Journal of Investigative Medicine High Impact Case Reports (Apr 2019)

Sustained Glycemic Control With Ivacaftor in Cystic Fibrosis–Related Diabetes

  • Francis Christian MD,
  • Andrew Thierman,
  • Erin Shirley,
  • Karen Allen MD,
  • Cory Cross MD,
  • Kellie Jones MD

DOI
https://doi.org/10.1177/2324709619842898
Journal volume & issue
Vol. 7

Abstract

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Cystic fibrosis–related diabetes (CFRD) is a common comorbidity in cystic fibrosis with pancreatic insufficiency occurring early in the disease process. Current treatment is exogenous insulin therapy as CFRD is due to impaired insulin secretion. Recent small studies have shown improvement in endogenous insulin secretion with a short period of ivacaftor therapy in primarily pediatric patients with cystic fibrosis transmembrane conductance regulator mutations amenable to potentiation. In this article, we present the case of an adult patient with long-standing CFRD who developed sustained improvement in glycemic control after initiation of ivacaftor.