Von Willebrand Disease is a common autosomal dominant disorder, with three subtypes.
Von Willebrand Disease usually presents with easy bruising, mucosal bleeding, post-operative bleeding or post traumatic bleeding. Menorrhagia and post-partum haemorrhage are common presentations in females.
Treatment for von Willebrand Disease is usually with:
DDAVP (Desmopressin) 0.3 microgram/kg diluted in 0.9% saline and given IV over 20 minutes for the common type-1 patients.
Patients with type-2 disorders (functional deficiency) and the rare type-3 disorder (homozygous disorder) will require factor replacement therapy with plasma-derived factor VIII (Biostate).
Recombinant factor VIII does not contain von Willebrand factor
Supplies of plasma-derived factor VIII (Biostate) are kept in the fridge in Transfusion Medicine (Haematology laboratory)
All presentations to the Emergency Department should be discussed with the on-call Haematologist before any treatment is instituted