NCT00909532

Brief Summary

The purpose of this study was to evaluate the efficacy and safety of ivacaftor in subjects with cystic fibrosis aged 12 years and older who have the G551D mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Ivacaftor is a potent and selective CFTR potentiator of wild-type, G551D, F508del, and R117H forms of human CFTR protein. Potentiators are pharmacological agents that increase the chloride ion transport properties of the channel in the presence of cyclic AMP-dependent protein kinase A (PKA) activation.

Trial Health

93
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
167

participants targeted

Target at P25-P50 for phase_3

Timeline
Completed

Started Jun 2009

Typical duration for phase_3

Geographic Reach
8 countries

65 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

May 26, 2009

Completed
2 days until next milestone

First Posted

Study publicly available on registry

May 28, 2009

Completed
4 days until next milestone

Study Start

First participant enrolled

June 1, 2009

Completed
1.1 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

July 1, 2010

Completed
2.1 years until next milestone

Results Posted

Study results publicly available

August 21, 2012

Completed
2 months until next milestone

Study Completion

Last participant's last visit for all outcomes

November 1, 2012

Completed
Last Updated

January 18, 2013

Status Verified

January 1, 2013

Enrollment Period

1.1 years

First QC Date

May 26, 2009

Results QC Date

February 27, 2012

Last Update Submit

January 14, 2013

Conditions

Keywords

FibrosisPancreatic DiseasesLung DiseasesRespiratory Tract DiseasesGenetic Diseases, InbornInfant, Newborn, DiseasesPathologic Processes

Outcome Measures

Primary Outcomes (1)

  • Absolute Mean Change From Baseline in Percent Predicted Forced Expiratory Volume in 1 Second (FEV1) Through Week 24

    Spirometry (as measured by FEV1) is a standardized assessment to evaluate lung function that is the most widely used endpoint in cystic fibrosis studies.

    baseline through 24 weeks

Secondary Outcomes (5)

  • Absolute Mean Change From Baseline in Percent Predicted FEV1 Through Week 48

    baseline through 48 weeks

  • Absolute Change From Baseline in Cystic Fibrosis Questionnaire-Revised (CFQ-R) Score Through Week 24 and Week 48 (Respiratory Domain Score, Pooled)

    baseline through 24 weeks and 48 weeks

  • Absolute Change From Baseline in Sweat Chloride Concentration Through Week 24 and Week 48

    baseline through 24 weeks and 48 weeks

  • Time-to-first Pulmonary Exacerbation Through Week 24 and Week 48

    baseline through 24 weeks and 48 weeks

  • Absolute Change From Baseline in Weight at Week 24 and Week 48

    baseline to 24 weeks and 48 weeks

Study Arms (2)

Placebo

PLACEBO COMPARATOR

Subjects who received placebo every 12 hours (q12h) for up to 48 weeks.

Drug: Placebo

150 mg Ivacaftor q12h

EXPERIMENTAL

Subjects who received 150 mg of ivacaftor q12h for up to 48 weeks.

Drug: Ivacaftor

Interventions

150-mg tablets given orally q12h for up to 48 weeks

Also known as: VX-770
150 mg Ivacaftor q12h

Tablet given orally q12h for up to 48 weeks

Placebo

Eligibility Criteria

Age12 Years+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Confirmed diagnosis of cystic fibrosis (CF) and G551D mutation in at least 1 allele
  • Forced expiratory volume in 1 second (FEV1) of 40% to 90% (inclusive) of predicted normal for age, gender, and height at Screening.
  • No clinically significant abnormalities that would have interfered with the study assessments, as judged by the investigator
  • Willing to use highly effective birth control methods during the study

You may not qualify if:

  • History of any illness or condition that might confound the results of the study or pose an additional risk in administering study drug to the subject
  • Acute respiratory infection, pulmonary exacerbation, or changes in therapy for pulmonary disease within 4 weeks of Day 1 of the study
  • History of alcohol, medication or illicit drug abuse within one year prior to Day 1
  • Abnormal liver function ≥ 3x the upper limit of normal
  • Abnormal renal function at Screening
  • History of solid organ or hematological transplantation
  • Pregnant, planning a pregnancy, breast-feeding, or unwilling to follow contraception requirements
  • Ongoing participation in another therapeutic clinical study or prior participation in an investigational drug study within 30 days prior to Screening
  • Use of inhaled hypertonic saline treatment
  • Concomitant use of any inhibitors or inducers of cytochrome P450 3A4 (CYP 3A4)

