Quality of life in adolescents and adults with cystic fibrosis

Introduction: As life expectancy increases in cystic fibrosis (CF), it becomes more important to recognize the quality of life (QoL) in adolescence and adulthood and to identify factors influencing QoL. Methods: 70 adolescents and adults with CF from four southwest-german outpatient clinics participated in this cross-sectional study. They answered three generic QoL- questionnaires (SF 36 Health Survey, Profil der Lebensqualitat chronisch Kranker, Fragebogen zur Lebenszufriedenheit) and the Trier Coping Questionnaire, additional clinical and sociodemographic data were collected. Results: Compared to healthy peers the QoL-level in the study group depends strongly on the method, because of different QoL-concepts. The main tendency shows minor restrictions in overall and health related QoL in the CF-patients. As expected deficits can be found primary in physical function. The subgroup aged 16-25 years has additional psychosocial problems. Relevant factors associated with overall QoL were the coping-styles rumination (r=-0.40) and seeking social support (r=0.48), the time-consumption for daily therapy (r=-0.41), lung function (FEV1%, r=0.47) and Body-Mass-index (r=0.37). 56% of the QoL-variance can be explained only by the coping and the time-consumption for therapy. Conclusion: Improvement of QoL in case of CF in adolescence and adulthood cannot be necessarily expected by intensifying therapy. In planning the individual therapy and developing new therapy strategies the limited coping ressources and the limited time budget of the patient has to be considered. Psychosocial rehabilitation and support for older CF-patients has to be improved to strengthen their coping ability.