How sad it was to learn of the recent passing of Prof Stephen Hawking. He requires little by way of introduction; he was quite simply the most famous theoretical physicist since Einstein, known for his ground-breaking work on the Big Bang theory, black holes, the theory of relativity and quantum mechanics. His 1988 book A Brief History of Time outlined articulately these complex concepts to a general readership, along with Prof Hawking’s search for a unifying theory that would pull everything together to describe the universe coherently. The book appeared on the Sunday Times bestseller list for more than five years, sold over 10 million copies and was translated into 35 languages by the turn of the millennium.

Prof Hawking was nothing less than an icon. His life story – as described in Jane Hawking’s memoir entitled Travelling to Infinity: My Life with Stephen – was adapted for the silver screen by Anthony McCarten in 2014. Directed by David Marsh, The Theory of Everything won much critical acclaim, not least Eddie Redmayne’s Academy Award for best actor. It is a moving tribute to Prof Hawking, charting his move from Oxford to Cambridge and his search for a thesis topic before eventually arriving upon his Big Bang theory. The film is partly a love story, but it also depicts a young man coming to terms with his own premature mortality, his lifelong struggle with disability and his gradual evolution into a life dominated by thinking.

In the film, Redmayne’s young Hawking is told by his doctor that he has two years to live. Although his illness will not affect his thoughts, it is said that eventually nobody will know what they are. Without a doubt, motor neurone disease is a devastating neurodegenerative disorder. Bäumer et al (2014)¹ wrote about recent advances in the disease (the commonest clinical presentation of which is amyotrophic lateral sclerosis [ALS]) in the Journal of the Royal Society of Medicine and remarked on an average one-year delay from first symptoms to diagnosis. The illness appears to be sporadic in nature, without any obvious environmental triggers, however recent research has identified a number of gene mutations linked to different functional pathways such that motor neurone disease as we know it might be best understood as a common final pathway rather than a single pathological entity. It seems that ALS is notably heterogenous in both its presentation and its rate of clinical progression, and around half of patients die within three to four years of onset, usually due to respiratory failure. A significant minority, according to the authors, survive into a second decade and beyond. Such individuals are usually diagnosed younger. 

So, wherein lies the hope? Aside from the growing scientific knowledge about motor neurone disease, narrative experiences such as that of Prof Hawking are a source of solace to many. He had one of the finest brains of the past century and was clearly unwilling to allow motor neurone disease to rob him of that. He himself admitted that the illness was to some degree a paradoxical gift that afforded him ample time to think. More than 50 years after his initial fateful diagnosis, he was still avidly researching while also dreaming about life and its place in the universe. In doing so, he achieved worldwide fame and recognition. Stephen Hawking died on March 14, 2018 at the age of 76. His story is the triumph of brilliance over disability.

References

1. Bäumer D, Talbot K, Turner MR. Advances in motor neurone disease. J Royal Society of Medicine 2014; 107(1):14-21