OBJECTIVE: To analyze pulmonary function parameters and pharmacodynamic response to a
bronchodilator, as well as the prescription of bronchodilators, in cystic fibrosis
(CF) patients. METHODS: This was a retrospective cohort study involving patients 6-18 years of age,
diagnosed with CF, and followed at a referral center between 2008 and 2010. We
evaluated only those patients who were able to perform pulmonary function tests
(PFTs). We analyzed FVC, FEV1, and FEF25-75%, expressed as
percentages of the predicted values, prior to and after bronchodilator tests
(pre-BD and post-BD, respectively), in 312 PFTs. Repeated measures ANOVA and
multiple comparisons were used. RESULTS: The study included 56 patients, divided into two groups: those whose PFT results
spanned the 2008-2010 period (n = 37); and those whose PFT results spanned only
the 2009-2010 period (n = 19). In the 2008-2010 group, there were significant
reductions in post-BD FEV1 between 2008 and 2010 (p = 0.028) and
between 2009 and 2010 (p = 0.036), as was also the case for pre-BD and post-BD
FEF25 75% in all multiple comparisons (2008 vs. 2009; 2008 vs. 2010;
and 2009 vs. 2010). In the 2009-2010 group, there were no significant differences
between any of the years for any of the variables studied. Among the 312 PFTs,
significant responses to the bronchodilator occurred in only 24 (7.7%), all of
which were from patients for whom no bronchodilator had been prescribed during the
study period. CONCLUSIONS: In the CF patients studied, there was loss of pulmonary function, indicating
progressive lung disease, over time. The changes were greater for
FEF25-75% than for the other variables, which suggests the initial
involvement of small airways.