Trial Outcomes & Findings for Novel Therapeutic Approaches for Treatment of CF Patients With W1282X Premature Termination Codon Mutations (NCT NCT03624101)

NCT ID: NCT03624101

Last Updated: 2025-04-16

Results Overview

Percent change in FEV1 from Baseline and 24 weeks

Recruitment status

TERMINATED

Study phase

PHASE4

Target enrollment

1 participants

Primary outcome timeframe

24 weeks

Results posted on

2025-04-16

Participant Flow

Participant milestones

Participant milestones
Measure
Symdeko
If the participant is not on a current modulator, they will take Symdeko for 28 days followed by a 28 day off period. This cycle will be continued for 168 days Symdeko: CFTR modulator
Symdeko/Ivacaftor
If the participant currently takes Symdeko and Ivacaftor, they will take Symdeko for a 28 day period followed by Ivacaftor for a 28 day period. This cycle will be continued for 168 days symdeko/Ivacaftor: CFTR modulator
Overall Study
STARTED
1
0
Overall Study
COMPLETED
1
0
Overall Study
NOT COMPLETED
0
0

Reasons for withdrawal

Withdrawal data not reported

Baseline Characteristics

Novel Therapeutic Approaches for Treatment of CF Patients With W1282X Premature Termination Codon Mutations

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure
Symdeko
n=1 Participants
If the participant is on symdeko, they will take symdeko for 28 days followed by a 28 day off period. This cycle will be continued for 168 day Symdeko: CFTR modulator
Symdeko/Ivacaftor
If the participant currently takes Symdeko , they will take Symdeko for a 28 day period followed by Ivacaftor for a 28 day period. This cycle will be continued for 168 days symdeko/Ivacaftor: CFTR modulator
Total
n=1 Participants
Total of all reporting groups
Age, Categorical
<=18 years
0 Participants
n=39 Participants
0 Participants
n=35 Participants
Age, Categorical
Between 18 and 65 years
1 Participants
n=39 Participants
1 Participants
n=35 Participants
Age, Categorical
>=65 years
0 Participants
n=39 Participants
0 Participants
n=35 Participants
Age, Continuous
37 years
n=39 Participants
37 years
n=35 Participants
Sex: Female, Male
Female
0 Participants
n=39 Participants
0 Participants
n=35 Participants
Sex: Female, Male
Male
1 Participants
n=39 Participants
1 Participants
n=35 Participants
Ethnicity (NIH/OMB)
Hispanic or Latino
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Ethnicity (NIH/OMB)
Not Hispanic or Latino
1 Participants
n=39 Participants
0 Participants
n=41 Participants
1 Participants
n=35 Participants
Ethnicity (NIH/OMB)
Unknown or Not Reported
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
American Indian or Alaska Native
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
Asian
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
Native Hawaiian or Other Pacific Islander
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
Black or African American
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
White
1 Participants
n=39 Participants
0 Participants
n=41 Participants
1 Participants
n=35 Participants
Race (NIH/OMB)
More than one race
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Race (NIH/OMB)
Unknown or Not Reported
0 Participants
n=39 Participants
0 Participants
n=41 Participants
0 Participants
n=35 Participants
Region of Enrollment
United States
1 participants
n=39 Participants
1 participants
n=35 Participants

PRIMARY outcome

Timeframe: 24 weeks

Population: no improvement was noticed and the study ended prematurely. No formal statistical analysis was performed

Percent change in FEV1 from Baseline and 24 weeks

Outcome measures

Outcome measures
Measure
Symdeko
n=1 Participants
If the participant is not on a current modulator, they will take Symdeko for 28 days followed by a 28 day off period. This cycle will be continued for 168 days Symdeko: CFTR modulator
Symdeko/Ivacaftor
If the participant currently takes Symdeko, they will take Symdeko for a 28 day period followed by Ivacaftor for a 28 day period. This cycle will be continued for 168 days symdeko/Ivacaftor: CFTR modulator
Percent Change in FEV1 From Baseline
0 change in percentage

Adverse Events

Symdeko

Serious events: 0 serious events
Other events: 1 other events
Deaths: 0 deaths

Symdeko/Ivacaftor

Serious events: 0 serious events
Other events: 0 other events
Deaths: 0 deaths

Serious adverse events

Adverse event data not reported

Other adverse events

Other adverse events
Measure
Symdeko
n=1 participants at risk
If the participant is not on a current modulator, they will take Symdeko for 28 days followed by a 28 day off period. This cycle will be continued for 168 days Symdeko: CFTR modulator
Symdeko/Ivacaftor
If the participant currently takes Symdeko , they will take Symdeko for a 28 day period followed by Ivacaftor for a 28 day period. This cycle will be continued for 168 days symdeko/Ivacaftor: CFTR modulator
Respiratory, thoracic and mediastinal disorders
Increased cough
100.0%
1/1 • Number of events 2 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
0/0 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
Respiratory, thoracic and mediastinal disorders
increased sputum production
100.0%
1/1 • Number of events 2 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
0/0 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
General disorders
Increased fatigue
100.0%
1/1 • Number of events 2 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
0/0 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
Metabolism and nutrition disorders
decreased appetite
100.0%
1/1 • Number of events 1 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
0/0 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
Endocrine disorders
low blood glucose
100.0%
1/1 • Number of events 1 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm
0/0 • 6 months
only 1 patient was entered into the study (Symdeko Arm). No patients were entered into the Symdeko/Ivacaftor Arm

Additional Information

Heather Hathorne

University of Alabama at Birmingham Child Health Research Unit

Phone: 2055032829

Results disclosure agreements

  • Principal investigator is a sponsor employee
  • Publication restrictions are in place