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Study to Evaluate the Safety and Efficacy of CTP-656 in Patients With Cystic Fibrosis With CFTR Gating Mutations

Phase 2
Terminated
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo
Drug: IVA
Registration Number
NCT02971839
Lead Sponsor
Vertex Pharmaceuticals Incorporated
Brief Summary

This study will evaluate the efficacy and safety of CTP-656 in patients with cystic fibrosis (CF) who have a cystic fibrosis transmembrane conductance regulator (CFTR) gating mutation.

Detailed Description

This is a randomized, parallel-group, double-blind, placebo controlled multicenter study to evaluate the safety and efficacy of CTP-656 in CF patients with CFTR gating mutations, compared to Kalydeco, for a total of 28 days. Subjects will be randomized to receive either double-blind CTP-656 or placebo, or open-label Kalydeco.

Recruitment & Eligibility

Status
TERMINATED
Sex
All
Target Recruitment
11
Inclusion Criteria
  • 18 years of age or older
  • Has a confirmed diagnosis of CF with at least one allele of the following CFTR gating mutations: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, and S549R.
  • Has been stable on Kalydeco therapy for at least 3 months prior to screening
  • Has FEV1 ≥ 60% of predicted normal for age, sex, and height at screening and baseline (Day 1) assessments
  • Weighs at least 40 kg at screening
  • Patients of either gender and women of childbearing potential must be willing to use a medically highly effective form of birth control during the treatment period and 30 days after the last dose of study treatment.
Exclusion Criteria
  • Acute upper respiratory infection or lower respiratory infection, pulmonary exacerbation, or changes in therapy within 4 weeks of study treatment
  • Uncontrolled type 2 diabetes, or uncontrolled CF-related diabetes
  • History of hepatitis C or chronic active hepatitis B infection
  • History of pulmonary tuberculosis, non-tuberculosis mycobacterial infections or allergic bronchopulmonary aspergillosis (ABPA) treated during screening or within 2 years prior to screening
  • Colonization with B. cenocepacia, B. dolosa, B. multivorans, and/or M. abcessus within 2 years prior to Screening
  • Abnormal liver function
  • History of abnormal renal function
  • History of prolonged QTcF > 450 msec for males or QTcF > 470 msec for females
  • History of solid organ or hematological transplantation
  • Using any inhibitor or inducer of cytochrome P450/3A during the study or within 30 days of screening
  • Women who are pregnant or lactating, or have plans to become pregnant during the study or within 1 month following the last dose

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlacebo-
VX-561 100 mgVX-561-
VX-561 150 mgVX-561-
IvacaftorIVA-
VX-561 20 mgVX-561-
Primary Outcome Measures
NameTimeMethod
Change From Baseline in Sweat Chloride at Day 28From baseline at Day 28
Secondary Outcome Measures
NameTimeMethod
Change From Baseline in Cystic Fibrosis Questionnaire-Respiratory Domain (CFQ-R) at Day 28From baseline at Day 28
Change From Baseline in Percent Predicted Forced Expiratory Volume in 1 Second (ppFEV1) at Day 28From baseline at Day 28

Trial Locations

Locations (14)

University of Southern California

🇺🇸

Los Angeles, California, United States

Stanford Hospital

🇺🇸

Palo Alto, California, United States

University of Miami

🇺🇸

Miami, Florida, United States

Rush University

🇺🇸

Chicago, Illinois, United States

Indiana University

🇺🇸

Indianapolis, Indiana, United States

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

Washington University

🇺🇸

Saint Louis, Missouri, United States

Cincinnati Children's Hospital

🇺🇸

Cincinnati, Ohio, United States

Nationwide Children's Hospital

🇺🇸

Columbus, Ohio, United States

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

New York Medical College

🇺🇸

Valhalla, New York, United States

Atlantic Health

🇺🇸

Morristown, New Jersey, United States

Children's National Health

🇺🇸

Washington, District of Columbia, United States

University of Massachusetts

🇺🇸

Worcester, Massachusetts, United States

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