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 and people with the condition have low levels or underperforming von Willebrand factor (VWF). VWF is an important protein which is involved in the blood clotting process. Women are more likely to experience symptoms due to the bleeding which occurs during menstruation, pregnancy, and post-partum.

VWF works with the clotting protein Factor VIII and both increase during pregnancy. 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 and 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.

Management of pregnant women with VWD is quite complex with little evidence to guide clinical decision-making and international practices. An example is controversy surrounding an ideal plasma VWF levels. However, recent research does recommend antenatal assessment of VWF levels and third trimester levels should be monitored.

References

Data and Statistics on von Willebrand Disease | CDC

Physiological Haemostasis - Mechanisms of Vascular Disease - NCBI Bookshelf (nih.gov)

Sadler, B., Castaman, G., & O’Donnell, J.S. von Willebrand disease and von Willebrand factor. Haemophilia. 2022;28(Suppl. 4):11–17

Lavin, M., Sánchez Luceros, A., Kouides, P. Abdul-Kadir, R., O’Donnell, J.S., Baker, R.I., Othman, M., & Haberichter, S.L. Examining international practices in the management of pregnant women with von Willebrand disease. J Thromb Haemost. 2022;20:82–91