Low‐dose intermittent factor replacement for post‐operative haemostasis in haemophilia

Abstract

Recommendations for factor replacement therapy for postoperative haemostasis in haemophilia are often empirical and based on the physiological understanding of haemostatic requirements. This report describes the haemostatic management of patients with severe haemophilia undergoing major surgery using lower than usually recommended levels of factor replacement therapy. Eighteen adults (11 with FVIII and seven with FIX deficiency) with an average weight of 52 kg (range: 27-69) underwent 20 major surgical procedures. Factor concentrates were administered by intermittent bolus infusions. The dose of FVIII infused before surgery was 76 mu kg-1 (range: 51-113) and that of FIX was 77 mu kg-1 (range: 50-104). The preoperative levels achieved were 107% (range: 80-180) and 73% (range: 60-90), respectively. Between days 1 and 3 after surgery, an average of 29 mu kg-1 day-1 (range: 20-46) of FVIII and 23 mu kg-1 day-1 (range: 12-42) of FIX was used resulting in mean trough levels of 36% (range: 12-62) and 34% (range: 11-52), respectively. After day 4, an average of 19 mu kg-1 day-1 (range: 15-25) of FVIII and 18 mu kg-1 day-1 (range: 10-37) of FIX was administered until wound healing. The average duration of factor replacement was 11 days (range: 8-16). The mean dose of factor concentrate per patient was 260 mu kg-1 (range: 179-338) of FVIII and 300 mu kg-1 (range: 183-524) of FIX. The total amount of factor used per patient ranged from 12,380 to 19,980 units of FVIII and 8000 to 23,600 units of FIX. Only one patient had post-operative bleeding which was due to a surgical cause. It is concluded that it may be possible to use much lower than recommended levels of factor replacement therapy for postoperative haemostasis in severe haemophilia.

Central venous access device insertion and perioperative management of patients with severe haemophilia A

“…Srivastava et al [4] from India addressed the efficiency of haemostatic coverage schedules that used lower factor levels during surgical procedures. Preoperatively, they used an average of 76 IU/kg per day of factor replacement achieving an average of 107% factor levels.…”

Comprehensive Care and Delivery of Care in Hemophilia: The Developing World

“…It has been the aim of therapy to establish a standard where damage to the joints, clinically and radiologically, can be completely prevented. In situations of extreme resource constraints, lower doses, aimed at maintaining 30-40% trough levels in the first 2-3 days, followed by 20-30% in the next 3-4 days and 10-20% levels during the subsequent days, can reduce factor usage to about 300 IU/kg per procedure even with intermittent infusion protocols for major surgical procedures, not including joint replacement surgery [29]. With continuous infusion of factor concentrates, this can be reduced to about 400-500 IU/ kg per procedure [28].…”

External fixators in haemophilia

“…Factor concentrates were replaced for haemostasis as described before [10] by intermittent bolus injections in seven patients and by continuous infusion in two patients. For surgery, a level of 80-100% was achieved for haemophilia A and 60-80% for haemophilia B.…”