Changing face of lung monitoring in CF: from tracking decline to maintaining healthy lungs

Cystic fibrosis (CF) has been transformed by therapies that correct and potentiate the cystic fibrosis transmembrane conductance regulator (CFTR) protein.1 Highly effective CFTR modulator therapies are now available for >90% of patients with CF in many countries, including the UK.2 This has hugely improved lung function and stabilised a condition previously characterised by progressive bronchiectasis, lung function decline and premature death. The majority of young people entering adult care now have spirometry within the normal range.3