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News
Cystic fibrosis drugs are changing who needs a lung transplant. Highly effective CFTR modulators have dramatically reduced the number of children with cystic fibrosis (CF) needing lung transplants, forcing clinicians to rethink referral criteria. A landmark 2025 ISHLT consensus is the first international guideline dedicated entirely to pediatric lung transplant candidate selection.
A revolution in cystic fibrosis (CF) treatment is rippling into the world of pediatric lung transplantation. The rise of highly effective CFTR modulators (HEMTs) — particularly the elexacaftor/tezacaftor/ivacaftor combination — has dramatically reduced the number of children with CF needing a transplant. CF once dominated pediatric lung transplant indications, peaking at over 50% of cases in the US in 2013. By 2020–2023, that figure had dropped to just 14.4%.
With CF stepping back, other conditions are stepping up. Childhood interstitial lung disease, pulmonary arterial hypertension (PAH), and obliterative bronchiolitis now make up a much larger share of pediatric transplant cases — each presenting unique clinical challenges around timing, disease progression, and bridging support.
By the Numbers
Why it matters: The old benchmark — an FEV1 below 30% — is no longer enough to guide transplant referral decisions in the HEMT era. The new 2025 ISHLT consensus provides the first dedicated international framework for pediatric candidate selection, helping clinicians navigate a rapidly evolving landscape.
