title: Deaths due to choking in Prader-Willi syndrome.
abstract: Prader-Willi syndrome (PWS) is the most common known syndromic cause of life-threatening obesity, yet few studies have examined the causes of death in PWS. The objective of this study was to examine the contribution of choking leading to mortality in PWS. In 1999, a brief survey was made available from the Prader-Willi Syndrome Association (USA) bereavement program, which documented demographic data and causes of death. Families were subsequently offered the opportunity to fill out a detailed questionnaire and additional forms to release medical records. Demographic information was available on 178 deceased individuals with PWS, and cause of death available on 152 individuals. Fifty-four families completed questionnaires. Of the deceased individuals with completed questionnaires, 34% reported a history of choking. Choking was listed by familial report as the cause of death in 12 (7.9%) of 152 subjects with an average age of 24 years (range 3-52 years; median 22.5 years) at death from choking. Only two of these individuals were less than 8 years of age. The data suggest that risks associated with choking are different in the PWS population compared with others. Potential causes of increased choking in PWS include poor oral/motor coordination, poor gag reflex, hypotonia, hyperphagia, decreased mastication, and voracious feeding habits. We recommend implementation of preventive measures and education for families and group home care providers for all individuals with PWS including the Heimlich maneuver, supervised meals, better food preparation, and diet modification to avoid high-risk choking items.
authors.affiliation: Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, Utah 84132, USA. david.stevenson@hsc.utah.edu
authors.forename: David A
authors.initials: DA
authors.lastname: Stevenson
authors.forename: Janalee
authors.initials: J
authors.lastname: Heinemann
authors.forename: Moris
authors.initials: M
authors.lastname: Angulo
authors.forename: Merlin G
authors.initials: MG
authors.lastname: Butler
authors.forename: Jim
authors.initials: J
authors.lastname: Loker
authors.forename: Norma
authors.initials: N
authors.lastname: Rupe
authors.forename: Patrick
authors.initials: P
authors.lastname: Kendell
authors.forename: Carol L
authors.initials: CL
authors.lastname: Clericuzio
authors.forename: Ann O
authors.initials: AO
authors.lastname: Scheimann
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011218:Prader-Willi Syndrome / Q000401:mortality*; D014839:Vomiting / Q000453:epidemiology