Updates in Cystic Fibrosis-Related Diabetes (CFRD)

Abstract

Cystic fibrosis-related diabetes is a unique form of diabetes caused by a combination of exocrine and endocrine pancreatic dysfunction. These changes lead to early problems with insulin secretion, disrupted communication within the islets, and an abnormal glucagon response. As a result, people develop a pattern of glucose abnormalities that is different from both type 1 and type 2 diabetes, often showing postmeal hyperglycemia and unpredictable fluctuations. Diagnosis still relies mainly on the oral glucose tolerance test, but continuous glucose monitoring is becoming increasingly helpful, especially for detecting early or intermittent dysglycemia in daily life. The introduction of highly effective cystic fibrosis transmembrane conductance regulator modulators has dramatically improved survival, lung health, and growth, yet it has also shifted the metabolic profile of adults with cystic fibrosis. Many are now experiencing greater weight gain, increased adiposity, and more insulin resistance. Insulin therapy remains the cornerstone of treatment because it improves nutrition, lung function, and overall clinical outcomes. Modern insulin delivery technologies, including hybrid automated systems, may help selected patients by reducing glucose variability and better supporting individual routines. There is also growing interest in the cautious use of certain noninsulin medications for specific adult phenotypes, although the evidence is still limited. As this population continues to age and live healthier lives, the management of cystic fibrosis-related diabetes will need to become more personalized, with careful attention to changing nutritional status, evolving insulin needs, and long-term health goals.

Keywords: cystic fibrosis; diabetes; insulin management; noninsulin management.