“Racing with Sam”

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The Story

“Racing with Sam”
by Robin Marantz Henig
The New York Times Magazine, January 30, 2005

The Pitch

Dear Vera,

In the pictures, children with progeria all look alike. They are tiny, usually no more than three feet tall, and there’s something raw and birdlike about their faces – bald heads, beaky noses, huge eyes and strangely delicate chins. Their voices are birdlike, too, high- pitched and twittery. Yet they act like children, think like children – in most cases, like precocious children – and are youthful in just about every way. Except physically.

Physically, they’re trapped in decrepit bodies, doomed by a cardiovascular system that ages at ten times the normal rate, and most of them die of heart attack or stroke by the age of twelve.

Last year, scientists found the gene that causes progeria – a single mutation on a single gene, in the middle of chromosome 1, one wrong nucleotide in a genome of three billion nucleotides. The finding suggests that maybe progeria can some day be cured.

But if it were just children with progeria who might benefit, this story would be interesting, but not that important: progeria, after all, is extremely rare, affecting no more than 40 people in the world, striking once every 4 to 8 million births. What makes this gene discovery exciting is that progeria is, according to some scientists, a model for normal aging. And developing a better understanding of why aging goes so badly awry in these children might some day help explain why normal aging happens – and how it can be slowed.

Scientifically, the mutation is also interesting, because it affects just one particular protein, which in turn affects the membrane that surrounds the nucleus of the cell. What it is about this nuclear membrane that’s so essential that a minor change in its permeability can have such a profound effect on every organ system in the body?

There’s also an interesting human interest spin to this story: one of the scientists who found the progeria gene is a young geneticist whose own son has progeria. This scientist, Leslie Gordon, re-directed her whole professional life when she heard her son Sam’s dismal diagnosis. She quit her residency in pediatrics, began a postdoc in genetics at Brown University, where she had just gotten an MD and a PhD, and with her attorney sister she formed the Progeria Research Foundation. As the foundation’s medical director, she lobbied hard for the National Human Genome Research Institute to mount a search for the progeria gene as a first step in the search for an explanation for aging.

The effort, which involved six medical centers and 20 scientists, paid off in just 11 months. And when the genome institute director, Francis Collins, stepped up to the microphone at a press conference in April 2003 announcing that they had found the single-gene defect that explained progeria, Leslie Gordon was standing to his left.

I’d like to write a 4000- or 5000-word article for the Magazine about what happens next, now that the progeria gene has been discovered. Scientists at a biotech company in Cambridge, called Elixir Pharmaceuticals, are collaborating with Collins and others at the National Institutes of Health to compare the genetic profiles of children with progeria, who age remarkably fast, with those of centenarians, their polar opposites on the aging spectrum. Gordon and her husband, pediatrician Scott Berns, are pushing for gene therapy or bone marrow transplants for children with progeria, knowing that time is running out for Sam, who is seven years old and in second grade and can count himself lucky for not yet having had a stroke. I’ve spoken to the CEO of Elixir, who is happy to cooperate with me on this piece, as well as to Francis Collins and his associate at the National Institute on Aging, Hubert Warner. Now what I’d like from you is a green light to go ahead and enlist the cooperation of Leslie Gordon and her family, as well as some of the other families with bizarrely aging children for whom time is, even more than it is for the rest of us, incredibly precious.

I look forward to hearing from you soon.

Best regards,

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