Poster Presentation ESA-SRB-APEG-NZSE 2022

Management of pancreatic agenesis in an infant: use of diabetes and telehealth technologies to overcome challenges of regional living and complex diabetes management (#471)

Jessica L Sandy 1 , Debra Sadie 1 , Carolyn Judge 1 , Nuala Harkin 1 , Carolyn Southey 1 , Jacqueline Askwith 2 , Marisa Bolton 2 , Rachel C Debono 3 , Yoon Hi Cho 1 , Maria Craig 1
  1. The Children's Hospital At Westmead, Annandale, NEW SOUTH WALES, Australia
  2. Paediatrics, Dubba Base Hospital, Dubbo, NSW, Australia
  3. Endocrinology and Diabetes, Sydney Children's Hospital, Randwick, NSW, Australia

This abstract describes the ongoing management of a previously reported infant with PTF1a (encoding pancreas-specific transcription factor 1a) mutation with pancreatic agenesis. She was born at 36 weeks gestation via Caesarean section weighing 1840g (5th percentile). Management of diabetes was complicated by reduced subcutaneous fat for sensor/pump cannulas as well as comorbid gastrointestinal dysmotility (requiring ileostomy from 3.5m to 12m and Total Parental Nutrition from birth until 2.5m) and variable carbohydrate absorption. Management included insulin infusion initially with introduction of insulin pump at 11w and Continuous Glucose Monitoring at 15w. After discharge from hospital at 5m, she was managed with dilute insulin until 7m, and Medtronic 770G Auto mode was commenced at 11m. 

 

This abstract and Table 1 describes the challenges in ongoing management of this case. The use of diabetes technologies including Auto mode has been integral to her care. There have been difficulties related to her co-morbidities and age (including frequent snacking) but these have been overcome with the use of such technologies and frequent meetings with her care teams. 

 

Management of intercurrent illness is challenging, especially in the context of the family living regionally. Sick day letters, parental and local team education, and frequent communication between the tertiary endocrine team, local paediatric services, and local emergency department has been important in ensuring her safety and wellbeing. Use of telehealth is essential in ensuring access to specialist care and importantly involving the family and local paediatric team in her management.

 

At most recent follow-up at 21 months of age, time in range was 50% with <4% time in hypoglycaemia on the 770G system. Total daily insulin dose was 8 units. Reassuringly, despite frequent hypoglycaemia and hyperglycaemia occurring in the neonatal period, this child is thriving and developmentally normal. There are current plans to convert to 780G system.

 

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