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 A cftr gene 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 severe cftr mutations, 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.
- Cystic Fibrosis
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Contributors LB-E planning of the study concept, data collection, statistical analysis and interpretation of the data, drafting of the article. AB-B initial planning of the study, data collection, critically reviewing of the article. LH initial planning of the study, critically reviewing of the article, drafting of the article. BM planning of the study concept, drafting/critically reviewing of the article. KS planning of the study concept, statistical analysis and interpretation of the data, drafting/critically reviewing of the article, PI. All authors approved the final version published, and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Funding The study was funded from Swedish Cystic Fibrosis Foundation (Riksförbundet Cystisk Fibros, RfCF), and Division of Psychology, Karolinska University Hospital is gratefully acknowledged.
Competing interests None declared.
Ethics approval Central Ethical Review Board, Stockholm, ethical permission, nr 2007/1266-31.
Provenance and peer review Not commissioned; externally peer reviewed.
Data sharing statement No additional data are available.