Medicines for Von Willebrand disease

What it is

Von Willebrand disease is the most common inherited bleeding disorder. It is caused by a problem with von Willebrand factor — a protein in the blood that helps platelets stick together and to blood vessel walls to form a clot, and that also helps carry another clotting factor. When this protein is reduced or does not work properly, the blood does not clot as effectively, leading to a tendency to bleed. The severity varies widely: most people have a mild form, and some may not realise they have it. Symptoms can include easy or excessive bruising, frequent or prolonged nosebleeds, bleeding gums, heavy or prolonged periods in women, and prolonged bleeding after cuts, dental work, surgery or childbirth. Because it is usually inherited, it often runs in families. Most people with von Willebrand disease lead normal lives, and the condition is usually mild and manageable, but it is important to know about — particularly before surgery, dental procedures, or pregnancy — so that bleeding can be prevented and managed.

How it is treated

Von Willebrand disease is diagnosed with blood tests that measure von Willebrand factor and clotting, often prompted by a personal or family history of easy bleeding. Management is guided by a haematologist and tailored to the type and severity, and for many people with mild disease, no day-to-day treatment is needed — instead, the focus is on being prepared for situations that could cause bleeding. Key measures include: informing doctors and dentists of the condition before any procedure or surgery, so bleeding can be prevented; avoiding medicines that increase bleeding risk (such as aspirin and some anti-inflammatory painkillers) unless advised; and knowing how to manage bleeds. When treatment is needed — for example to cover surgery, dental work, injury, or heavy periods — options include a medicine that boosts the body's own von Willebrand factor (desmopressin) in suitable people, treatments that replace the factor, medicines that help stabilise clots, and, for heavy periods, specific treatments. Wearing or carrying information about the condition is helpful. The reassuring message is that von Willebrand disease is usually mild and manageable, most people lead normal lives, and effective treatments are available to prevent and control bleeding when needed.