There are few scientists whom have been as well-known as Professor Stephen Hawking over the last 100 years. What’s less known by many people is Hawking’s battle with Amyotrophic lateral sclerosis (ALS).
This debilitating neurodegenerative disease has no cure and the life expectancy after initial diagnosis is on average only two to five years.
So how did someone like Stephen Hawking manage to live to the age of 76, despite being diagnosed with ALS in his early 20s?
Here we take a closer look at ALS, its impact on Hawking and why he managed to live so long.
Amyotrophic lateral sclerosis, also known as Lou Gehrig’s Disease, is a neurodegenerative disease that is progressive in nature and eventually leads to death. It affects the neurons in the brain and spinal cord.
In normal circumstances, the brain will send messages to the nerves to move muscles so that we can pick things up, walk and sit down. ALS causes the degeneration of the neurons that form these connections, gradually destroying them and blocking any transmission.
This has a huge effect on the individual. Over time, they lose the ability to move, speak, eat and even breath. The final stages of the disease result in paralysis.
Our understanding of ALS is far from complete. While 5-10% of cases may have a genetic component, the vast majority are sporadic or seem to occur for no reason at all.
ALS tends to affect people between the ages of 40 and 70 and it’s estimated that around 30,000 people in the USA have the disease. Although there are treatments available to help slow down the progression of ALS as well as drugs that can help manage the symptoms associated with the disease, there is currently no known cure for this condition.
In what is an unusual occurrence for ALS, Professor Stephen Hawking was diagnosed with the disease at the age of only 21, when he was studying at Cambridge University in 1963.
During this time, much less was known about the disease, which meant Hawking did not have the sort of support and medical help that is available to people these-days.
Despite the diagnosis, Hawking went on to spend 30 years as professor of mathematics at Cambridge University. He became famous for his books including ‘A Brief History of Time’ and ‘The Theory of Everything’, and, even though for most of his adult life he was confined to a wheelchair, his mental faculties and singular mind continued to influence science until his death in 2018.
At university, Hawking had experienced bouts of clumsiness such as falling down stairs but it wasn’t until his speech began to become slurred that medical investigations began. At the time of his diagnosis, he was given just two more years to live.
While bucking expectations when it came to ALS, Hawking began to see a dramatic decline in his physical abilities by the late 1960s. By the end of the 1970s, he was using a wheelchair and by 1985 had lost most of his ability to speak.
He initially communicated by lifting his eyebrows to choose letters on a card. This was replaced shortly after by the ‘Equalizer’, invented by a friend, that allowed Hawking to press a switch to select phrases, words and numbers. It was a method he used throughout the rest of his life.
Even though he had such a serious medical condition, Hawking continued to work and made significant contributions to the scientific field of general relativity.
The statistics of the disease are pretty stark. The majority of those affected by ALS will die between 3 and 5 years following their diagnosis. Around 20% will live longer than five years and only 10% will typically live more than 10 years. It’s no wonder that ALS life expectancy is such an emotive issue when someone is first diagnosed.
Professor Stephen Hawking lived for 55 years with ALS and much has been written about how he managed to outlast expectations. Some have suggested that, because he was diagnosed with the disease at such a young age, this may well have had an impact. While there is some evidence that people diagnosed in their teens experience a slower progression, more research needs to be done in this area.
ALS has a number of different forms and while the average prognosis looks bleak on paper, there are others who have lived quite a long time with the disease.
The common reason that people die from the disease is that the nerves serving the lungs become damaged, which means they can no longer breath. If these stay intact, the prognosis is much better. The other factor is if the muscles involved in swallowing lose their neurons, which means the individual is unable to eat, although this can be counteracted by using a feeding tube.
In truth, living a long time with the disease may simply have been down to biological chance. Hawking had a disease with slow progression and it didn’t affect these vital areas until later on in his life.
The only thing we can be sure of is that Stephen Hawking is an example of how incredibly variable ALS can be. Perhaps we should leave the final word to the professor himself:
“My advice to other disabled people would be, concentrate on things your disability doesn’t prevent you doing well and don’t regret the things it interferes with. Don’t be disabled in spirit as well as physically.”