Table 2

Potential predictors of death or the need for transplantation (where applicable, medians and ranges are shown)

DeathTransplant
Number of patients118
FEV1 last recorded33 (12–69)24 (22–29)
FEV1 1 year prior32 (6–64)34 (19–44)
FEV1 5 years prior62 (30–96)30 (20–54)
Absolute fall/year in FEV1 over 5 years8.9 (6.1–12.5)10.7 (4.7–16.5)
FVC last recorded45 (22–80)52 (38–69)
FVC 1 year prior45 (21–83)44 (30–57)
FVC 5 years prior66 (34–104)61 (39–82)
Absolute fall/year in FVC over 5 years4.3 (0.5–8.5)7.7 (1.5–18)
Exercise tolerance*
 Desaturation (SpO2≥4%)1 (25%)6 (86%)
 No desaturation3 (75%)1 (14%)
 Not tested71
 Wheelchair use3 (27%)3 (38%)
  • Lung function shown as % predicted (2/11 children who died were unable to perform spirometry).

  • *Exercise testing was either modified shuttle test or 3-min step test.

  • FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity.