Impact of Discontinuing Chronic Therapies in People With Cystic Fibrosis on Highly Effective CFTR Modulator Therapy

NCT ID: NCT04378153

Last Updated: 2024-12-04

Study Results

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Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

987 participants

Study Classification

OBSERVATIONAL

Study Start Date

2020-08-25

Study Completion Date

2022-07-11

Brief Summary

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Despite the increasingly common use of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies in treating CF, it is still largely unknown whether or not other chronic therapies can be safely stopped. The SIMPLIFY study is being done to test whether or not it is safe to stop taking inhaled hypertonic saline or Pulmozyme® (dornase alfa) in those people that are also taking Trikafta™.

Trikafta (elexacaftor/tezacaftor/ivacaftor) is a combination CFTR modulator therapy that was approved by the Food and Drug Administration for people with CF who have at least one F508del mutation. The three drugs that make up Trikafta work together to allow many more chloride ions to move into and out of the cells, improving the balance of salt and water in the lungs. These changes result in better clearance of mucus from the lungs and improvements in lung function.

Inhaled hypertonic saline and Pulmozyme (dornase alfa) also improve clearance of mucus from the lungs to support lung function and have been available to people with CF for many years. Both therapies are considered to be relatively burdensome and it is not known whether either therapy can improve or maintain lung function above what is already gained through Trikafta use.

The goal of the SIMPLIFY study is to get information about whether or not it is safe to stop either inhaled hypertonic saline or Pulmozyme (dornase alfa) by testing if there is a change in lung function in subjects with cystic fibrosis (CF) who are assigned to stop their chronic medication (either hypertonic saline or Pulmozyme) as compared to those who are assigned to keep taking their medication while continuing to take Trikafta.

Detailed Description

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SIMPLIFY-IP-19 is a master protocol is designed to evaluate the independent effects of discontinuing hypertonic saline or dornase alfa in people with cystic fibrosis (CF) age 12 and older currently taking the highly effective modulator elexacaftor/tezacaftor/ivacaftor (ETI). The hypertonic saline and dornase alfa sub studies are identical open label two-arm randomized non-inferiority trials consisting of a 2-week run-in period, randomization to continue or discontinue hypertonic saline or dornase alfa, followed by a 6-week study period. Subjects taking only hypertonic saline (HS) or dornase alfa at trial entry will be randomized 1:1 to either continue or discontinue the applicable therapy (i.e. HS or dornase alfa). Subjects taking both hypertonic saline and dornase alfa at study entry will be randomized to participate in either the hypertonic saline or dornase alfa and will be randomized (1:1) to continue or discontinue the applicable therapy (i.e. HS or dornase alfa). After completion of the first study, eligible subjects may subsequently enroll in the alternative study.

This clinicalrials.gov registration, NCT04378153, summarizes the 2-week run-in and adherence period prior to participant enrollment in either the Hypertonic Saline or Dornase Alfa sub studies.

Two additional clincialtrials.gov records document the sub study specific enrollment and outcome measures.

Hypertonic Saline Study Record

* NCT06350461
* Brief Title: Impact of Discontinuing Hypertonic Saline in People With CF on Highly Effective CFTR Modulators- A SIMPLIFY Sub-Study (SIMPLIFY-HS)

Dornase Alfa Study Record

* NCT06350474
* Brief Title: Impact of Discontinuing Dornase Alfa in People With CF on Highly Effective CFTR Modulator Therapy-A SIMPLIFY Sub-Study (SIMPLIFY-DN)

Conditions

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Cystic Fibrosis

Study Design

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Observational Model Type

OTHER

Study Time Perspective

PROSPECTIVE

Study Groups

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Master Study Run-in

Participants enrolled in the run-in phase of the master protocol.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of CF.
* Age ≥ 12 years at the Screening Visit.
* Forced expiratory volume in 1 second (FEV1) ≥ 70 % predicted at the Screening Visit if \< 18 years old, and ≥ 60 % predicted at Screening Visit if ≥ 18 years old.
* Clinically stable with no significant changes in health status within the 7 days prior to and including the Screening Visit.
* Current treatment with elexacaftor/tezacaftor/ivacaftor (ETI) for at least the 90 days prior to and including the Screening Visit and willing to continue daily use for the duration of the study.
* Currently taking hypertonic saline (at least 3%) and/or dornase alfa for at least the 90 days prior to and including the Screening Visit and willing to continue daily use for the 2-week screening period.

