Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use

Pediatr Pulmonol. 2019 Aug;54(8):E16-E18. doi: 10.1002/ppul.24353. Epub 2019 May 7.


Pancreatic exocrine insufficiency in cystic fibrosis is genetically determined and generally felt to be irreversible. However, recent studies in young children started on cystic fibrosis transmembrane conductance regulator (CFTR) modulators have suggested improvement of pancreatic functioning over time. Here, we present the case of a 10-year-old child with pancreatic exocrine insufficiency since birth who regained pancreatic functioning after 4 years on the CFTR corrector drug, ivacaftor.

Keywords: cystic fibrosis; modulator therapy; pancreatic insufficiency.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aminophenols / therapeutic use*
  • Child
  • Chloride Channel Agonists / therapeutic use*
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Exocrine Pancreatic Insufficiency / drug therapy*
  • Humans
  • Male
  • Quinolones / therapeutic use*
  • Treatment Outcome


  • Aminophenols
  • CFTR protein, human
  • Chloride Channel Agonists
  • Quinolones
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor