When Two Worlds Collide: Navigating Diabetes in Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) gene on the long (q) arm of chromosome number 7. It is characterized by the buildup of thick, sticky mucus that can damage various body organs. The most commonly affected organs are the pancreas, liver, intestines, and lungs. Diabetes mellitus is a complex multi-system metabolic disorder. It is characterized by the relative or absolute insufficiency of insulin secretion with or without concomitant insulin resistance, leading to high blood sugar levels. One of the feared complications of CF is cystic fibrosis-related diabetes (CFRD). In this narrative review, we examine various treatment options, their mechanisms of action, their side effects, and their impact on the lives of patients with CFRD. In conclusion, insulin remains the cornerstone of treatment in the management of CFRD. However, oral medications for diabetes can also be considered safe and effective, in selected patients, with stable liver function and preserved lung capacity.