Table 1

Patient demographics (where applicable, medians and ranges are shown)

DeathTransplant
Number of patients118
Gender9F/2M6F/2M
Genotype
 Δ508/Δ5084 (36%)4 (50%)
 Δ508/Other3 (27%)4 (50%)
 Other/Other4 (36%)0
 Age at CF diagnosis (years)0.17 (0–1.1)0.54 (0.04–3)
 Age at death (years)14.2 (3.5–16.5)
 Age at transplant (years)13.0 (9.2–15.5)
Nutritional status
 Height z score−1.51 (−4.41 to −0.64)−1.14 (−2.49 to +0.99)
 Weight z score−1.02 (−6.63 to +0.11)−1.04 (−2.13 to +0.66)
 BMI z score−0.78 (−3.57 to +1.35)−0.3 (−2.03–+0.3)
 Gastrostomy7 (73%)6 (75%)
 Lung function (last recorded)33 (12–69)24 (22–29)
FEV1 (% predicted)
 FVC (% predicted)45 (22–80)34 (3–44)
Infection status
 Chronic Pseudomonas aeruginosa11 (100%)8 (100%)
 Chronic Staphylococcus aureus7 (64%)6 (75%)
 Chronic Stenotrophomonas maltophilia4 (36%)3 (38%)
 Chronic Burkholderia cepacia complex4 (36%)0
 Chronic Aspergillus fumigatus6 (55%)2 (25%)
 Non-tuberculous mycobacteria1 (9%)0
 No. IV antibiotic courses in last year6 (3–10)5 (3–6)
 TIVAD in situ6 (64%)7 (88%)
Comorbidities
 CF-related diabetes1 (9%)2 (25%)
 ABPA2 (18%)1 (13%)
 CF liver disease00
 Gastro-oesophageal reflux4 (36%)4 (50%)
 Nissen's fundoplication3 (27%)3 (38%)
Home respiratory support
 Supplemental oxygen6 (55%)8 (100%)
 Non-invasive ventilation01 (13%)
  • Lung function measured in 9/11 children who died.

  • ABPA, allergic bronchopulmonary aspergillosis; BMI, body mass index; CF, cystic fibrosis; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; IV, intravenous; TIVAD, totally implantable intravenous device.