Abstract

BackgroundCystic fibrosis (CF) is characterized by recurrent respiratory infections and progressive lung disease. Whereas exercise may contribute to preserving lung function, its benefit is difficult to ascertain given the selection bias of healthier patients being more predisposed to exercise. Our objective was to examine the role of self-reported exercise with longitudinal lung function and body mass index (BMI) measures in CF.MethodsA total of 1038 subjects with CF were recruited through the U.S. CF Twin-Sibling Study. Questionnaires were used to determine exercise habits. Questionnaires, chart review, and U.S. CF Foundation Patient Registry data were used to track outcomes.ResultsWithin the study sample 75% of subjects self-reported regular exercise. Exercise was associated with an older age of diagnosis (p = 0.002), older age at the time of ascertainment (p < 0.001), and higher baseline FEV1 (p = 0.001), but not CFTR genotype (p = 0.64) or exocrine pancreatic function (p = 0.19). In adjusted mixed models, exercise was associated with both a reduced decline in FEV1 (p < 0.001) and BMI Z-score (p = 0.001) for adults, but not children aged 10–17 years old.ConclusionsIn our retrospective study, self-reported exercise was associated with improved longitudinal nutritional and pulmonary outcomes in cystic fibrosis for adults. Although prospective studies are needed to confirm these associations, programs to promote regular exercise among individuals with cystic fibrosis would be beneficial.Electronic supplementary materialThe online version of this article (doi:10.1186/1471-2466-14-159) contains supplementary material, which is available to authorized users.

Highlights

  • Cystic fibrosis (CF) is characterized by recurrent respiratory infections and progressive lung disease

  • The group that reported no exercise had a younger age of diagnosis (1.5 ± 4.7 years) than the exercise group (2.8 ± 6.0 years; p = 0.002); no differences were seen with exocrine pancreatic function status (p = 0.19) or CFTR genotype(p = 0.64)

  • Using mixed models with interaction terms adjusted for baseline lung function, we found that adults who reported exercising had a less rapid decline in lung function and body mass index (BMI) Z-scores compared to their nonexercising peers

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Summary

Introduction

Cystic fibrosis (CF) is characterized by recurrent respiratory infections and progressive lung disease. A number of factors have been shown to have an impact on lung function in CF, including secondhand smoke exposure [3,4,5], health insurance [6,7,8,9], household income [10], adherence [11], air pollution [12], and ambient temperature [13], only two of these are readily modifiable: secondhand smoke and adherence. Another potential modifiable factor to improve outcomes may be exercise.

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