Cystic fibrosis-related diabetes: The patient perspective

Abstract

Cystic fibrosis-related diabetes (CFRD) affects nearly 20% of adolescents and 40–50% of adults. However, the impact on patients and their families is poorly understood. Here, we examine how patients perceive CFRD and identify gaps in our understanding of the patient experience.

Despite its relatively high prevalence, data suggest that many individuals are not aware of the possibility of developing CFRD or compare it to other types of diabetes. Annual oral glucose tolerance testing (OGTT) may serve as an opportunity to provide education and prepare individuals for the possibility of developing abnormalities in glucose tolerance.

Many cite lack of awareness of CFRD as the most difficult part of the diagnosis. While factors such as older age and a strong support system promote acceptance, most individuals view the diagnosis negatively and struggle to balance the demands of diabetes with other obligations, including airway clearance, nebulizer therapies, supplementation nutrition, and administration of vitamins and medications. Relatively few people with CFRD monitor their blood glucoses consistently, which is attributed to time constraints or an attempt to avoid pain. In addition, many feel that they are not prone to hypoglycemia and are not concerned with long-term complications, anticipating that they will succumb to their pulmonary disease before these become problematic. The adolescent period presents unique challenges for adherence as children work to develop autonomy.

Factors that promote CFRD adherence include incorporating management into daily CF routines and the support of knowledgeable providers to help develop an individualized approach to management. Diabetes technology has the potential to reduce treatment burden and improve glycemic control, but data in CFRD are limited, and additional study is needed.

Given that CFRD is associated with a decline in health-related quality of life, it is critical that providers understand patients’ perspectives and address gaps in understanding and barriers to management.