GUIDELINES FOR CLOTTING FACTOR REPLACEMENT IN SEVERE HEMOPHILIA A

(Modified from Pediatr Clin NA 1996;43:709-736) (see below for notes regarding dosing in hemophilia B)
SITE OF BLEED
TARGET FACTOR LEVEL
FACTOR DOSING
COMMENTS
Joint 40-50% 20-40 U/kg/day Rest, immobilization, physical therapy
Muscle 40-50% 25-30 U/kg/day Calf or forearm bleeds may be limb-threatening. Femoral or retroperitoneal bleeds may cause significant blood loss
Oral mucosa Initially 50%, then antifibrinolytic Rx until healed 25 U/kg/day Adjuvant antifibrinolytic Rx (Amicar) 
Epistaxis Initially 80-100%, then 30% until healed 40-50 U/kg, then
30-40 U/kg/day
Pressure; packing and cautery for severe/ recurrent bleed
Gastrointestinal Initially 100%, then 30% until healed 40-50 U/kg, then
30-40 U/kg/day
Endoscopy (lesion often found)
Antifibrinolytic Rx
Genitourinary Initially 100%, then 30% until healed 40-50 U/kg, then
30-40 U/kg/day
R/O stone, UTI
Consider adjuvant prednisone 1-2 mg/kg/day x 5-7 d
CNS Initially 100%, then
50-100% x 10-14 d
50 U/kg, then 25 U/kg/12 or by continuous infusion Consider prophylactic anticonvulsants.

LP requires prophylactic infusion

Trauma or Surgery Initially 100%, then 50% until wound healing begins, then 30% until healing complete 50 U/kg, then dose q12h or by continuous infusion Evaluate for inhibitor prior to elective surgery
See also Furie et al, Blood 1994;84:3-9

Factor VIII level will increase by approximately 2% for every unit of clotting factor per kg body weight. Factor VIII half-life is 8-12 hours. When the goal is to maintain factor levels above a threshold for more than a day, Factor VIII should be given every 12 hours, or by continuous infusion. Factor levels should be drawn near the end of a dosing period. If recovery of factor is substantially less than expected, consider the possibility of a factor VIII inhibitor.  [Note: recombinant factor VIII concentrates may give lower-than-expected recovery, typically about 80% of expected.]

Please notify the special coagulation lab (3-5005) in advance if you anticipate ordering factor VIII or IX levels at night or on weekends.

Factor IX has longer half-life (18-24 hours) but greater volume of distribution, so factor level increases by only 1% per unit of clotting factor per kg body weight. The net effect is that steady-state dosing is approximately the same as for factor VIII, although initial doses need to be somewhat higher. Recombinant factor IX concentrates may require somewhat higher dosing than plasma-derived concentrates to achieve comparable blood levels.  Factor IX concentrate should be given every 12-24 hours. 

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
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 concentrate.
More information about the program

Reviewed March 2010