Exercise in People With Cystic Fibrosis on CFTR Modulator Therapy (FIQMODE)

Polytechnic University of Madrid (UPM)

Status: Enrolling

Conditions: Cystic Fibrosis

Treatments: Other: Exercise

Study type: Interventional

Funder types: Other

Identifiers

NCT06322446

PI23/00299

Details and patient eligibility

About

Recently, the treatment of Cystic Fibrosis (CF) incorporated new modulators/enhancers of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). It is thus increasingly important to study the side effects of these drugs, their extrapulmonary effects and possible interaction with other drugs and with exercise. For this purpose, a randomized controlled trial is proposed to determine the effects of a telematic exercise intervention on muscle health, in a group of 48 children and adolescents with CF treated with these new generation CFTR modulators. They will be randomly assigned to two groups (exercise and control group). The effect of the intervention will be analysed measuring the variables of muscle health, cardiorespiratory fitness, lung function, body composition, inflammatory biomarkers and miRNAs. After completion of the intervention program, adherence to exercise and clinical evolution after one year will be analysed.

Full description

Specific objectives

1. To determine the effects of a strength exercise intervention in a group of children and adolescents with cystic fibrosis treated with new generation CFTR modulators on: (1.i) peripheral muscle strength; (1.ii) respiratory muscle strength; (1.iii) muscle mass and (1.iv) biomarkers of muscle damage.
2. To determine the effects of a strength exercise intervention in a group of children and adolescents with cystic fibrosis being treated with new generation CFTR modulators on: (2.i) cardiorespiratory fitness; (2.ii) body composition and (2.iii) lung function.
3. To determine the effects of a strength-based exercise intervention in a group of children and adolescents with cystic fibrosis treated with new-generation CFTR modulators on: (3.i) biomarkers of inflammatory status and (3.ii) expression of associated miRNAs.
4. To determine the effects of a strength-based exercise intervention in a group of children and adolescents with cystic fibrosis treated with the new generation CFTR modulators on the levels of Elexaxcaftor/Ivacaftor/Tezacaftor and their metabolites.
5. To assess the clinical evolution and adherence to exercise after 6 months of the programme in children and adolescents with cystic fibrosis treated with the new generation of CFTR modulators.

Enrollment: 48 estimated patients

Sex: All

Ages: 6 to 20 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• patients diagnosed with CF.
• patients between 6-20 years of age.
• patients receiving treatment with new CFTR protein modulating medication
• reading, acceptance and signing of the informed consent form.

Exclusion criteria

• CF patients with symptoms of pulmonary exacerbation during the last four weeks.
• with a diagnosis of other cardiorespiratory lung diseases progressing to a symptom of persistent respiratory dysfunction.
• CF patients with musculoskeletal alterations that influence assessments.
• CF patient who is pregnant during the time of the study
• CF patient with cognitive impairment;
• CF patient with incomplete dosing of modulator therapy;

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: Single Blind

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48 participants in 2 patient groups

Intervention

Experimental group

Description:

Telematic Exercise: A remotely supervised resistance exercise program will be carried out for 16 weeks, with two weekly sessions lasting approximately 60 minutes each. Training will be performed in groups of four patients, according to their lung function/physical fitness. The first training session will be on site (University) for familiarization, planning and adjustment of the exercises, and the following sessions will be performed online. Each session is divided into: (i) Warm-up and joint mobility; (ii) main part: strength exercises for different muscle groups; and (iii) cool down: stretching and breathing exercises.

Treatment:

Other: Exercise

Control

No Intervention group

Description:

Control group will follow routine recommendations from the multidisciplinary CF team based on WHO´s guidelines