Adverse Event: any untoward medical occurrence in a participant during the study, including any unfavorable and unintended sign, symptom, or disease whether or not it was considered to be study drug related. This included any newly occurring event or previous condition that increased in severity or frequency after obtaining informed consent and assent (where applicable). SAE: medical event or condition, which resulted in any of following, regardless of its relationship to the study drug: death, life threatening adverse experience, in-patient hospitalization/prolonged hospitalization, persistent/significant disability/incapacity, congenital anomaly/birth defect, important medical event. Non-Serious AEs included all AEs except SAEs.

Safety Set included all participants who received at least 1 dose of study drug during Study 105.

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. Predicted FEV1 (for age, gender, and height) was calculated using the Knudson method. Baseline was defined as Study 105 Day 15.

Full Analysis Set.

FEV1 is the volume of air that can forcibly be blown out in one second, after full inspiration. Predicted FEV1 (for age, gender, and height) was calculated using the Knudson method. Baseline was defined as the most recent measurement prior to intake of the first dose of study drug in Study 105. Absolute Change at Week 48 and Week 96 are reported.

Full Analysis Set. Here, number of participants analyzed signifies participants who were evaluable for this outcome measure and n signifies participants who were evaluable for specified categories for each arm, respectively.

The CFQ-R is a validated patient-reported outcome measuring health-related quality of life for subjects with cystic fibrosis. Respiratory domain assessed respiratory symptoms (for example, coughing, congestion, wheezing), score range: 0-100; higher scores indicating fewer symptoms and better health-related quality of life. Absolute Change at Week 48 and Week 96 are reported.

Full Analysis Set. Here, number of participants analyzed signifies participants who were evaluable for this outcome measure and n signifies participants who were evaluable for specified categories for each arm, respectively.

Annualized event rate was calculated by regression with negative binomial distribution.

Full Analysis Set.

Full Analysis Set.

Weight is a measurement of nutritional status. Absolute change in weight, measured in kilograms (kg), at Week 48 and Week 96 are reported.

Full Analysis Set. Here, number of participants analyzed signifies participants who were evaluable for this outcome measure and n signifies participants who were evaluable for specified categories for each arm, respectively.