Improvements in Glucose Regulation in Children and Young People with Cystic Fibrosis-Related Diabetes following Initiation of Elexacaftor/Tezacaftor/Ivacaftor.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are increasingly used in children and young people with cystic fibrosis (CF). Data in adults show there may be an impact on glycaemic control in those with CF-related diabetes (CFRD). Paediatric data are rare. Case Series/Presentation: Children aged >12 years with CFRD, who were eligible for elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) were commenced on treatment. Glucose monitoring via the FreeStyle Libre system was commenced prior to, immediately after, and several months after commencing ELX/TEZ/IVA. Glycaemic control, shown by time in range (3-10 mmol/L), percentage of time spent hypoglycaemic (<3 mmol/L), and percentage of time spent hyperglycaemic (>10 mmol/L) on Insulin doses were recorded. Following ELX/TEZ/IVA, four of seven children stopped insulin, two required substantially reduced doses of insulin, one showed no response. Glycaemic control remained similar on lower doses or no insulin. Hypoglycaemia was detected in those not requiring insulin. CONCLUSION: ELX/TEZ/IVA has a positive impact on glycaemic control and insulin requirements in children with CFRD. Close monitoring is required when commencing treatment. Children with CFRD need counselling regarding possible reductions in insulin requirement and re-education regarding symptoms, signs, and management of hypoglycaemia.

Eliminating birth trauma at Ascension Health.

BACKGROUND: Ascension Health identified perinatal safety as one of eight priorities for action in a systemwide effort to achieve zero preventable injuries and deaths by July 2008. IMPLEMENTATION: Three alpha sites developed and implemented transformational practices aimed at eliminating preventable birth trauma. Standardized order sets linked to all major areas of obstetrical care were either updated or developed and then tested and incorporated into the work flow of the labor and delivery units. Best practices were shared via team meetings and conference calls. Each site created systems to ensure that evidence-based practices were reliably followed for high-risk conditions associated with perinatal harm, that robust strategies for communication were adopted, and that collaborative practice was promoted among caregivers. RESULTS: By June 2006, all facilities achieved birth trauma rates that were at or near zero in conjunction with the implementation of these practices. DISCUSSION: Three alpha sites of differing size, patient demographics, and available resources, using a combined uniform and facility-specific approach, achieved a significant reduction in the incidence of birth trauma. Yet each site adopted unique site-specific processes designed to enhance practice on the basis of unit or institutional culture, market challenge, and/or the prospect for success.