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Inborn errors of metabolism are a specialised area, requiring very specific and robust dietary management. With the majority of inborn errors of metabolism involving defects in enzymes required to break down macronutrients including protein, fats or carbohydrates, it is crucial that these disorders are diagnosed very early on in life. Untreated or diagnosed metabolic disorders can result in life-threatening injuries and cause significant intellectual delay.

In this fascinating and informative presentation, Accredited Practising Dietitian Kiera Batten explains the management of inborn errors in metabolism through outlining the dietary management of key metabolic disorders. She describes the challenges involved in this, as these diets are often very restrictive, thus requiring specific nutrition support products to ensure that energy and micronutrient targets are met. Kiera outlines the key goals in the management of metabolic disorders including maintaining nutritional adequacy, normalising feeding development and practices, and optimising growth. 

In addition to the dietary management of metabolic conditions, Kiera also explains the challenges faced by parents of children born with these conditions as it can be a cause of significant stress and anxiety for them in ensuring that their child can receive appropriate nutrition. Food refusal, force-feeding and conflict can also result, and hospital admissions are also a possibility if inappropriate foods are consumed, further impacting on both the family and the child.

However, in collaboration with a metabolic dietitian, as well as other supports, inborn errors of metabolism are treatable conditions which can be appropriately managed to optimise quality of life.

Summary:

• Inborn errors of metabolism are a complex but very rewarding area to work in, with dietetics involvement having a direct and major impact on the management of the condition.
• Untreated metabolic disorders can result in significant intellectual delay and can be life-threatening.
• The treatment diets are often very restrictive, thus requiring specific nutrition support products to ensure that energy and micronutrient targets are met.
• Routine blood tests and screening are crucial for iron, vitamin D and B12 as the intake of these micronutrients are most likely to be inadequate in those effected by metabolic disorders.

This presentation is useful for dietitians seeking to learn more about the dietary management of metabolic disorders to either work with patients in an acute setting, or even in a rural community setting where a specialised metabolic dietitian may not be readily accessible. 

Kiera Batten has a BSc (Nutrition Dietetics Hons I) & BAppSc (Exercise Sport Science) from Sydney University. Kiera has been a dietitian for over ten years, and is a senior metabolic dietitian with the Genetic Metabolic Disorders Service and Department of Nutrition and Dietetics at the Children’s Hospital Westmead, Sydney. Kiera has served as dietetic representative on the Australasian Society for Inborn Errors of Metabolism executive committee, and will be commencing a Doctor of Philosophy in 2022 focusing on paediatric metabolics. Her areas of interest include practical optimisation of dietary management, novel therapeutics, development of training modules, and exercise physiology.

To register for the presentation and associated documents including the assessment quiz click here