title: Psychological and family functioning and quality of life in adolescents with cystic fibrosis.
abstract: BACKGROUND
  The life expectancy of individuals with CF has increased to 33 years. Thus, issues such as quality of life and psychological well-being, previously thought to be of lesser importance than physical well-being, are now recognised as significant factors. This study examined the interrelationships between quality of life, family functioning, individual psychopathology and optimism of adolescents with CF.
  
  
  METHODS
  Adolescents attending the CF clinic completed a number of questionnaires. Quality of Life was measured using the Cystic Fibrosis Questionnaire, family functioning by the Family Environment Scale (3rd edition), general psychopathology with the Symptom Checklist-90-Revised and optimism for the future by the Hunter Opinions and Personal Expectations Scale. Disease severity was assessed using the Shwachman score and spirometry at the time of questionnaire completion.
  
  
  RESULTS
  The level of psychopathology (12.5% of those 13 years and over) in the group was lower than that reported for young people in Australia (15-20%). The results indicated that young people with a delayed diagnosis and those who are alienated from their families may be in need of additional psychosocial support. The group was hopeful and positive about their future and these attributes were independent of clinical measures of disease severity. In general, these young people scored relatively highly on the quality of life scale. For example the mean standardised score for physical functioning was 70 points, for respiratory symptoms was 63 points and for emotional state was 78 points. Increased levels of psychopathology and lack of hope for the future were however associated with lower ratings on a number of quality of life measures. Family cohesiveness, expressiveness and organization were associated with better psychological functioning in the young people.
  
  
  CONCLUSIONS
  Adolescents with CF appear to be a psychologically well functioning and well-adjusted group. These findings support the importance of a more sophisticated model of well-being for adolescents with CF, which explores the young person's views on their quality of life and wider support frameworks rather than relying solely on measures of physical health to gauge well-being.
authors.affiliation: Department of Adolescent Medicine, The Children's Hospital at Westmead, New South Wales, Australia.
authors.forename: Janina E
authors.initials: JE
authors.lastname: Szyndler
authors.forename: Susan J
authors.initials: SJ
authors.lastname: Towns
authors.forename: Peter P
authors.initials: PP
authors.lastname: van Asperen
authors.forename: Karen O
authors.initials: KO
authors.lastname: McKay
mesh_terms: D000293:Adolescent; D003550:Cystic Fibrosis / Q000523:psychology*; D005190:Family / Q000523:psychology*; D006801:Humans; D011788:Quality of Life; D012944:Social Support