Abstract

Objective: To determine individual/institutional practices at hemophilia treatment centers (HTCs) regarding management of severe hemophilia patients during minor surgery (e.g., central venous line insertion, cataract surgery, lymph node biopsy), both in the absence and presence of low and high titer inhibitors to Factor VIII (FVIII).Background: The management of hemophilia patients undergoing surgery has changed over the past 20 years as product choice and availability has evolved. Individual preference and experience may affect treatment decisions in the absence of data from clinical trials.Methods: The survey was distributed to both adult and pediatric hematologists at HTCs listed on the CDC website. 93 surveys were successfully emailed. Survey questions evaluated management of minor surgery, both in the absence and presence of a low and high titer inhibitor to FVIII.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 FVIII 50 U/kg (73%, 22/30; range 25–50 U/kg); only 3 respondents reported a continuous infusion (range 4–8 u/kg/hr). For the post-operative dose, most (n=22) prefer a bolus dose (25–50 U/kg); only 6 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 in 88% (23/26) respondents; days of post operative treatment ranged from 1–14 with a median of 4 days (n=22). Desired FVIII level ranged from 50–100% for 1–10 days. Fifty percent (15/30) reported adjuvant use of Amicar. For patients with high inhibitor levels no one reported a pre or post operative FVIII bolus or continuous infusion rate. Fifty percent (9/18) reported adjuvant Amicar use. The reported rFVIIa dose in patients with high titer inhibitors, ranged from 90–200 mcg/kg (most commonly 90 mcg/kg), usual frequency of every 2 hours, for a range of 1–10 days. The reported FEIBA dose in patients with high titer inhibitors, ranged from 50–100 U/kg (most commonly 75–100 U/kg with a frequency of every 12 hours, for 1–10 days.Conclusion: Substantial practice variations exist in managing minor surgeries in hemophilia patients, both with and without low and high titer inhibitors, as is evident with this survey.

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