(DCP034) CYSTIC FIBROSIS-RELATED DIABETES: THE FIRST CANADIAN GUIDELINES

Background: Cystic Fibrosis (CF)-related diabetes (CFRD) is a specific type of diabetes associated with an elevated risk of nutritional (weight loss), pulmonary complications, and early mortality. A recent Canadian survey of health care professionals (HCP) shows great heterogeneity in screening and treatment practices across the country. In addition, North American recommendations are more than 10 years old and do not reflect new knowledge about the increase in life expectancy and weight, especially with the arrival of the genetic therapy era (modulators). The objective of these new guidelines written by the Canadian CFRD Working Group including persons living with CF (pwCF) is to provide an update of the scientific evidence regarding the screening, diagnosis and care of CFRD.

METHODS AND RESULTS: Thematic subgroups were established with Canadian HCPs, experts, and leaders in the field of CFRD. The guidelines have been developed between 2021 and 2023 based on recent developments in CF knowledge.

The new guidelines recommend the inclusion of a person trained in CFRD in the multidisciplinary CF team. Annual CFRD screening process is now based on a 2-step approach: glycated hemoglobin (A1c) becomes the first screening step, oral glucose tolerance test (OGTT) is no longer required if A1c is < 5.5%, a 2nd step based on OGTT is recommended if A1c is between 5.5 and 6.5% and finally if A1c is > 6.5% a second test (HbA1c or OGTT) is done to confirm the diagnosis of CFRD. Nutritional guidance and support remain a central part of CFRD management. For pharmacological treatment, insulin is no longer systematically recommended but should rather be individualized and recommended for persons with compromised nutritional and/or pulmonary status as well as for prepubertal pwCF to provide both and anabolic effect and glucose control. If glucose control is the only objective some non-insulin anti-diabetic medications could be considered. For pwCF starting the new modulating therapies (Kaftrio/Trikafta), close monitoring of blood glucose levels and antidiabetic therapy is required. Technologies such as continuous glucose monitors (CGMs) and insulin pumps may be useful for the diagnosis and management of CFRD.

Conclusion: The first Canadian CFRD guidelines propose significant developments in the screening and management with the objective of reducing disease burden and improve care.