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The following article is reproduced from the Toronto Star (Torstar Syndication Services)
May 21, 2005. 01:00 AM
Child's obscure disease like living in a minefield
JENNIFER WELLS
Noah's kiss flutters by. A whisper brushing my cheek.
How is it that a rambunctious 2 1/2-year-old, in a plaid shirt and jeans, showing off his trucks and stuff, can transform in a microsecond to the gentlest giver of the kindest gifts?
Children have a knack for mastering such transcendent moments, and in this Noah Thomas Shulist is no exception.
There are some things about Noah that are very exceptional. He has tubers growing throughout his brain and on his heart. There used to be tubers growing on his kidneys, but these have receded. There are lesions on his arms, which you cannot see, under his working-man shirt. And this spring he underwent a three-stage operation at NYU Medical Center in Manhattan — each surgery lasting a minimum of five hours — to remove one of the tubers in his brain. In a strangely simple twist of fate, the growths in Noah's brain that should not be there were "firing off," as Noah's mother says, providing neurologists with a vivid snapshot of where the workings in Noah's head were going wrong.
Noah's mom is Karen Shulist and, as soon as you meet her, you pay mental penance for the ease with which most lives are lived compared with what she has been through. She has a pixie blond haircut to go with her elfin form, both of which belie the fierce energy with which she has taken on public awareness of Noah's disease.
The disease is tuberous sclerosis complex, and this is tuberous sclerosis awareness month, though you'd never know it. TSC has not made it to marquee status in Canada, unlike in the United States, where actress Julianne Moore went before Congress last month making the case for increased funding for research. Here in Canada, the affliction, genetic and insidious, is all but unknown.
Karen weaves the story back to a day almost two years ago. It is early afternoon. Karen's husband Lyle is at work. Noah's older brother Connor is then 4.
Noah is 6 months old. Karen had noticed in advance of this day the odd tick, a twitching of an eyelid, that has made her wonder whether something is going on with Noah. But, on this day, the day she will always remember, Noah has a full-blown seizure.
The heart-stopping trip to the hospital is followed by a CT scan and then, within two hours, the news that will forever shape Noah's life. "They found out that he has tubers throughout his brain and he had quite a few," Karen recalls. "They diagnosed him with TSC. I had never heard of it before."
I hadn't even heard of tubers before. When I heard "tuberous" it made me think of roses, not the calcified rooted growth that surgeons pulled from Noah's brain in March. Karen describes it as having the look of a round piece of quartz.
"The disease attacks every organ of the body," Karen says. "And it's awful. When I describe it to somebody, I say it's like walking through a desert, not knowing where the land mines are. Something can blow at any time. You never know with Noah."
Noah was having a great deal of seizure activity. Some were mild — his doctors believe he was experiencing a seizure roughly every hour — and would go undetected. A video EEG revealed that in a 20-hour span Noah had 17 seizures in the right hemisphere of his brain. He was having seizures in his sleep. Other seizures were prolonged, extending to half an hour, even an hour.
"We'd never know whether he was going to wake up from it," says Karen. "The doctors would say we have to wait and see."
Noah was not deemed a candidate for surgery in Canada because, Karen says, specialists could not determine with any accuracy where in the brain the seizure activity was coming from. At NYU, electrodes were placed on Noah's brain. "Within a day, they basically came to us and said, `We know which tuber is firing off and we are going to resection it,'" says Karen. "They had to remove a portion of the brain, not just the tuber."
Since the last day of surgery, March 24, Noah has not had another seizure. Knock wood. Yet TSC will forever mark his life. If the tubers on the heart prove problematic, that could mean more surgery. It's not uncommon for tubers to root themselves behind the eyes, though this has not happened in Noah's case.
"We have a cardiologist, we have a pediatrician, we have a neurologist, we have an ophthalmologist," Karen says. "We have a doctor for every single organ and I'm constantly running from one to the other."
Around the Shulists' Ajax home are brightly coloured reminders of words and the objects they represent. A glass is drawn on a piece of yellow construction paper; a drawing of a fork on a pink sheet; a drawing of a bowl on a green one. Noah's motor skills are very fine, but his neurological development is still unknown. Whatever he gained in language and communication skills he would then lose at the onset of a seizure. "Nobody can say to me this is where Noah is going to be," says Karen. "This is a waiting game."
The vigilance required of Karen and Lyle is exhausting and endless. They are constantly monitoring. Does Noah appear unbalanced? Does he seem to be zoning out?
You should know that Noah is full of joy. And the boy has spunk, as he exhibited when he pulled out his catheter after surgery, and again when he said, "Done! done!" after the procedures, and when he woke up from surgery and said, for the first time, "Mum mum," followed by, "Boos Coos" (That's code for Blue's Clues.)
Noah is Karen's Number 1 job. Her Number 2 job is raising awareness. "We need people to take notice," she says. TSC research is woefully underfunded. A high-profile spokesperson would help greatly. "It doesn't have to be Julianne Moore, but someone who will say, `You know what? We'll help you out.'"
Noah has snuggled in for his nap. Karen is busily trailblazing. If you want to know more, http://www.tscst.org.
Jennifer Wells' column appears Saturday in the Life section. Email:
jwells@thestar.ca.
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