Second Open Label Extension to Bridging Study CTBM100C2303

An AE was defined as any unfavorable and unintended sign, symptom, or disease temporally associated with the use of study drug, whether or not related to study drug.

Time frame: From time of first administration of study drug until study completion (up to 169 days)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study.

A SAE was defined as an event which was fatal or life threatening, required or prolonged hospitalization, was significantly or permanently disabling or incapacitating, constituted a congenital anomaly or a birth defect, or encompassed any other clinically significant event that could jeopardize the participant or require medical or surgical intervention to prevent one of the aforementioned outcomes.

Time frame: From time of consent to 4 weeks after study completion (up to 199 days)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study.

Airway Reactivity \>= 20% relative decrease in FEV1% predicted from pre-dose to 30 minutes post-dose. Relative Change = 100 \* (30 minutes Post-dose - Pre-dose)/Pre-dose assessed by the number and percentage of participants with a decrease of ≥ 20% in FEV1 % predicted from pre-dose to 30 minutes post-dose.

Time frame: Pre-dose and post-dose of Day 1 and Day 29 of every Cycle (5, 6, 7)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

Auditory acuity of participants was measured using a standard dual-channel audiometer at frequencies from 250 to 8000 Hertz, and an audiogram (pure-tone air conduction) and tympanogram were performed by an audiologist. The categories reported includes \>= 10dB decrease in 3 consecutive frequencies in either ear, \>= 15dB decrease in 2 consecutive frequencies in either ear, and \>= 20dB decrease in at least one frequency in either ear

Time frame: Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: All participants included in the safety population with at least one audiology testing. Number analyzed is the number of participants with data available at the given timepoint.

Pseudomonas Aeruginosa Density refers to overall density, defined as the sum of Biotypes (mucoid, dry and small colony variant). Absolute change was determined using the formula; Change = Post-baseline value- baseline value. Absolute Change in Pseudomonas Aeruginosa Sputum density is measured in log 10 Colony Forming Units per gram (Log 10 CFU/g).

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

Minimum Inhibitory Concentration (MIC) is defined as the lowest concentration of an antimicrobial agent required to inhibit the visible growth of a microorganism after overnight incubation.

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

The average number of days patients were hospitalized due to respiratory events during the study.

Time frame: From time of consent to 4 weeks after study completion (up to 199 days)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

The rate of anti-pseudomonal antibiotics use were determined from the collection of concomitant medication during the study Treatment period.

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study.

A SAE was defined as an event which was fatal or life threatening, required or prolonged hospitalization, was significantly or permanently disabling or incapacitating, constituted a congenital anomaly or a birth defect, or encompassed any other clinically significant event that could jeopardize the participant or require medical or surgical intervention to prevent one of the aforementioned outcomes.

Time frame: From time of consent to 4 weeks after study completion (up to 199 days)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

Forced Expiratory Flow Rate Over 25 and 75 Percent (FEF25-75%) is the forced expiratory flow from 25% to 75% of the Forced Vital Capacity (FVC). Relative change in FEF25-75% from baseline to pre-dose day X = (pre-dose day X FEF25-75 - baseline FEF25-75) / baseline FEF25-75) • 100.

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

Forced expiratory volume in one second (FEV1) is the amount of air which can be forcibly exhaled from the lungs in the first second of a forced exhalation. FEV1 % predicted was a normalized value of FEV1 calculated using the Knudsen equation, based upon participant's age, gender and height. Relative change in FEV1 % predicted from baseline to pre-dose day X = ((pre-dose day X FEV1 % predicted - baseline FEV1 % predicted) / baseline FEV1 % predicted) x 100.

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.

Percent Predicted Forced Vital Capacity (FVC%) is the maximal exhaled breath volume following a maximal inhaled breath. Overall change in percent predicted FVC = (observed value)/(predicted value) \* 100%. A higher value indicates a greater response.

Time frame: Baseline, Cycles 5, 6, 7 (Days 1, 29) and Follow-up (Week 57/Day 57)

Population: Safety population included all participants who completed their participation in C2303E1, who gave their consent to enter the extension 2 study and who received at least one dose of study drug in the extension 2 study. Number analyzed is the number of participants with data available at the given timepoint.