[Comment] How early should CFTR modulators be initiated to prevent structural cystic fibrosis lung disease?

When first described, cystic fibrosis was a disease that portended death in childhood. However, with the establishment of care centres and standard of care therapies, the quality and quantity of life of people with cystic fibrosis in high-income countries has markedly improved. In recent decades, discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene and consequent understanding of the range of protein dysfunction that can occur has led to a new era of therapeutics, to address the basic protein dysfunction (via CFTR modulators) and the consequences that most likely lead to premature death—namely abnormal salt, water, and bicarbonate transport; decreased mucociliary clearance; exuberant inflammation; and airway wall thickening, dilatation, and destruction.