title: Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung's disease.
abstract: OBJECTIVE
  The first objective was to compare the quality of life of adult patients with anorectal malformations (ARM) or Hirschsprung's disease (HD) and to compare both groups with healthy people. And the second objective was to examine the factors that affect the quality of life of patients with ARM or HD, using a theoretical model in which patients' background characteristics explain quality of life via mediating disease-specific functioning and psychosocial functioning.
  
  
  METHODS
  Three hundred and forty-one patients completed a questionnaire, which assessed sociodemographic characteristics, disease-specific and psychosocial functioning, and quality of life. Clinical factors were extracted from the medical records.
  
  
  RESULTS
  Patients with ARM or Hirschsprung's disease did not differ in their quality of life. Compared to healthy people, both patient groups reported more limitations in their "overall" physical quality of life, but only patients with ARM reported impaired quality of life on several specific domains (e.g., physical role-functioning, pain). The model was largely accepted. Most striking were the strong effects of the psychosocial functioning factors in contrast to weak effects of the disease-specific "constipation" and "fecal continence" factors.
  
  
  CONCLUSIONS
  The quality of life of patients with ARM or Hirschsprung's disease was found to be comparable. Compared to healthy people, both patient groups encountered "overall" physical health problems, but only patients with ARM reported additional pain and limitations in role functioning due to physical problems. It appeared that psychosocial functioning had the most important effect on the quality of life of patients with ARM or Hirschsprung's disease, while fecal incontinence and constipation had almost no effect on their quality of life.
authors.affiliation: Division of Medical Psychology of the University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Esther E
authors.initials: EE
authors.lastname: Hartman
authors.forename: Frans J
authors.initials: FJ
authors.lastname: Oort
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Aronson
authors.forename: Marianne J G
authors.initials: MJ
authors.lastname: Hanneman
authors.forename: David C
authors.initials: DC
authors.lastname: van der Zee
authors.forename: Paul N M A
authors.initials: PN
authors.lastname: Rieu
authors.forename: Gerard C
authors.initials: GC
authors.lastname: Madern
authors.forename: Zacharias J
authors.initials: ZJ
authors.lastname: De Langen
authors.forename: L W Ernest
authors.initials: LW
authors.lastname: van Heurn
authors.forename: M
authors.initials: M
authors.lastname: van Silfhout-Bezemer
authors.forename: N
authors.initials: N
authors.lastname: Looyaard
authors.forename: Mirjam A G
authors.initials: MA
authors.lastname: Sprangers
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001003:Anal Canal / Q000002:abnormalities; D016022:Case-Control Studies; D004065:Digestive System Abnormalities / Q000175:diagnosis* / Q000523:psychology*; D005260:Female; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011584:Psychology; D011788:Quality of Life; D012007:Rectum / Q000002:abnormalities*; D012016:Reference Values; D012307:Risk Factors; D012737:Sex Factors; D018434:Sickness Impact Profile; D011795:Surveys and Questionnaires