Metabolism Disorder – FSSAI allows import of special food

Giving a ray of hope to the babies born with genetic metabolism disorders in the country, the Food Safety and Standards Authority of India (FSSAI) has allowed import of special foods meant to cater their nutritional need.

The top food regulation body has recently identified 15 Inborn errors of Metabolism (IeM) disorders, for which specialty foods can be imported for the next two years — by the time which the FSSAI hopes to frame standards for such products.

IeM are rare genetic (inherited) disorders in which the body cannot properly turn food into energy. The disorders are usually caused by defects in specific proteins (enzymes) that help break down (metabolise) parts of food. Long term treatment requires a special diet. The diet includes a man-made infant formula with low levels of the amino acids depending on the metabolism disorder.

IEMs can lead to permanent neurological, cognitive, tactile, and physical damage in the child but if detected early enough, they can all be treated leading to completely normal development. One of the factors contributing to their high prevalence in India is said to be consanguineous marriages and marriage within communities over generations.

Studies indicate that up to 15 per cent of new born sick babies could have treatable metabolic disorders which go unknown leading to mental retardation and death.

“Such specialty foods for such babies are not readily available in India. Moreover, in the absence of the standards for such items, it is difficult to import them in the country. FSSAI has been receiving representation from various organisations for allowing these foods in the country”, a senior official from the authority said.

While we have already started the process to work in this direction (framing standards), in the meanwhile, to ensure patients do not suffer, we have allowed the import of such foods with certain conditions so that the move is not misused, he said.

As per circular issued in this regard, the import has  been allowed for two years or till the standards for such food items are framed. The labels on such foods should clearly mention the medical conditions for which they have to be used. The importers will also have to ensure that they are given to the patients under the supervision of the medical professionals.

 Some of the IeMs identified by the FSSAI are Maple Series Urine Disease, Glutaric  Acedmia Type 1, Amino Acid Metabolic Disorders, Urea Cycle disorders and Hypoallergenic conditions like severe allergy due to multiple food protein intolerance that  cannot be effectively managed by extensively hydrolysed formula, severe allergy due to intact milk protein and soya protein. 


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