ABSTRACT
The UK treatment strategy for von Willebrand disease (VWD) is based on consensus guidelines
produced by the United Kingdom Haemophilia Centre Doctors' Organization (UKHCDO) relating
to the diagnosis and management of VWD. Selection of therapeutic products suitable
for treatment of this complex inherited bleeding disorder is based on the observed
response. Desmopressin (DDAVP), an analog of vasopressin, is the recommended treatment
in individuals who respond to this drug on trial infusion. DDAVP clearly has no effect
in type 3 VWD but may have variable clinical effect in individuals with other subtypes
or may be contraindicated in some cases. 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 United Kingdom,
and treatment is administered by a network of 67 hemophilia treatment centers that
also provide specialist care for individuals diagnosed with VWD. Patients diagnosed
with the condition are registered on a national inherited bleeding disorder database
administered by the UKHCDO on behalf of the Department of Health to aid in service
planning and commissioning. Genetic testing is employed in the United Kingdom in certain
situations, which is also performed in accordance with current UKHCDO guidelines.
KEYWORDS
von Willebrand disease - inherited bleeding disorders - service specification - UK
Haemophilia Centre Doctors' Organization - National Haemophilia Database
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Steve KeeneyPh.D.
Deputy Head, Molecular Diagnostics Centre, Manchester Royal Infirmary
Oxford Road, Manchester M13 9WL, UK
Email: steve.keeney@cmft.nhs.uk
