Cystic Fibrosis-related Diabetes: The Unmet Need

Cystic fibrosis (CF) is a common autosomal recessive disease. Life expectancy of patients with CF continues to improve mainly driven by the evolving therapies for CF-related organ dysfunction. The prevalence of CF-related diabetes (CFRD) increases exponentially as patients' age. Clinical care guidelines for CFRD from 2010, recommend insulin as the mainstay of treatment. Many patients with CFRD may not require exogenous insulin due to the heterogeneity of this clinical entity. Maintenance of euglycemia by enhancing endogenous insulin production, secretion and degradation with novel pharmacological therapies like glucagon-like peptide-1 agonist is an option that remains to be fully explored. As such, the scope of this article will focus on our perspective of glucagon-like peptide-1 receptor agonist in the context of CFRD. Other potential options such as sodium-glucose cotransporter-2 and dipeptidyl peptidase 4 inhibitors and their impact on this patient population is limited and further studies are required.