Cystic Fibrosis Related Diabetes Mellitus (CFRD) drives excess pulmonary morbidity and mortality in patients with cystic fibrosis (CF). The recommended treatment is insulin therapy. Insulin therapy in CF should be customized to the specific patient. CF patients typically require intensive insulin regimens such as multiple daily injections or insulin pump therapy, but frequently require lower doses than in type 1 diabetes mellitus. Patients with CF may also need insulin to cover intravenous or enteral feedings. Pre-diabetic glycaemic abnormalities are also associated with clinical decline in cystic fibrosis prior to the diagnosis of CFRD, however, whether and how this should be treated is not fully determined. There is also interest, but inadequate data regarding other treatments besides insulin (i.e., oral medications) for treatment of pre-diabetes or CFRD. CFTR potentiator and corrector therapy has yet to demonstrate an effect on the rate of CFRD, but may improve insulin secretion. There is great opportunity for further research to better understand when and how best to treat glycaemic abnormalities in cystic fibrosis.
Bibliographical noteFunding Information:
KLO, CLC, AG and AM received grant support through the Cystic Fibrosis Foundation , Emerging Leaders in CF Endocrinology (EnVision) Program. The authors would like to thank the Cystic Fibrosis Foundation and all the faculty memebers of the EnVison: Emerging Leaders in CF Endocrinology program for support and mentorship, especially Dr. Moran.
- Cystic fibrosis
- Cystic fibrosis related diabetes mellitus
- Insulin pump
- Oral hypoglycaemic agents