Von Willebrand disease (VWD) is an inherited bleeding disorder and unfortunately cannot be cured. It is a disease where the blood does not clot properly. People with the condition have low levels or underperforming von Willebrand factor (VWF), which is an important protein involved in the blood clotting process.

Types of VWD Type 1 – VWD factor levels are low – the most common. Type 2 – VWD doesn’t work properly. Type 3 – VWD factor is missing – the rarest form.

Facts about VWD

VWD is one of the most common bleeding disorders worldwide and affects approximately 1% of the population, both male and female. However, women are more likely to experience symptoms associated with VWD due to the bleeding which occurs during menstruation, pregnancy, and post-partum.

VWD and women

VWF, along with another important clotting factor, Factor VIII, increases during pregnancy and these changes aim to facilitate haemostasis after childbirth. Haemostasis is the body’s response to stop bleeding and involves complex interactions between cells, platelets and coagulation proteins such as Factor VIII.

Excess gynaecological bleeding is increased in women with VWD, with post-partum haemorrhage increasing 1.5 times. There is also a 5-fold risk of blood transfusion and a higher risk of death by 10-fold. Women with VWD also have an increased risk of post-partum bleeding up to 6-weeks following delivery; 30% compared to 2% among women without the condition.

Symptoms

If you have VWD, you may have the following general symptoms:

• Excessive bleeding from an injury or after dental work or surgery.
• Frequent nosebleeds which do not stop after about 10 minutes.
• Blood in the urine or stool.
• Easy bruising or lumpy bruises.

For women, the following symptoms may present:

• Heavy or long menstrual bleeding.
• Heavy bleeding during labour and delivery.
• Blood clots greater than 1 inch in diameter in your menstrual flow.
• The need to change your menstrual pad or tampon more than once an hour; or needing to use double pad protection.
• Anaemia, tiredness, fatigue or shortness of breath.

VWD in adolescents

Adolescents with low VWF are particularly vulnerable, as the diagnosis is often delayed due to the potential presence of other diseases or medical conditions such as iron deficiency anaemia and associated symptoms. Research found that up to 42% of adolescents with low VWF experience nosebleeds, 45% have bleeding under the skin from burst bleed vessels, 35% bleed from minor wounds, and 32% suffer from oral cavity bleeding. Anaemia was found in 21% of the patients, with 60% having iron deficiency.

The bleeding phenotype (or observable characteristics or traits) in adolescents results in a significant decline in quality of life due to iron deficiency anaemia causing fatigue. Iron deficiency also is associated with reduced cognition in adolescents and girls. A retrospective study found that adolescents presenting with heavy menstrual bleeding (HMB), 50.9% noted to have depression, compared to 24.2% of patients without HMB.

In 2021, VWD Guidelines for diagnosis and management were published by the American Society of Hematology (ASH), International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH). ASH ISTH NHF WFH VWD Guidelines

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References

• Kashinath, S.K. & Kouides P.A. The diagnosis, natural history, and management of von Willebrand disease in women in the age of guidelines. Expert Rev Hematol. 2023;12:1-16. doi: 10.1080/17474086.2023.2166925.
• Sadler, B., Castaman, G., & O’Donnell, J.S. von Willebrand disease and von Willebrand factor. Haemophilia. 2022;28(Suppl. 4):11–17
• Data and Statistics on von Willebrand Disease | CDC
• Physiological Haemostasis - Mechanisms of Vascular Disease - NCBI Bookshelf (nih.gov)
• Von Willebrand disease - Symptoms and causes - Mayo Clinic
• Von Willebrand disease - types, symptoms, diagnosis and treatment | healthdirect