QUALITY-OF-LIFE IN ADULTS WITH CYSTIC-FIBROSIS

Background - Cystic fibrosis is an inherited condition with a high mor tality and morbidity. The aims of this study were to assess quality of life in a population of adults with cystic fibrosis, to compare quali ty of life with published scores from a and other patient examine the relation between quality of life and other measured clinical variables . Methods - Patients over 16 years of age attending an adult cystic fi brosis outpatient clinic were surveyed at a time when they were clinic ally stable. A self-complete questionnaire was administered which comp rised the Nottingham Health Profile (NHP) together with six additional questions related to cystic fibrosis. Results - Completed questionnai res were obtained from 240 subjects (100 women) of median age 26 years (range 16-56). Mean (SD) forced expiratory volume in one second (FEV, ) was 49 (26)% predicted, forced vital capacity (FVC) was 68 (26)% pre dicted, and the FEV,:FVC ratio was 59 (16)%. In this cross sectional s tudy different patterns of perceived quality of Life were seen in men and women. In part 1 of the NHP there was an age related trend compare d with norms in men, with more distress/disability in the dimensions o f emotion, sleep, and social isolation in the older age groups. In wom en there was no age related trend in the degree of distress/disability compared with norms. The mean score was different from norms in the d imensions of pain, emotion and sleep. For the patients with cystic fib rosis as a whole the scores in part 1 were comparable with published s cores of patients with minor non-acute conditions. Scores in part 2 of the NHP for men were different from norms in six of the seven areas o f daily living (all except home life). For women the scores were diffe rent from norms in the areas of looking after the home, social life, h obbies, and holidays. There were correlations between several of the q uality of life dimensions and other measured variables such as FEV(1), breathlessness score, and the time spent on home treatment. Conclusio ns - Men and women with cystic fibrosis have different patterns of per ceived quality of Life, and there is an age related trend of perceived quality of life in men in some dimensions. Quality of life scores in this group, as assessed by the NHP, are similar to those reported in s ubjects with minor non-acute conditions.