A Very Happy Life : Ends at 17

Last Friday, 10th January 2014, Sam Berns, an American citizen, passed away at the age of 17 years.

He suffered from a disease known as progeria: that is, premature ageing. It is an extremely rare disease, and there are approximately 350 people with this disease in the world (according to Sam).

The condition is caused by a change in a gene which leads to the production of a protein called progerin; it damages the cells of which the body is made up. As a result, rapid ageing takes place, at seven times the normal rate. Thus, a child aged 11 suffering from progeria will look like someone in their 70s and would have similar problems of old people.

The disease is usually diagnosed between 18 to 24 months, in the case of Sam it was 22 months. The average age at which the affected people die, usually from cardiovascular complications, is 13 years, so Sam lived four years beyond the average. But he looked like all victims of progeria do, with wrinkled skin tightly stretched over his stunted body, prominent ears and eyes and a beaked nose, without eyelashes and having a big, bald head besides speaking with a high-pitched voice.

However, mentally those affected by progeria are normal and although there is no known treatment, they can lead a practically normal life with good family and professional support. In fact, this is the core message that came through during a TEDtalk that Sam was invited to give shortly after he turned 17. He referred to a question he was asked earlier, namely ‘what is the most important thing to know about you?’ And his answer was clear, succinct and straightforward: ‘I have a very happy life.’

An amazing answer indeed when one considers that his end was not far away, but he meant it and he expanded on that during the TEDtalk. He presented a slide with four points, which read:

– Be OK with what you ultimately can’t do, because there is so much you CAN do.

– Surround yourself with people you want to be around.

– Keep moving forward.

– Never miss a party if you can help it.

He spoke with great confidence and lightheartedly, but it was serious talk with powerful messages, all the more appealing because of his comeliness despite his progeric look and the evident maturity at such a young mental age. In this very short span he had clearly packed in a lot of experience that was reflected in his messages, which he put across with great conviction. He positively bubbled with optimism, and he received a standing ovation at the end of his talk that lasted about 12 minutes. A very moving talk, and very inspiring indeed.

He said that he didn’t want people to feel bad for him, because he had been able to overcome most of the obstacles caused by his progeria. He was OK with what he could not do as there was so much he could do – his first point in the slide. He preferred to focus on activities that he was passionate about, such as music, scouting and his role as section leader in the percussion band at Foxboro High School in Foxboro, Massachusetts.

He spoke about the ‘quality people’ he surrounded himself with, which started with his ‘amazing family’, and included his close group of friends at Junior High School and his friends in the band. They shared things and helped each other, and looked at each other ‘from the inside.’ They were happy making music which ‘supersedes progeria.’ He did not think about his progeria all the time.

He also believed in always having something to look forward to. He thought of a bright future ahead and did not ‘waste energy feeling bad about myself.’ In fact he had wanted to study cell biology or genetics, and he had met with Dr Francis Collins, famous for his work on the Human Genome Project.

‘Sometimes I had to be brave,’ he said, ‘but it was not always very easy to be brave. Sometimes I faltered, I had bad days. I realized that being brave is not supposed to be easy. But it was the key way to keep moving forward.’

When we come across people with minor ailments who throw up their arms in despair instead of facing their situation with some realism and serenity, the courage of Sam is remarkable to say the least. He reminded me of a young boy of about 14 with a cancer of the shoulder bone whom I had had to operate many years ago. The parents, simple, unschooled folks took it in their stride, accepting that their child’s upper limb had to be removed completely (an amputation at the level of the shoulder). Next morning when I visited the lad in the ward and asked him whether he had any pain, he answered with a quasi-beatific smile that I can still recall, ‘ene tigit meme.’ Within a few months he was no more, the cancer having spread to his lungs.

So Sam’s testimony is to me nothing short of stupendous. Certain, premature death trailed him like a shadow, ready to pounce any time. But his focus was life. He had conquered death, truly.

‘Hang on,’ he said, making his last point,’ there’s one more piece of advice – never miss a party if you can help it! I have my school dance party tomorrow night, and I am going to be there!’ No wonder he got that standing ovation.

Earlier, he had formulated the wish that all who were listening to him could lead as happy a life as his regardless of their obstacles. Compared to him, they were all in good health and in theory should be equally happy if not happier. But how many disgruntled, perpetually complaining people there are out there in spite of having their health – and many other things besides? Plenty. No, but we really fail to appreciate that health is our true wealth. We don’t realise how precious it is simply to be alive. Instead, we are all the time chasing the will-o’-the-wisp.

Let us, therefore, heed Sam’s words of hope and encouragement. Never mind that he was only 17. If we are true to ourselves, we should be prepared to learn from anyone who speaks with sincerity, from the heart but intelligently, irrespective of the age. For that, we must keep an open mind, for ‘minds, like parachutes, function best when they are open.’ There is no age to learn, and special regard is due to those who have unique experiences to share, like Sam.

In medicine, we learnt the lesson very early on, in ancient times, from the one who is considered to be the father of modern medicine, Hippocrates. This was his advice in 400 BCE: ‘Life is short and the art is long; the opportunity fleeting; experiment dangerous and judgement difficult. Yet we must be prepared not only to do our duty ourselves, but also the patient, attendants and external circumstances must cooperate.’

Indeed, life is too short and time limited. And there are myriads of things that we need to know about, and to do. That is why we must be prepared to learn all the time, from anyone and everyone. Because knowledge is vast, and we cannot possibly undergo the immense range of human experience in our single lifetime. Learning from others with alternative and different experiences, and knowledgeable in fields other than ours but of interest and importance to mankind, is both enriching and rewarding.

Even posthumously, Sam deserves our admiration, love and gratitude. He is surely at peace now.

 


* Published in print edition on 16 January 2014

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