Living with ALS: Learning to Live with Limits

US News

This is the fourth installment in a series a about Hollister Lindley, a 62-year-old resident of Richmond, Va., and how she is changing the way she lives after being diagnosed with amyotrophic lateral sclerosis, a fatal condition also known as Lou Gehrig's disease. Read "Living with ALS" part one "One Day at a Time," part two "Money Issues Need Care as Well" and part three "Medical Gains Can't Come Fast Enough." See Lindley's "Life in Pictures."

Imagine you only had a certain number of miles left in your legs. How would you choose to use them? This is only one of Hollister Lindley's dilemmas as she grapples with how to live the rest of her life with amyotrophic lateral sclerosis. ALS is fatal with no known cure - a degenerative disease that targets the body's motor neurons, causing them to die, and with them, the muscles and bodily functions they control.

For reasons researchers don't yet understand, ALS does not affect muscles in the eyes, which is sometimes the only body part late-stage sufferers can still control. The disease also spares bowel and bladder functions until near the end of patients' lives.

While the disease is fatal, the speed with which it takes away nearly every bodily function differs broadly among its victims. Hollister has a slowly progressing form of ALS, which means she could have many years left. Or not. Even if she has many years left, she does not know how many of them will be of sufficient quality to make her want to get out of bed in the morning. Hollister does not volunteer to talk about the hardships of ALS, but she is characteristically honest and blunt when asked about them.

Walking is just one of many challenges, since it is painful for Hollister, and she has grown increasingly unsteady on her feet. Falls, in fact, are a major source of serious ALS injuries and sometimes lead to death. For safety and mobility, Hollister has a high-end motorized wheelchair that costs more than most automobiles. Yet when she was puttering around her house on a recent afternoon, she got up out of the wheelchair to gather some personal papers from a nearby bookshelf.

"The less you walk, the longer you can," she notes. When healthy people walk, they destroy a lot of neurons, she explains. But neurons are regenerated in a healthy body. When Hollister walks and destroys motor neurons, her body doesn't make many new ones. So, the more she walks, the sooner she will be unable to walk at all.

Breathing is another challenge for ALS patients due to the progressive weakening of lung muscles. When Hollister goes to the Richard R Dart ALS Clinic at the University of Virginia Health System for treatment, one of the key metrics she seeks is a measure of her breathing capacity that estimates how much carbon dioxide a person can expel from his or her lungs. Hollister, a former swimmer, says her lung capacity once was "unbelievably high" but has been dropping.

"For the last four months, I kind of feel like I've moved back to Denver," she says. "I cannot exhale all the carbon [dioxide] that's in my lungs, and that is one of the ways we die."

Following Hollister's ALS diagnosis, she says her first breathing test last spring showed strong lung function at 108 percent of normal. The value dropped in later tests, and her last reading, in late April, was 70 percent.

Hollister tried a breathing aid that involves placing a mask over her face, but Lawrence Phillips, head of the ALS clinic at the University of Virginia, says the device made her uncomfortable and claustrophobic, and she just couldn't tolerate it.

"I'm getting increasingly impaired in what I can do for myself," she says. "It's taken my two great passions and two great hobbies."

Sewing was one.

Hollister, who's 6 feet all, struggled to find clothes that fit where she grew up in Hawaii, so she learned how to sew her own clothing. That's all in the past. "I haven't been able to thread a needle in three years," she says, adding that even dressing herself can be a challenge. "My best friend had to dress me for my wedding" in 2010, she recalls. "I just couldn't button anything."

The second passion Hollister lost was her delight in all things related to food. She loved to hunt for the right ingredients at a newly discovered hole-in-the-wall ethnic grocery store. She enjoyed preparing elaborate meals for company. She read exhaustively about all aspects of food - from the ingredients, to the recipes, to the cultural and even evolutionary role of food in history. Until recently, she was a local food critic for a Richmond magazine.

"It was a very time-consuming passion for me," she says, "but the last time I had a chef's knife in my hand, I nearly cut off a finger." Hollister has not been able to cut her own food for a year. Her fine crystal has given way to plastic. Her extensive cookbook collection is of little use. She can't even open a Ziploc bag. "My friends come over and say, 'We'll cook for you.' And that just makes me madder," she says. It is also a challenge to navigate through a restaurant and impossible to arrive unannounced or unnoticed, which is how most food critics prefer to do their jobs.

"The frustrating thing is you lose the ability to do things almost daily," Hollister says. Opening a pill bottle with a safety cap was the most recent thing that came to her mind. "Last week I could do it; this week I can't."

At the same time, she appreciates what computers and technology permit her to continue to do. Computers are a godsend, and the phrase "assistive technology" is now a very real part of Hollister's daily life - as cellphones, voice-activated apps, touch screens and other devices have emerged as lifesavers. For example, Hollister could recite an ode to vice-grip pliers, which allow her to securely grip objects her hands are no longer strong enough to lift or move.

And she is constantly appreciative of her husband of three years, Rich Kern. It's the little things he does that make Hollister's life easier. In the morning, he makes breakfast for Hollister, including coffee - "and this from a guy who has never had a cup of coffee in his life," she jokes. He fills a small container with milk because Hollister no longer has the arm strength to lift larger milk bottles, and he places it on a lower shelf of the refrigerator so she can reach it.

Her motorized wheelchair - with an odometer and impressive array of features - has also made an enormous difference in the quality of her daily life. "I've put about 45 miles on that chair in this house since March," she says.

Hollister is very aware her daily challenges pale in comparison with ALS patients facing far more serious disabilities. "So many other ALS patients can't speak or breathe," she says. "And so many people I've met online in ALS chat rooms have died."

Yet, at the end of the day, Hollister is acutely aware that she is dying. While some aspects of life may appear a little sweeter, feeling all warm and fuzzy about life's small pleasures is just not her.

I don't have any kids. I don't have any brothers or sisters, and both of my parents are dead. So I'm not a person that would take anything to fight this disease. ... I may go to sleep and not wake up. There is a whole bunch worse. It can be a whole bunch bigger than this. For me right now, this is incredibly inconvenient and remarkably frustrating, but there is very little pain involved. What there is, is a muscle spasm, and I got enough muscle relaxers here to take out a South African bull elephant.

So I don't know how long I will be alive. Obviously, I can't ignore it. But I try not to let it own me. I'm going to own this disease. It is not, I repeat, not going to own me.

Twitter: @PhilMoeller

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