Congratulations to the 2024 GMDI Research Grant Recipient
Kiera Batten
Kiera Batten is a senior metabolic dietitian with the Genetic Metabolic Disorders Service and Department of Nutrition and Dietetics at the Children’s Hospital Westmead, Sydney, Australia. Kiera has a BSc (Nutrition Dietetics Hons I) & BAppSc (Exercise Sport Science) from Sydney University, and is currently undertaking a PhD at the University of New South Wales. Kiera’s areas of interest include practical optimisation of dietary management, novel therapeutics, exercise physiology, and teaching and training.
Project Title: The impact of exogenous ketones on exercise capacity in children with inborn errors of muscle energy metabolism (IEMEM)
Description: This study is investigating whether a novel commercially available ketone product improves exercise capacity in children with IEMEM. Secondary objectives will be to determine if the ketone product is acceptable and tolerated by participants. Children with a LC-FAOD or HMG-CoA-lyase deficiency under the care of the Sydney Children’s Hospital Network (SCHN) will be offered recruitment to the study. We will combine the clinical expertise of the staff within the Genetic Metabolic and Children's Hospital Institute for Sports Medicine services, to test whether a commercially available ketone product is more beneficial than a control carbohydrate product, in improving exercise capacity in children with IEMEM. Pre and post blood analysis will be undertaken, to determine metabolic changes with single-dose substrate ingestion via metabolites of lipids and carbohydrates; as well as safety markers such as creatine kinase and liver function tests. A questionnaire will also be used to determine if participants find the ketone product acceptable and tolerable. This will be the first randomised controlled double blinded crossover trial assessing the impact and acceptability of a commercially available ketone product in children with LC-FAOD or HMG-CoA-lyase deficiency.