Demographics and pre-infection characteristics of people with cystic fibrosis (CF)
| Total | Non-lung transplant | Lung transplanted# | ||||
| n (%)¶ | Missing | n (%)¶ | Missing | n (%)¶ | Missing | |
| Subjects n | 828 | 750 | 78 | |||
| Sex | 0 | 0 | 0 | |||
| Female | 427 (51.6) | 384 (51.2) | 43 (55.1) | |||
| Male | 401 (48.4) | 366 (48.8) | 35 (44.9) | |||
| Median age years | 24.0 | 0 | 23.0 | 0 | 34.5 | 0 |
| 0–11 years | 134 (16.2) | 134 (17.9) | 0 (0) | |||
| 12–17 years | 113 (13.6) | 111 (14.8) | 2 (2.6) | |||
| 18–29 years | 291 (35.1) | 268 (35.7) | 23 (29.5) | |||
| 30–39 years | 164 (19.8) | 135 (18.0) | 29 (37.2) | |||
| 40–49 years | 87 (10.5) | 67 (8.9) | 20 (25.6) | |||
| ≥50 years | 39 (4.7) | 35 (4.7) | 4 (5.1) | |||
| CFTR genotype | 0 | 0 | 0 | |||
| F508del/F508del | 218 (26.3) | 180 (24.0) | 38 (48.7) | |||
| F508del/other | 262 (31.6) | 236 (31.5) | 26 (33.3) | |||
| Other/Other | 348 (42) | 334 (44.5) | 14 (17.9) | |||
| BMI, z-score+ | 39 | 36 | 3 | |||
| < −2 | 54 (7.1) | 40 (5.8) | 14 (18.7) | |||
| −2–2 | 692 (90.6) | 631 (91.6) | 61 (81.3) | |||
| >2 | 18 (2.4) | 18 (2.6) | 0 (0) | |||
| Lung disease FEV1 % pred§ | 28 | 26 | 2 | |||
| Severe (≤40) | 76 (10.3) | 65 (9.8) | 11 (14.5) | |||
| Moderate (>40–70) | 221 (29.9) | 206 (31.0) | 15 (19.7) | |||
| Mild (>70) | 443 (59.9) | 393 (59.2) | 50 (65.8) | |||
| Pancreatic insufficiency | 660 (80.6) | 9 | 584 (78.8) | 9 | 76 (97.4) | 0 |
| CF-related diabetes | 206 (26.1) | 39 | 153 (21.4) | 34 | 53 (72.6) | 5 |
| ABPA | 47 (7.3) | 188 | 41 (6.9) | 158 | 6 (12.5) | 30 |
| Chronic liver GI disease | 163 (26.6) | 215 | 148 (26.7) | 196 | 15 (25.4) | 19 |
| Systemic arterial hypertension | 32 (5.1) | 199 | 20 (3.4) | 156 | 12 (34.3) | 43 |
| Treatment | ||||||
| CFTR modulator therapy | 260 (31.5) | 2 | 260 (34.8) | 2 | 0 (0.0) | 0 |
| Iva | 43 (5.2) | 43 (5.7) | 0 (0.0) | |||
| Lum/Iva | 72 (8.7) | 72 (9.6) | 0 (0.0) | |||
| Tez/Iva | 75 (9.1) | 75 (10.0) | 0 (0.0) | |||
| Elexa/Tez/Iva | 63 (7.6) | 63 (8.4) | 0 (0.0) | |||
| Yes, type unknown | 4 (0.5) | 4 (0.5) | 0 (0.0) | |||
| Yes, other | 3 (0.4) | 3 (0.4) | 0 (0.0) | |||
| Inhaled antibiotics | 332 (50.7) | 173 | 313 (50.6) | 131 | 19 (52.8) | 42 |
| Oral antibiotics | 234 (38.5) | 220 | 215 (37.3) | 174 | 19 (59.4) | 46 |
| Inhaled steroid | 318 (42.0) | 71 | 302 (43.7) | 59 | 16 (24.2) | 12 |
| Azithromycin | 307 (38.1) | 22 | 253 (34.7) | 21 | 54 (70.1) | 1 |
| DNase | 382 (58.3) | 173 | 377 (60.9) | 131 | 5 (13.9) | 42 |
| Hypertonic saline | 338 (51.4) | 171 | 334 (53.8) | 129 | 4 (11.1) | 42 |
| Flu vaccine | 207 (57.8) | 470 | 180 (55.6) | 426 | 27 (79.4) | 44 |
| Microbiology | ||||||
| Pseudomonas aeruginosa | 346 (43.4) | 31 | 313 (42.6) | 15 | 33 (53.2) | 16 |
| Staphylococcus aureus | 420 (57.7) | 100 | 403 (59.0) | 67 | 17 (37.8) | 33 |
| Burkholderia cepacia complex | 29 (4.4) | 168 | 28 (4.5) | 122 | 1 (3.1) | 46 |
| MRSA | 65 (9.3) | 126 | 63 (9.5) | 84 | 2 (5.6) | 42 |
| Non-tuberculous mycobacteria | 28 (5.2) | 292 | 28 (5.5) | 242 | 0 (0.0) | 50 |
| Stenotrophomonas maltophilia | 65 (8.8) | 90 | 63 (9.1) | 61 | 2 (4.1) | 29 |
| Achromobacter species | 60 (8.1) | 89 | 54 (7.8) | 61 | 6 (12.0) | 28 |
| Aspergillus colonisation | 102 (14.0) | 99 | 94 (13.8) | 71 | 8 (16.0) | 28 |
CFTR: cystic fibrosis transmembrane conductance regulator; BMI: body mass index; FEV1 % pred: per cent predicted forced expiratory volume in 1 s; ABPA: allergic bronchopulmonary aspergillosis; GI: gastrointestinal; Iva: ivacaftor; Lum: lumacaftor; Tez: tezacaftor; Elexa: elexacaftor; MRSA: methicillin-resistant Staphylococcus aureus. #: 10 recipients of other solid organ transplants were included in this group (7 liver, 2 kidney, 1 unspecified). ¶: percentages are computed excluding missing data. +: BMI z-score was only calculated for patients aged 2 years and over, using Centers for Disease Control and Prevention reference values [40]. §: FEV1 % pred was only calculated for patients aged 6 years and over.