Mumbai had a progeria case in 2003; two-year-old boy looked like an old man
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The Mumbai-based two-year-old baby's pictures, with a large head, and skin that appears wrinkled, from Dr Amar Surjushe's records. |
"She cried for a long time," says Dr Amar Surjushe, an associate professor of dermatology at Shri Vasantrao Naik government college in Yavatmal. "She had come looking for a cure and here we were telling her that this was to be her child's lifelong condition that her baby boy was going to be trapped in the body of an old man."
That was in 2003, when Dr Surjushe was a postgraduate student at J J Hospital. They were treating their first case of a person, a two-year-old baby boy, suffering from the Hutchinson-Gilford syndrome, more commonly known as progeria. This year, Dr Surjushe alongwith co-authors and doctors Minal Thakre, Resham Vasani and Dattatray Saple published a paper on progenia inu00a0 the Indian Journal of Dermatology.
Meanwhile, we are greeted with the familiar image of Amitabh Bachchan as Auro in forthcoming movie Paa, in which he plays a toothy-smiled 13-year-old trapped in an old man's body.
Family disappeared
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Amitabh Bachchan in Paa |
u00a0Much more awareness is needed for progeria, and Surjushe is hoping that this will be accomplished with Paa. In the film, written and directed by R Balki, Bachchan Sr plays the 13-year-old son of Abhishek Bachchan and Vidya Balan.
"The baby was stable. His mother had come in because his head was so much larger than other children and because of some lesions on his stomach which showed sclerodermoid changes. In simple terms, his skin was parched and tight. Most progeria patients die because of excessive accumulation of fat in their blood vessels, leading to a stroke, usually fatal. Research tells us that the average life expectancy is about 13 years, but there are not more than a hundred cases documented so far," he says.
The movie will raise awareness, he says. "More parents can come forward after they realise that this is a known condition and can seek counselling to be better parents to such children. This boy was never acutely aware of his condition considering that he was very young at the time of diagnosis. But it can open doors for other parents and children dealing with the disease alone," Dr Surjushe said.
What is progeria?
A genetic condition where the signs of aging are visible from a very early age. Very few cases are reported from India and 97 per cent patients reported have been Caucasians. The first case was detected in 1886. In patients, intelligence is normal, but sexual maturation is absent and sexual development is retarded. The average life expectancy is 13 years (which probably gives an inkling of what Auro's fate will be in Paa), but there's an age range of 7-27 years.
'We're open to partnering with Paa'
In an email interview, the US-based Progeria Research Foundation (PRF) said about the movie Paa, "While the main leadership of PRF has not spoken with the director (of Paa), we would be open to discussing partnering together to raise awareness of progeria in India. PRF recently launched a global campaign to find undiagnosed children with progeria around the world to ensure that they can receive the medical support that they need. If there is a proper connection to progeria in its story line, the release of this film could play a hand in identifying children with progeria in India."
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