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Glucagon-like-peptide-1 agonist therapy in adults with cystic fibrosis

Sanghoon Park1, Raksha Jain2, Sasan Mirfakhraee3

  • 1University of Texas Southwestern Medical Center, Department of Internal Medicine, Dallas, TX, United States.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|August 30, 2024
PubMed

Insights

Glucagon-like-peptide-1 (GLP-1) agonists show promise for people with cystic fibrosis (pwCF), aiding weight loss and improving glycemic control. Most pwCF experienced enhanced lung function and reduced insulin needs, though some reported side effects.

Area of Science:

  • Endocrinology
  • Pulmonology
  • Metabolic Diseases

Background:

  • Glucagon-like-peptide-1 (GLP-1) agonists are established treatments for type 2 diabetes mellitus and obesity.
  • Evidence for GLP-1 agonist use in people with cystic fibrosis (pwCF) is limited.
  • Cystic fibrosis often involves metabolic complications and respiratory decline.

Purpose of the Study:

  • To investigate the effects of GLP-1 agonist therapy in people with cystic fibrosis.
  • To assess changes in weight, glycemic control, and pulmonary function in pwCF treated with GLP-1 agonists.

Main Methods:

  • Retrospective case series of 11 people with cystic fibrosis treated with GLP-1 agonists (semaglutide or tirzepatide).
  • Data collected on weight, BMI, pulmonary function (ppFEV1, ppFVC), and glycemic control (insulin dose, glucose time in range) for patients with cystic fibrosis-related diabetes (CFRD).

Main Results:

  • All 11 pwCF experienced significant weight loss (median -7.2 kg) and BMI reduction.
  • Eight pwCF showed improvements in percent predicted forced expiratory volume in 1 second (ppFEV1) and nine in percent predicted forced vital capacity (ppFVC).
  • Seven pwCF with CFRD demonstrated a mean 31.5% decrease in daily insulin dose, with improved glucose time in range for most.

Conclusions:

  • GLP-1 agonist therapy in this cohort of pwCF resulted in universal weight loss, reduced insulin requirements, and improved pulmonary function for the majority.
  • While generally well-tolerated, some pwCF discontinued therapy due to adverse events like nausea and vomiting.
  • Further multi-center studies are warranted to confirm the efficacy and safety of GLP-1 agonists in the cystic fibrosis population.
Keywords:
Cystic fibrosisCystic fibrosis transmembrane conductance regulator modulatorsCystic fibrosis-related diabetes

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