Exercise in People With Cystic Fibrosis on CFTR Modulator Therapy
NCT ID: NCT06322446
Last Updated: 2024-03-21
Study Results
Results pending
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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Recruitment Status
RECRUITING
Clinical Phase
NA
Total Enrollment
48 participants
Study Classification
INTERVENTIONAL
Study Start Date
2024-02-27
Study Completion Date
2025-12-23
Brief Summary
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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.
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Detailed Description
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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.
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.
Conditions
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Cystic Fibrosis
Study Design
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Allocation Method
RANDOMIZED
Intervention Model
PARALLEL
Primary Study Purpose
TREATMENT
Blinding Strategy
SINGLE
Outcome Assessors
Study Groups
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Intervention
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.
Group Type
EXPERIMENTAL
Exercise
Intervention Type
OTHER
16-week exercise intervention: At the beginning of the intervention, we will conduct a face-to-face familiarisation session with the exercises of the training programme. The intervention will consist of 2 sessions/week for 16 weeks. Each session consists of three stages: (i) Warm-up: 10 min of joint mobility and low intensity exercises involving the musculature to be worked in that session; (ii) Main part: circuit training mainly composed of strength exercises targeting the different muscle groups of the body and playing activities (iii) Cool down: 10 min with guided breathing work and stretching of the main muscle groups worked
Control
Control group will follow routine recommendations from the multidisciplinary CF team based on WHO´s guidelines
Group Type
NO_INTERVENTION
No interventions assigned to this group
Interventions
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Exercise
16-week exercise intervention: At the beginning of the intervention, we will conduct a face-to-face familiarisation session with the exercises of the training programme. The intervention will consist of 2 sessions/week for 16 weeks. Each session consists of three stages: (i) Warm-up: 10 min of joint mobility and low intensity exercises involving the musculature to be worked in that session; (ii) Main part: circuit training mainly composed of strength exercises targeting the different muscle groups of the body and playing activities (iii) Cool down: 10 min with guided breathing work and stretching of the main muscle groups worked
Intervention Type
OTHER
Eligibility Criteria
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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.
* 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;
* 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;
Minimum Eligible Age
6 Years
Maximum Eligible Age
20 Years
Eligible Sex
ALL
Accepts Healthy Volunteers
No
Sponsors
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Carlos III Health Institute
OTHER_GOV
Sponsor Role
collaborator
Universidad Europea de Madrid
OTHER
Sponsor Role
collaborator
Hospital Universitario La Paz
OTHER
Sponsor Role
collaborator
Hospital Universitario Ramon y Cajal
OTHER
Sponsor Role
collaborator
Hospital Infantil Universitario Niño Jesús, Madrid, Spain
OTHER
Sponsor Role
collaborator
Universidad Politecnica de Madrid
OTHER
Sponsor Role
lead
Responsible Party
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Margarita Pérez-Ruiz
full professor in Physical Activity and Health
Responsibility Role
PRINCIPAL_INVESTIGATOR
Principal Investigators
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Margarita Perez Ruiz, PhD
Role: PRINCIPAL_INVESTIGATOR
Universidad Politecnica de Madrid
Locations
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Facultad de Ciencias de la Actividad Física y Deporte - INEF UPM
Madrid, , Spain
Site Status
RECRUITING
Countries
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Spain
Central Contacts
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Facility Contacts
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References
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RESULT
King JA, Nichols AL, Bentley S, Carr SB, Davies JC. An Update on CFTR Modulators as New Therapies for Cystic Fibrosis. Paediatr Drugs. 2022 Jul;24(4):321-333. doi: 10.1007/s40272-022-00509-y. Epub 2022 May 16.
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RESULT
Rodriguez-Miguelez P, Seigler N, Ishii H, Crandall R, McKie KT, Forseen C, Harris RA. Exercise Intolerance in Cystic Fibrosis: Importance of Skeletal Muscle. Med Sci Sports Exerc. 2021 Apr 1;53(4):684-693. doi: 10.1249/MSS.0000000000002521.
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RESULT
Wells GD, Wilkes DL, Schneiderman JE, Rayner T, Elmi M, Selvadurai H, Dell SD, Noseworthy MD, Ratjen F, Tein I, Coates AL. Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia. Pediatr Res. 2011 Jan;69(1):40-5. doi: 10.1203/PDR.0b013e3181fff35f.
Reference Type
RESULT
Bene Z, Fejes Z, Macek M Jr, Amaral MD, Balogh I, Nagy B Jr. Laboratory biomarkers for lung disease severity and progression in cystic fibrosis. Clin Chim Acta. 2020 Sep;508:277-286. doi: 10.1016/j.cca.2020.05.015. Epub 2020 May 16.
Reference Type
RESULT
Stachowiak Z, Wojsyk-Banaszak I, Jonczyk-Potoczna K, Narozna B, Langwinski W, Szczepankiewicz A. Extracellular vesicles-derived miRNAs as mediators of pulmonary exacerbation in pediatric cystic fibrosis. J Breath Res. 2023 Feb 10;17(2). doi: 10.1088/1752-7163/acb792.
Reference Type
RESULT
Pantoja-Arevalo L, Yvert T, Iturriaga T, Sanz-Santiago V, Barcelo O, Quesada-Gonzalez C, Morales-Tirado A, Santiago-Dorrego C, Lopez-Neyra A, Ruiz de Valbuena M, De Manuel Gomez C, Rubio Alonso M, De Vidania S, Ramirez-Castillejo C, Gonzalez-Gross M, Perez-Ruiz M. Strength-Oriented Virtual Exercise Training Intervention in Children and Adolescents with Cystic Fibrosis Under CFTR Modulators (the FIQMODE Study): Study Protocol for a Randomized Controlled Trial. Curr Protoc. 2025 Sep;5(9):e70202. doi: 10.1002/cpz1.70202.
Reference Type
DERIVED
Other Identifiers
Review additional registry numbers or institutional identifiers associated with this trial.
PI23/00299
Identifier Type: -
Identifier Source: org_study_id
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