The long-term effect of elexacaftor/tezacaftor/ivacaftor on cardiorespiratory fitness in adolescent patients with cystic fibrosis: a pilot observational study.
Humans
Cystic Fibrosis
/ drug therapy
Adolescent
Male
Female
Prospective Studies
Pilot Projects
Indoles
/ therapeutic use
Benzodioxoles
/ therapeutic use
Quinolones
/ therapeutic use
Aminophenols
/ therapeutic use
Pyrazoles
/ therapeutic use
Pyridines
/ therapeutic use
Drug Combinations
Cardiorespiratory Fitness
Exercise Test
Pyrroles
/ therapeutic use
Exercise Tolerance
/ drug effects
Oxygen Consumption
Child
Pyrrolidines
Cardiopulmonary exercise testing
Cystic fibrosis
Elexacaftor/tezacaftor/ivacaftor
Journal
BMC pulmonary medicine
ISSN: 1471-2466
Titre abrégé: BMC Pulm Med
Pays: England
ID NLM: 100968563
Informations de publication
Date de publication:
28 May 2024
28 May 2024
Historique:
received:
19
03
2024
accepted:
21
05
2024
medline:
29
5
2024
pubmed:
29
5
2024
entrez:
28
5
2024
Statut:
epublish
Résumé
Physical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO We performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients. We observed significant improvement in peak workload, VO Exercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.
Sections du résumé
BACKGROUND
BACKGROUND
Physical activity is a crucial demand on cystic fibrosis treatment management. The highest value of oxygen uptake (VO
METHODS
METHODS
We performed a single group prospective observational study of 10 adolescent patients with cystic fibrosis who completed two CPET measurements between January 2019 and February 2023. During this period, elexacaftor/tezacaftor/ivacaftor treatment was initiated in all of them. The first CPET at the baseline was followed by controlled CPET at least one year after medication commencement. We focused on interpreting the data on their influence by the novel therapy. We hypothesized improvements in cardiorespiratory fitness following treatment. We applied the Wilcoxon signed-rank test. The data were adjusted for age at the time of CPET to eliminate bias of aging in adolescent patients.
RESULTS
RESULTS
We observed significant improvement in peak workload, VO
CONCLUSION
CONCLUSIONS
Exercise tolerance improved after elexacaftor/tezacaftor/ivacaftor treatment initiation. We suggest that the CFTR modulator alone is not enough for recovering physical decondition, but should be supplemented with physical activity and respiratory physiotherapy. Further studies are needed to examine the effect of CFTR modulators and physical therapy on cardiopulmonary exercise tolerance.
Identifiants
pubmed: 38807122
doi: 10.1186/s12890-024-03069-8
pii: 10.1186/s12890-024-03069-8
doi:
Substances chimiques
Indoles
0
Benzodioxoles
0
Quinolones
0
Aminophenols
0
Pyrazoles
0
Pyridines
0
Drug Combinations
0
Pyrroles
0
elexacaftor
RRN67GMB0V
Pyrrolidines
0
Types de publication
Journal Article
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
260Informations de copyright
© 2024. The Author(s).
Références
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