Cystic Fibrosis (CF) requires lifetime multidisciplinary care to manage both pulmonary and extra pulmonary manifestations. The median age of survival for people with CF is rising and the number of adults with CF is expected to increase dramatically over the coming years. People with CF have better outcomes when managed in specialty centers, however access can be limited. Telemedicine and technology-based care solutions may help to overcome barriers to availability and improve access. This review outlines the use of telehealth for CF management. Telehealth has been utilized for CF across a broad variety of indications, even prior to the COVID-19 pandemic, and in general has been well accepted by patients and providers. There are a paucity of data, however, related to health outcomes, and the healthcare utilization specific to CF and its related comorbidities. Future studies are needed to address the questions of health outcomes, cost, burdens of telehealth and barriers to implementation.

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Cystic fibrosis related diabetes (CFRD) generally reflects insufficient and/or delayed production of insulin, developing slowly over years to decades. Multiple mechanisms have been implicated in the pathogenesis of CFRD. CFTR function itself is a strong determinant of CFRD risk. Variants in CFTR that result in residual CFTR function and exocrine pancreatic sufficiency reduce the risk of CFRD by ten to twenty fold. Two groups of hypotheses have been proposed for the mechanism of CFTR impairing insulin secretion in CFRD: (1) β-cell dysfunction results from β cell intrinsic CFTR-dependent mechanisms of insulin secretion. (2) β-cell dysfunction results from factors outside the β cell. Genome-wide association studies have identified multiple susceptibility genes for type 2 diabetes, including TCF7L2, CDKN2A/B, CDKAL1, and IGF2BP2, as containing genetic modifiers of CFRD. These findings support the presence of intrinsic β cell defects playing a role in CFRD pathogenesis. Oxidative stress and inflammation are β cell-extrinsic mechanisms involved with CFRD. CFTR mutations render β cells more susceptible to oxidative stress and also leads to defects in α-cell function, resulting in reduced suppression of glucagon secretion. Furthermore, CFRD is characterized by β cell loss secondary to intra-islet inflammation. Recent studies have demonstrated the presence of multiple inflammatory mediators within the human CF islet. This review presents a concise overview of the current understanding of genetic modifiers of CFRD, oxidative stress, islet inflammation, and the controversies about the role of CFTR in the islet.

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