REPLACEMENT THERAPY FOR VON WILLEBRAND DISEASE

Humate-P® is an intermediate-purity factor VIII preparation that contains significant amounts of von Willebrand factor.  It is the treatment of choice for patients with type II and type III von Willebrand disease (VWD) and patients with type I VWD who have major bleeding, or in whom treatment with desmopressin (DDAVP) is ineffective.  Humate-P is labelled in both factor VIII units and ristocetin cofactor units; the latter should be used to calculate dosage in VWD.   The plasma factor VIII activity can be used to monitor treatment. Please notify the special coagulation lab (3-5005) in advance if you anticipate ordering factor VIII activity at night or on weekends.

TREATMENT OF VON WILLEBRAND DISEASE WITH HUMATE-P®

Classification of VWD Indication for treatment Dosage (ristocetin cofactor units/kg)
Mild type I (VWF >30%) Major bleeding or major invasive procedure/surgery 1. Load with 40-60 U/kg
2. 40-50 U/kg every 8-12 h (keep VIII >50%)
3, 40-50 U/kg/day for up to 7 days
Moderate type I (VWF < 30%) Minor bleeding, or minor invasive procedure 40-50 U/kg x 1-2 doses
Moderate or severe type I Major bleeding or surgery 1. Load with 50-75 U/kg
2. 40-60 U/kg every 8-12 h for 3 days (keep VIII >50%)
3. 40-60 U/kg/day for up to 7 days
Type II or III Minor bleeding, or minor invasive procedure 40-50 U/kg x 1-2 doses
Type II or III Major bleeding or major invasive procedure/surgery 1. Load with 60-80 U/kg
2. 40-60 U/kg every 8-12h for 3 days (keep VIII >50%)
3. 40-60 U/kg/day for up to 7 days
(Based on manufacturer's guidelines; see also Mannuci PM, Blood 2001;97:1915)

The UWHC Comprehensive Program For Bleeding Disorders (608-890-9493) can facilitate delivery of clotting factor concentrates for home use, and provide general assistance in the outpatient management of patients with hemophilia.
Contacts (with pager #s):
Jessica Pindilli RN: 890-9493, pager 9493
Stephanie Youngbauer RN 890-9494, pager 9494
Jeff Amond MSSW 890-9495
After hours or on weekends patients will need to come to UW Hospital to get clotting factor.
More information about the program

Reviewed March 2010