title: Associations between genetics, medical status, physical exercise and psychological well-being in adults with cystic fibrosis.
abstract: BACKGROUND
  Cystic fibrosis (CF) is the most common autosomal recessive, life-shortening disease among people of European origin. Type of genetic mutation and regular physical exercise has an impact on clinical outcome. This cross-sectional study explores the associations between genetics, medical status, physical exercise and psychological well-being in adult patients with CF.
  
  
  METHODS
  Adult patients with CF (N=68; mean age: 32.2; range 18-67 years; 46% women) completed the Cystic Fibrosis Questionnaire-Revised and Hospital Anxiety Depression Scale. Measures about lung function/forced expiratory volume in 1 s per cent predicted, body mass index, physical working capacity, immunoglobulin G, CF Transmembrane Conductance Regulator (CFTR) mutations, and physical exercise were obtained. structural equation modelling was used to fit models to data.
  
  
  RESULTS
  Acftrgene mutation×age interaction effect indicates a psychological disadvantage increasing with age of having more severe CFTR mutations; >65% of the effect is mediated by medical status. Physical exercise has a positive effect on psychological well-being, but >75% of the effect is mediated by medical status.
  
  
  CONCLUSIONS
  Psychological well-being decreases with age in patients with more severecftrmutations, to a large extent due to a parallel deterioration of medical status. Physical exercise has a positive effect on psychological well-being if resulting in better health only. To manage the complexity of these patients' needs, the CF-care should emphasise a holistic approach and offer individualised exercise/treatment programmes and psychological competence.
authors.affiliation: Department of Clinical Neuroscience, Division of Psychology, Karolinska Institutet, Solna, Sweden; Karolinska University Hospital, Stockholm CF-center, Stockholm, Sweden.
authors.forename: Lena
authors.initials: L
authors.lastname: Backström-Eriksson
authors.affiliation: Karolinska University Hospital, Stockholm CF-center , Stockholm , Sweden.
authors.forename: Agneta
authors.initials: A
authors.lastname: Bergsten-Brucefors
authors.affiliation: Karolinska University Hospital, Stockholm CF-center, Stockholm, Sweden; Department of Clinical Science, Intervention and Technology, Division of Pediatrics, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Lena
authors.initials: L
authors.lastname: Hjelte
authors.affiliation: Department of Clinical Neuroscience, Division of Psychology , Karolinska Institutet , Solna , Sweden.
authors.forename: Bo
authors.initials: B
authors.lastname: Melin
authors.affiliation: Department of Clinical Neuroscience, Division of Psychology , Karolinska Institutet , Solna , Sweden.
authors.forename: Kimmo
authors.initials: K
authors.lastname: Sorjonen
keywords: Cystic Fibrosis; Exercise; Psychology