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.
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
Main outcome measures: Physical and
psychosocial summary scores and individual scale scores for the Child
Health Questionnaire and Short Form-36.
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