In this section
von Willebrand disease
Ref: White GC et al. Definitions in Hemophilia: Recommendation of the Scientific Subcommittee on Factor VIII and Factor IX of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001;85:560
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.
Recombinant clotting factor concentrates are the product of choice. Rarely will patients be treated with plasma-derived concentrates e.g. tolerisation regimes used to treat inhibitors (check ED alert system).
Note that there are two recombinant Factor VIII products available. Whilst it is recommended that patients maintain treatment with their established brand of FVIII product, in an emergency situation administration of any brand of recombinant FVIII is acceptable.
Product name (Brand)
ED alert system will indicate patient's treatment product.
Plasma derived FVIII(8) and von Willebrand Factor
Instructions for preparation and administration of clotting factor concentrates
For muscle and joint bleeds R.I.C.E.S will limit bleeding and reduce pain. Initiate on arrival.
Please ask families to make contact with the Haemophilia Nurse on the next working day for a telephone review.
Referral to physiotherapist for joint and muscle bleeds is essential. Following severe joint/muscle bleeds, the affected joint should be rested. For other joint bleeds, physiotherapy should commence as soon as pain and swelling has subsided and bleed has been controlled.