Effective management of Type 1 diabetes can be challenging. We would like to discuss the management of a 7 year old boy with Type 1 Diabetes, Lissencephaly and global severe developmental delay with medically refractory epilepsy who was introduced to and managed on a Ketogenic diet to try and improve his seizure control. He is non mobile and non-verbal and purely gastrostomy fed. He has diagnoses including. Lissencephaly and band heterotopia, absent corpus callosum, microcephaly, cortical visual impairment, severe global developmental delay and medically refractory epilepsy not controlled on multi drug therapy. His Diabetes was managed prior the ketogenic diet with rapid acting insulin aspart via an insulin pump with Continuous glucose monitoring, sensor augmented pump therapy. His Diabetes control was satisfactory as evidenced by his HbA1c of 53 mmol/mol in October 2017. As his seizure control was worsening a classical ketogenic diet therapy was proposed. He was started on a diet that would provide 930 Kcal and 24 g protein, 9060 g Carbohydrate and 5260 g LCT fat. Gradually introduced over many weeks it was proposed to maintain his blood ketones between 2 and 3 mmol. This presented many challenges including identifying and differentiating ketosis which was diet induced and appropriate but identifying diabetic ketoacidosis (DKA) which would be fatal if untreated. We did weekly venous blood gases to check his blood pH to see if rising ketone levels would affect his pH and blood gas as there was very little data available in the literature regarding this on a patient out of an intensive care setting. Blood ketones between 2 and 3 mmol were attained by week 5 of diet. His blood gases remained stable and non-acidotic. We established that maintaining blood ketones between 2 and 3 mmol consistently did not make him acidotic on the blood gas. We continued with his ketotic diet for the next 4 months with no improvement of his seizures. Therefore the diet was gradually discontinued. His diabetes control improved during this time with the recent HbA1c being 45 mmol/mol. This patient demonstrated the challenges in delivering ketogenic diet to a patient with Type 1 Diabetes and global developmental delay and inability to communicate.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology