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Published Thursday, February 18, 2016

Progeria: Sam’s Fight Goes On

By Todd Feinburg

Sam Berns and his aunt, Audrey Gordon, director of the Progeria Foundation, attended the New York premiere of HBO’s “Life According To Sam” in 2013.

Sam was emaciated and weak when he died two years ago, weighing only about 50 pounds. Though he was 17, he had the appearance of a man in his 80s, and he suffered from the ailments of that age group as well, like weak bones and heart disease.

“Being brave isn’t supposed to be easy,” Sam Berns liked to say. For him, the challenge was to focus on the positives that life had to offer – like family, friends and pursuing his passions – rather than letting himself be defined by his disease. A wise old man at an early age, Sam shared his philosophy in a TedX Talk shortly before his death that has been viewed by thousands.

Perhaps you only learned about progeria – a genetic condition that causes young people to age at a rapid rate and to die early in life – on TV, as so many in the Boston area did, because Sam Berns was raised in Framingham and had relatives on the North Shore. But he also became the worldwide face of the malady.

Progeria is extremely rare – only about 300 children have it worldwide at any given time – so awareness of the disease, and funding for research, has only developed in the last several years – mostly because of Sam’s family. His aunt, Audrey Gordon, is executive director of the Progeria Research Foundation in Peabody, founded by Sam’s parents and extended family after his diagnosis. The organization’s gala fundraiser, called “Night of Wonder,” takes place April 9 at the Boston Waterfront Hotel.

Sam Berns was honored at TD Garden before a Boston Bruins game in 2013. He is pictured with Zdeno Chara, captain of the team.

Sam’s parents, Leslie Gordon and Scott Berns, are both doctors, and when Sam was diagnosed at age two, they discovered there was virtually no support for families like theirs. “Little information was available, there was no Progeria Foundation in place, and no research on the disease was being funded,” said Audrey Gordon. So the foundation, which now spends about $2.5 million annually, according to Audrey, was born. “We said we’re not going to accept the idea that there’s nothing we can do.” They have raised more than $20 million, in total, since 1999.

Part one of the effort had to do with raising awareness of the disease. That included getting media attention from big outlets like the Wall Street Journal and CNN, as well as an HBO documentary. Social media has also played an important role – the foundation has over a million followers on Facebook. “But there are still 165 or so kids suffering from the disease around the world we don’t even know about,” said Audrey, “so we still have lots to do.”

The second part of their focus at PRF had to do with creating a center of information, fundraising and research for finding treatments and a cure for progeria.

There is no cure yet for the disease, and that fact keeps Sam’s family and the foundation working hard. But a drug called lonafarnib, originally tested as a cancer treatment, has been found to be effective in slowing the development of progeria. It was the foundation which lobbied the drug maker to test the drug for its efficacy in fighting the disease. Now, that treatment has extended the lives of progeria victims by about a year-and-a-half according to a study.

“‘Night of Wonder’ is what has made this progress possible,” said Audrey, referring to the upcoming fundraiser. “Without the money we raised with ‘Night of Wonder,’ we could never run multi-million dollar drug trials,” she said. “And kudos to Merck for agreeing to continue to manufacture the drug and to supply it to us at no cost,” added Gordon.

The money the organization spends on research – a million dollars last year alone – is a point of pride. “We’ve got to keep up with things – I never want to say no to a good research project or say no to a kid who wants to come here for a drug trial. That’s why things like this gala are so important.”

“It’s tons of fun, and people get really engaged in the night and in the cause.”

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