Living with ALS

Keith James has watched his body succumb bit by bit to amyotrophic lateral sclerosis, more commonly known as Lou Gehrig’s disease, since he was diagnosed with the fatal ailment five years ago. Now paralyzed but able to breathe on his own, James, 41, goes through the final stage of the disease as best he can. And so do the people who care for him—his wife and parents.

One centimeter—less than half an inch—the gap created by squeezing thumb and forefinger together on the steel tabs of a medium binder clip. That’s the diameter of a dime, the kind life can turn on. And turn it did, one hot August day in 2007 as Keith James picked up a clip and squeezed.
Eight pounds of pressure, not much more than it takes to perform a routine chore like trimming your toenails. But try as he might, the clip wouldn’t budge. His brain sent the signal, but his hand never got the message. At Bright House Networks, where Keith worked as community relations director, they have a term for such a problem. They call it a service disruption.

The muscles in his hand rippled and twitched, as if something were boiling beneath the skin. It was like one of those random muscle twitches everyone gets, except it wouldn’t stop. Keith flashed back to a dark night, more than a decade before, when he lay paralyzed next to the wreckage of a friend’s Nissan Pathfinder on a desolate stretch between Tallahassee and Pensacola, thinking he’d sacrificed his independence for a chance to see Pearl Jam in New Orleans.

In that instance, paramedics attributed the temporary paralysis to shock. He was taken to a hospital in Dothan, Ala., where doctors, without the benefit of an MRI or CT scan, diagnosed him with a pinched nerve. That time, he regained the use of his arms and legs and was released with just a little residual numbness in his fingertips.

He wondered, as he considered the binder clip, if the two events could be related.

When the problem persisted, Keith looked up his symptoms on the Internet and made a startling discovery. The weakness he was experiencing was listed among the telltale signs of amyotrophic lateral sclerosis, or ALS. A lifelong Yankee fan, James knew the disorder by its more common tag—Lou Gehrig’s disease.

Keith shared his suspicions with his boss, who advised him to go see a doctor. She offered a more practical assessment: fatigue, caused by the long hours they’d all logged preparing for the opening of Bright House Stadium at the University of Central Florida—that, or middle age catching up with him at 36.

Keith mentioned the weakness to his father, Larry James, on a family trip home to Connecticut. There being no blood, and no bone sticking out, Larry dismissed the ailment “dad”-style. “I told him don’t worry, it will go away.”

Death Sentence

There is no test for ALS. The disease is diagnosed with a battery of tests designed to identify other nerve problems. This if-it’s-not-anything-else-it-must-be-ALS method has resulted in misdiagnoses, possibly including for Gehrig, whom recent research suggests may not have had ALS but suffered from a fatal disease caused by concussionlike trauma. Gehrig died in 1941, two years after being diagnosed with ALS.
Keith took these tests in the fall of 2007. One is a mild form of electrocution, in which doctors place electrodes and clips—a little smaller than medium binder clips—on a patient’s limbs and fingers and fire jolts of electricity into the weak spots, trying to jumpstart a motor response.
One by one, the tests came back negative, until all other causes had been ruled out.

The verdict: ALS.
The sentence: Loss of all muscle movement, leading to total paralysis, then death by suffocation. He had two to five years to live.

Looking back, Keith’s wife, Amy, says she thinks doctors could have been more tactful. But really, is there a nice way to tell someone who walks into your office with a little binder clip problem that he will die, mute and paralyzed, before his twin daughters take their first Holy Communion?

“When u first hear ur prognosis and the phrase ‘2-5 years,’ it’s truly devastating. Cried for weeks n still cry myself to sleep from time to time,” Keith, now 41, writes out on a computer screen mounted on his wheelchair. Keith pecks out words on the screen using a Wii-style transponder attached to an empty eyewear frame. Using his head and neck to guide a cursor around a grid, he selects letters and common words. It’s a tedious process. A single sentence can take several minutes to construct. To talk, he puts the cursor over “enter” and the computer speaks what he has written in a phonetic voice that sounds like that of a child’s electronic spelling game.
“My death is surreal at this point cause my breathing is pretty good,” he writes. “When that breaks down further I’m sure my thoughts will change. That being said, it’s [death] on ur mind daily.”

