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Study of Cavosonstat (N91115) in Patients With CF Homozygous for the F508del-CFTR Mutation

Phase 2
Completed
Conditions
Cystic Fibrosis
Interventions
Drug: Placebo
Registration Number
NCT02589236
Lead Sponsor
Nivalis Therapeutics, Inc.
Brief Summary

This will be a double-blind, randomized, placebo-controlled, parallel group study. The purpose of this study is to investigate the efficacy and safety of Cavosonstat (N91115) in adult patients with CF who are homozygous for the F508del-CFTR mutation and being treated with lumacaftor/ivacaftor (Orkambi™).

Detailed Description

Primary Objective:

* Assess the efficacy of N91115 at 12 weeks when added to preexisting treatment with lumacaftor/ivacaftor in adult patients with CF who are homozygous for the F508del-CFTR mutation

Secondary Objectives:

* Assess the effect of N91115 added to lumacaftor/ivacaftor on safety

* Assess the effect of lumacaftor/ivacaftor added to N91115 on the pharmacokinetics of N91115, lumacaftor, and ivacaftor

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
138
Inclusion Criteria
  • Patients must have been treated with lumacaftor/ivacaftor for at least 8 weeks prior to Day 1 (start of dosing)
  • A history of Sweat Chloride (SC) ≥ 60 mEq/L by quantitative pilocarpine iontophoresis test (QPIT) (either before or after starting lumacaftor/ivacaftor treatment)
  • Body weight ≥ 40 kg
  • ppFEV1 40 - 85 % predicted (inclusive) at screening
  • Oxygen saturation ≥ 90% breathing ambient air at screening
Exclusion Criteria
  • Any acute infection that requires treatment or hospitalization within 2 weeks of Study Day 1
  • Colonization with organisms associated with more rapid decline in pulmonary status, such as Burkholderia cenocepacia, Burkholderia dolosa, and Mycobacterium abscessus
  • Any change in the regimen for chronic therapies for CF lung disease (e.g., Pulmozyme®, hypertonic saline, Azithromycin, TOBI®, Cayston®) within 4 weeks of Study Day 1
  • Are pregnant, planning a pregnancy, or breast-feeding at screening
  • Blood hemoglobin < 10 g/dL at screening
  • Serum albumin < 2.5 g/dL at screening
  • Abnormal liver function defined as ≥ 3 x upper limit of normal (ULN)
  • History of abnormal renal function within 3 months of screening
  • History of ventricular tachycardia or other clinically significant ventricular arrhythmias
  • History, including the screening assessment, of prolonged QT and/or QTcF (Fridericia's correction) interval
  • History of solid organ or hematological transplantation
  • History of alcohol abuse or drug abuse
  • Ongoing participation in another therapeutic clinical trial
  • Use of continuous (24 hr/day) or nocturnal supplemental oxygen

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Cavosonstat (N91115) 400 mgCavosonstatCavosonstat (N91115) 400 mg BID
PlaceboPlaceboPlacebo Capsule
Cavosonstat (N91115) 200 mgCavosonstatCavosonstat (N91115) 200 mg twice daily (BID)
Primary Outcome Measures
NameTimeMethod
Absolute change from baseline in percent predicted FEV1 (ppFEV1)From baseline to 12 weeks

Forced Expiratory Volume in one second (FEV1) from before study (Baseline) to after 12 weeks of N91115 treatment

Secondary Outcome Measures
NameTimeMethod
Absolute change from baseline in Cystic Fibrosis Questionnaire -Revised CFQ-R (respiratory symptom scale)baseline to 16 weeks

Comparison of the Questionnaire from study start to 16 weeks

Absolute change from baseline in Patient Global Impression of Change (PGIC)baseline to 12 weeks

Patient reported outcome journal

Relative change from baseline in ppFEV1baseline to 12 weeks

Forced Expiratory Volume in one second (FEV1) from before study (Baseline) to after 12 weeks of N91115 treatment

Absolute change from baseline in sweat chloridebaseline to 12 weeks

A sweat chloride measurement on the skin at study start and after 12 weeks of N91115

Absolute change from baseline in body mass index (BMI)baseline to 12 weeks

Assessment of change in body mass index from study start to after 12 weeks of N91115

Incidence of Treatment-Emergent Adverse Events [Safety and Tolerability])baseline to 16 weeks

Any adverse events assessment including clinical laboratory values, electrocardiogram (ECG), pulmonary exacerbations, or vital sign changes

Pharmacokinetic Measurements of Maximum Plasma Concentration [Cmax], of N91115, lumacaftor, and ivacaftorbaseline to 12 weeks

Maximum Plasma Concentration \[Cmax\] measurements of N91115, lumacaftor and ivacaftor

Pharmacokinetic Measurements of Area Under the Curve (AUC) for N91115, Ivacaftor and lumacaftorbaseline to 12 weeks

AUC measurements of N91115, lumacaftor and ivacaftor

Trial Locations

Locations (46)

University of Alabama

🇺🇸

Birmingham, Alabama, United States

Banner University of Arizona Medical Center

🇺🇸

Tucson, Arizona, United States

Children's Hospital Los Angeles

🇺🇸

Los Angeles, California, United States

Stanford University

🇺🇸

Palo Alto, California, United States

UC Davis Medical Center

🇺🇸

Sacramento, California, United States

Children's Hospital Colorado

🇺🇸

Aurora, Colorado, United States

National Jewish Health

🇺🇸

Denver, Colorado, United States

Yale University

🇺🇸

New Haven, Connecticut, United States

Tampa General Hospital

🇺🇸

Tampa, Florida, United States

St. Luke's CF Center of Idaho

🇺🇸

Boise, Idaho, United States

Scroll for more (36 remaining)
University of Alabama
🇺🇸Birmingham, Alabama, United States

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