His illness defined British physicist Stephen Hawking as much as his genius. Hawking was diagnosed with a degenerative motor-neuron disease called Amyotrophic lateral sclerosis (ALS) when he was just 22 and given a few years to live.
He beat the prognosis and lived for another fifty-four years after that, dying at the age of 76.
In 2014, Hawking’s family took the ice bucket challenge and posted the video on social media to raise awareness and funds for ALS. The video was watched by several millions, which helped raise $128 million for the ALS Association that year.
However, the prognosis wasn’t wrong. “Most people die within three to five years of being diagnosed with ALS. Only about 10% of those diagnosed live for more than a decade,” said Dr JD Mukherji, head of neurology, Max Superspeciality hospital, Saket, New Delhi.
What is ALS?
ALS is one of the four types of rare degenerative motor-neuron diseases. “In people with ALS, the neurons in the brain and spinal cord that control motor functions start degenerating. With the neurons gone, brain cells cannot communicate with the muscles, which stop functioning. It is a progressive disease that slowly affects all motor functions, including eating and breathing, finally leading to the death of the patient,” said Dr Mukherji.
“Even as the muscle die, the cognitive abilities of the brain remain intact, so the patient retains complete mental faculties, which makes it very difficult for them,” he said.
The disease can happen at any age, but is more common in people above the age of 55. “It is more common in men than women. To date, the cause is not known, but genetic factors or, perhaps, an infection may play a role,” he said.
Few people survive beyond a decade.“There are medicines that slow the progression of the disease and certain medical procedures like ventilator support or feeding tube help in prolonging life, but there is no cure,” said Dr Vineet Suri, neurologist at Indraprastha Apollo hospital.
In India, ALS affects around 5 persons per 100,000, with men twice as likely to be diagnosed with the disease, according to the Foundation for Research on Rare diseases and Disorders.
Two medicines can delay the progression of the disease. “Both the medicines are very expensive, upwards of Rs 40,000 a month. But a pharmaceutical company in India provides medicines free to people who can’t afford it. So, when it comes to medicines for ALS, most people in India can get it,” said Dr Suri.
However, once the disease progresses, patients need round the clock care, respiratory support and nutritional support.
“Sadly, there aren’t any nursing homes or other facilities in the country that can help people with the prolonged care that is needed. But, all the technology and the life support systems are available in India for people who can afford to care for the terminal patients at home. But, less than 10% patients can actually afford such care,” said Dr Suri.