In this section
See also: Haemophilia
The most common inherited bleeding disorder affecting 0.1 - 1% of the population. Caused by a deficiency (either quantitative or functional) in von Willebrand Factor (vWF). Deficiency of vWF causes inadequate platelet adhesion and secondary deficiency of Factor FVIII. Affects males and females equally.
Characterised by easy bruising, bleeding from mucous membranes (particularly epistaxis, oral mucosa, menorrhagia) and post-op bleeding.
There are three main phenotypes of vWD.
Notify Haem Inpatient Registrar (pager 5030) of patients presenting to Emergency between 0900-1700hrs, Monday to Friday. Notify Haematologist on call of patients presenting after hours with bleeding problems.
Assess site and extent of bleeding.
Assess type and severity of vWD, if known. Refer to the RCH Emergency Department alert system for individual treatment plan.
Please note that the vial will state both Factor VIII and von Willebrand Factor units.
Check that dosage is according to Factor VIII units,eg prescribe as 'Biostate® 2000 Factor VIII units'.
If there is uncertainty regarding correct dosage please contact Haem Inpatient Registrar (pager 5030) between 0900-1700hrs, Monday to Friday or Haematologist on call (via switch) after hours.
Please ask families to make contact with the Haemophilia Nurse on the next working day for a telephone review.
Instructions for preparation and administration of Clotting factor products