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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Multitreatment | Experimental | 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). |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Exercise | Behavioral | Surpervised exercise program: Includes a concurrent exercise intervention (strength training and aerobic training), 3 days a week, 60 minutes sessions. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change in Strength | Changes in strength will be measured using a five repetition maximum test (5RM) | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Change in Cardiorespiratory Fitness | Changes in cardiorespiratory fitness will be measured using a cardiopulmonary exercise test (CPET) | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Measure | Description | Time Frame |
|---|---|---|
| Changes in Forced expiratory volume in 1 second (FEV1) | Changes in FEV1 will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019) | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Changes in Forced vital capacity (FVC) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Margarita Perez-Ruiz, MD | Universidad Politecnica de Madrid | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Escuela de Doctorado e Investigacion, Universidad Europea | Villaviciosa de Odón | 28670 | Spain |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| D015444 | Exercise |
| ID | Term |
|---|---|
| D009043 | Motor Activity |
| D009068 | Movement |
| D009142 | Musculoskeletal Physiological Phenomena |
| D055687 | Musculoskeletal and Neural Physiological Phenomena |
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Single-group non-randomized, prospective. Within-subject multitreatment
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| Unsupervised exercise | Behavioral | Unsupervised exercise program: The same exercise program learned during the supervised phase will be practiced at home. |
|
| CFTR Modulators | 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 |
|
|
Changes in FVC will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019) |
| Four assessment points throughout the study: baseline and after each 8-week intervention |
| Changes in FEV1/FVC ratio (FEV1%) | Changes in FEV1/FVC ratio (FEV1%) will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019) | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Changes in Forced expiratory flow (FEF) | Changes in FEF will be measured using Spirometry (z-score based on Global Lung Function Initiative reference DOI: 10.1016/j.arbres.2017.07.019) | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Changes in Physical Activity Questionnaire (PAQ) for children and adolescents | Changes in physical activity levels will be measured using PAQ-C for children under 14 years of age and PAQ-A for adolescents over 14 years of age. Items 1 to 9 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-C activity summary score. Items 1 to 8 will be used in the physical activity composite score, and means will be calculated to obtain the final PAQ-A activity summary score. A score of 1 indicates low physical activity, whereas a score of 5 indicates high physical activity. | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Change in quality of life: Cystic Fibrosis-Questionnaire-Revised (CFQ-R) | Changes in quality of life will be measures with the Cystic Fibrosis-Questionnaire-Revised (CFQ-R). Scores for each health related quality of life domain are calculated; after recoding, each item is summed to generate a domain score and standardized. Scores range from 0 to 100, with higher scores indicating better health. | Four assessment points throughout the study: baseline and after each 8-week intervention |
| Sweat chloride level | Chloride concentration in sweat (mEq/L) will be measured in the laboratory using an MK II Chloride Analyzer 926S | Four assessment points throughout the study: baseline and after each 8-week intervention |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |