The impact of subtotal pancreatectomy on people with congenital hyperinsulinism and their caregivers.

Introduction: Congenital hyperinsulinism (HI) is a rare disorder characterized by severe, recurrent hypoglycemia. Subtotal pancreatectomy remains a treatment option for diffuse HI, but post-surgery quality of life is largely undescribed. Methods: This mixed-methods study utilized quantitative data from the HI Global Registry, including individuals with diffuse HI and ≥75% pancreatectomy (n = 34). Of these, 13 (38%) completed qualitative interviews to capture the patient and caregiver perspectives. This is the first study to investigate long-term clinical and lived impacts of pancreatectomy for diffuse HI as reported by affected families. Results: Most participants underwent subtotal pancreatectomy before two months of age (24, 71%). Only three (9%) had normal glucose status at discharge. At follow-up by median (range) age 9 (1.5-32) years, diabetes was reported in 15 (44%), pancreatic insufficiency in 14 (41%), and 24% reported ongoing medication use for hypoglycemia. Continuous glucose monitoring data demonstrated suboptimal time in range, regardless of diabetes status. Interviews revealed variability in surgical decision-making based on preoperative medical management, genetics, and imaging of the pancreas. Hospitalization and post-discharge periods were described as stressful and challenging to mental health for caregivers. At follow-up, children reported less perceived burden than caregivers. Of the six parents interviewed whose children have transitioned to diabetes, four stated that diabetes was easier to manage than HI, although hypoglycemia could still be frequent. Discussion: Subtotal pancreatectomy for diffuse HI may be necessary when medical therapy is not sufficient to prevent severe hypoglycemia. However, surgery was not curative and was associated with life-long consequences and management.