A survey of perioperative management of sickle cell disease in North America

Paul G. Firth, Kristen N. McMillan, Charles M. Haberkern, Myron Yaster, Michael A. Bender, Salvatore R. Goodwin

Research output: Contribution to journalArticlepeer-review

Abstract

Summary Background: Children with sickle cell disease frequently undergo surgical procedures that are associated with acute exacerbations of the disease. Current perioperative management practices are unclear. Objectives: We aimed at describing the current management. Methods: We conducted an electronic survey of North American members of the Society for Pediatric Anesthesia, in which we asked about their perioperative management of sickle cell disease. Results: The response rate to valid addresses was 25% (n = 510/2006). In four scenarios, (a patient with mild disease undergoing a minor procedure; a patient with mild disease undergoing a more invasive procedure; a patient with severe disease undergoing a minor procedure; and a patient with severe disease undergoing a more invasive procedure) 80%, 38%, 27%, and 16% of respondents, respectively, would rely on oral fluids to hydrate patients during the preoperative fast, while 13%, 34%, 44%, and 59%, respectively, would use intravenous fluid. For the same four scenarios, 64%, 28%, 33%, and 10%, respectively, would not transfuse patients in an attempt to prevent sickle cell exacerbations, while 17%, 49%, 36%, and 51%, respectively, would transfuse to a hemoglobin concentration of 10 g·dl-1. The tendencies to administer preoperative intravenous fluid and to transfuse blood increased with disease severity and procedure invasiveness (P < 0.001). Although 89% felt comfortable managing patients with sickle cell disease, 73% thought an advisory statement on optimal perioperative management was needed. Conclusions: There is a wide variation in the management of children with sickle cell disease. Clinicians differentiate management based on disease severity and procedure type.

Original languageEnglish (US)
Pages (from-to)43-49
Number of pages7
JournalPaediatric anaesthesia
Volume21
Issue number1
DOIs
StatePublished - Jan 2011

Keywords

  • acute chest syndrome
  • anesthesia
  • hemoglobinopathy
  • pain crisis
  • sickle cell disease
  • surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine

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