Combined Effect of CFTR Protein Modulator Drugs and Exercise in Cystic Fibrosis

NCT ID: NCT04415268

Last Updated: 2023-12-11

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

WITHDRAWN

Clinical Phase

NA

Study Classification

INTERVENTIONAL

Study Start Date

2020-06-09

Study Completion Date

2021-05-15

Brief Summary

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This study aims to assess the effects of programmed exercise combined with CFTR protein modulator drugs in the cardiorespiratory fitness, strength, functional capacity and agility in a group of young patients with Cystic Fibrosis.

Detailed Description

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The present study is a new clinical trial that extends our previous work intended to assess the effect of programmed exercise with or without electrical stimulation on cardiorespiratory fitness, strength, functional capacity and agility in a group of young patients with Cystic Fibrosis (ECOMIRIN) registered at ClinicalTrials.gov (Identifier: NCT04153669).

After finishing the study, a subset of the participants was treated with either TEZ/IVA or LUM/IVA per standard of care, as they were considered candidates according to the AEMPS (https://www.clinicaltrialsregister.eu/ctr-search/trial/2019-000833-37/ES). Those patients are expected to complete five months of pharmacological treatment without exercise by June 2020.

Primary Objective To evaluate the effect of an exercise program on physical fitness in cystic fibrosis patients after 5 months of administration of LUM/IVA or TEZ/IVA in comparison to the effect produced by the same exercise program before administration of the drug.

Secondary Objectives Other specific objectives include comparing the beneficial effects of exercise and LUM/IVA or TEZ/IVA on: 1) cardiorespiratory function and muscle strength; 2) the concentration of chlorine in sweat; and 3) quality of life.

Conditions

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Cystic Fibrosis in Children

Keywords

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Cystic Fibrosis Electrostimulation Combined Training Physical Fitness

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Single-group non-randomized, prospective. Within-subject multitreatment
Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Multitreatment

Pharmacological treatment per standard of care (whole study length, starting on week 1).

Supervised exercise protocol (phase 1, 8 weeks starting on week 9). Unsupervised exercise protocol (phase 2, 8 weeks starting on week 17).

Group Type EXPERIMENTAL

Exercise

Intervention Type BEHAVIORAL

Surpervised exercise program: Includes a concurrent exercise intervention (strength training and aerobic training), 3 days a week, 60 minutes sessions.

Unsupervised exercise

Intervention Type BEHAVIORAL

Unsupervised exercise program: The same exercise program learned during the supervised phase will be practiced at home.

CFTR Modulators

Intervention Type DRUG

Standard of care: Lumacaftor with Ivacaftor or Tezacaftor combined with Ivacaftor.

For children between 6 and 11 years of age, Lumacaftor 400 mg combined with Ivacaftor 500 mg in total per day, divided into 2 doses per day; for those over 11 years of age, Tezacaftor 100 mg combined with Ivacaftor 150 mg in the morning, adding a dose of Ivacaftor 150 mg at night.

https://www.clinicaltrialsregister.eu/ctr-search/trial/2019-000833-37/ES

Interventions

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Exercise

Surpervised exercise program: Includes a concurrent exercise intervention (strength training and aerobic training), 3 days a week, 60 minutes sessions.

Intervention Type BEHAVIORAL

Unsupervised exercise

Unsupervised exercise program: The same exercise program learned during the supervised phase will be practiced at home.

Intervention Type BEHAVIORAL

CFTR Modulators

Standard of care: Lumacaftor with Ivacaftor or Tezacaftor combined with Ivacaftor.

For children between 6 and 11 years of age, Lumacaftor 400 mg combined with Ivacaftor 500 mg in total per day, divided into 2 doses per day; for those over 11 years of age, Tezacaftor 100 mg combined with Ivacaftor 150 mg in the morning, adding a dose of Ivacaftor 150 mg at night.

https://www.clinicaltrialsregister.eu/ctr-search/trial/2019-000833-37/ES

Intervention Type DRUG

Other Intervention Names

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EudraCT Number 2019-000833-37

Eligibility Criteria

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Inclusion Criteria

* In order to be included, patients must have participated in the ECOMIRIN clinical trial (ClinicalTrials.gov Identifier: NCT04153669).
* They must have a previous CF diagnosis, defined as: sweat chlorine ≥ 60 mEq/L and/or carry two variants of the CFTR gene characterized as pathogenic.
* Age 6-18 years; eligible to receive treatment with TEZ/IVA or LUM/IVA according to the AEMPS (LUM/IVA for 6-11 year-old children homozygous for the F508del mutation; TEZ/IVA for ≥ 12 year-old children homozygous for the Phe508del mutation, as well as heterozygous for the F508 mutation plus one of the following mutations: P67L, R117C, L206W, R352Q, A455E, D579G, 711 + 3A G, S945L, S977F, R1070W, D1152H, 2789 + 5G A, 3272-26A G, and 3849 + 10kbC T);
* Agreement to collaborate in performing static and dynamic pulmonary function tests.
* Consent of children and/or parents or legal guardians to participate in the study after having read and understood the informed consent form.

Exclusion Criteria

* CF patients not consenting to participate in the study at any time after reading and understanding the informed consent form will be excluded.
* CF patients who are not eligible to receive treatment with TEZ/IVA or LUM/IVA according to the AEMPS during the course of the study will be excluded.
* Study participants will be excluded if they test positive for any contagious viral infection that may affect the outcomes (e.g. SARS-CoV-2).
Minimum Eligible Age

6 Years

Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Universidad Europea de Madrid

OTHER

Sponsor Role lead

Responsible Party

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Margarita Perez

Full Professor MD, PhD

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Margarita Perez-Ruiz, MD

Role: PRINCIPAL_INVESTIGATOR

Universidad Politecnica de Madrid

Locations

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Escuela de Doctorado e Investigacion, Universidad Europea

Villaviciosa de Odón, , Spain

Site Status

Countries

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Spain

Other Identifiers

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CIPI/20/119

Identifier Type: -

Identifier Source: org_study_id