TABLE 1

Baseline characteristics (n=401)

CFTR modulator treatment, n (%)
 Lumacaftor/ivacaftor (LUM/IVA)401 (100)
 Transition to tezacaftor/ivacaftor (TEZ/IVA)208 (51.9)
 Time (years) to transition from LUM/IVA to TEZ/IVA, mean±sd2.0±0.6
Death, n (%)2 (0.5)
Lung transplantation, n (%)11 (2.7)
Sex, n (%)
 Male233 (58.1)
 Female168 (41.9)
Age (years), median (IQR)24.5 (18.0–31.5)
Age 12–18 years, n (%)116 (28.9)
Age >18 years, n (%)285 (71.1)
Missing, n (%)0
FEV1 % pred, mean±sd70.5±23.4
FEV1 <40% pred, n (%)51 (12.7)
FEV1 40–70% pred, n (%)128 (31.9)
FEV1 70–90% pred, n (%)129 (32.2)
FEV1 ≥90% pred, n (%)90 (22.4)
Missing, n (%)3 (0.8)
BMI adults (kg·m−2) ≥19 years, mean±sd21.4±2.5
 Missing, n (%)5 (1.8)
BMI Z-score children 12–19 years, mean±sd−0.5±0.8
 Missing, n (%)0
Received intravenous antibiotic treatment, n (%)
 Yes149 (37.3)
 No201 (50.0)
 Missing51 (12.7)
Duration of intravenous antibiotic treatments in days, median (IQR)23 (17–42)
Pseudomonas aeruginosa sputum culture status, n (%)
 Positive179 (44.6)
 Negative209 52.2)
 Missing13 (3.2)
Staphylococcus aureus sputum culture status, n (%)
 Positive196 (48.9)
 Negative192 (47.9)
 Missing13 (3.2)
Cystic fibrosis-related diabetes, n (%)
 Yes156 (38.9)
 No234 (58.4)
 Missing11 (2.7)
Cystic fibrosis-related liver disease, n (%)
 Yes89 (22.2)
 No255 (63.6)
 Missing57 (14.2)

Definitions: age was calculated at the date of CFTR modulator initiation (baseline). FEV1 % pred, BMI, BMI Z-score, number and duration of received intravenous antibiotic treatment, Pseudomonas aeruginosa and Staphylococcus aureus sputum culture status, CF-related diabetes and CF-related liver disease status reported at the last annual measurement preceding CFTR modulator initiation. The median duration of intravenous treatments was calculated for the 149 participants who received intravenous antibiotics in the last year prior to CFTR modulator initiation. BMI: body mass index. CFTR: cystic fibrosis transmembrane conductance regulator; FEV1 % pred: % predicted forced expiratory volume in 1 s; IQR: interquartile range.