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Phase 3 N=307 Randomized Quadruple-blind Treatment

Evaluation of Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis Subjects Without an F508del Mutation

Cystic Fibrosis

Enrolled (actual)
307
Serious AEs
10.8%
Results posted
Aug 2024
Primary outcome: Primary: Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) — -0.4; 8.9 percent predicted FEV1 — p=< 0.0001

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
ELX/TEZ/IVA (Drug); IVA (Drug); Placebo (matched to ELX/TEZ/IVA) (Other); Placebo (matched to IVA) (Other)
Age
Pediatric, Adult, Older Adult · 6+ yrs
Sex
All
Sponsor
Vertex Pharmaceuticals Incorporated
Primary completion
Jul 2023

Outcome Measures

OutcomeResultp-value
PRIMARY
Absolute Change in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1)
-0.4; 8.9 < 0.0001 sig
SECONDARY
Absolute Change in Sweat Chloride (SwCl)
0.5; -27.8 < 0.0001 sig
SECONDARY
Absolute Change in Cystic Fibrosis Questionnaire-Revised (CFQ-R) Respiratory Domain (RD) Score
-2.0; 17.5 < 0.0001 sig
SECONDARY
Absolute Change in Body Mass Index (BMI)
0.35; 0.81 < 0.0001 sig
SECONDARY
Absolute Change in Weight
1.2; 2.4 < 0.0001 sig
SECONDARY
Number of Pulmonary Exacerbations (PEx)
40; 21
SECONDARY
Safety and Tolerability as Assessed by Number of Participants With Adverse Events (AEs) and Serious Adverse Events (SAEs)
97; 193; 15; 18

Summary

This study evaluated the efficacy, pharmacodynamics (PD) and safety of ELX/TEZ/IVA in participants 6 years of age and older with a non-F508del ELX/TEZ/IVA-responsive cystic fibrosis transmembrane conductance regulator gene (CFTR) mutation.

Eligibility Criteria

Key Inclusion Criteria

  • Participant has a qualifying ELX/TEZ/IVA-responsive CFTR mutation and does not have an exclusionary CFTR mutation
  • Forced expiratory volume in 1 second (FEV1) value >=40% and <=100% of predicted mean for age, sex, and height

Key Exclusion Criteria

  • History of solid organ or hematological transplantation
  • Clinically significant cirrhosis with or without portal hypertension
  • Lung infection with organisms associated with a more rapid decline in pulmonary status

Other protocol defined Inclusion/Exclusion criteria may apply.

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT05274269). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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