Quality of Life in Cystic Fibrosis: the Impact of Gender, General Health Perceptions and Disease Severity

Background: Disease progression in cystic fibrosis (CF) is marked by deterioration across a number of physiological systems. In addition, there is evidence that females have a worse prognosis than males. The current work assesses the impact of both these factors on health related quality of life (HRQoL).

Methods: Two hundred and twenty-three adolescents and adults completed the cystic fibrosis quality of life (CFQoL) questionnaire with a further 185 approached and not responding by non-completion of the questionnaire. The CFQoL is divided into nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career. Measurement of objective clinical status included, body mass index (BMI), and percentage of predicted forced expiratory volume in one second (FEV1). General health perceptions (GHP) were also measured.

Results: Patients were sub-divided by gender and disease severity (mild > 70% FEV1, moderate 40-69% and severe < 40%). Factorial analysis of variance indicated significant main effects for FEV1 (F = 587.98, P < or = 0.001) and BMI (F = 17.29, P < or = 0.001) as a function of disease severity. Post hoc tests revealed significant two-group differences for FEV1 and BMI between disease severity groups. No differences were observed for gender across FEV1 or BMI. Differences emerged across most CFQoL domains for disease severity, with the exception of concerns for the future, which was consistently low throughout. Gender differences emerged for chest symptoms, emotional functioning, concerns for the future, body image and career. With the exception of body image, females exhibited poorer HRQoL. Pearson correlations indicated that females' perception of health was more closely related to clinical status than males.

Conclusions: Disease severity has an impact on HRQoL in adolescents and adults with CF. Some differences emerged between males and females, with females generally reporting poorer HRQoL. Evidence indicated that males and females perceived their health status differently, with females having a more accurate perception of objective clinical health status.