Table 1

Baseline demographics of the 35 study patients compiled from clinic letters at the time of commencing ivacaftor

Demographic (n=35)Mean±SDMedian (IQR)
Age at starting ivacaftor (years)29.06±9.5427.9 (21–33.2)
Age at CF diagnosis (years)2.94±3.882 (0–4.5)
Baseline BMI (kg/m2)21.85±3.4622.1 (19–23.5)
Baseline FEV1 (% predicted)58.41±22.6463 (42–75.2)
N (%)
Male sex20 (57)
Exocrine pancreatic insufficiency33 (94)
CF-related diabetes mellitus9 (26)
Osteoporosis/bone disease5 (15)
ABPA3 (9)
CKD1 (3)
CF-related liver disease1 (3)
Baseline therapiesN (%)
Long-term oral macrolide antibiotic28 (90)
Inhaled/nebulised antibiotic16 (52)
Inhaled corticosteroid16 (52)
Mucolytic (DNase/hypertonic saline)15 (48)
Oral supplements7 (21)
Enteral feeding (PEG)2 (6)
Home oxygen4 (11)
NIV0 (0)
MicrobiologyN (%)
Pseudomonas aeruginosa 23 (66)
Staphylococcus aureus 13 (37)
Aspergillus5 (14)
Burkholderia multivorans 4 (11)
Burkholderia cenocepacia 2 (6)
Achromobacter xylosoxidans 2 (6)
Haemophilus influenzae 1 (3)
MutationN (%)
Phe508del/Gly551Asp29 (83)
Gly551Asp/Gly551Asp3 (9)
Arg117His/Gly551Asp2 (6)
Asn1303Lys/Gly551Asp1 (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.