The Unique Management of Cystic Fibrosis–Related Diabetes and the Importance of Glycemic Control

• Cystic fibrosis–related diabetes is a diagnosis different from type 1 and type 2 diabetes in pathophysiology, diagnosis, and management.
• Sufficient evidence is present to support the benefits of glycemic control on lung function in patients with cystic fibrosis–related diabetes.
• Additional research is required to identify the mechanisms by which cystic fibrosis–related diabetes affects pulmonary function.

Cystic fibrosis (CF) is a rare autosomal recessive lung disease that decreases the ability of chloride channels to function properly, leading to complications such as salt loss and mucus accumulation in different organs. Mucus accumulation leads to lung infections and decreased pancreatic function, nutrition, and sperm production. Treatment developments have prolonged the median life expectancy, but, with increasing age, a condition known as CF-related diabetes (CFRD) is more likely to develop. CFRD is a distinct condition that is different from type 1 and type 2 diabetes in key ways, including its pathophysiology, diagnosis, and management. It is essential for nurse practitioners in the primary-care setting to recognize these differences, because CFRD is becoming more common in CF patients. Obtaining glycemic control and recognizing its importance in patients with CFRD is especially significant, because glycemic control is correlated with lung function. This review focuses on recognizing CFRD and the rationale behind treatment differences.