Muscle Function and Physical Activity in the Modern Era of Cystic Fibrosis

Cystic Fibrosis Clinical Trial

— MUCOMUSCLE  

Official title:

Comparison of Muscle Function and Physical Activity Levels Between People With Cystic Fibrosis and Healthy Individuals in the Modern Era of CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Modulators: A Case-Control Study

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT06251622
• Other study ID #: 69HCL23_1226
• Status: Recruiting
• Start date: February 19, 2024
• Est. completion date: October 1, 2025

Study information

• Verified date: April 2024
• Source: Hospices Civils de Lyon
• Contact: Laurent MD MELY
• Phone: 04 94 38 17 52
• Email: laurent.mely[@]chu-lyon.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

Cystic fibrosis (CF) is characterized by various extrapulmonary manifestations, including altered skeletal muscle function, with both quantitative (e.g. reduced muscle mass) and qualitative (e.g. impaired oxidative function) impairments that may have a negative impact on exercise tolerance and quality of life. These abnormalities have traditionally been related to disease (e.g. systemic inflammation) or behavioral factors (e.g. increased physical inactivity). However, most of the studies that observed these abnormalities and tried to shed light on the underlying factors were either small or conducted before the widespread of CFTR (Cystic fibrosis transmembrane conductance regulator) modulators that have profound impact on the trajectory of the disease. While several studies suggested that the major recent improvements in therapeutics, including highly effective CFTR modulators, may have positive effects on skeletal muscle function, either directly (e.g. improved mitochondrial function) or indirectly (e.g. reduction in physical inactivity), no studies to date have thoroughly investigated this issue in a representative sample of people with CF. The absence of recent data on muscle function and physical activity levels casts doubt on the relevance of recommendations on exercise training in this population that were published before the widespread use of highly effective CFTR modulators. This study aims to compare muscle function, measured according to the latest recommendations of the European Cystic Fibrosis Society (Saynor et al., 2023), and physical activity of children and adults with CF under CFTR modulators, compared to age- and sex-matched healthy individuals. We hypothesize that the strength, endurance, muscle power, and physical activity levels of individuals with cystic fibrosis, treated with CFTR modulators, remain reduced compared to healthy individuals.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 112
• Est. completion date: October 1, 2025
• Est. primary completion date: August 1, 2025
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 10 Years and older

Eligibility

Inclusion Criteria:

• Criteria for inclusion of CF patients:
• Diagnosis of cystic fibrosis
• Treated with CFTR modulator therapy
• Men and women (children or adults) aged 10 years or older
• Affiliated with or entitled to social security coverage
• For people = 18 years old: participant expressing its oral non-opposition
• For minor child (<18 years old): participant and its legal representative expressing their oral non-opposition
• Criteria for inclusion of healthy subjects:
• Men and women (children or adults) aged 10 years or older
• Affiliated with or entitled to social security coverage
• For people = 18 years old: participant expressing its oral non-opposition
• For minor child (<18 years old): participant and its legal representative expressing their oral non-opposition

Exclusion Criteria:

• Exclusion criteria for CF patients:
• Lack of a stable clinical condition (defined here as the presence of a pulmonary exacerbation and/or a significant change in treatments in the three weeks preceding inclusion in the study).
• Medical contraindication to engage in moderate-intensity physical activity.
• Knee joint pain incompatible with the measurement of quadriceps strength.
• Pregnancy.
• Exclusion criteria for healthy subjects:
• Known chronic respiratory, cardiovascular, metabolic, renal, or neuromuscular diseases.
• Presence of benign illnesses and/or acute infections requiring a doctor's visit and occasional treatment in the three weeks preceding inclusion in the study.
• Medical contraindication to engage in moderate-intensity physical activity.
• Knee joint pain incompatible with the measurement of quadriceps strength.
• Pregnancy.

Related Conditions & MeSH terms

• Cystic Fibrosis
• Fibrosis

Intervention

Other:
• Volumetric ultrasound of the quadriceps and fitting of an accelerometer
The research procedure will simply involve a volumetric ultrasound of the quadriceps and the fitting of an accelerometer (watch worn on the wrist) for one week (collection of usual physical activities).

Locations

Country	Name	City	State
France	HCL - Hôpital Renée Sabran	Hyères

Sponsors (1)

Lead Sponsor	Collaborator
Hospices Civils de Lyon

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Measurement of isometric quadriceps strength (expressed in Newton), performed at René Sabran Hospital (as part of routine clinical practice for people with CF)	The quadriceps strength will be measured on an isometric chair according to the last recommendations of the European Cystic Fibrosis Society (Saynor et al., 2023). Briefly, participants will perform at least three maximal voluntary contractions of the knee extensors, each lasting 4-6s, with a minimal recovery of 1-min between each attempt, with the aim to obtain three maximal quadriceps strength values varying less than 5% (that is usually obtained in less than 6 trials). The highest strength value among these three attempts will be kept for analysis.	Day 0