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NCT Number: NCT07314229

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

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.

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Key information

Conditions

Cystic Fibrosis (CF) Behavior Congenital, Hereditary, and Neonatal Diseases and Abnormalities Cystic Fibrosis Digestive System Diseases Genetic Diseases, Inborn Infant, Newborn, Diseases Lung Diseases Motor Activity Mucoviscidosis Pancreatic Diseases Respiratory Tract Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

University Hospital

Lille, 59037, France

Location status: Recruiting

Location contact

Camille Audousset, Doctor

CONTACT

[email protected]

0320445544

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • 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

Exclusion criteria

  • 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

Treatment and study plan

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

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.

    Time frame: duration of 56 weeks starting in December 2025

    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%

Secondary outcomes

  1. 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.

    Time frame: duration of 56 weeks starting in December 2025

    The assessment criterion is 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 (VE in L/min) using a portable spirometer during exercise divided by the theoretical VM x100 less than 15%.

  2. 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

    Time frame: duration of 56 weeks starting in December 2025

    The criterion for judgement is a decrease in inspiratory capacity (L) of more than 150 mL within 30 seconds after the end of the effort compared to the pre-effort inspiratory capacity (L) measured by spirometry.

Study contacts

Contact information is provided by the study sponsor or research team.

Camille AUDOUSSET, Doctor

CONTACT

[email protected]

+33 3.20.44.41.45 ext. +33

Sponsors and collaborators

Lead sponsor

University Hospital, Lille

Other

Collaborators

  • Vaincre la Mucoviscidose

Registry information

Acronym: MUCOLIMEX

Important dates

Study start
2025
Primary completion
2027
Study completion
2027
First posted
Jan 2, 2026
Registry last updated
Apr 22, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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