VWF has two major jobs in the body. It helps platelets stick to an injured blood vessel to form the first seal, and it protects factor VIII, another important clotting protein. When VWF is too low or does not work well, bleeding becomes harder to control.

Role 1

Platelet adhesion

When a blood vessel is damaged, VWF acts like a bridge or glue, helping platelets stick to the injured area and to one another. This creates the first platelet plug that helps slow bleeding.

Role 2

Factor VIII protection

VWF carries and protects factor VIII in the bloodstream. If VWF is not available, factor VIII can break down too quickly, making the clotting process even less effective.

Types of VWD

Three main types — with different levels of severity

VWD is grouped into three main types based on whether the body makes too little VWF or whether the VWF present does not function as it should.

Type 1

The mild form

Type 1 is the most common form. People with Type 1 have lower-than-normal levels of VWF, but the VWF they do have generally works normally.

  • Usually the mildest form
  • Often inherited
  • Symptoms may be mild or overlooked
  • VWF levels are reduced rather than absent
Type 2

The qualitative form

Type 2 means VWF is present, but it does not work properly. It includes subtypes 2A, 2B, 2M, and 2N, each affecting clotting in a different way.

  • Amount may be normal or near normal
  • Function is the main problem
  • Subtyping matters for treatment decisions
  • Can sometimes be confused with other bleeding disorders
Type 3

The severe form

Type 3 is rare and severe. People with Type 3 have very little or no VWF, and factor VIII levels are often very low as well.

  • Rarest form
  • Usually identified early in life
  • Can involve serious bleeding episodes
  • Often needs regular specialist treatment
Symptoms & Signs

Symptoms can range from mild to severe

Some people only experience occasional bleeding, while others have more frequent or serious symptoms. Severity often depends on the type of VWD and how low or dysfunctional the VWF is.

Common symptoms

Everyday signs

  • Easy bruising
  • Frequent or prolonged nosebleeds
  • Bleeding from cuts that lasts longer than expected
  • Prolonged bleeding after dental work
Women’s health

Symptoms women may notice first

  • Heavy or prolonged periods
  • Bleeding after childbirth or miscarriage
  • Ovulation-related bleeding
  • Iron deficiency from repeated blood loss
Severe symptoms

More serious bleeding

  • Spontaneous joint bleeding
  • Muscle bleeds
  • Severe bleeding after minor injuries
  • Life-threatening bleeding after surgery or trauma
When to act

Speak to a doctor

If unusual bleeding runs in your family, or if you or your child experience unexplained bruising, prolonged nosebleeds, heavy periods, or excessive bleeding after procedures, it is worth asking for further assessment.

Causes & Inheritance

How VWD is inherited

VWD is usually genetic. Most Type 1 and many Type 2 cases follow an autosomal dominant pattern, while Type 3 usually follows an autosomal recessive pattern. Rarely, a person can develop acquired von Willebrand syndrome later in life because of another medical condition.

Autosomal dominant

Types 1 and most Type 2

  • One altered gene from one parent can be enough
  • Each child of an affected parent has a 50% chance of inheriting it
  • It affects males and females equally
  • It may appear in multiple generations of the same family
Autosomal recessive

Type 3 and some Type 2

  • Two altered genes are needed
  • Parents may be carriers without symptoms
  • Each child of two carriers has a 25% chance of having Type 3
  • This pattern is much rarer than Type 1
Diagnosis

Diagnosis often needs specialist testing

Diagnosing VWD is not always straightforward. Symptoms can be mild, and VWF levels can change because of stress, illness, exercise, pregnancy, inflammation, and even blood group. Multiple tests may be needed over time.

History

Medical and family history

Doctors will ask about your bleeding history, family history, medications, and any recent illnesses or procedures.

Test 1

VWF antigen

Measures how much von Willebrand factor is present in the blood.

Test 2

VWF activity

Checks how well the VWF you have is actually working.

Test 3

Factor VIII activity

Looks at another clotting protein that is closely connected to VWF.

Test 4

Multimer analysis / RIPA

These tests help identify the structure and function of VWF, especially when Type 2 subtypes are suspected.

Genetics

Genetic testing

Sometimes used to confirm diagnosis, clarify subtype, or support family planning decisions.

Treatment Options

Treatment depends on the type, severity, and situation

Some people only need treatment before dental work, surgery, or after an injury. Others — especially those with severe disease — may need more regular support or preventive treatment.

First line

Desmopressin (DDAVP)

Often used for Type 1 VWD and some Type 2A cases. It helps release stored VWF into the bloodstream for a short period.

Replacement

VWF concentrates / recombinant VWF

Used when DDAVP is not suitable or not effective, especially in Type 3 and more severe Type 2 cases.

Clot support

Antifibrinolytics

Medicines such as tranexamic acid can help stop clots from breaking down too quickly, particularly for nose, mouth, or menstrual bleeding.

Women’s care

Hormonal treatment

Hormonal therapy may help reduce heavy menstrual bleeding and improve day-to-day quality of life.

Topical care

Local treatments

Fibrin glue, thrombin, and other local treatments can sometimes help control minor bleeding or support dental and surgical care.

Avoid with care

Medicines that may worsen bleeding

Aspirin, NSAIDs such as ibuprofen or naproxen, and other blood-thinning medicines may increase bleeding risk. Always check new medication with your clinical team.

Living with VWD

Everyday management, confidence, and planning ahead

Most people with VWD can live full, active lives. The key is knowing your diagnosis, understanding your treatment plan, and preparing for situations such as injury, surgery, dental work, travel, pregnancy, or school and work needs.

Daily life

Simple habits that help

  • Wear medical alert identification
  • Carry an emergency card
  • Keep a record of bleeding episodes
  • Use a soft toothbrush and safer grooming tools
Activity

Staying active safely

  • Swimming, walking, cycling, yoga, and tennis are often good options
  • Higher-contact sports may need extra planning or may not be suitable
  • Protective gear and emergency plans matter
Procedures

Surgery and dental care

Always tell your dentist, surgeon, and wider care team about your VWD. Procedures often need planning in advance with a haematologist.

Advocacy

School, work, and family life

Sharing the right information with schools, employers, coaches, and relatives can make day-to-day life safer and reduce anxiety.

Complications & Support

Know when to seek urgent help

Most bleeding episodes can be managed safely with the right plan, but some symptoms should never be ignored. If you are unsure, contact your care team or seek emergency medical advice.

Seek urgent medical care if you experience:

  • Bleeding that does not stop after 10–15 minutes of pressure
  • Head injury followed by headache, confusion, vomiting, or loss of consciousness
  • Severe pain, swelling, or limited movement in a joint
  • Blood in vomit or stool
  • Severe abdominal pain
  • Dizziness, weakness, rapid heartbeat, or signs of significant blood loss

Contact your doctor if:

  • Your bleeding pattern changes
  • You need treatment more often than usual
  • You have signs of iron deficiency or anaemia
  • You are planning surgery, dental work, pregnancy, or childbirth
  • You are starting new medication
  • You have concerns about your emergency plan or diagnosis
Medical disclaimer: This content is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek advice from your doctor, haematology team, or another qualified healthcare professional about any medical concern.
VWD Alliance Community

You don’t have to navigate VWD alone.

Whether you are newly diagnosed, supporting a child, managing symptoms as an adult, or looking for better information, the VWD Alliance community is here to offer support, education, advocacy, and connection.

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