Toddler bites into own flesh

A two-and-a-half-year old child has been diagnosed with Lesch Nyhan Syndrome (LNS), an extremely rare disease caused by enzyme deficiency, where patients chew their own flesh.

According to the medical fraternity in Kolkata, there is no treatment for the disease.

The child in question is Saheb, son of Mahadeb and Ruma Roy of Uluberia’s Baniban Nurullapara village, around 40 km from Kolkata. The toddler bites into his own flesh, which leaves gaping wounds that bleed profusely.

“We’ve visited several doctors. But no one has been able to offer us any hope.

Everybody says it’s a very rare disease. We are helpless. You, too, will be horrified if you see his wounded hands,” Ruma told Deccan Herald. “I don’t know if my son will be cured. It’s painful to see my baby biting his fingers and wrists. He bleeds so much.”

“The disease, mainly found in male infants, arises from a deficiency in the production of the enzyme hypoxanthine phosphoribosyl transferase, which results in over-production of purine and accumulation of uric acid in the victims’ systems. No medical treatment exists to alleviate the symptoms of self-mutilation in LNS patients,” said Anindya Dasgupta, head of the Department of Biochemistry at Calcutta National Medical College.  

“Hardly one case of LNS is found in every 5,00,000 babies. This may even be the first case of LNS ever recorded in Bengal. Only two or three cases have been reported in the country so far,” said Snehangshu Dholey, medical officer at Brindabanpur BPHC, where Saheb was first treated.

“I’ve never heard of an LNS case being reported in Bengal throughout my professional career of more than 25 years. It’s a very rare congenital enzyme-related disorder,” Dasgupta said.

“Genetic therapy is the only confirmed treatment for LNS but it is still in trial stage. Enzyme replacement method could be an alternative treatment, but it’s highly expensive and there are hardly one or two medical institutes in the country which have the necessary facilities.”