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (65)

University of Alabama

Birmingham, Alabama, 35233-1711, United States

Location

Kaiser Permanente Medical Care Program

Oakland, California, 94611, United States

Location

Cystic Fibrosis Research Office, Stanford University

Palo Alto, California, 94304, United States

Location

Rady Children's Hospital

San Diego, California, 92123-5070, United States

Location

National Jewish Medical and Research Center

Denver, Colorado, 80206, United States

Location

Emory Cystic Fibrosis Center

Atlanta, Georgia, 30322, United States

Location

St. Luke's CF Clinic

Boise, Idaho, 83712, United States

Location

Children's Memorial Hospital

Chicago, Illinois, 60614, United States

Location

Indiana University

Indianapolis, Indiana, 46202, United States

Location

University of Iowa

Iowa City, Iowa, 52242, United States

Location

Johns Hopkins University

Baltimore, Maryland, 21205, United States

Location

Massachusetts General Hospital

Boston, Massachusetts, 02114, United States

Location

Children's Hospital Boston

Boston, Massachusetts, 02115, United States

Location

University of Michigan

Ann Arbor, Michigan, 48109, United States

Location

Pulmonary, Allergy & Critical Care Medicine, University of Minnesota

Minneapolis, Minnesota, 55455, United States

Location

The Children's Mercy Hospital

Kansas City, Missouri, 64108, United States

Location

Washington University

St Louis, Missouri, 63110, United States

Location

Adult Pulmonary/ CF, University of Nebraska Medical Center

Omaha, Nebraska, 68198-5300, United States

Location

Monmouth Medical Center

Long Branch, New Jersey, 07740, United States

Location

Women and Children's Hospital of Buffalo

Buffalo, New York, 14222, United States

Location

Long Island Jewish Medical Center

New Hyde Park, New York, 11042, United States

Location

SUNY Upstate Medical University

Syracuse, New York, 13210, United States

Location

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, 27599, United States

Location

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, 45229, United States

Location

Pediatric & Pulmonary Division, Rainbow Babies/Case Western

Cleveland, Ohio, 44106, United States

Location

Nationwide Children's Hospital

Columbus, Ohio, 43205, United States

Location

Toledo Children's Hospital

Toledo, Ohio, 43606, United States

Location

Oregon Health & Sciences University

Portland, Oregon, 97239-3098, United States

Location

Hershey Medical Center

Hershey, Pennsylvania, 17033, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

