Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis

Objective: To compare the health-related quality of life (HRQOL) of people with cystic fibrosis (CF) to the general population, and to determine the relationship between HRQOL and clinical and demographic factors. Design: Cross-sectional analysis of observational cohort. Setting: Outpatient clinics of a Midwestern CF center. Subjects: One hundred sixty-two subjects with CF aged 5 to 45 years. Main outcome measures: Physical and psychosocial summary scores and individual scale scores for the Child Health Questionnaire and Short Form-36. Results: Compared with the general population, people with CF reported similar scores for most psychosocial measures, but lower scores for most physical measures, with the lowest scores on the general health perceptions scale. In multivariable analyses, pulmonary exacerbations in the past 6 months were strongly associated with the physical (p = 0.001) and psychosocial (p = 0.0003) scores. The physical score fell, on average, 6 points per exacerbation and the psychosocial score fell 3 points. Lung function, nutrition, 6-min walk distance, age, gender, and insurance status were not significantly associated with HRQOL in this study population. Those who declined to participate had significantly lower FEV₁ percent predicted and nutritional indexes. Our findings may not be generalizable to the entire CF population. Conclusion: Recent pulmonary exacerbations have a profound negative impact on HRQOL that is not explained by differences in lung function, nutritional status, or demographic factors.