Stephen Hawking’s 55-year battle with ALS was inspiring, but most people with the disease don’t live more than 5 years and don’t have the medical care he did.
I had the privilege of interviewing Stephen Hawking in the early 1990s.
The meeting took place in a conference room in the southwest corner of the University of California Berkeley.
Hawking was promoting his book, “A Brief History of Time,” and four or five journalists were there to talk with him.
The renowned scientist, who died March 14 at the age of 76, rolled into the room in his motorized wheelchair after we assembled.
He wasn’t quite as physically affected or restricted from amyotrophic lateral sclerosis (ALS) as he was in his later years.
But his movements were still quite limited.
He couldn’t talk or move his arms or legs. He communicated by pressing a button with one of his fingers as letters scrolled on a screen.
It took three to four minutes for him to craft an answer to our questions. A mechanical voice would recite his words when he was done.
His answers were eloquent and most ended with a joke of some sort. Hawking would then unfurl that crooked smile that Eddie Redmayne perfected in the movie, “The Theory of Everything.”
I came away from the interview with wonderment. Hawking was one of the most impressive people I’ve ever met.
However, his life isn’t typical for most people who live with ALS. He lived far longer and had more medical assistance than most.
Nonetheless, he seemed to inspire the ALS community, including those who have the disease as well as those working on a cure.
“The way he chose to live his life gave me hope that I too can live a productive life. He was certainly a unique and curious person,” said Shelly Hoover, who was diagnosed with ALS in 2013 at the age of 47.
“Maybe Mother Nature is trying to tell us something with Stephen Hawking’s life,” added Dr. Richard Bedlack, director of the Duke ALS Clinic. “It’s there if we can just unlock the doors.”
About 6,000 people in the United States are diagnosed with ALS every year, according to the ALS Association.
It’s estimated that 20,000 Americans are currently living with the debilitating disease that usually progresses steadily and robs people of the use of their limbs and other bodily functions.
About 60 percent of people with ALS are men and 93 percent are Caucasian. Military veterans are twice as likely to develop the ailment, also known as “Lou Gehrig’s disease.”
Most people are diagnosed with ALS between the ages of 40 and 70 with the average age being 55.
About half of people with ALS live at least three years. About 20 percent live more than five years. And only about 10 percent live more than 10 years.
Some believe Hawking may hold the world record for ALS longevity by surviving for 55 years after his initial diagnosis when he was a 21-year-old student at Cambridge University in 1963.
There’s a theory that one reason Hawking lived for so long was because he developed ALS at such a young age.
Bedlack said this may be a component, but he told Healthline that there’s a lot more to it than that.
He said Hawking had a strong desire to live and was willing to accept his deteriorating condition as a way of life. Not everyone with ALS has this motivation to carry on.
Bedlack has observed this phenomenon over the years with the 2,000 ALS patients he has treated. On average, he tells five people a week that they have this disease.
He sometimes uses Hawking’s example as encouragement.
“I have to break the news to them, but I also want to give them hope,” he said.
Bedlack added that Hawking also had full-time professional care and some of the best equipment, including ventilators, available.
“The equipment can’t be underestimated. Some people don’t have access to the options he did,” he noted.
Finally, Bedlack said, ALS varies greatly from person to person and Hawking seemed to have something different going on.
“There is a tremendous amount of variety in this disease,” he said.
Even with advanced equipment and a strong desire, life with ALS is difficult and frustrating to manage.
Hoover knows that all too well.
Before 2013, she was a healthy 47-year-old woman with a husband and two grown children. She was working as a middle school principal and was physically active.
Her first symptom was increased difficulty walking. Hoover told Healthline she was “shocked” by her eventual diagnosis. She was told at the time that she had two to five years to live.
Hoover’s story was posted in a YouTube video in 2014 with her in a wheelchair discussing her disease. Since then, she’s also lost the use of her arms and hands. That means she can’t drive, feed herself, or get dressed by herself.
“I can still control my power chair with some movement in my right hand, but require assistance for everything else,” Hoover said. “My morning routine takes at least two hours. My husband or a paid caregiver uses a lift to transfer me from my hospital bed to a shower chair. I go to the bathroom and get showered, and transfer to my power chair. Then teeth, hair, face, clothes, meds, and breakfast. My nightly routine takes about an hour with passive range-of-motion exercises to keep my unused joints from freezing.”
To do the interview with Healthline, Hoover sent her answers to questions via email, a process she accomplished by “writing with my eyes.”
Hoover said she tries to have as active a life as she can with her husband, Steve. Their son and daughter are both married and live close by. Hoover and her husband now have three grandchildren with a fourth expected in July.
“We travel, go to plays and concerts,” she said. “I liken my life to that of a retired person, but I move slowly and the simplest of outings requires great effort and planning.”
Hoover is using the “eye typing” to write a novel and is involved in advocacy causes.
“I still think I live a productive life. It’s just more cerebral than physical,” she said.
She said the 2016 film “Gleason,” which details former National Football League player Steve Gleason’s life with ALS, gives an accurate portrait of daily life with the disease.
Bedlack said most people may not realize just how restrictive a life with ALS is.
He tells people to imagine waking up in the morning with ALS. You can’t move your arm to shut off the alarm. You also can’t sit up, much less get out of bed.
From there, someone has to help you go to the bathroom, shower, get dressed, and eat breakfast.
Bedlack said the act of swallowing can even be difficult. Most people generate a healthy amount of saliva every day and simply swallow it. Many people with ALS can’t do that. The saliva must be removed from inside their mouth or cleaned after it drools out.
“There are a million things that we take for granted,” he said.
There’s also the financial burden.
Hoover noted that care for some people with ALS can top $200,000 a year.
“Most families are devastated financially and struggle to meet basic needs,” she said.
Despite the overwhelming effects of the disease, there’s hope in the near future for a cure for ALS.
Last summer, the Food and Drug Administration (FDA) approved the drug Radicava to treat ALS. It was the first new drug for the disease in 22 years.
Bedlack noted that about 50 percent of people with ALS have been self-experimenting with alternative treatments they find on the internet. Rather than discourage them, he set up ALSUntangled to encourage shared decision-making on whether these alternative treatments are worth trying.
Some of these experimentations have led to cases of “ALS reversals” where people with the disease regain some normal functions. Bedlack said he knows of 36 such cases.
He discussed these discoveries in a blog last year.
Bedlack said genome work is also making great strides. At the moment, about 30 genes have been identified as potential causes for ALS.
“I’m excited to see what develops there,” he said.
Bedlack said that gene editing techniques such as CRISPR may be able to produce a cure for some types of ALS in the next 5 to 10 years.
“The hope is we’ll be able to stop the disease in its tracks,” he said.
Hoover would be among the thousands of people living with ALS who would like to see that.
“I believe there are effective treatments in the FDA’s pipeline,” she said. “But, I think the FDA is protecting me to death with their regulatory process. People with ALS 15 to 20 years from now will most certainly have an effective treatment as stem cell science matures.”