Children's Hospital of Pittsburgh of UPMC

Pittsburgh, Pennsylvania, 15224, United States

Location

East Tennessee Children's Hospital

Knoxville, Tennessee, 37916, United States

Location

Vanderbilt University Medical Center

Nashville, Tennessee, 37232-5735, United States

Location

University of Utah

Salt Lake City, Utah, 84132, United States

Location

University of Virginia

Charlottesville, Virginia, 22908, United States

Location

Seattle Children's Hospital

Seattle, Washington, 98105, United States

Location

Division of Pulmonary and CCM, University of Washington

Seattle, Washington, 98195-6522, United States

Location

West Virginia University

Morgantown, West Virginia, 26506, United States

Location

Medical College of Wisconsin

Milwaukee, Wisconsin, 53226, United States

Location

The Children's Hospital Westmead

Westmead, New South Wales, 2145, Australia

Location

The Prince Charles Hospital

Chermside, Queensland, 4032, Australia

Location

Royal Children's Hospital Brisbane

Herston, Queensland, 4026, Australia

Location

Mater Adult Hospital

South Brisbane, Queensland, 4101, Australia

Location

Royal Children's Hospital Melbourne

Parkville, Victoria, 3052, Australia

Location

Lung Institute of Western Australia

Nedlands, Western Australia, 6009, Australia

Location

Princess Margaret Hospital for Children

Subiaco, Western Australia, 6008, Australia

Location

Queen Elizabeth II Health Sciences Centre

Halifax, Nova Scotia, B3H 3A7, Canada

Location

St. Michael's Hospital

Toronto, Ontario, M5B 1W8, Canada

Location

CF Center, Hospital for Sick Children

Toronto, Ontario, M5G 1X8, Canada

Location

Montreal Children's Hospital - MUHC

Montreal, Quebec, H3H 1P3, Canada

Location

FN Motol

Prague, 15006, Czechia

Location

Hopital Cochin

Paris, 75014, France

Location

Hopital Necker

Paris, 75015, France

Location

Centre de Perharidy

Roscoff, 29684, France

Location

Kinder- und Jugendklinik Universitätsklinikum Erlangen

Erlangen, 91054, Germany

Location

Mukoviszidose-Zentrum am Klinikum der Friedrich-Schiller-Universität Jena, Klinik für Kinder- und Jugendmedizin

Jena, 07740, Germany

Location

Klinikum der LMU München, Dr. von Haunersches Kinderspital (CHA)

Munich, 80337, Germany

Location

Universitäts-Kinderklinik Würzburg

Würzburg, 97080, Germany

Location

Cork University Hospital

Cork, Ireland

Location

Our Lady's Children's Hospital

Dublin, 12, Ireland

Location

The National Children's Hospital

Dublin, 24, Ireland

Location

St. Vincent's University Hospital

Dublin, 4, Ireland

Location

Beaumont Hospital

Dublin, 9, Ireland

Location

Belfast City Hospital

Belfast, Northern Ireland, BT9 7AB, United Kingdom

Location

Imperial College London

London, SW3 6LR, United Kingdom

Location

Related Publications (4)

  • Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordonez C, Elborn JS; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.

    PMID: 22047557BACKGROUND
  • Flume PA, Wainwright CE, Elizabeth Tullis D, Rodriguez S, Niknian M, Higgins M, Davies JC, Wagener JS. Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor. J Cyst Fibros. 2018 Jan;17(1):83-88. doi: 10.1016/j.jcf.2017.06.002. Epub 2017 Jun 24.

  • Solem CT, Vera-Llonch M, Liu S, Botteman M, Castiglione B. Impact of pulmonary exacerbations and lung function on generic health-related quality of life in patients with cystic fibrosis. Health Qual Life Outcomes. 2016 Apr 21;14:63. doi: 10.1186/s12955-016-0465-z.

  • Quittner A, Suthoff E, Rendas-Baum R, Bayliss MS, Sermet-Gaudelus I, Castiglione B, Vera-Llonch M. Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial. Health Qual Life Outcomes. 2015 Jul 2;13:93. doi: 10.1186/s12955-015-0293-6.

Related Links

MeSH Terms

Conditions

Cystic FibrosisFibrosisPancreatic DiseasesLung DiseasesRespiratory Tract DiseasesGenetic Diseases, InbornInfant, Newborn, DiseasesPathologic Processes

Interventions

ivacaftor

Condition Hierarchy (Ancestors)

Digestive System DiseasesCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesPathological Conditions, Signs and Symptoms

Results Point of Contact

Title
Medical Monitor
Organization
Vertex

Study Officials

  • Bonnie W. Ramsey, MD

    Children's Hospital and Regional Medical Center, Seattle, Washington, USA

    PRINCIPAL INVESTIGATOR
  • Stuart Elborn, MD

    Respiratory Medicine Group, Queen's University of Belfast, Belfast, Northern Ireland, UK

    PRINCIPAL INVESTIGATOR

Publication Agreements

PI is Sponsor Employee
No
Restriction Type
GT60
Restrictive Agreement
Yes

Study Design

Study Type
interventional
Phase
phase 3
Allocation
RANDOMIZED
Masking
QUADRUPLE
Who Masked
PARTICIPANT, CARE PROVIDER, INVESTIGATOR, OUTCOMES ASSESSOR
Purpose
TREATMENT
Intervention Model
PARALLEL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

May 26, 2009

First Posted

May 28, 2009

Study Start

June 1, 2009

Primary Completion

July 1, 2010

Study Completion

November 1, 2012

Last Updated

January 18, 2013

Results First Posted

August 21, 2012

Record last verified: 2013-01

Locations