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Medical science benefits from children like Verona baby with accelerated aging disease

2.4 from 5 votes
Thursday, October 28, 2010

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VERONA -- Zoey Penny is 13 months old and 13 1/2 pounds, a peanut of a toddler.

Cute and happy, as cute and happy can be.

She bounces and giggles through the toy-strewn house she shares with her mother and father, two big brothers, a dog and three cats. Her only care, like all toddlers, is to get to the next thing. The little slide, the push car, the activity table, busy as busy can be.

This is life in the Penny house. Three kids under 5, a chaotic tornado of plastic toys, and sing-song TV, and children’s laughter.

Only when Zoey needs her pin-prick injection of Saizen, human growth hormone, does reality set in, and then only for the moment.

"I mostly think of her as healthy, her personality is tremendous, she is smart, she is adorable," said Laura Penny, her mother.

She is all those things. And more. Because in that little body, Zoey Penny holds the secrets of human aging.

She is one of 71 known children worldwide with progeria, the disease of accelerated aging. It is one of the world’s rarest afflictions so the chance to study it is rare as well.

Children like Zoey are a gift from God.

On the human side, the children are heart-achingly fragile. They become little, old people before kindergarten. Most die by 13, of old age, vulnerable at a time when their peers seem indestructible. In this way, they teach us compassion and tolerance, even love, of those most peculiar.

"I can’t believe how giving people are," Laura Penny said. After she was diagnosed last spring, Team Zoey was launched by family and friends to raise funds for progeria research. The goal was $400,000. Through things like golf outings, rummage sales, swim-a-thons, and a website (, the group has raised $355,545.

"People have been very generous with their two most precious things: time and money," Laura said.

On the science side, children like Zoey benefit us all. Because of them, the study of aging can be compressed; what once could only be accomplished by following a patient for roughly three-quarters of a century, can now be done in few short years.

The first case of progeria was documented in 1886, but so rare that no large body of science could be gathered.

That changed when Sam Berns was born with disease 14 years ago. His parents, both physicians, pulled together help from family, friends and the medical community and began the Progeria Research Foundation (PRF), in Peabody, Mass., in 1999. With Sam’s mother, Leslie Gordon, leading the way, PRF created a medical database with tissue and cell banks. By 2003, the gene that caused progeria was isolated.

"It took us four years to discover the defective gene, then another four years to study the defect and look to correct it," said Audrey Gordon, Leslie’s sister, and the executive director of PRF.

A drug that may slow, or even cure, progeria was developed by Merck and a clinical trial involving 45 children with the disease is now underway. PRF has also began a program to locate more children with progeria wordwide, Two years ago, there were 54 known cases. The PRF campaign has located 17 more.

The children also revealed a link to a protein, named progerin, which causes heart disease, arteriosclerois and thrombosis. The protein becomes more prevalent in all people as they age. Children with progeria are born with extremely high levels.

"The children have given us a unique insight into how these proteins cause heart disease, and, obviously, once we understand these things, we can find cures," Audrey Gordon said. "It’s just one example of how this rare disease can help us all."

Laura Penny takes life with Zoey "day-to-day," like any other mother of three small children. But every now and then, she is awed by the outpouring of love shown Zoey.

"I just want to thank all the people who have helped Zoey," she said.

But, the truth is, we should be thanking Zoey. Her secrets in her little body, her little life, are large sacrifice for the rest of us.


© 2009 New Jersey On-Line LLC.

Author: By Mark Di Ionno
2.4 from 5 votes
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