Objective
To characterize spirometry and height changes in cohorts of 6-year-olds with cystic fibrosis (CF).
Study design
Global Lung Initiative (GLI) forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC) and FEV1/FVC and CDC height-for-age (HFA) Z-scores were generated for 6-year-olds in the from the CF Foundation Patient Registry (CFFPR) each year from 1994 and 2012. Z-score mean differences were analyzed by t-test and time trends of means by least squares regression for all children and for subgroups (sex, F580del mutation genotype, Medicaid insurance, and prenatal/newborn screening identification). Z-score distributions were compared by two-sample Kolmogorov-Smirnov tests.
Results
11,670 children with CF were studied, of whom 50.5% were males, 50.2% had the F508del/F508del genotype, and 46.6% were insured by Medicaid. Mean HFA, FEV1 and FVC Z-scores increased significantly over the period in the entire population and all subgroups (P<0.001), but FEV1/FVC Z-scores were below normal and did not change significantly. In 2012, children identified by screening had significantly higher mean HFA (P=0.002), FEV1 (P<0.001) and FVC (P<0.001) Z-scores than those not screened, with 90% of FVC and 71.4% of FEV1 Z-scores greater than predicted by the Normal distribution; FEV1/FVC Z-scores were not different between screening groups.
Conclusions
Consistent, significant increases in HFA, FEV1, and FVC occurred from 1994–2012, but FEV1/FVC, a measure of airway obstruction, did not appreciably change. FVC and FEV1 Z-score distributions suggest that normative equation reference populations under-predict lung volumes of children with CF, but the reason(s) for this remain unclear.