Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor

J Cyst Fibros. 2019 Jan;18(1):144-149. doi: 10.1016/j.jcf.2018.07.010. Epub 2018 Aug 10.


Background: The effects of lumacaftor-ivacaftor therapy on glycemia have not been thoroughly investigated. Continuous glucose monitoring (CGM) provides detailed information about glycemic patterns and detects glucose abnormalities earlier than traditional screening tools for diabetes.

Methods: CGM measures, HbA1c, and oral glucose tolerance test (OGTT) results were collected and within-subject results compared in F508del homozygous youth with CF before and after initiation of lumacaftor-ivacaftor using the Wilcoxon signed-rank test.

Results: Nine youth with CF (6 males, median age 12.7 years) were enrolled. CGM was performed in all participants before (median 26 weeks) and after lumacaftor-ivacaftor (median 29 weeks). HbA1c and fasting plasma glucose increased (p = .02) after lumacaftor-ivacaftor initiation. No changes in OGTT 1 h or 2 h glucose nor CGM measures were observed overall. When analyzed by sex, males showed lower glycemic variability, as reflected by the mean amplitude of glycemic excursions, on the post-treatment CGM.

Conclusions: Glycemic abnormalities persisted in CF patients treated with lumacaftor-ivacaftor, although sex-dependent differences in glycemic response to treatment may exist.

Keywords: Continuous glucose monitoring; Cystic fibrosis related diabetes; Cystic fibrosis transmembrane conductance regulator protein modulator.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Aminophenols / therapeutic use*
  • Aminopyridines / therapeutic use*
  • Benzodioxoles / therapeutic use*
  • Blood Glucose / metabolism*
  • Child
  • Cystic Fibrosis / blood*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • DNA / genetics
  • Drug Combinations
  • Female
  • Forced Expiratory Volume / physiology
  • Glucose Tolerance Test / methods
  • Glycated Hemoglobin / metabolism
  • Homozygote
  • Humans
  • Male
  • Mutation
  • Prognosis
  • Quinolones / therapeutic use*
  • Treatment Outcome


  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • Blood Glucose
  • Drug Combinations
  • Glycated Hemoglobin A
  • Quinolones
  • hemoglobin A1c protein, human
  • lumacaftor, ivacaftor drug combination
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • DNA