Baseline demographics
| CF centre | |||
| 2015–2016 | 2016–2017 | 2017–2018 | |
| Patients (n) | 134 | 135 | 141 |
| Age (years) (mean±SD) | 12.7±4.3 | 13.0±4.2 | 13.4±4.4 |
| Female, n (%) | 73 (54) | 72 (53) | 74 (52) |
| Caucasian, n (%) | 126 (94) | 126 (93) | 131 (93) |
| Hispanic, n (%) | 32 (24) | 31 (23) | 33 (23) |
| CFTR genotype, n (%) | |||
| F508del homozygous | 59 (44) | 47 (35) | 58 (41) |
| F508del heterozygous | 49 (37) | 59 (44) | 52 (37) |
| Other/other | 26 (19) | 29 (21) | 31 (22) |
| Diagnosis, n (%) | |||
| Identified by prenatally or by NBS | 32 (24) | 39 (29) | 45 (32) |
| History of meconium ileus | 33 (25) | 31 (23) | 31 (22) |
| BMI percentile (mean±SD) | 65.9±22.6 | 66.7±22.2 | 68.3±23.7 |
| Pancreatic insufficient, n (%) | 108 (81) | 109 (80) | 116 (82) |
| Microbiology, n (%) | |||
| P. aeruginosa positive | 49 (37) | 47 (35) | 48 (34) |
| MRSA positive | 17 (13) | 22 (16) | 13 (9) |
| Pulmonary function, median (IQR) | |||
| FEV1% predicted | 98 (89–113) | 99 (88–109) | 98 (88–109) |
| FEF25–75% predicted | 87 (72–110) | 87 (68–106) | 83 (66–111) |
| Pulmonary exacerbations, n (%) | |||
| Per cent with one or more Pex | 42 (31) | 38 (28) | 43 (30) |
| Primary airway clearance technique, n (%) | |||
| High-frequency chest wall oscillation | 109 (81) | 113 (84) | 116 (82) |
| Oscillating PEP | 24 (18) | 20 (15) | 25 (18) |
| Postural drainage with clapping (CPT) | NA | 2 (1) | NA |
| Maintenance therapy use, n (%) | |||
| Dornase alpha | 126 (94) | 123 (90) | 136 (96) |
| Hypertonic saline | 66 (49) | 73 (54) | 86 (61) |
| Azithromycin | 71 (53) | 66 (49) | 70 (50) |
| Ibuprofen | 7 (5) | 6 (4) | 6 (4) |
| Inhaled antibiotics | 64 (48) | 63 (46) | 63 (45) |
| Inhaled steroids | 39 (29) | 44 (32) | 40 (28) |
| CFTR modulator | 42 (31) | 59 (43) | 69 (48.9) |
| Comorbidities, n (%) | |||
| Asthma | 47 (35) | 49 (39) | 55 (39) |
| ABPA | 10 (8) | 11 (8) | 11 (8) |
| CFRD | 13 (10) | 15 (11) | 16 (11) |
| Impaired glucose tolerance | 24 (18) | 25 (18) | 30 (21) |
Best BMI for each patient during that study year.
Any culture positive in the study year.
Best value for each patient in study year.
ABPA, Allergic Bronchopulmonary Aspergillosis; BMI, body mass index; CFRD, Cystic Fibrosis Related Diabetes; CFTR, cystic fibrosis transmembrane regulator; CPT, Chest Physical Therapy; FEF25%–75%, forced expiratory flow at 25%–75%; FEV1, forced expiratory volume in the first second; MRSA, methicillin-resistant staph aureus; NBS, newborn screening; P. aeruginosa, Pseudomonas aeruginosa; PEP, Positive Expiratory Pressure.