Pancreatitis in a patient with cystic fibrosis taking ivacaftor

Abstract

Pancreatitis is rare in pancreatic insufficient cystic fibrosis patients. While pancreatic insufficiency has been considered irreversible until now, in the current era of new therapies with modulators of the Cystic Fibrosis Transmembrane Regulator CFTR channel, there are reports of improvement of pancreatic exocrine function. We describe the case of an adolescent with cystic fibrosis who developed pancreatitis after the partial recovery of pancreatic function while taking ivacaftor. This case adds to the limited body of evidence that CFTR modulators lead to the improvement of pancreatic exocrine function in cystic fibrosis.