Background for 'The Methuselah Paradox
World-building: WHY PROGERIA?
Author of 'The Methuselah Paradox', EJ Jackson, explains how learning about Progeria changed the focus of her first full-length novel:
When I finished the first draft of 'All Our Tomorrows' - as the story was titled then - in early 2012, it was pretty much a love story with a vaguely science-fiction twist to it. Expanding the story which first saw the light of day as the titular short story in my first anthology. 'The Journey & Other Short Stories', I wanted to tell Eva's story of tragic loss and how she finds a new love in the form of Tom, and how they have a daughter together, Emma. And keeping a similar theme to what happened to Tom in 'The Journey', I knew from quite early on that he would also have suffered a terrible loss - his son Nathan had been born with a condition which ultimately took his life, and Nathan's twin Chloe had been born healthy, sadly Tom's marriage to Nathan's mother, Alice had not survived the loss .
The question was, which disease would poor Nathan have? Whatever it was, I didn't want it to be just a vague reference in Tom's back story, and I didn't want it to be a cliche, either. I started to look around on the web to see what I might be able to use, aware as I did so of an uncomfortable feeling that I was seeking to make capital on someone else's misfortune...
Then I came across a website which talked about a disease I had never heard of before - Progeria. As I read about the short -but often happy- lives of the young sufferers, I was moved to tears. Perhaps because of its low incidence rate (compared with diseases such as cancer, that is) Progeria did not, it seemed to me, seem to attract as much in the way of research funding as more prevalent conditions. I clicked away from family-run websites feeling nothing but admiration for the efforts being made by parents whose children were affected. I felt sad too, and helpless.
Then I started to think... We live in a society where youth, strength, health and beauty are prized: we spend millions each year on products and treatments designed to keep us looking young, even though ageing is inevitable. If someone could find the golden key to keep us eternally young... I did some reading into genetic cures, remembering the film 'Blade Runner' (adapted from the novel 'Do Androids Dream of Electric Sheep?' by Philip K Dick) in which artificial humans are created to do mankind's dirty work, but only live for three years - and how the 'replicants' began asking questions, and -perhaps more pertinent to my research - began to demand a longer life span from their creator, Eldon Tyrell. Phrases like 'recombinant DNA' and 'virus' were used by Tyrell in conversation with Roy Batty, leader of a rebel group (and with whom my sympathies firmly lay!) and slowly an idea began to form...
One of the characters in the film, a toy-maker named J.F. Sebastian, suffered from a condition he called 'Methuselah Syndrome' - "I grow old too fast." This got me thinking: what if, in the future, a genetic cure for ageing could be found? And what if that cure were to be discovered during research into a cure for Progeria? The idea that it might be turned from a medical cure into something much more marketable wouldn't go away... and that is how Nathan came to be born with Progeria, and how the rest of the story fell into place.
Science makes advances all the time, and as I was completing another edit in the spring of 2015, an international consortium of scientists announced that a 'Methuselah Gene' (co-incidentally one of the working titles for my novel!) would very soon be discovered, and that we might all be able to live four hundred years or even longer... if I'd had any doubts about writing this story, that announcement put them to rest once and for all!
For more information about Progeria, see the Progeria Research Foundation website. Research is ongoing, and you can read about one breakthrough in treatment here. Donations are always welcome, so if you'd like to help, please donate via PayPal to:
I have also set up a JustGIving page.
Progeria is a rare, fatal, genetically determined disease of childhood characterised by dramatic, premature ageing that occurs at about seven to ten times the normal rate. Because of this accelerated ageing, a child of ten years will have similar respiratory, cardiovascular, and arthritic conditions to someone in their 70's or 80's. Progeria is the most radical of the ageing illnesses.
Its name is derived from Greek and means “prematurely old”. The condition is estimated to affect one in 8 million newborns worldwide with a total reported incidence of just over 100 in the century since it’s been identified. There are currently around 90 known cases worldwide of Progeria. In the past 15 years, children with Progeria have been reported all over the world, including in Algeria, Argentina, Australia, Austria, Canada, China, Cuba, England, France, Germany, Israel, Italy, Mexico, the Netherlands, Poland, Puerto Rico, South Africa, South America, South Korea, Switzerland, Turkey, the US, Venezuela, Vietnam, and Yugoslavia.
The most severe form of the disease is Hutchinson-Gilford Progeria Syndrome, recognising the efforts of Dr. Jonathan Hutchinson, who first described the disease in 1886, and Dr. Hastings Gilford who did the same in 1904.
As newborns, children with Progeria usually appear normal. However, within a year, their growth rate slows and they soon are much shorter and weigh much less than others their age. While possessing normal intelligence, affected children develop a distinctive appearance characterised by baldness, aged-looking skin, a pinched nose, and a small face and jaw relative to head size. They also often suffer from symptoms typically seen in much older people: stiffness of joints, baldness, hip dislocations and severe, progressive cardiovascular disease. Death occurs on average at age 13, usually from heart attack or stroke. Most children with Progeria don’t live beyond their early teenage years, though one or two have lived to their early 20s.
Children with Progeria are genetically predisposed to premature, progressive heart disease. Death occurs almost exclusively due to widespread heart disease, one of the leading causes of death worldwide. As with any person suffering from heart disease, the common events for Progeria children are high blood pressure, strokes, angina (chest pain due to poor blood flow to the heart itself), enlarged heart, and heart failure, all conditions associated with ageing. Thus, there is clearly a tremendous need for research in Progeria. Finding a cure for Progeria will not only help these children, but may provide keys for treating millions of adults with heart disease and stroke associated with the natural ageing process.
(text taken from the 'about' page of Progeria UK )
cover by www.lawstondesign.co.m
(c) EJ Jackson 2016 All Rights Reserved