Abstract
Objective: To determine individual/institutional practices at hemophilia treatment centers (HTCs) regarding management of severe hemophilia patients during major surgery (e.g., knee replacement, appendectomy), both in the absence and presence of low and high titer inhibitors to Factor VIII (FVIII).
Background: There are considerable variations in the management of hemophilia patients undergoing surgery. The use of alternative products like Amicar, recombinant Factor VIIa (rFVIIa) and FEIBA is also growing.
Methods: The survey was distributed to both adult and pediatric hematologists at HTCs listed on the CDC website. Ninety-three surveys were successfully emailed. Survey questions evaluated management of hemophilia patients during major surgical procedures, both in the absence and presence of a low and high titer inhibitor.
Results: Surveys from 32 HTCs were received. Twenty-two respondents were affiliated with a University Hospital. For patients without inhibitor, the majority of respondents prefer a pre-operative bolus dose of 50 U/kg (84%, 22/28, range 40–70 U/kg); 15 respondents prefer a continuous dose (range 2–8 U/kg/hr). For the post-operative dose, 11 reported a bolus dose, usually 25–50 u/kg; 17 reported using a continuous dose (range 2–8 U/kg/hr). Total number of days treated pre-operatively was reported as less than a day by 90% (18/20) respondents; days of post operative treatment ranged from 7–42 with a median of 13 days (n=22). Desired FVIII level ranged from 50–100% for 3– 15 days. Median number of days inpatient was 6 (n=21). Eight percent (2/25) reported adjuvant use of Amicar. Thirty-nine percent (11/28) reported use of thrombotic prophylaxis after surgery. For patients with high titer inhibitor levels, only 1 respondent reported a pre or post operative FVIII bolus or continuous dose. The reported rFVIIa dose in patients with high titer inhibitor, ranged from 90–270 mcg/kg (most reporting 90 mcg/kg), with most reporting a frequency of every 2 hours for a range of 1–14 days. The reported FEIBA dose in patients with high titer inhibitors ranged from 50–100 U/kg (most 75 U/kg) with most reporting a frequency of every 12 hours for 6–14 days. Sixty four percent (7/11) reported use of thrombotic prophylaxis after surgery.
Conclusion: Substantial practice variations exist in managing major surgeries in hemophilia patients, both with and without low and high titer inhibitors. This data will assist in developing future clinical trials and practice guidelines for management of hemophilia patients undergoing surgical procedures.
Disclosure: No relevant conflicts of interest to declare.
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