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A Jehovah's Witness child with hemophilia B and factor IX inhibitors undergoing scoliosis surgery: [Chirurgie de la scoliose chez un enfant Temoin de Jehovah souffrant d'hemophilie B et presentant des inhibiteurs du facteur IX].

Chau A, Wu J, Ansermino M, Tredwell S, Purdy R.
Staff Anesthesiologist, Department of Pediatric Anesthesia, Room 1L7, British Columbia’s Children’s Hospital, 4480 Oak Street, Vancouver, British Columbia V6H 3V4, Canada. rpurdy@cw.bc.ca.

PURPOSE: To describe the successful perioperative hemostatic management of a Jehovah's Witness patient with hemophilia B and anaphylactic inhibitors to factor IX, undergoing scoliosis surgery.

CLINICAL FEATURES: A 14 (1/2)-yr-old boy with severe hemophilia B who had a history of anaphylactic inhibitors to factor IX was scheduled to undergo corrective scoliosis surgery. He was initially started on epoetin alfa and iron supplementation to maximize preoperative red cell mass. Additionally, he was placed on a desensitization protocol of recombinant coagulation factor IX (rFIX) and was then treated with activated recombinant coagulation factor VII (rFVIIa) during the postoperative period. Tranexamic acid was given concomitantly. The intraoperative blood loss was approximately 350 mL. The nadir hemoglobin concentration was 111 g.L(-1) on postoperative days one and two. On postoperative day 11, the patient was stable and discharged home with a hemoglobin of 138 g.L(-1). He did not require blood transfusion and no adverse events were observed.

CONCLUSIONS: The use of rFIX, rFVIIa, erythropoetin, iron, and tranexamic acid before, during and after scoliosis surgery may be a viable and safe option for hemophilia patients with inhibitors, who refuse blood products.

PMID: 18166748 [PubMed - as supplied by publisher]

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Source: A Jehovah's Witness child with hemophilia B and fa...[Can J Anaesth. 2008] - PubMed Result