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Walk-a-thon offers hope to those braving rare disease

Ten-year-old Amanda Zampella likes to read. But getting through just a couple of paragraphs is a tiresome chore.

During a special reading comprehensive class at Cotee River Elementary School in New Port Richey, she takes her turn reading aloud about life in Alaska. The words come out haltingly, barely audible to the other students who tower over her. Still, she makes her way to the end of the paragraph and flashes a smile when she is finished. There is little doubt she is doing her best.

In another classroom, kindergartener Michael Zampella has been writing a story about spiders. His words, written across the board in the classroom, come together in a plausible tale that pleases his teacher.

When it comes time to read his story aloud to his classmates, he breezes through and finishes with a smile to match his sister's. He, too, is offering up his best effort.

To a casual observer, Michael's reading ability seems miles ahead of that of his sister, who is four years older.

But who knows how things might be if, like her brother, Amanda had been spared _ if she had not inherited a rare genetic disease called ataxia-telangiectasia.

The fourth-grader from Port Richey with the ever-present smile has extraordinary resolve that would undoubtedly set her above the rest, says her inclusion teacher, Cookie Levenson.

"She's so determined even when she's worn out. We try to get her to rest, but she doesn't want to."

When it comes to writing assignments, says Levenson, "We'll say, "We'll do this for you,' but she (Amanda) usually says, "No, I want to do it by myself.'

"She doesn't want to be different."

Known simply as A-T, ataxia-telangiectasia is a degenerative disease that has parents like Adrienne and Mike Zampella struggling in a race against time.

The disease, which affects a wide range of body systems, mimics many other diseases: cystic fibrosis, cerebral palsy, leukemia and lymphoma.

A-T children appear normal at birth. But around the age of 2, the signs, including a lack of balance and slurred speech, appear and worsen from there. Most A-T children are using wheelchairs by the time they are 10 years old. Few survive their teens.

In the six years since their daughter was diagnosed with A-T, the Zampellas have witnessed her steady decline. It has been a painful process, watching the brown-eyed toddler who at first just seemed a little clumsy now have to maneuver with the help of a walker on wheels.

"It's hard because we see it every day," said Amanda's father, Mike Zampella. "It's extremely hard because you know the way it's going."

Since she was featured in a Times article one year ago, Amanda has had increasing difficulty with her school work. Because A-T affects the neurological system, Amanda has a tough time focusingher eyes, which affects her daily studies. Processing information has definitely slowed.

"She can read all right," said Levenson. "She just has difficulty getting it (the words) out."

Amanda's health also has worsened. Because the disease has weakened her immune system, she now is on a constant regimen of antibiotics.

Like cystic fibrosis patients, Amanda has to undergo respiratory therapy for a persistent cough. After a frightening weight loss triggered by her various illnesses and inability to swallow, she must now be nourished through a feeding tube. And there was recent surgery for spleen removal that made her miss two months of school.

Then came the wheelchair.

"We try to keep her active and keep her strength," said Leslie Jacobs, the physical therapist assistant who works with Amanda at Cotee Elementary and has watched her student weaken.

"We know that once they're in a wheelchair, they don't ever get up and walk again."

Acquiring the power wheelchair is bittersweet, says Amanda's mom, Adrienne Zampella. In an attempt to slow her daughter's decline, she allows Amanda to use it on a limited basis. Amanda is delighted by her newfound ability to get about using a joystick control and anticipates being able to race it against other physically impaired children. Mom, on the other hand, is not so enamoured.

"It's almost like admitting defeat. You know by a certain age they're going to be in a wheelchair, and you're fighting not to get there," said Mrs. Zampella.

Part of that fight includes raising money for A-T research through a walk-a-thon that the Zampella family is hosting today. This is the second annual A-T Walk for a Cure for the Zampellas. Last year, they and the local community raised $16,800 for the Children's A-T Project, far exceeding their goal of $5,000.

This year the Zampellas have set a goal of $10,000. "But we're hoping to at least match what we raised last year," said Mrs. Zampella.

The results of fundraising efforts have gone a long way toward bringing renewed hope to the Zampellas. Families throughout the country hosting similar walk-a-thons are the primary source of funding for A-T research.

In the six years since the A-T Children's Project began, funds have helped establish A-T clinics to serve families at St. Jude Children's Research Hospital in Memphis, Tenn., and Johns Hopkins Hospital in Baltimore.

Researchers have also been able to isolate the A-T gene, conduct swallowing studies that have led to improved treatments and more recently launched research to reseed stem cells into the brains of A-T mice and monkeys, a treatment that could potentially halt or reverse the disease.

Mrs. Zampella recently attended a conference in Texas that presented the stem cell research.

"We were all sitting there amazed," she said. "This was the first time I could envision her (Amanda) walking and running with her brother."

"It's just amazing what they're doing. They've come so far in a very short time," said Mr. Zampella, adding that the progress in A-T research could affect those suffering from those diseases that A-T mimics.

"What is exciting is that they're starting to see that this disease touches other diseases, and that brings more awareness. Maybe in a shorter time they will find a cure or a very good treatment. We're very optimistic about that. Now we can see some light."