NCT04732910

Brief Summary

This observational study evaluates the effect of therapy with cystic fibrosis transmembrane regulator (CFTR) modulators on CFTR function measured by the CFTR biomarker intestinal current measurement (ICM), nasal potential difference (NPD) and sweat chloride in a post-approval setting in patients with cystic fibrosis (CF).

Trial Health

77
On Track

Trial Health Score

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

Enrollment
500

participants targeted

Target at P75+ for all trials

Timeline
17mo left

Started Jul 2018

Longer than P75 for all trials

Geographic Reach
1 country

4 active sites

Status
recruiting

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

Study Progress85%
Jul 2018Dec 2027

Study Start

First participant enrolled

July 1, 2018

Completed
2.6 years until next milestone

First Submitted

Initial submission to the registry

January 25, 2021

Completed
7 days until next milestone

First Posted

Study publicly available on registry

February 1, 2021

Completed
6.2 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

March 31, 2027

Expected
9 months until next milestone

Study Completion

Last participant's last visit for all outcomes

December 31, 2027

Last Updated

February 24, 2026

Status Verified

February 1, 2026

Enrollment Period

8.8 years

First QC Date

January 25, 2021

Last Update Submit

February 20, 2026

Conditions

Keywords

CFTRICMNPD

Outcome Measures

Primary Outcomes (1)

  • Intestinal current measurement (ICM)

    Absolute change from baseline of the chloride secretory ion current induced by cyclic adenosine monophosphate (cAMP) stimulation (forskolin/3-isobutyl-1-methylxanthine (IBMX)) in rectal tissue determined by intestinal current measurement (ICM) as a cystic fibrosis transmembrane conductance regulator (CFTR) biomarker

    12 weeks

Secondary Outcomes (20)

  • Forced expiratory volume in 1 second (FEV1)

    quaterly in the first year, yearly thereafter up to 5 years

  • Nasal potential Difference (NPD)

    12 weeks

  • Sweat chloride

    12, 52 weeks, yearly thereafter up to 5 years

  • Lung clearance index (LCI)

    quaterly in the first year, yearly thereafter up to 5 years

  • Lung magnetic resonance imaging (MRI)

    12, 52 weeks, yearly thereafter up to 5 years

  • +15 more secondary outcomes

Interventions

Eligibility Criteria

Age6 Months+
Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Cystic Fibrosis patients

You may qualify if:

  • Decision for cystic fibrosis (CF) transmembrane regulator (CFTR)-modulator therapy by the patient and the caring CF physician
  • Signed informed consent form (ICF) and, where appropriate, signed assent form.

You may not qualify if:

  • Ongoing participation in an investigational drug study (including studies investigating lumacaftor, tezacaftor or ivacaftor)

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (4)

Charité - Universitätsmedizin Berlin

Berlin, State of Berlin, 13353, Germany

RECRUITING

Justus-Liebig-University Giessen

Giessen, Germany

RECRUITING

Hannover Medical School

Hanover, Germany

RECRUITING

University of Heidelberg

Heidelberg, Germany

RECRUITING

Related Publications (4)

  • Dohna M, Voskrebenzev A, Klimes F, Kaireit TF, Glandorf J, Pallenberg ST, Ringshausen FC, Hansen G, Renz DM, Wacker F, Dittrich AM, Vogel-Claussen J. PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study. Radiol Cardiothorac Imaging. 2024 Apr;6(2):e230104. doi: 10.1148/ryct.230104.

  • Schaupp L, Addante A, Voller M, Fentker K, Kuppe A, Bardua M, Duerr J, Piehler L, Rohmel J, Thee S, Kirchner M, Ziehm M, Lauster D, Haag R, Gradzielski M, Stahl M, Mertins P, Boutin S, Graeber SY, Mall MA. Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis. Eur Respir J. 2023 Aug 3;62(2):2202153. doi: 10.1183/13993003.02153-2022. Print 2023 Aug.

  • Graeber SY, Renz DM, Stahl M, Pallenberg ST, Sommerburg O, Naehrlich L, Berges J, Dohna M, Ringshausen FC, Doellinger F, Vitzthum C, Rohmel J, Allomba C, Hammerling S, Barth S, Ruckes-Nilges C, Wielputz MO, Hansen G, Vogel-Claussen J, Tummler B, Mall MA, Dittrich AM. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med. 2022 Aug 1;206(3):311-320. doi: 10.1164/rccm.202201-0219OC.

  • Graeber SY, Vitzthum C, Pallenberg ST, Naehrlich L, Stahl M, Rohrbach A, Drescher M, Minso R, Ringshausen FC, Rueckes-Nilges C, Klajda J, Berges J, Yu Y, Scheuermann H, Hirtz S, Sommerburg O, Dittrich AM, Tummler B, Mall MA. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med. 2022 Mar 1;205(5):540-549. doi: 10.1164/rccm.202110-2249OC.

Biospecimen

Retention: SAMPLES WITH DNA

Sputum, Blood

MeSH Terms

Conditions

Cystic Fibrosis

Interventions

Therapeuticslumacaftor, ivacaftor drug combinationtezacaftor, ivacaftor drug combinationelexacaftor, ivacaftor, tezacaftor drug combinationdeutivacaftor, tezacaftor , vanzacaftor

Condition Hierarchy (Ancestors)

Pancreatic DiseasesDigestive System DiseasesLung DiseasesRespiratory Tract DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesInfant, Newborn, Diseases

Study Officials

  • Marcus A Mall, MD

    Charite University, Berlin, Germany

    PRINCIPAL INVESTIGATOR
  • Simon Y Graeber, MD

    Charite University, Berlin, Germany

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Clinician Scientist

Study Record Dates

First Submitted

January 25, 2021

First Posted

February 1, 2021

Study Start

July 1, 2018

Primary Completion (Estimated)

March 31, 2027

Study Completion (Estimated)

December 31, 2027

Last Updated

February 24, 2026

Record last verified: 2026-02

Locations