From pancreatitis to Puestow: The winding path of cystic fibrosis

Abstract

Individuals with cystic fibrosis (CF) who possess milder and less common mutations can have preservation of exocrine pancreatic function (EPF) but are at risk for recurrent pancreatitis, chronic pancreatic damage, and loss of EPF. They may develop sequelae secondary to pancreatic disease including malabsorption, chronic pain, and disordered eating. In severe cases, endoscopic and surgical intervention is necessary. With advancement of CFTR modulators, individuals with more unique mutations are eligible for modulator therapy, leading to preservation of pancreatic function and elimination of further pancreatitis and co-morbidities. Our case illustrates an adolescent female with CF and chronic pancreatitis with associated malabsorption, loss of EPF, chronic pain and nausea. She underwent a Puestow procedure and subsequent initiation of ivacaftor/tezacaftor, which dramatically improved her medical course and quality of life. With earlier intervention, including modulator therapy, patients with CF and pancreatic disease can potentially preserve EPF and obtain an improved quality of life.