Prevalence of Exercise-induced Ventilatory Limitation and Associated Factors in Patients With Cystic Fibrosis Receiving Elexacaftor-Tezacaftor-Ivacaftor

NCT07314229 | Recruiting

• 2 other identifiers: 2024_02772024-A02709-38
• Study Type: interventional
• Target: 130
• Locations: 1 country
• Sites: 1

Brief Summary

Cystic fibrosis is a genetic disorder affecting the entire body and associated with respiratory exacerbations, impaired quality of life and reduced life expectancy. The therapeutic management of cystic fibrosis has been profoundly changed by the recent arrival of a combination of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators, Elexacaftor-Tezacaftor-Ivacaftor (ETI), which improve quality of life, respiratory function and reducing the number of exacerbations. The impact of these treatments on exercise adaptation has not been clearly identified. The main objective is to estimate the prevalence of ventilatory reserve amputation during submaximal exercise testing assessed by the 6-minute walk test (6MWT) in patients with cystic fibrosis treated with ETIs.

Conditions

Cystic Fibrosis (CF); Mucoviscidosis

Keywords

Cystic fibrosis; physical activity; CFTR modulator treatment; exercise limitation; respiratory physiology; ventilatory limitation; reduction in ventilatory reserve; dynamic distension

Outcome Measures

Primary Outcomes (1)

• Estimate the prevalence of ventilatory limitation measured by a portable spirometer during a submaximal exercise test (6-minute walk test) in patients with cystic fibrosis undergoing ETI.

  The primary endpoint is ventilatory limitation defined as the difference between the estimated theoretical maximun minute ventilatory volume (VMM in L/min) estimated by multiplying FEV1 (L) x 35 and the measurement of external ventilation during exercise (VE, L/min) using a portable spirometer divided by the theoretical VM x100 less than 15%. Ventilatory limitation = (MMV (L/min) - VE (L/min) /MMV (L/min))\*100 \< 15%

  duration of 56 weeks starting in December 2025

Secondary Outcomes (2)

• Estimate the prevalence of ventilatory limitation measured using a portable spirometer during a maximal incremental step test (A-STEP) with progressive steps in patients with cystic fibrosis undergoing ETI.

  duration of 56 weeks starting in December 2025

• Assessment of the prevalence of dynamic distension during a submaximal (TM6) or maximal (incremental step test according to the A-Step protocol) exercise test by spirometry

  duration of 56 weeks starting in December 2025

Study Arms (1)

patients treated with ETI (Kaftrio-Kalydeco©)

EXPERIMENTAL

Device: Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6

Interventions

Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6 DEVICE

Measurement of resistance through forced oscillations and continuous measurement of ventilation and inspiratory capacity during TM6

patients treated with ETI (Kaftrio-Kalydeco©)

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Male or female
• Adult aged 18 or over
• Suffering from cystic fibrosis
• Treated at the CRCM in Lille and Créteil
• Treated by ETI
• Be covered by social security
• Be able to understand the requirements of the study, provide written informed consent, and comply with the study's data collection procedures

You may not qualify if:

• Medical contraindication or inability to perform a stress test according to ERS recommendations
• Absolute contraindications
• Relative contraindications:
• Exacerbation of the condition in the 4 weeks preceding the V1 visit (27).
• Pregnant or breastfeeding women
• Administrative reasons
• Persons deprived of their liberty
• Minors or protected adults
• Persons who have refused or are unable to give informed consent
• Persons in emergency situations

Sponsors & Collaborators

• University Hospital, Lille: lead
• Vaincre la Mucoviscidose: collaborator

Study Sites (1)

University Hospital

Lille, 59037, France

RECRUITING

MeSH Terms

Conditions

Cystic Fibrosis; Motor Activity

Condition Hierarchy (Ancestors)

Pancreatic Diseases; Digestive System Diseases; Lung Diseases; Respiratory Tract Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Infant, Newborn, Diseases; Behavior

Central Study Contacts

Camille AUDOUSSET, Doctor

CONTACT

+33 3.20.44.41.45; DRC@chu-lille.fr

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: HEALTH SERVICES RESEARCH
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Model Details: No control group. Patients treated with ETI (Kaftrio-Kalydeco©) are treated as part of the management of their condition in accordance with the indications for marketing authorisation (MA), temporary authorisation for use (ATU) or French compassionate access.

Study Record Dates

• First Submitted: November 24, 2025
• First Posted: January 2, 2026
• Study Start: December 17, 2025
• Primary Completion (Estimated): February 2, 2027
• Study Completion (Estimated): April 4, 2027
• Last Updated: April 22, 2026

Record last verified: 2026-04

Locations

FR (1)