ABSTRACT
OBJECTIVES: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL. PATIENTS AND METHODS: A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age). The clinical characteristics (disease severity, presence of congenital anomalies) were extracted from medical records. RESULTS: More than 50% (316, 64%) of patients with anorectal malformations or Hirschsprung disease completed the questionnaire. On average, children and adolescents in both patient groups reported no differences in QL domains compared with the reference groups. However, standard deviations revealed considerable individual variation, indicating the presence of patients with high levels of QL as well as patients with low levels of QL. Children and adolescents in both patient groups reported psychosocial problems in all domains, compared with the reference groups. Females, older patients, and those with a severe form of the disease reported lower levels of perceived self-competence and global disease-specific functioning, which in turn predicted QL. CONCLUSIONS: Our results should alert clinicians to patients who are at risk for QL problems and may therefore be in need of extra care. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.
Subject(s)
Abnormalities, Multiple/physiopathology , Abnormalities, Multiple/psychology , Anal Canal/abnormalities , Hirschsprung Disease/physiopathology , Hirschsprung Disease/psychology , Quality of Life , Rectum/abnormalities , Adolescent , Age Factors , Child , Female , Humans , Male , Risk Factors , Self Concept , Sex Factors , Sickness Impact Profile , Surveys and QuestionnairesABSTRACT
OBJECTIVES: The purpose of this work was to examine changes in quality of life, disease-specific functioning, and psychosocial competencies of children and adolescents (8-16 years of age) with anorectal malformations or Hirschsprung disease and to identify predictors of change in quality of life by testing an explanatory model in which background variables explained changes in quality of life via changes in disease-specific functioning and psychosocial competencies. METHODS: Questionnaires were administered to 129 patients with anorectal malformations and 121 patients with Hirschsprung disease within a 3-year interval. Clinical and sociodemographic background variables were measured on the first occasion. Quality of life (physical and mental), disease-specific functioning (defecation-related), and psychosocial competencies (self-esteem, athletic competencies, and school attitude) were measured on both occasions. RESULTS: Patients improved in disease-specific functioning and mental quality of life. Changes in quality of life were indeed explained by the explanatory model. Among other things, the results indicated that patients with a severe form of the disease or with additional congenital diseases showed worsening of school attitude, which in turn affected change in mental quality of life negatively. CONCLUSIONS: Children and adolescents with anorectal malformations or Hirschsprung disease reported better quality of life over time. To improve and maintain an optimal level of children's and adolescents' quality of life, it is important to direct treatment both to reducing symptoms and enhancing psychosocial competencies, in particular by paying attention to school attitude.
