Importance of the field: Cystic fibrosis-related diabetes (CFRD) is a unique type of diabetes. In this article I review the pathophysiology of CFRD to gain insight as to why these patients have clinical features of both type 1 and 2 diabetes (DM). The reader will also learn that, although CFRD is different from type 1 and 2 DM, the development of diabetes-induced complications is similar to other types of DM. These complications can include retinopathy, nephropathy and neuropathy resulting from uncontrolled hyperglycemia; however, hyperglycemia and/or insulin deficiency in people with CFRD may exacerbate underlying CF problems, such as decreased pulmonary function and weight loss.
Areas covered in this review: Review medical therapy of CFRD including the over-riding goal of maintaining blood glucose levels in a range as close to normal as possible. The other important goal for diabetes management is to prevent diabetes complications and to encourage psychological wellbeing of the patient. However, as reviewed in this article, the underlying medical condition of people with CF and the basic metabolic differences caused by the disease often provide challenges in maintaining optimal diabetes control.
What the reader will gain: The reader will gain an understanding of how CFRD physically affects the patient with CF and the various pharmacologic therapies available for treatment of this type of diabetes. Furthermore they will gain insight into areas where more research is needed.
Take home message: Cystic fibrosis-related diabetes is unique to CF and thus deserves disease specific medical therapy.