Von Willebrand Disease Treatment

Women and von Willebrand disease

Women with VWD usually have more bleeding symptoms than men, particularly during menstruation and childbirth. Girls may experience very heavy menstrual bleeding during puberty and their first period, lasting longer than usual. As a result, anaemia may develop — a condition in which haemoglobin concentration in the blood is reduced, leading to decreased oxygen delivery to tissues. During pregnancy and childbirth, women with von Willebrand disease are in the care of specialist haematologists and gynaecologists who look after both the foetus and the mother throughout pregnancy and delivery.

Risks during surgery and injuries

All people with von Willebrand disease are at increased risk of bleeding during surgery, injuries, polytrauma and similar situations. Even minor procedures such as tonsillectomy or piercing can be risky.

In the event of a bleeding episode, prompt initiation of treatment in cooperation with a haemophilia centre haematologist is important. A prerequisite for home treatment is good cooperation between the family, the patient and the haemophilia centre.

Treatment by VWD type

The treatment approach differs according to the type and severity of the disease. In patients with Type I, where von Willebrand factor is present in sufficient amounts but at reduced concentrations, stimulation of the release of the body's own VWF stores using desmopressin (DDAVP) is often sufficient. In Types II and III, where VWF is qualitatively altered or nearly absent, replacement therapy with VWF-containing concentrates is necessary.

Patients with Type III may be treated prophylactically — with regular administration of VWF concentrate at intervals of several days, similar to severe haemophilia. Prophylaxis significantly reduces the frequency of bleeding episodes and improves quality of life.

Desmopressin (DDAVP)

Desmopressin (DDAVP) is a very effective medication for most people with VWD. It stimulates the release of von Willebrand factor from endothelial cells into the bloodstream and temporarily increases its level. It can be administered intravenously, subcutaneously or as a nasal spray. A DDAVP response test must be performed before first use, as not all patients respond adequately. High-concentration desmopressin for haemostatic use (Octostim) may have limited availability in the Czech Republic — check with your haematologist for the current situation.

VWF concentrates

For patients who do not respond to desmopressin or in whom it is contraindicated, plasma-derived concentrates containing von Willebrand factor and factor VIII are used. Concentrates containing VWF alone without added FVIII are also available and may be a suitable choice for patients at risk of excessive FVIII elevation.

Supportive medications

Supportive medications used in VWD treatment include antifibrinolytics (e.g. tranexamic acid — PAMBA, EXACYL), which stabilise the formed blood clot, and platelet function-supporting drugs (e.g. Dicynone). Antifibrinolytics are particularly useful for mucosal bleeding, after dental procedures and for menstrual bleeding. Treatment is always managed by a haematologist and can differ significantly between individual patients with VWD.

Further information

• About von Willebrand disease
• Haemophilia treatment — a related treatment approach