Our findings support the importance of initiating CFTR modulator therapy early in life to maximize long-term therapeutic efficacy, as younger patients may benefit from rescue of higher levels of CFTR function and less structural epithelial remodeling and organ damage, two factors that may facilitate restoration of epithelial homeostasis, as recently supported by single cell RNA sequencing studies of nasal epithelial cells from children with CF who initiated ETI therapy (Loske et al., 2024). This evidence concerns the gene CFTR and cystic fibrosis.