Article Abstract

Diagnosis and management of von Willebrand disease in the United Kingdom

Authors: Steve Keeney, Anne Goodeve

Abstract

Treatment of von Willebrand disease (VWD) in the United Kingdom (UK) is based on consensus guidelines, subject to regular review, relating to the diagnosis and management of the condition. Those diagnosed with VWD are registered on a national inherited bleeding disorder database administered by the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) which also records treatment history and aids service planning and commissioning. Selection of therapeutic products suitable for treatment of VWD is based on the observed clinical response. For those individuals where it is not contra-indicated or inappropriate, desmopressin (DDAVP) remains the recommended treatment. In patients where DDAVP treatment is unsuitable, replacement factor concentrate containing von Willebrand factor (VWF) is the recommended alternative. Relevant concentrates are available for all patients in the UK and treatment is administered by a network of 59 haemophilia treatment centres that also provide specialist care for individuals diagnosed with VWD. The treatment guidelines and specialist care network exist to develop comprehensive management plans specific to each patient. Genetic testing is employed in the UK in certain situations, particularly for type 2 VWD and this is also performed in accordance with current guidelines.