An IIPM Initiative
Saturday, November 27, 2021

A Giant spirit


Issue Dated: May 17, 2009
Tags : Elevator | shaft | rude | insensitive | cruel | dead | dry | branches | regular | kid | Faridabad | cricket | martial arts | studio | cruelly | deformed | body | precision | waist | Manav | waved | game | embarrassed | pitying | handicap | kick-boxing | gym | basement | street lights | blinking | taekwondo | finals | artistes | Vin Diesel | Morquio | syndrome | International MPS Day | Mucopolysaccharidoses | diseases | Type IV | Morquio Syndrome | paediatrician | Uruguay | essential | enzyme | crucial | mucopolysaccharides | sugar molecules | keratan sulphate | insufficiently | unaffected | physical | impairment | spine | development | hypermobile | enzyme | replacement | therapies | Clinical | trials | cure | agenda |
He looked like he had been trapped under an elevator in an elevator shaft. I know it sounds rude and insensitive, perhaps even cruel, but it is important for you to know how he looked… and there’s no other way to describe it… His arms and legs had withered and twisted out of shape, like dead dry branches and his torso seemed to have been squeezed into an oblong shape by a pair of huge hands… almost like nature changed her mind and tried to make a dwarf out of a regular kid… And yet, it wasn’t his body that I noticed first…

I was driving up a lane in Faridabad, where a bunch of boys were playing cricket and as my car approached, the kids stopped playing and stepped aside to let the car pass. I slowed to a stop next to them and asked them if they knew about a certain martial arts studio in the area (I was looking for a friend who ran the studio). The boys seemed a little confused… and then I heard a tiny voice. “Take a u-turn and go straight down and take the second right”, it said. I peered over their heads and saw a happy smiling face that beamed with confidence and poise. But as he walked towards me, I couldn’t help notice the rest of him…

He was a pitiful sight with that handsome face sitting atop that cruelly deformed body. The precision in his answer surprised me, especially since the boys around him seemed so unsure. So I asked him how he could be so sure… “I used to train with him,” came the calm reply. I thanked him, reversed and saw the kids go back to their game. I was impressed by the quiet dignity with which this deformed boy carried himself. He was tiny, no taller than my waist, and yet he spoke with such poise, seemed so self assured… It was impossible to not respect this fellow. I called out to him as he marked out his run-up… “What’s your name?” He looked up, seemed a little surprised, and then he smiled, “Manav!” he replied, waved, and went back to his game.

I felt embarrassed for pitying him. Not only did this boy feel no self-pity but he also seemed to have ensured that all his friends treated him as an equal too… no quarters given, none asked.

All the while I spoke to my friend, my thoughts kept running back to Manav and then finally I asked him if it was true that this little boy with that severe handicap actually trained with my friend in the kick-boxing gym that he’d set up in his basement.. “Oh Manav… yes he does come in every now and then … His mum’s not too keen on him training with us actually. You know how he is, and she’s worried that he might come in and maybe get hurt… But that gutsy little fellow sneaks away and drops by whenever he can.”

I drove back to that street. It was dark now and the street lights were blinking back to life. A group of mothers were standing together, discussing the treasures and travails of motherhood while their toddlers huffed and puffed around them on their tricycles. I asked them if they knew where Manav stayed… they seemed confused… then I described him to them and their furrowed brow eased into a smile… “Ah, our black belt… that’s his house.” I walked up towards the house where a bespectacled woman, having heard our conversation, turned towards the house. “Manu, tumhare liye aaye hain…” Manu sauntered out, that radiant smile still there… “Hi! What are you guys doing here?” I felt a little awkward… these days if I told a kid I admired him for being the way he was, one might get taken for a paedophile, so I just told him I’d come to meet my friend who really praised him and I wanted to find out if he really was that good… “Well, I’m fourteen-years-old and I beat a guy who was 19 in the taekwondo finals in school…” he replied with a smug smile. Honestly, I didn’t believe him. He was tiny, shorter than my eight-year old nephew by a handful of inches. I couldn’t believe this tiny thing could beat people half his age, let alone trained taekwondo artistes. “Are you serious?” I asked. He must have seen I found it hard to believe him. “I could show you”. I nodded and braced myself… little Manav began bouncing on his toes and then whipped out a series of roundhouse kicks that slapped my upper thigh… and though there wasn’t a lot of weight behind the kicks, boy did it sting… it almost made me wince. Now to put things in perspective, though I’m no Vin Diesel, I’m no wimp either… I mean I stand 1.8 metres tall and weigh about 170 lbs… and if he made me wince, it’s likely, he’ll make you wince too. He knew I was convinced but he finished off with a series of light punches and hooks for good measure… “I could go harder you know…” he said with a naughty gleam in his eye.

I smiled… “How do you stay so upbeat Manav? I really admire your spirit…” His mother had joined us by now… “He’s got Morquio syndrome. It’s a form of dwarfism, but since the dwarfism’s only partial and the limbs get affected, it ends up making a caricature of my son. But he’s a brave boy…” and she gently ran her hand through his hair and looked at him… “…he wants to join the army he says. He is very ambitious… with big dreams…”

“I have three dreams,” said Manav. “One, I want to get into the army; two, become a software engineer and three, play cricket professionally… and getting into the army isn’t as difficult as mum likes to believe… I’d love to be able to become a soldier but if I can’t, I could join the army as a software guy, right?” I smiled, “yes, you could…” This boy’s spirit was indomitable… invincible… “I’ve got to go… saxophone class…” and with that he drummed out a beat on his mother’s arm and went inside.

“Ma’am, I truly admire your son and his spirit… I wish all his dreams come true…”

“I hope so too” she said quietly. “I’m sure they will,” I insisted… she nodded quietly… I turned to go… “Do you know that most people with this disease don’t live beyond twenty… thirty if they’re lucky…” I was stunned… “Does he know…?” I asked. “You saw him… he’s a smart kid…” she sighed… “…he knows!” Gosh, he knew… and yet, “…and yet..?” I said it out loud. Her face broke into a smile, “…yes, and yet he doesn’t let it stop him, overcome him… he refuses to stop dreaming, and he refuses to stop chasing those dreams… and his dreams help us dream too…” A Giant spirit The slip stream


May 15 is International MPS Day. One of the Mucopolysaccharidoses (MPS) and related diseases – specifically, Type IV – is Morquio Syndrome, named after a paediatrician in Uruguay who had first described the conditions.

Affecting one in 200,000-300,000 live births (according to National MPS Society in North Carolina, USA), Morquio Syndrome is a genetic disorder wherein an essential enzyme crucial to breaking down mucopolysaccharides – or, sugar molecules, in this case keratan sulfate – is missing. The resulting build-up of insufficiently broken down sugars in the cells causes progressive damage to the heart, bones, nervous system etc. over the growing years. Although cognitive functions remain largely unaffected, physical impairment is acute including stunted spine development, enlarged liver and head, hypermobile joints and vision problems.

Genes occur in pairs, and it is indeed a rather unfortunate rarity to inherit the defective gene from both the parents (who may be merely carriers i.e. one normal and one affected gene), so then again the probability of the affected genes coming together is one in four. While enzyme replacement therapies are available, MPS patients usually have a medically arduous road ahead. Clinical trials towards a cure are on the agenda for this year.
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Issue Dated: Feb 5, 2017