Endocrine Abstracts

JOINT1773

Congenital hyperinsulinism (HI) is a rare condition where excessive insulin secretion leads to persistent hypoglycemia. Uncontrolled hypoglycemia can cause neurological damage, but prompt detection minimizes this risk. Continuous glucose monitors (CGM) are not approved for use in HI, however, they are increasingly used off-label to monitor glucose levels. In the HI Global Registry, 51% of participants reported using CGM (n = 255). Early studies in HI suggest CGMs can support euglycemic control, enable timely interventions during hypoglycemia, and reduce the frequency of severe hypoglycemia, mirroring successes in diabetes. Importantly, CGMs may improve independence and quality of life for HI families by decreasing frequent glucose checks, alleviating anxiety around unrecognized hypoglycemia, and enabling safer participation in daily activities. There are no formal measures to assess the potential benefits of CGM use for people with HI. The Congenital Hyperinsulinism International Collaborative Research Network (CRN) is a patient-led, multi-disciplinary group comprised of clinicians, researchers, caregivers, industry partners, and people affected by HI. One CRN workgroup focuses on the importance of accurate and available glucose monitoring tools. The CRN developed a mutual decision-making tool for physicians and families to evaluate nine factors of life with HI— fasting duration before hypoglycemia, continuous feeding, injectable medications/pumps, glucometer use, hypoglycemia frequency, hypoglycemia unawareness, need for external decision-making, independence, and pancreatectomy status. The tool generates a score to facilitate conversations about the value of adding CGM to glucometer use. Six HI Centers of Excellence used the suggested scoring system to retrospectively assess up to 20 HI patients at each center. Individuals received a distinct CGM benefit score. Comparing the tool’s assessment with real-world decisions physicians had made previously for that individual resulted in 87/99 (88%) agreements, including instances where both the tool and the physician agreed the patient would and would not benefit from CGM. Most disagreements occurred when the tool suggested benefit, but the physician had not recommended CGM. Of those scored as “may confer benefit” by the tool, 5/13 (38%) received a CGM. Five tool questions were significantly associated with CGM benefit using chi-square statistics. The success of the tool’s pilot testing exemplifies the benefit of utilizing the CHI CRN to ensure that all stakeholder perspectives are considered and reflected in the creation of the tool. This tool provides a structured framework for facilitating clinician-family discussions about CGM use, with the potential to improve HI management and enhance quality of life for people with HI and their families.