Down the Rabbit Hole

Keith broke the news to his parents by cell phone from the parking lot of his office at Bright House. They all cried together. It was one of the few times he and his parents have discussed his death directly.
“He’s had to rehash this a thousand times in his own mind, probably every waking day,” his father says. “Why bring it up and put him through it again? I just won’t do it. Even though I’m curious, I don’t want him to have to relive it for one moment. It’s not worth it to me.”

People reacted differently to Keith’s illness.

Keith’s mother, Nancy, turned to prayer, asking God for a miracle. Even now, with Keith approaching the end, she holds out hope, although she has broadened her definition of a miracle to include “miracles of the spirit”—her way of acknowledging that things aren’t looking so good.

Larry finds little solace in the spiritual, disbelieving there could be a god who would let something like this happen. He’s pledged to do everything in his power to comfort his son in his last days.
Amy, whose faith tends toward Larry’s side of the ledger, fashioned a raft of routines that she clings to for dear life, holding body and soul together with a strict regimen of fitness, part-time work and time out with friends to help fill the empty spaces where bitterness grows. Routine is good. Routine is normal. And normal is important—not only for the girls, Cameron and Sydney, twins, who, at 7 years old, don’t yet know how the story of Keith’s life ends, but also for Amy, who does.

The Bucket List

Amy operates an overhead lift on a track to transport Keith to the bathroom.

At about the same time Keith received the news of his imminent death, The Bucket List broke big in theaters. The movie asks the question: “What would you do if money were no object and you only had a few months to live?”

It was a smash hit, a shot in the arm for travel writers and tour operators, who responded with countless lists of “must sees” and “things to do before you die.” Keith and Amy didn’t see the movie, nor did they come up with a bucket list, per se, though it was the thing to do in the wake of the film’s success, even for people who weren’t on the clock. An ambitious professional couple in their mid-30s when Keith was diagnosed, they had every reason to believe their dreams would come true, in time. And when Keith found out how little time he had left, travel was no longer a priority.

It was friends, horrified to learn of Keith’s prognosis and helpless to do anything about it, who lavished the couple with gifts. Amy’s boss signed over her frequent flyer miles, enough to send the couple on a dream trip to Rome. Senior managers at Bright House arranged for Keith and Amy to fly to California to meet Robert Downey Jr. and Gwyneth Paltrow on the set of Iron Man 2, and go backstage at a concert to meet members of the couple’s favorite band, Live.

There were other perks: He and his father took a pre-opening tour of the new Yankee Stadium and Keith got to throw out the ceremonial first pitch on Lou Gehrig Day at a Tampa Bay Rays game in St. Petersburg.

These were wonderful gifts—memories that scroll on a digital frame near Keith’s wheelchair at his Altamonte Springs home.

Stealing Pieces

When he was diagnosed, Keith James tipped the scales at 185. He was a strapping man of medium height, with a thousand-watt smile and a prosperous paunch. Ginger hair and an Irish complexion gave him a robust summer blush and made him look preternaturally young.

He wasn’t wealthy, but he was on his way—well-connected and highly regarded as a hard worker, a straight shooter and a loyal friend, the kind of guy you wouldn’t mind seeing on a campaign poster some day.

He and Amy were opposites, as their choice in professions would seem to indicate. They met while working at Walt Disney World—he the gregarious marketing intern from Connecticut who made friends easily, and she the bookish planner from accounting studying for her master’s degree at UCF. The first thing she noticed about Keith was his smile. It was the first thing everyone noticed about him.

“He had a great sense of humor,” says Amy, who struggles to find strength to talk about the Keith she fell in love with. “We were a great fit with very different temperaments.”

Keith, she continues, was “a wonderful hands-on father. He did everything around the house.”

Not much changed that first year with ALS. Work kept Keith from dwelling on his problems. One doctor thought he might have lucked into a slow-moving type of ALS, which would allow him to enjoy many years of relatively good health. It was a nice thought.

By the middle of 2009, however, Keith was walking with a cane. He decided it was time to retire. His boss asked him to stay, but he declined, insisting he wanted to be able to walk out of the building under his own power.

