Illness, surgery or injury - LC-FAOD

Emergency Regimen:

Standard protocol for intercurrent illness aims for the best possible intake of fluid, energy and methionine-free amino acid substitution, with special attention for higher need of energy, while taking amino acid substitutes in these periods may be a real.

In general such regime consists of very frequent feeds of a glucose polymer, during daytime and night and be started immediately. LCT fat is contraindicated in this situation.

Contact with the treatment center is warranted.

Emergency dietary management in long chain fatty acid oxidation disorders^1,2
Age (years)	% glucose polymer in fluid	Volume/hour/ml	Daily total volume/ml
0-1	10	35-80	150-200 /kg bodyweight
1-2	15	50-125	100 /kg bodyweight
2-6	20	50-125	1200-1500 ml/day
6-10	20	65-165	1500-2000 ml/day
>10	25	90-180	2000 ml/day

References:

Dixon MA, Leonard JV. Intercurrent illness in inborn errors of intermediary metabolism. ;Arch Dis Child 1992;67(11):1387-1391
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