Understanding your Von Willebrand disease (VWD) type: a step-by-step guide

If you or a loved one has been diagnosed with Von Willebrand Disease (VWD), an important step in effective management is understanding the type. Identifying the specific type is crucial because it impacts treatment and management strategies. While only a hematologist can officially determine your VWD type, there are key steps you can take to better understand where you might fall within the classification.

Step 1: Understand the types of VWD

Von Willebrand Disease is a genetic bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (VWF), a protein that helps blood clot. There are three main types of VWD:

• Type 1 – Lower levels of VWF, but the protein functions normally.
• Type 2  – The protein is present but does not function properly. It is further divided into subtypes (2A, 2B, 2M, 2N) specific to how the VWF protein is defective. 
• Type 3 – Little to no VWF, leading to severe, sometimes spontaneous bleeding issues.
• Acquired VWD - results after a diagnosis of an autoimmune disease.

Step 2: Review your bleeding history

One of the most important clues to your VWD type is your personal and family bleeding history. The National Bleeding Disorders Foundation has put together this form for patients to track their bleeding history and any relevant family history, which can be especially helpful when working with your healthcare provider to determine type and get follow-up testing orders. 

Step 3: Get comprehensive blood tests

Standard tests include:

• Von Willebrand factor antigen test – Measures the amount of VWF in your blood.
• Ristocetin cofactor activity (VWF activity test) – Evaluates how well VWF functions.
• Factor VIII activity test – Assesses Factor VIII levels, which can help distinguish VWD from hemophilia.
• Von Willebrand multimer analysis – Determines the structure and function of VWF, which is crucial for diagnosing Type 2 subtypes.

Your hematologist may order these tests multiple times, as VWF levels fluctuate due to factors like stress, hormones, or illness.

Step 4: Identify your VWD type

Based on your lab results, your doctor can determine your VWD type:

• Type 1: If your VWF levels are lower than normal but function properly, Type 1 is most likely.
• Type 2: If your VWF is present but not working correctly, additional tests can pinpoint whether you have 2A, 2B, 2M, or 2N.
• Type 3: If your VWF is nearly undetectable, it’s likely Type 3.

Step 5: Confirm with genetic testing if recommended

Genetic testing isn’t always required but can be useful for confirming Type 2 and Type 3 VWD. It may also be recommended if you have a strong family history of bleeding disorders.

Step 6: Discuss your diagnosis with a specialist

A hematologist specializing in bleeding disorders is your best resource for interpreting your results. They can:

• Confirm your VWD type.
• Explain how your diagnosis affects treatment.
• Provide personalized management strategies.

Final thoughts

Understanding your VWD type is key to managing your condition effectively. If you suspect you have VWD but don’t yet know your type, talk to a specialist, track your bleeding symptoms, and get the appropriate lab tests. With the right diagnosis, you can take control of your health and prevent complications.

Do you have any questions about VWD testing or diagnosis? Share your thoughts in the comments below!

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