Exclusion Criteria

* Active smoking or vaping.
* Use of an investigational drug within 28 days prior to and including the Screening Visit.
* Changes to chronic therapy (e.g., ibuprofen, azithromycin, inhaled tobramycin, aztreonam lysine) within 28 days prior to and including the Screening Visit. This includes new airway clearance routines.
* Acute use of antibiotics (oral, inhaled or IV) or acute use of systemic corticosteroids for respiratory tract symptoms within 7 days prior to and including the Screening Visit.
* Chronic use of systemic corticosteroids at a dose equivalent to ≥ 10mg per day of prednisone within 28 days prior to and including the Screening Visit.
* Antibiotic treatment for nontuberculous mycobacteria (NTM) within 28 days prior to and including the Screening Visit.
Minimum Eligible Age

12 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Cystic Fibrosis Foundation

OTHER

Sponsor Role collaborator

Dartmouth-Hitchcock Medical Center

OTHER

Sponsor Role collaborator

University of Washington

OTHER

Sponsor Role collaborator

Nicole Hamblett

OTHER

Sponsor Role lead

Responsible Party

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Nicole Hamblett

Professor of Pediatrics, Division of Pulmonary and Sleep Medicine, University of Washington School of Medicine Adjunct Professor, Biostatistics, University of Washington School of Medicine Co-Executive Director, Cystic Fibrosis Therapeutics Development

Responsibility Role SPONSOR_INVESTIGATOR

Principal Investigators

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Nicole Mayer-Hamblett, PhD

Role: PRINCIPAL_INVESTIGATOR

University of Washington/Seattle Children's

Alex Gifford, MD, FCCP

Role: PRINCIPAL_INVESTIGATOR

Dartmouth-Hitchcock Medical Center

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

Providence Alaska Medical Center

Anchorage, Alaska, United States

Site Status

Tucson Cystic Fibrosis Center

Tucson, Arizona, United States

Site Status

Arkansas Children's Hospital

Little Rock, Arkansas, United States

Site Status

Miller Children's and Women's Hospital Long Beach

Long Beach, California, United States

Site Status

CHOC Children's Hospital

Orange, California, United States

Site Status

Stanford University Medical Center

Palo Alto, California, United States

Site Status

Rady Children's Hospital and Health Center at the University of California San Diego

