(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 |

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 *