What Is Cystic Fibrosis-Related Diabetes? 

Cystic fibrosis is a genetic disorder that causes thick mucus to build up throughout various parts of the body.¹ Cystic fibrosis-related diabetes (CFRD) is a form of diabetes that results as a complication of cystic fibrosis.² It is the most common comorbidity in individuals with cystic fibrosis, with a prevalence of 40-50% in adults and 20% in adolescents.³ Insulin insufficiency is identified as the primary cause, although cystic fibrosis–associated infection and inflammation may contribute to the development of insulin resistance and ultimately CFRD.

Who Should Be Screened for CFRD?

• Anyone >10 years old with CFRD should be screened annually
• Anyone, regardless of age, who fails to gain expected weight or loses weight

As early diagnosis and treatment of CFRD have long-term benefits in preserving lung function, it is important that screening recommendations be met.³ The American Diabetes Association recommends that anyone with cystic fibrosis should be annually screened for CFRD by age 10. Although screening for CFRD is not typically conducted before the age of 10, children who fail to gain expected weight or experience weight loss should be screened. 

The oral glucose tolerance test, measuring the 2-hour plasma glucose level with 75 g of oral glucose, is currently the only recommended approach for screening for CFRD. Although recent studies suggest that A1C may also be accurate, it is not yet recommended. 

How Is CFRD Managed?  

Although CFRD is typically associated with worse inflammatory lung disease, lower nutritional status, and greater mortality when compared to those with type 1 or type 2 diabetes, CFRD can be effectively managed with insulin therapy.³ Currently recommended treatment for CFRD is insulin therapy, as it has shown to help achieve glycemic targets while promoting retention of macronutrients and weight gain. In addition to receiving insulin therapy, individuals with CFRD should be annually monitored for diabetes-related complications from 5 years after initial CFRD diagnosis.

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