QUALITY-OF-LIFE IN PATIENTS WITH CYSTIC-FIBROSIS AND THEIR PARENTS - WHAT IS IMPORTANT BESIDES DISEASE SEVERITY

Background-Cystic fibrosis is the most common inherited disease with a fatal outcome in industrialised nations. With the improvement in life expectancy, supporting patients and their families in adapting to lif e with this chronic progressive disease has become increasingly import ant. The aim of the present study was to investigate the relationship between health related quality of life (HRQOL) in this population, sev erity of disease, and cognitive/behavioural factors such as subjective health perception and ways of coping. Methods-A sample of 89 adolesce nt and adult patients with cystic fibrosis and 125 parents of younger patients with cystic fibrosis completed questionnaires on health relat ed quality of life and on ways of coping with the illness. Parents wer e asked to fill out the questionnaires regarding their own quality of life and coping. Multiple regression analyses were performed to examin e the relationship between different predictor variables and quality o f Life. Results-After accounting for the impact of disease severity an d hours of treatment per day, the subjective health perception of pati ents significantly explained variance in their quality of life. Ways o f coping were also significantly correlated with HRQOL. In parents the most important factor in explaining variance of HRQOL seems to be the coping style, whereas disease severity of the child and subjective he alth perception did not show any influence. Conclusions-The findings s upport the important role of cognitive and behavioural factors in spec ific subjective health perception and ways of coping in the adaptation to this severe chronic disease, both in patients themselves and in pa rents. The results call for a careful assessment of issues of coping a nd professional support for families of patients with cystic fibrosis in the early course of disease.