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.

Efficacy of advanced hybrid closed loop systems in cystic fibrosis related diabetes: a pilot study

“…Even though CFRD is the most common comorbidity in CF, many patients are unaware of the possibility to develop it and CFRD diagnosis may be seen as a further increase in therapy burden, which is already a complex, time-consuming medical regimen involving airway clearance, inhaled therapies and antibiotics, enzyme replacement and caloric supplementation ( 34 , 35 ). The use of AHCL systems in T1DM has been associated with an improvement in Quality of Life (QoL), quality of sleep and reduced impact of diabetes on daily life ( 36 , 37 ).…”

Oral Glucose Tolerance Testing Using Candy: A Sweet Solution to Improve Screening in Children with Cystic Fibrosis?

“…As many as 40% of adolescents did not know how CFRD was different from other types of diabetes mellitus or that it shared characteristics of both type 1 and 2 diabetes nor what the pathophysiology was to cause the condition [ 8 , 9 ]. It is also a shared belief of patients with CF that care teams could improve the level of education and mental preparation imparted to patients for the lifelong daily management of CFRD, including daily injections, blood glucose monitoring, and potential carbohydrate counting [ 10 ]. One is left to assume that perhaps the earlier introduction of CFRD-related education to families is important to improve overall buy-in and adherence to treatments.…”