Table 1.
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
---|---|---|---|---|---|
Age at CF diagnosis | 2 years | 5 years | 5 weeks | 8 years | 2 years |
Mutations | Δ508/G551D | Δ508 | Δ508 | Δ508 | Δ508/S549R |
Age at CFRD diagnosis | 20 | 29 | 16 | 30 | 20 |
Age at ivacaftor initiation | 28 | 30 | 29 | 32 | 29 |
Specific drug | ivacaftor | combined** | combined | combined | ivacaftor |
Time to CFRD resolution | 9 months | 1 month | 8 months | 12 months | 12 months* |
Continued hypoglycemia | Yes | Yes | No | No | Yes |
Follow up post CFRD resolution | 5 years | 1 year | 8 months | 2 years | 4 years* |
Change in FEV1 (% predicted) | ND*** | +3% | +3% | +13% | +8% |
Change in BMI | ND | −1.0 | +1.5 | +0.7 | 0 |
Patient had reduction in insulin use from short-acting insulin three times a day to once a week, not complete resolution
lumacaftor/ivacaftor combination drug
ND = no data