Study of Ivacaftor in Cystic Fibrosis Subjects 2 Through 5 Years of Age With a CFTR Gating Mutation

NCT ID: NCT01705145

Last Updated: 2016-04-05

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

35 participants

Study Classification

INTERVENTIONAL

Study Start Date

2013-01-31

Study Completion Date

2014-03-31

Brief Summary

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The purpose of this study is to evaluate the safety, pharmacokinetics (PK), and pharmacodynamics (PD), of ivacaftor in children with cystic fibrosis (CF) who are 2 through 5 years of age and have a CF Transmembrane Conductance Regulator (CFTR) gating mutation in at least 1 allele.

Part A is designed to evaluate the safety and PK of multiple-dose administration of ivacaftor in participants 2 through 5 years of age and to confirm the doses for Part B. Part B is designed to evaluate the safety, PK, PD, and efficacy of ivacaftor in participants 2 through 5 years of age.

Detailed Description

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Conditions

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Cystic Fibrosis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Ivacaftor

Part A: Ivacaftor 50 milligram (mg) (for participants weighing less than \[\<\] 14 kilograms \[kg\]) or 75 mg (for participants weighing greater than or equal to \[\>=\] 14 kg) every 12 hours (q12h) from Day 1 through Day 3 and 1 morning dose on Day 4 during Part A of the study..

Part B: Ivacaftor 50 mg (for participants weighing \<14 kg) or 75 mg (for participants weighing \>=14 kg) q12h for 24 weeks during Part B of the study.

Group Type EXPERIMENTAL

Ivacaftor

Intervention Type DRUG

Interventions

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Ivacaftor

Intervention Type DRUG

Other Intervention Names

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Kalydeco VX-770

Eligibility Criteria

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Inclusion Criteria

* Male or female with confirmed diagnosis of CF
* Must have a CFTR gating mutation in at least 1 allele
* Aged 2 through 5 years at screening and Day 1
* Weight \>= 8 kg at screening and Day 1
* Hematology, serum chemistry, coagulation, and vital signs results at screening with no clinically significant abnormalities that would interfere with the study assessments, as judged by the investigator

Exclusion Criteria

* History of any illness or condition that, in the opinion of the investigator, might confound the results of the study or pose an additional risk in administering study drug to the participant
* An acute upper or lower respiratory infection, or pulmonary exacerbation, or changes in therapy for pulmonary disease within 4 weeks before Day 1
* Abnormal liver function, at screening
* History of solid organ or hematological transplantation
* Use of any moderate or strong inducers or inhibitors of cytochrome P450 (CYP) 3A within 2 weeks before Day 1
* Participation in a clinical study involving administration of either an investigational or a marketed drug within 30 days or 5 terminal half-lives before screening
Minimum Eligible Age

2 Years

Maximum Eligible Age

5 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Cystic Fibrosis Foundation

OTHER

Sponsor Role collaborator

Vertex Pharmaceuticals Incorporated

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Margaret Rosenfeld, MD

Role: PRINCIPAL_INVESTIGATOR

Seattle Children's Hospital

Jane Davies, MD

Role: PRINCIPAL_INVESTIGATOR

Imperial College London

Locations

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Birmingham, Alabama, United States

Site Status

Aurora, Colorado, United States

Site Status

Atlanta, Georgia, United States

Site Status

Indianapolis, Indiana, United States

Site Status

Lexington, Kentucky, United States

Site Status

Boston, Massachusetts, United States

Site Status

Detroit, Michigan, United States

Site Status

Grand Rapids, Michigan, United States

Site Status

Minneapolis, Minnesota, United States

Site Status

Kansas City, Missouri, United States

Site Status

Omaha, Nebraska, United States

Site Status

Pittsburgh, Pennsylvania, United States

Site Status

Salt Lake City, Utah, United States

Site Status

Charlottesville, Virginia, United States

Site Status

Richmond, Virginia, United States

Site Status

Seattle, Washington, United States

Site Status

Vancouver, , Canada

Site Status

Edinburgh, , United Kingdom

Site Status

Liverpool, , United Kingdom

Site Status

London, , United Kingdom

Site Status

Countries

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United States Canada United Kingdom

References

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Davies JC, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Robertson S, Green Y, Cooke J, Rosenfeld M; KIWI Study Group. Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. Lancet Respir Med. 2016 Feb;4(2):107-15. doi: 10.1016/S2213-2600(15)00545-7. Epub 2016 Jan 21.

Reference Type DERIVED
PMID: 26803277 (View on PubMed)

Other Identifiers

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KIWI

Identifier Type: -

Identifier Source: secondary_id

VX11-770-108

Identifier Type: -

Identifier Source: org_study_id

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