Patient 1 | Patient 2 | |
---|---|---|
Current age (years) | 32 | 47 |
Gender | Female | Female |
Genotype | 3272-26A→G /E585X | 3849+10kbC→T /F508del |
Sweat chloride (mmol/L) Before Ivacaftor | 82 | 57 |
After Ivacaftor | 83 (6 and 18 months) | 19 (6 months) |
FEV1 (% PREDICTED) Before Ivacaftor | 29 | 38 |
After Ivacaftor | 44 (6 months); 52 (18 months) | 50 (6 months) |
6MWT (meters) Before Ivacaftor | 500 | 508 |
After Ivacaftor | 630 (6 and 18 months) | 572 (6 months) |
Days of antibiotic therapy 12 months Before Ivacaftor | 152 (67 IV and 85 oral) | 88 (36 IV and 52 oral) |
After Ivacaftor | 49 (18 months) oral | 0 (6 months) |
CFQ-R Respiratory Domain | 33 (Basal) 78 (18 months) | 72 (Basal) 83 (6 months) |