Factors in childhood associated with lung function decline to adolescence in cystic fibrosis

Nelufa Begum; Catherine A Byrnes; Joyce Cheney; Peter J Cooper; Emmanuelle Fantino; Nicholas Gailer; Keith Grimwood; Diana GutierrezCardenas; John Massie; Colin F Robertson; Peter D Sly; Harm Awm Tiddens; Claire E Wainwright; Robert S Ware

doi:10.1016/j.jcf.2022.03.008

Factors in childhood associated with lung function decline to adolescence in cystic fibrosis

J Cyst Fibros. 2022 Nov;21(6):977-983. doi: 10.1016/j.jcf.2022.03.008. Epub 2022 Mar 24.

Authors

Affiliations

¹ Children's Health and Environment Program, Child Health Research Centre, The University of Queensland, 62 Graham St, South Brisbane, QLD 4101, Australia.
² Starship Children's Hospital and The University of Auckland, Auckland, New Zealand.
³ Children's Health Queensland Hospital and Health Service, Brisbane, QLD, Australia.
⁴ The Children's Hospital at Westmead, Westmead, NSW, Australia.
⁵ Griffith University and Gold Coast Health, Southport, QLD, Australia.
⁶ Royal Children's Hospital, Melbourne, VIC, Australia.
⁷ Children's Health and Environment Program, Child Health Research Centre, The University of Queensland, 62 Graham St, South Brisbane, QLD 4101, Australia. Electronic address: p.sly@uq.edu.au.
⁸ Erasmus MC, Sophia Children's Hospital, Rotterdam, Netherlands.
⁹ Children's Health Queensland Hospital and Health Service, Brisbane, QLD, Australia; Child Health Research Centre, The University of Queensland, South Brisbane, QLD, Australia.
¹⁰ Griffith University, Nathan, QLD, Australia.

PMID: 35341694
DOI: 10.1016/j.jcf.2022.03.008

Abstract

Background: Despite improvements in general health and life expectancy in people with cystic fibrosis (CF), lung function decline continues unabated during adolescence and early adult life.

Methods: We examined factors present at age 5-years that predicted lung function decline from childhood to adolescence in a longitudinal study of Australasian children with CF followed from 1999 to 2017.

Results: Lung function trajectories were calculated for 119 children with CF from childhood (median 5.0 [25%-75%=5.0-5.1]) years) to early adolescence (median 12.5 [25%-75%=11.4-13.8] years). Lung function fell progressively, with mean (standard deviation) annual change -0.105 (0.049) for forced vital capacity (FVC) Z-score (p<0.001), -0.135 (0.048) for forced expiratory volume in 1-second (FEV₁) Z-score (p<0.001), -1.277 (0.221) for FEV₁/FVC% (p<0.001), and -0.136 (0.052) for forced expiratory flow between 25% and 75% of FVC Z-score (p<0.001). Factors present in childhood predicting lung function decline to adolescence, in multivariable analyses, were hospitalisation for respiratory exacerbations in the first 5-years of life (FEV₁/FVC p = 0.001, FEF_25-75p = 0.01) and bronchoalveolar lavage neutrophil elastase activity (FEV₁/FVC% p = 0.001, FEV₁p = 0.05, FEF_25-75p = 0.02). No examined factor predicted a decline in the FVC Z-score.

Conclusions: Action in the first 5-years of life to prevent and/or treat respiratory exacerbations and counteract neutrophilic inflammation in the lower airways may reduce lung function decline in children with CF, and these should be targets of future research.

Keywords: ACFBAL study; Aspergillus; CF-FAB study; Hospitalisation; Neutrophilic inflammation; Respiratory exacerbations.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Child
Child, Preschool
Cystic Fibrosis* / complications
Forced Expiratory Volume
Humans
Longitudinal Studies
Lung
Spirometry
Vital Capacity