What is Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic lateral sclerosis (ALS) is a progressive and neurodegenerative disease. It affects the nerve cells in the brain and spinal cord that make the body’s muscles move.

With disease, these nerve cells lose the ability to initiate and control muscle movement, leading to paralysis and later death. People with this disease lose control of their muscle movements and sometimes also lose the ability to eat, speak, walk and finally breathe.

One of the most famous patients with the disease was physicist Stephen Hawking, who died in 2018 at the age of 76.

ALS is also known as Lou Gherig’s disease, named after a baseball player who retired because of the disease. Other well-known people who have suffered from the disease include actor David Niven, NBA player George Yardley, jazz musician Charles Mingus and Argentine politician Esteban Bullrich.

It was recently reported that Sandra Bullock’s partner, photographer Bryan Randall, had “passed away peacefully” after a private three-year battle with amyotrophic lateral sclerosis. He was 57 years old.

Little is known about the causes of this disease, and there is currently no cure. Men suffer more from the disease than women.

Extraordinarily long duration

Stephen Hawking, diagnosed with the disease in 1963, lived with it for over 50 years: an unusually long duration for an ALS patient.

The disease left him completely paralyzed and dependent on others and technology for everything: bathing, dressing, eating, moving and talking. He was only able to move a few fingers on one hand.

“I try to live as normal a life as possible and not think about my condition or regret the things it prevents me from doing, which are not many,” he wrote on his website.

“I was lucky because my condition progressed more slowly than it normally does. But it is still necessary not to lose hope, ”she added.

Hawking’s life, including his battle with ALS, was made into a movie in 2014: in the film ‘The Theory of Everything’, starring Eddie Redmayne.

The ‘Ice Bucket Challenge’

The US Centers for Disease Control (CDC) estimate that between 20,000 and 30,000 people in the US suffer from ALS, with about 5,000 new cases each year. Diagnosis usually occurs when patients are between 55 and 75 years of age. On average, patients live between two and five years from the onset of symptoms.

There are two types of ALS: sporadic, which is more common, and familial. The latter is hereditary: the patients’ children have a 50% chance of inheriting the disease, and people with familial ALS live on average only one to two years after the onset of symptoms. However, this is much rarer: 90% of cases are sporadic ALS.

The disease became widely known in 2014, when Pete Frates, a former Boston College basketball player who had been living with ALS since 2012, started the ice bucket challenge. The challenge went viral and greatly increased awareness of this disease, as well as generating a huge increase in donations to the ALS Association.

“We’ve never seen anything like this in the history of the disease,” said Barbara Newhouse, president and CEO of the association, in a press release at the time.

The unknown cause of ALS

No one knows what causes this disease, and for reasons still unknown, military veterans are twice as likely to be diagnosed, according to the ALS Association.

Scientists have studied many factors that may be linked to ALS, such as genetics and environmental exposures,” says the CDC.

“Other scientists have studied diet or injury. No cause has been found for most cases of ALS. In the future, scientists may discover that many factors together cause ALS.”

Until 2017, there was only one drug approved by the Food and Drug Administration (FDA) for ALS. This only prolonged survival by a few months. In May 2017, the FDA approved the first new drug in over 20 years to treat the disease.