Inborn Errors of Metabolism (IEM) is a rare genetic (inherited or congenital) disorder, in which the human body cannot properly turn the food into energy for the body consumption. This type of a disorder is usually caused by defects in specific proteins or enzymes that helps breakdown or metabolize the parts of food. Children are affected by this are unable to survive beyond the age of 1 due to complications caused by protein build-up in the body.
The FSSAI has begun to recognise genetic conditions such as IEM as an issue and has set up a new initiative – Diet4Life – to address this issue. The platform addresses various issues such as diet, healthcare, and support groups to adopt the right approach towards IEM management. The five chosen companies to address the issue are Abbott Nutrition, Mead Johnson Nutrition, Nestle and Danone (Nutricia) and Pristine. Pristine Organics, a 25 years old Bangalore-based food technology & Nutraceutical company, pioneering in Food Research & Development is the only Indian company, to address Inborn Errors of Metabolism (IEM) issue.
In India, the diagnosis is extremely limited because of lack of awareness amongst the people. IEM cases in India often go undiagnosed or misdiagnosed. Children with IEM Disorders tend to reflect different symptoms and signs based on the type of enzymes that is lacking in their metabolism. Typically, 1 in 2497 new-born suffers from this condition, and often don’t survive due to misdiagnosis and lack of awareness about the condition.
A special IEM Diet is required for these children to be able to process the food, and get the required nutrition for growth at the same time. The diets can be formulated for specific condition. As the diets helps in management of the condition and increases the lifespan of the child, recent technological advancements such as enzyme replacement, gene therapy, and organ transplantation have opened doors for treatment of IEM.
K C Raghu, Founder & MD at Pristine Organics, said, “We have children from AIIMS, Delhi, Rainbow hospital, Hyderabad, and hospitals in Bangalore, Chennai using diets for IEM. Their nutrition, growth and development are no longer stunted.”
India’s Food Safety & Standards Authority of India (FSSAI) has entered into a partnership with professional organizations, healthcare professionals and corporate to address the challenge that plagues our society. The treatment process in the initial years of IEM discovery was the restriction of dietary proteins which resulted in malnourished children with further complications.
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The CSR Journal Team