Health service utilisation data and rates of change of pulmonary function and weight in the year prior to and the year following commencement of lumacaftor/ivacaftor in 24 adults with cystic fibrosis

Year prior to commencementYear following commencementp-value
Health service utilisation data
 Pulmonary exacerbations requiring hospitalisation n3 (2–4)1.5 (1–2)0.0002
 Hospitalisation (on ward) days27 (18–58) (range 10–103)17 (10–25) (range 1–70)0.0002
 IVAB usage days45 (21–75) (range 10–280)27 (11–52) (range 1–167)0.0007
Annual rate of change in pulmonary function and weight
 FEV1 L−0.084±0.0460.027±0.0410.077
 FVC L−0.04±0.100.00±0.060.75
 FEF25–75% L−0.07±0.020.02±0.020.007
 FEV1 % pred−2.10±1.181.45±1.130.035
 FVC % pred−0.69±2.111.27±1.510.46
 FEF25–75% % pred0.07±0.200.07±0.050.97
 Weight kg−0.62±0.892.60±0.880.013

Health service utilisation data are presented as median (interquartile range); pulmonary function and weight data show mean change per year (slope±se) using linear regression modelling of all clinical measurements in each year-long period. IVAB: intravenous antibiotic; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; FEF25–75%: forced expiratory flow at 25–75% of FVC. p-value for difference between year prior and year following commencement (Wilcoxon signed-rank test for health service utilisation data; weighted linear regression analyses for pulmonary function and weight data).