Managing the diet - LC-FAOD

Tabel 4: Nutricia Metabolic formula characteristics Example
Product	Monogen
Form	Powder
Availability
USE: Scoop or measurements
Recommended age
Added vit/min
Protein equivalent
Carbohydrate
Fat
DHA
Fiber
Energy/kcal

Diet calculations of a newly diagnosed infant^1,2,3,4
Diagnosis	LC-FAOD
LCHADD and MTPD and severe VLCHAD	Monogen is targeted at symptomatic infants Total fat restriction 25-30 % of total calories; 20 – 25 En % MCT; 5 – 10 En % LCT
Mild VLCAD	Total fat restriction max 30 % of total calories; 15 En % MCT; 15 En % LCT
Protein recommendation	Age adequate, based on bodyweight
Energy provision	Age adequate, based on bodyweight
Volume	150-180 ml/kg/day Frequency of feeding depends on the severity of the disorder, age and weight. Children with severe defects are fed 3-4 hourly during the first 4 months of life, and additionally one our per month of age thereafter (5 hours for 5 months etc).
Monogen	Standard solution: .. grams (or ..scoops) Monogen + .. ml water provides Monogen formula, providing: …. Calories Protein LCT fat MCT fat carbohydrates
Breast feeding	Exclusive breastfeeding is contraindicated except for asymptomatic VLCADD infants diagnosed on newborn screening
Transition to solid products/weaning	In line with recommendations on food introduction for healthy population, generally around 5-6 months of age Solid foods should contain minimal levels of fat and high carbohydrate levels, such as vegetables, fruit, rice, cereals and potatoes Further introduction of weaning foods should be monitored based on fat content Allthough fasting tolerance over the day will increase with aging, frequent meals and bedtime snacks will be a continuous part of the daily diet

Gillingham MB. Nutrition Management of patients with inherited Disorders of Mitochondrial Fatty Acid Oxidation. In: Acosta PB. Ed. Nutrition Management of Patients with Inherited. Metabolic Disorders. Boston, Massachusetts: Jones and Barlett, 2010:369-403.
Dixon M, MacDonald A, et al. Disorders Of Mitochondrial Energy Metabolism, Lipid Metabolism and Other Disorders. In: Clinical Paediatric Dietetics. 4^th ed: Shaw V. West Sussex: John Wiley & Sons Limited, 2014:588-636
Spiekerkoetter U, Duran M. Mitochondrial Fatty Acid Oxidation Disorders. In: Physician’s guide to the diagnosis, treatment and follow up of Inherited Metabolic Diseases 2^nd ed: Blau N. Springer Heidelberg New York 2014:247-266
Spiekerkoetter U, Lindner M, Santer R et al. Treatment recommendations in longchain fatty acid oxidation defects: consensus from a workshop. J Inherit Metab Dis,2009, 32 (4), pp. 498-505.