Sam Berns, a Massachusetts high school junior whose life with the illness progeria was the subject of a documentary film recently shortlisted for an Academy Award, died on Friday in Boston. He was 17.
His death, from complications of the disease, was announced by the Progeria Research Foundation, which Sam’s parents, both physicians, established in 1999.
Extremely rare — it affects one in four million to one in eight million births — progeria is a genetic disorder resulting in rapid premature aging. Only a few hundred people have the disease, whose hallmarks include hair loss, stunted growth, joint deterioration and cardiac problems.
Though the gene that causes progeria was isolated in 2003 by a research team that included Sam’s mother, there is still no cure. Patients live, on average, to the age of 13, typically dying of heart attacks or strokes.
The feature-length documentary “Life According to Sam,” directed by Sean Fine and Andrea Nix Fine, was released last year. They won an Oscar for their 2012 short documentary “Inocente,” about a homeless teenager.
“Life According to Sam” has been shown at film festivals, including Sundance, and it was broadcast on HBO in October. The Academy of Motion Picture Arts and Sciences said it is among 15 documentaries considered for Oscar nominations.
Through the film, through a profile in The New York Times Magazine in 2005and through a talk he gave last year at a TEDx conference (a community-based incarnation of the TED talks) that gained wide currency on the Internet, Sam became progeria’s best-known public face.
“Life According to Sam” opens when its subject, who lived in Foxborough, is 13 and follows him for three years. He agreed to participate on one condition, which he sets forth firmly in the film: “I didn’t put myself in front of you to have you feel bad for me,” he says. “You don’t need to feel bad for me. Because I want you to get to know me. This is my life.”
Diminutive and bespectacled, Sam was a riot of enthusiasms: for math and science, comic books, scouting (he was an Eagle Scout), playing the drums and Boston-area sports teams.
In his TEDx talk, he spoke of his heart’s desire: to play the snare drum with the Foxborough High School marching band. The trouble was that the drum and its harness weighed 40 pounds. Sam weighed 50 pounds. His parents engaged an engineer to develop an apparatus weighing just six pounds. Sam marched.
The only child of Dr. Scott Berns, a pediatrician, and Dr. Leslie Gordon, then a pediatric intern, Sampson Gordon Berns was born in Providence, R.I., on Oct. 23, 1996. He received a diagnosis of progeria shortly before his second birthday.
Finding little medical literature about progeria, his parents, with Dr. Gordon’s sister Audrey Gordon, started the research foundation. As a result of its work, clinical trials of a drug, lonafarnib, which appears to ameliorate some effects of progeria, began in 2007. Though preliminary results are considered encouraging, the drug does not constitute a cure.
Besides his parents, Sam’s survivors include his grandparents, Alice and Lewis Berns and Barbara and Burt Gordon.
At his death, Sam had been planning to apply to college, where he hoped to study genetics or cell biology.
“No matter what I choose to become, I believe that I can change the world,” he said in his TEDx talk last year. “And as I’m striving to change the world, I will be happy.”