Managing the diet - PA/MMA

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

Diet calculations of a newly diagnosed infant^1,2
Diagnosis	MMA/PA
Dietary management in newborn MMA/PA infants is depending on clinical presentation at initiation and centers preference for disease management	The main aim is to restore and prevent metabolic decompensation and catabolism and at a stable situation maintain metabolic stability and normal growth to prevent metabolic decompensation. The amount of natural whole protein tolerated is determined individually by the severity of the disorder and monitoring parameters such as growth, control of acidosis, excretion of abnormal metabolites, blood amino acid levels, and testing for body protein stores. Consider continuous dripfeeding in case of feeding problems.
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
Methionine free infant formula	Standard solution: 15 grams (or 3 scoops) isoleucine, valine threonine and methionine free infant formula (MMA/PA Anamix Infant) 3 scoops + 90 mls water provides 100 ml isoleucine, valine threonine and methionine free formula
Breast feeding	Mostly 6-8 feeds are advised daily. Consider naso-gastric tube feeding at an early stage of inadequate intake Techniques for breastfeeding Fixed frequency of breast feeding and isoleucine, valine, threonine and methionine free formula alternating Isoleucine, valine threonine and methionine free formula prior to each breast feeding Measured expressed breast milk
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 breastfeeding (depends on the condition of the child and the strictness of feeding on the severity of the disorder) Mixing the standard infant formula with the isoleucine, valine threonine and methionine 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	Daily, reducing to 2-3 times a week Weighing: daily
Increasing methionine 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 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 M, MacDonald A, White F, Stafford J. Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Disorders. In: Clinical Paediatric Dietetics. 4th ed: Shaw V. West Sussex: John Wiley & Sons Limited, 2014:381-525
Baumgartner et al. Proposed guidelines for the diagnosis and management of methylmalonic and propionic academia. Orphanet Journal of Rare Diseased, 2014;9:130
E-IMD. Methylmalonic and propionic acidurias: quick reference guide. http://www.e-imd.org