Baseline demographics of the 35 study patients compiled from clinic letters at the time of commencing ivacaftor
| Demographic (n=35) | Mean±SD | Median (IQR) |
| Age at starting ivacaftor (years) | 29.06±9.54 | 27.9 (21–33.2) |
| Age at CF diagnosis (years) | 2.94±3.88 | 2 (0–4.5) |
| Baseline BMI (kg/m2) | 21.85±3.46 | 22.1 (19–23.5) |
| Baseline FEV1 (% predicted) | 58.41±22.64 | 63 (42–75.2) |
| N (%) | ||
| Male sex | 20 (57) | |
| Exocrine pancreatic insufficiency | 33 (94) | |
| CF-related diabetes mellitus | 9 (26) | |
| Osteoporosis/bone disease | 5 (15) | |
| ABPA | 3 (9) | |
| CKD | 1 (3) | |
| CF-related liver disease | 1 (3) | |
| Baseline therapies | N (%) | |
| Long-term oral macrolide antibiotic | 28 (90) | |
| Inhaled/nebulised antibiotic | 16 (52) | |
| Inhaled corticosteroid | 16 (52) | |
| Mucolytic (DNase/hypertonic saline) | 15 (48) | |
| Oral supplements | 7 (21) | |
| Enteral feeding (PEG) | 2 (6) | |
| Home oxygen | 4 (11) | |
| NIV | 0 (0) | |
| Microbiology | N (%) | |
| Pseudomonas aeruginosa | 23 (66) | |
| Staphylococcus aureus | 13 (37) | |
| Aspergillus | 5 (14) | |
| Burkholderia multivorans | 4 (11) | |
| Burkholderia cenocepacia | 2 (6) | |
| Achromobacter xylosoxidans | 2 (6) | |
| Haemophilus influenzae | 1 (3) | |
| Mutation | N (%) | |
| Phe508del/Gly551Asp | 29 (83) | |
| Gly551Asp/Gly551Asp | 3 (9) | |
| Arg117His/Gly551Asp | 2 (6) | |
| Asn1303Lys/Gly551Asp | 1 (3) | |
Baseline microbiology includes all organisms isolated from sputum samples in the year prior to commencing ivacaftor.
PEG - percutaneous endoscopic gastrostomy
ABPA, allergic bronchopulmonary aspergillosis; BMI, body mass index; CF, cystic fibrosis; CKD, chronic kidney disease; NIV, non-invasive ventilation.