Exercise in People With Cystic Fibrosis on CFTR Modulator Therapy

Specific objectives

• 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.
• 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.
• 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.
• 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.
• 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.

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;