CFTR dysfunction causes hyperconcentration of pancreatic secretions and plugging of the ducts, leading to a backlog of digestive enzymes and auto-digestion of pancreatic tissue, which in turn causes severe pancreatitis and fibrosis with exocrine pancreatic insufficiency already present in ∼85% of infants with CF (Wilschanski and Novak, 2013; Ramsey and Galante, 2024). Here, CFTR is linked to cystic fibrosis.