Quality of life in cystic fibrosis

In recent years the role of behavioural and psychological variables in the management of patients with cystic fibrosis has been noted.1-3 This interest has grown from the observation that improved diagnosis and treatment mean that many patients can now anticipate burdensome daily management routines continuing for some decades. Recent studies in this and other publications have included demonstrations that the health related quality of life (HRQOL) of adults with cystic fibrosis is in some respects impaired,1 3 and that HRQOL is a valuable outcome measure in clinical trials.4 5 Such findings are in contrast with earlier literature which tended to shown little evidence of psychosocial impact in children6 and adolescents7 with cystic fibrosis. There is, however, an important distinction between the psychosocial outcomes identified in the 1980s, often using instruments intended to detect psychopathology, and more recent conceptualisations of HRQOL.

HRQOL is a multidimensional construct encompassing a range of impacts including physical, functional, social, and emotional components. Definitions frequently emphasise not only this breadth of impact but the subjectivity of reports8—that is, HRQOL differs from reports of psychological adjustment or functional status in targeting the importance of the impairment to patients, assessed through their own reports where possible. This reliance upon self reporting is one reason for the dearth of paediatric studies since clinicians and researchers have doubted the capacity of children to self report.9 Even supposing the …