Managing the diet - IVA

Tabel 4: Nutricia Metabolic formula characteristics Example
Product
Form	Powder
Availability	400g/can
USE: Scoop ormeasurements
Recommended age	0-12 months or as a supplementary feed up to 3 years
Added vit/min	Yes
Protein equivalent
Carbohydrate
Fat
DHA
Fiber
Energy/kcal

Diet calculations of a newly diagnosed infant^1,2
Diagnosis	IVA
Depending clinical presentation at initiation: The main aim is to correct leucine levels in the normal range as soon as possible	Leucine does not accumulated in plasma, so it is not possible to use Leucine levels to determine protein intake. The newly diagnosed infant may be already very sick, requiring intensive care admittance. Prevention and management of catabolism is a key part of disease management to stabilize the infant.
Protein recommendation	Age adequate, based on bodyweight
Energy provision	Age adequate, based on bodyweight
Volume	Age adequate, based on bodyweight 6-8 feeds in 24 hours/on demand
Leucine free infant formula	Standard solution: 15 grams (or 3 scoops) Leucine free infant formula+ 90 mls water provides 100 ml leucine free formula
Breast feeding	Some infants can be exclusively breastfed or given a leucine free infant formula in combination with breastfeeding to limit natural protein intake from breastmilk.Mostly 6-8 feeds are advised daily. Techniques for breastfeeding Fixed frequency of breast feeding and leucine free formula alternating Leu free formula prior to each breast feeding Measured expressed breast milk The volume will vary according to plasma levels, age of diagnosis and frequency of breast feeds.
Standard infant formula	Preparation of the standard infant formula as indicated. Mostly 6-7 feeds are advised daily, the total daily amount is divided by the amount of feeds. Techniques for bottle feeding Start with the standard infant formula feed to ensure the prescribed amount of leucine is provided Mixing the standard infant formula with the leucine free infant formula requires the infant need to finish the full amount and does not get used to the taste of the amino acids, which can be a problem once low protein foods are introduced and the standard infant formula amount is lowered. But it assures a balanced provision of essential amino acids
Frequency of monitoring	Weighing: daily
Increasing leucine intake	Increase amount of standard formula Or increase frequency of feeding or volume of breast milk
Transition to solid products/weaning	In line with recommendations on food introduction for healthy population Ensure total leucine intake will stay in line with tolerance during introduction of protein containing solid foods Start the introduction on low protein foods gradually in line with local recommendations, like vegetables and fruits Provide the low protein foods after the breast milk or formula feeds Manage energy intake, low protein foods contain often lower energy density than breast or bottle feeds Once 8 – 12 tablespoons are consumed at one time, this can be given instead of the equivalent of the standard infant formula or breast feed Gradually introduce more texture and lumpier foods, starting at 6-8 months Finger foods can be introduced from 7 months onwards From 9 months the low protein pasta, low protein bread and potato (products) can be introduced

References:

Dixon MD, MacDonald A, White F, Stafford J. In: Clinical Paediatric Dietetics. 4^th ed: West Sussex: John Wiley & Sons Limited, 2014:381-525.
Knerr I, Vockley J, Gibson KM. Disorders of leucine, isoleucine and valine metabolism. In; Blau N (ed) Physician’s guide to the diagnosis, treatment and follow-up of inherited metabolic diseases, 2^nd Springer Heidelberg, 2014103-142