Communicating a new congenital hyperinsulinism diagnosis — OSCE

The parents of a nine-day-old girl sit with you on the postnatal ward. She was born large for gestational age and developed severe persistent hypoglycaemia from 24 hours of age, presenting with a seizure and a glucose of 0.8 mmol/L. The critical sample confirmed congenital hyperinsulinism with a measurable insulin, suppressed ketones, and a positive glucagon response, and genetic testing is pending to distinguish focal from diffuse disease. She currently needs 14 mg/kg/min of glucose and has not yet responded to diazoxide. The parents are frightened, feel they did something wrong, have read about experimental gene cures online, and are terrified of surgery. Counsel them.