Avery Hart’s mother, Lisa Hart, met with Rep. Jerry Moran earlier this month to educate Moran on tuberous sclerosis complex and enlist help to fund TSC research.
As she cheerfully toddles through the first years of her life, Avery Hart’s body is fighting — and, for the moment, largely winning — a very serious battle.
Avery, whose family moved to Emporia last June, looks and acts like a healthy, normal 2-year-old. A pair of red lesions on her face are the only outward clue of what’s going on underneath her skin.
Avery suffers from tuberous sclerosis complex, or TSC, a genetic mutation disorder that causes tumors to grow on different organs in the body. A tumor on her brain that caused her to suffer daily seizures literally by the dozen has already been surgically removed, and another tumor still causes her to suffer one to two seizures per day. Not only can the tumors that TSC patients have cause seizures, they can also impede flow of blood and bodily fluids and can contribute to such conditions as mental retardation and autism.
Tuberous sclerosis is considered a rare disorder, but approximately one in 6,000 live births have it, according to the Tuberous Sclerosis Alliance — meaning 50,000 people in the United States and more than 1 million people worldwide.
For Avery’s parents, Britton and Lisa Hart, raising awareness and support for TSC research has gone hand in hand with the drive to keep their daughter as healthy as possible.
“As a family, we’ve just decided that we’re going to do everything in our power to make Avery have a happy life,” Lisa said. “Whatever that means, whatever that takes.”
The Harts — Britton, Lisa, now-5-year-old Kaylen and Avery — were living in Olathe when Avery was born early in 2006. Until she was about 15 weeks old, Lisa Hart said, Avery seemed healthy. But on Good Friday 2006, she had a “massive seizure” in her mother’s arms.
“From nowhere, she just got really, really stiff, and her eyes rolled up in her head, and she started convulsing,” Lisa Hart said.
Photo by Carly Pearson
Avery Hart, 2, plays with a toy while at home. Hart has tuberous sclerosis which is a disorder that causes tumors to grow on different organs of the body.
The Harts took Avery to Children’s Mercy Hospital, where a series of tests and an MRI revealed that she probably had tuberous sclerosis complex. Lisa Hart said she had never heard of the disorder before.
“At that time, when she was 3 months old, when we were at Children’s Mercy (Hospital), they were able to get the seizures under control with medication,” Lisa Hart said. “But they basically said to us, ‘Children with this disease are usually seriously physically handicapped, mentally handicapped. Most likely, she will never walk, she will never talk, and at some point you’ll probably want to consider a place for her — she’ll need to be institutionalized, because you won’t be able to care for her at home.’”
The Harts couldn’t believe that Avery faced that kind of future. The hospital, Lisa Hart said, told the Harts to bring Avery back in a year for her next consultation.
Once they got home, Lisa began doing research on the Internet and found Dr. David Franz, a Cincinnati neurologist who specializes in TS cases.
“There was a parent message board,” she said, “and his name came to pop up most frequently when parents were talking about success stories and talking about their kid doing well."
The Harts made the drive to Cincinnati for a consultation with Franz’s office. What they found out, Lisa said, was that the Kansas City doctors had put Avery on the wrong medication. When Avery’s meds were adjusted, Lisa said, she did very well — until August 2006, when she began having seizures again. Soon, Lisa said, Avery went from having one to two seizures per day to having more than could be counted — sometimes even around 100 per day.
“And I think probably the first thing you think of (is), ‘How is that even possible?’ because you’re picturing the full-out convulsing,” Lisa said. “And a seizure in a child like Avery, or any kid, can be real subtle. They can just get a real blank look on their face, and for Avery, she just gets this blank look, and then her eyes kind of start to look left, and then her head just tilts and jerks a little bit. Lasts about 30 seconds.”
The Harts ended up taking Avery, still less than 10 months old, back to Cincinnati for testing. The family wanted surgery to be a last resort, but finally Avery underwent a surgical procedure to first pinpoint the tumor that was causing most of the seizures and then remove it. The process took seven days.
Avery battled dangerous complications, including an infection and clot, following the surgery, but gradually pulled through. She stayed in the hospital three weeks before the Harts came home.
Today, Lisa said, Avery is back to having one to two seizures per day. She said the current step in her treatment is deciding if Avery would benefit from another surgery to find and remove the tumor causing the current convulsions.
There’s about a 50 percent autism rate among people with TSC, but Franz said Avery shows no signs of autism. He said tumors on other organs in her body, such as her kidneys and heart, need to simply be monitored at this point. Avery has been developmentally affected by TSC, Franz said, but her case is fairly typical.
“Her language is not what you’d expect from a child of her age, and she had some delays in terms of hitting her motor milestones and things,” he said. “So it’s more she’s making progress, but just not at the rate that most kids would that haven’t had to have seizures and brain surgery and all the times in the hospital like she’s had.”
Franz himself is closely involved in much of the TSC research going on right now. One source of hope is the drug rapamycin, which is now used to prevent the bodily rejection of organ transplants. Franz said it’s been found, through testing, that rapamycin treatment can shrink kidney and lung tumors.
“We’ve also found the tumors in the brain in TS patients will shrink with rapamycin, and there’s preliminary evidence that it can help with the seizures and the behavior problems that these kids have,” he said.
Along with other mothers of children with TSC, Hart has been making the rounds with Kansas legislators to raise awareness and push for government help in funding research. A recent meeting with Rep. Jerry Moran resulted in Moran encouraging the House Appropriations Committee to legislate $10 million annually to be budgeted for TSC research.
“I think the importance of this (disorder) is, many of the diseases that we do fund with medical research are ones that me, as a member of Congress, have heard about,” he said. “What was so useful about this meeting is, this is a disease that certainly I didn’t know about, and my guess is that many of my colleagues didn’t.
“So Mrs. Hart is doing exactly what she should be doing for moving this process along for ultimately getting dollars.”
For now, as she continues the drive to raise money, hearts and minds, Lisa Hart is thankful that their smiling young daughter is doing more than they were originally told she would ever do.
“I mean, when you are dealt with a blow as devastating as a disease like this,” Lisa said, “you can sit around and you can feel sorry for yourself and say, ‘Oh, woe is me, my child has this horrible disease.’ Or, you can say, ‘OK, what do we do to fix this, what do we do to make this better?’
ON THE NET
Tuberous Sclerosis Alliance: www.tsalliance.org.
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