San Diego, California, United States

Site Status

University of California, San Francisco - Adult Center

San Francisco, California, United States

Site Status

University of California, San Francisco - Peds Center

San Francisco, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

National Jewish Health

Denver, Colorado, United States

Site Status

Yale University School of Medicine

New Haven, Connecticut, United States

Site Status

University of Florida

Gainesville, Florida, United States

Site Status

Nemours Children's Clinic

Jacksonville, Florida, United States

Site Status

Central Florida Pulmonary Group

Orlando, Florida, United States

Site Status

The Nemours Children's Clinic - Orlando

Orlando, Florida, United States

Site Status

Nemours Children's Clinic - Pensacola

Pensacola, Florida, United States

Site Status

All Children's Hospital

St. Petersburg, Florida, United States

Site Status

Tampa General Hospital

Tampa, Florida, United States

Site Status

Emory University

Atlanta, Georgia, United States

Site Status

Saint Luke's Cystic Fibrosis Center of Idaho

Boise, Idaho, United States

Site Status

Northwestern University

Chicago, Illinois, United States

Site Status

OSF Saint Francis Medical Center

Peoria, Illinois, United States

Site Status

Riley Hospital for Children

Indianapolis, Indiana, United States

Site Status

University of Iowa

Iowa City, Iowa, United States

Site Status

University of Kansas Medical Center

Kansas City, Kansas, United States

Site Status

University of Kentucky

Lexington, Kentucky, United States

Site Status

University of Louisville

Louisville, Kentucky, United States

Site Status

Tulane University

Metairie, Louisiana, United States

Site Status

Maine Medical Partners Pediatric Specialty Care

Portland, Maine, United States

Site Status

John Hopkins Hospital

Baltimore, Maryland, United States

Site Status

Boston Children's Hospital, Brigham & Women's Hospital

Boston, Massachusetts, United States

Site Status

University of Michigan, Michigan Medicine

Ann Arbor, Michigan, United States

Site Status

Wayne State University Harper University Hospital

Detroit, Michigan, United States

Site Status

Helen DeVos Children's Hospital

Grand Rapids, Michigan, United States

Site Status

Children's Hospitals and Clinics of Minnesota

Minneapolis, Minnesota, United States

Site Status

The Minnesota Cystic Fibrosis Center

Minneapolis, Minnesota, United States

Site Status

Children's Mercy Kansas City

Kansas City, Missouri, United States

Site Status

Washington University School of Medicine

St Louis, Missouri, United States

Site Status

Billings Clinic

Billings, Montana, United States

Site Status

Dartmouth Hitchcock Medical Center

Lebanon, New Hampshire, United States

Site Status

Monmouth Medical Center

Eatontown, New Jersey, United States

Site Status

Morristown Medical Center

Morristown, New Jersey, United States

Site Status

Rutgers - Robert Wood Johnson Medical School

New Brunswick, New Jersey, United States

Site Status

Cohen Children's Medical Center of New York

Lake Success, New York, United States

Site Status

Beth Israel Medical Center

New York, New York, United States

Site Status

Columbia University Cystic Fibrosis Program

New York, New York, United States

Site Status

University of Rochester Medical Center Strong Memorial

Rochester, New York, United States

Site Status

SUNY Upstate Medical University

Syracuse, New York, United States

Site Status

New York Medical College at Westchester Medical Center

Valhalla, New York, United States

Site Status

University of North Carolina at Chapel Hill

Chapel Hill, North Carolina, United States

Site Status

Wake Forest University Baptist Medical Center

Winston-Salem, North Carolina, United States

Site Status

Children's Hospital Medical Center of Akron

Akron, Ohio, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Rainbow Babies and Children's Hospital/University Hospitals Cleveland Medical Center

Cleveland, Ohio, United States

Site Status

Cleveland Clinic Cystic Fibrosis Program

Cleveland, Ohio, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Dayton Children's Hospital

Dayton, Ohio, United States

Site Status

Oregon Health Sciences University

Portland, Oregon, United States

Site Status

Hershey Medical Center Pennsylvania State University

Hershey, Pennsylvania, United States

Site Status

University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status

University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, United States

Site Status

Medical University of South Carolina

Charleston, South Carolina, United States

Site Status

Dell Children's Medical Center of Central Texas

Austin, Texas, United States

Site Status

University of Texas Southwestern / Children's Health

Dallas, Texas, United States

Site Status

University of Texas Southwestern

Dallas, Texas, United States

Site Status

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status

University of Texas Health Center at Tyler

Tyler, Texas, United States

Site Status

Primary Children's Cystic Fibrosis Center

Salt Lake City, Utah, United States

Site Status

University of Vermont Medical Center

Burlington, Vermont, United States

Site Status

University of Virginia

Charlottesville, Virginia, United States

Site Status

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

University of Washington Medical Center

Seattle, Washington, United States

Site Status

Providence Medical Group, Cystic Fibrosis Center

Spokane, Washington, United States

Site Status

West Virginia University - Morgantown

Morgantown, West Virginia, United States

Site Status

University of Wisconsin

Madison, Wisconsin, United States

Site Status

Children's Hospital of Wisconsin

Milwaukee, Wisconsin, United States

Site Status

Froedtert & Medical College of Wisconsin

Milwaukee, Wisconsin, United States

Site Status

Countries

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United States

References

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Gold LS, Hansen RN, Mayer-Hamblett N, Nichols DP, Gifford AH, Kloster M, Goss CH, Kessler L. The cost of simplifying treatments for cystic fibrosis: Implications of the SIMPLIFY trial. J Manag Care Spec Pharm. 2024 Jan;30(1):26-33. doi: 10.18553/jmcp.2024.30.1.26.

Reference Type DERIVED
PMID: 38153868 (View on PubMed)

Mayer-Hamblett N, Ratjen F, Russell R, Donaldson SH, Riekert KA, Sawicki GS, Odem-Davis K, Young JK, Rosenbluth D, Taylor-Cousar JL, Goss CH, Retsch-Bogart G, Clancy JP, Genatossio A, O'Sullivan BP, Berlinski A, Millard SL, Omlor G, Wyatt CA, Moffett K, Nichols DP, Gifford AH; SIMPLIFY Study Group. Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials. Lancet Respir Med. 2023 Apr;11(4):329-340. doi: 10.1016/S2213-2600(22)00434-9. Epub 2022 Nov 4.

Reference Type DERIVED
PMID: 36343646 (View on PubMed)

Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2021 Mar 18;3(3):CD001127. doi: 10.1002/14651858.CD001127.pub5.

Reference Type DERIVED
PMID: 33735508 (View on PubMed)

Mayer-Hamblett N, Nichols DP, Odem-Davis K, Riekert KA, Sawicki GS, Donaldson SH, Ratjen F, Konstan MW, Simon N, Rosenbluth DB, Retsch-Bogart G, Clancy JP, VanDalfsen JM, Buckingham R, Gifford AH. Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design. Ann Am Thorac Soc. 2021 Aug;18(8):1397-1405. doi: 10.1513/AnnalsATS.202010-1336SD.

Reference Type DERIVED
PMID: 33465316 (View on PubMed)

Provided Documents

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Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

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SIMPLIFY-IP-19

Identifier Type: -

Identifier Source: org_study_id