title: A survey of perioperative management of sickle cell disease in North America.
abstract: 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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Massachusetts General Hospital, Boston, MA 02114, USA. pfirth@partners.org
authors.forename: Paul G
authors.initials: PG
authors.lastname: Firth
authors.forename: Kristen N
authors.initials: KN
authors.lastname: McMillan
authors.forename: Charles M
authors.initials: CM
authors.lastname: Haberkern
authors.forename: Myron
authors.initials: M
authors.lastname: Yaster
authors.forename: Michael A
authors.initials: MA
authors.lastname: Bender
authors.forename: Salvatore R
authors.initials: SR
authors.lastname: Goodwin
mesh_terms: D000755:Anemia, Sickle Cell / Q000628:therapy*; D000758:Anesthesia; D001803:Blood Transfusion; D002648:Child; D005440:Fluid Therapy; D019538:Health Care Surveys; D006801:Humans; D007430:Intraoperative Care; D009656:North America; D019990:Perioperative Care; D011300:Preoperative Care; D012017:Referral and Consultation