Cystic Fibrosis-Related Diabetes

Cystic fibrosis-related diabetes (CFRD) is an increasingly prevalent complication of cystic fibrosis (CF), as people with CF live longer particularly in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies. Though our understanding of the underlying pathophysiology of CFRD continues to evolve, the primary defect is relative insulin deficiency resulting from reduced pancreatic beta-cell mass and impaired beta-cell function, and insulin therapy is the standard of care.Management includes individualized insulin regimens and use of diabetes technology (e.g. insulin pumps and continuous glucose monitoring). Increasingly, dietary management, and, in select cases, non-insulin therapies can be beneficial, although long-term studies of non-insulin therapies in CFRD are few and monitoring for side effects are needed. Individuals with CFRD should be monitored for traditional microvascular and macrovascular complications of diabetes mellitus and a multidisciplinary team approach is essential. Additional long-term studies are needed to understand how CFTR modulator therapies may influence the natural history of CFRD.