Progeria and a Boy Called Sam

Sam Berns dies of progeria, aged 17.

In a world obsessed with eternal youth, how did Sam Berns stand tall as a beacon of inspiration despite suffering from progeria? The answer lies in two key questions: what is progeria?And who is Sam Berns?

Understanding progeria

Also called Hutchinson-Gilford Progeria Syndrome, this rare genetic abnormality affects one infant out of four million across the world. The best known aspect of this condition is that the affected person develops many symptoms of accelerated aging in early childhood. The word “progeria” itself is derived from the Greek term “geras” which means “old age.”

A child who contracts this condition, is born healthy and normal. Characteristic symptoms such as slowing growth, reduced weight gain and skin aging typically begin to develop as the child nears his or her first birthday, and sometimes not until even later. Other symptoms associated with this condition are baldness, a small face and jaw, stiff joints and weak bones resulting in frequent dislocations. Perhaps the most serious and tragic complication arising from this illness is the rapidly progressive cardiovascular failure caused by atherosclerosis. Children afflicted with this disorder have an average life expectancy of 12-14 years and death is usually the result of a stroke or heart attack.

Sam Berns: A rare spirit

The positive attitude and zest for life displayed by Jewish youngster Sam Berns is as rare as the disease that claimed his life on January 10, 2014. Born in Boston to parents who are both pediatricians, Sam began to manifest the symptoms of the illness when he was around 22 months old.

Although progeria dug its inexorable tentacles into the young child’s frail body, Sam’s mind and spirit soared high above the ravages of the disease. Although now he is a household name for being the latest victim of this illness, to his family and friends he was so much more. According to Rabbi Barry Starr, Sam desired above all else: “not to define himself (as) a victim of 
a disease which we’re 
just beginning to understand. He defined himself as a young man of dignity and strength. He defined himself as a wonderful son, as a great friend, a family member. He wanted to have fun and he wanted to be a kid and he wanted to live his life. And he defined himself by that — not by what happened to him.”

Sam loved playing the snare drum and enjoyed hockey and football. He never missed a game if he could help it. At a recent New England Patriots game, He even gave an impromptu speech about motivation.

Perhaps Sam’s biggest legacy to the world is by becoming the face of progeria in recent times. He ushered in a new era of awareness, understanding and a concerted push for intensified research and clinical trials. His very public existence has enabled even physicians from remote parts of the world to accurately diagnose a disease that for decades was misunderstood. He was also the topic of a HBO documentary called Life According to Sam.

Sam Berns, builder of Lego cities, member of the school marching band, “band geek” and Eagle Scout succumbed to progeria at the age of 17. But the quality of life that he insisted on and the scientific work done by his parents and their organization, the ‘Progeria Research Foundation’ will continue to inspire and benefit many generations to come. John Seng, a board member of the Foundation says, “Sam recognized that he alone had the talent, the maturity, and the mission to not only promote awareness of progeria, but [also] to show how a person can live his life with any disease.”

By Grace Stephen

Sources:

Times of Israel

Genome

National Geographic

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