The disease entered an aggressive phase, and by August 2010 he was in a wheelchair and his speech was starting to go.

By the time I met him, in June 2011, for the first of what would become several interviews, he could no longer eat solid food—except for an occasional tiny morsel to taste. His weight had plunged to 155, and he could barely say a word. Hard consonants had been replaced with glottal yawps, indecipherable to a stranger but still largely recognizable to Amy. He resorted to computer-assisted communication to fill in the blanks.

He was still using a joystick to maneuver his wheelchair, and the family enjoyed weekly dinners out at a local restaurant. That ended in August, when he lost the use of his hands and switched to a head control that caused his chair to lurch.

August was also the date of Keith’s last long-distance trip, a rattle-bang nightmare of being tossed around on the Amtrak Auto Train, and a van ride the rest of the way to his hometown in Connecticut. There, friends from high school were holding a golf tournament to raise money to help with medical and family expenses, as they have done for four years now. He returned from that trip sore and dispirited.

“The last of my independence is gone,” he typed, his trademark smile uncharacteristically absent. Even if he’s alive this August, he won’t make the trip home for the fundraiser, he says.

Master of Routine

According to the ALS Association, 30,000 Americans suffer from Lou Gehrig’s disease. That’s not entirely true. For every incapacitated victim confined to a wheelchair, there is at least one caregiver tethered to that same chair, equally unable to live independently. When it comes to suffering, ALS is a team sport.

The disease brings with it a loss of intimacy in a marriage, and there’s a gradual shift in relationship from husband and wife to—in Keith and Amy’s case—patient and caregiver. It is a terrible burden, made worse by the fact that the needs of the caregiver are often overlooked.

“I know it’s all about him, but sometimes I wish it could be about me,” Amy admits. “I love him very much, but you can’t be a caregiver in the way I am every day and still see the person the same.”

It’s a common lament, whispered in shame and sobbed through guilty tears by other caregivers at the local ALS support group, which meets on the first Thursday of each month at the Center for Independent Living in Winter Park. Amy doesn’t attend the meetings, preferring to cope with what life has dealt her in her own way.

Like ALS, there is no cure for the caregiver’s ordeal, but there can be relief—in the form of family members, home health-care nurses, friends and others willing to shoulder some of the load.

Keith’s parents lifted a big part of Amy’s burden, selling their home in Connecticut and moving to Central Florida in 2009 to help care for Keith, the youngest of their four children, two days a week. Friends get Amy out of the house regularly for girl talk and a few hours of time off the care clock. And then there’s The Routine.

An accountant by trade, Amy has always found comfort in structure and balance. Even before Keith got sick, she managed the family finances with a sharp pencil. By the time Keith was no longer able to work, the couple were debt-free except for their mortgage, and had a paid-up long-term disability policy in place to cover the unthinkable—which, as it turned out, happened.

It was only natural then, when life went down the ALS rabbit hole, that Amy would seek relief where she had found it before. Thus was born The Routine.

Most days Amy is up before first light, getting the girls ready for school. On Mondays, Wednesdays and Fridays, after she drops them off, she goes to the gym. Tuesdays and Thursdays she goes to work at an accounting firm.

On the days she doesn’t work, she gets Keith up around 9 o’clock, using an overhead lift on a track to transport him to the shower and toilet. She dresses him in loose-fitting gym shorts and a T-shirt—functional attire chosen mainly for ease of access. She feeds him a can of liquid nutrients through a tube in his stomach, and flushes the tube to keep it from clogging. Once he is in his wheelchair and in his usual spot by the fireplace, usually by 10 a.m., she will leave him to watch television and cruise the Internet while she runs household errands.

Tuesdays and Thursdays, Larry and Nancy take care of Keith while Amy works. The money from her job goes into savings. Household expenses are paid through a combination of Keith’s Social Security and disability insurance. Funds raised through the Keith James Family Fund—established by high school friends and supported by the annual golf tournament—cover medical expenses and any equipment not paid for by insurance or that isn’t available through the ALS Association’s equipment loaner closet.

Amy is home to meet the girls by 3 p.m., and they spend the afternoon doing homework and playing before dinner and bed.

Weekends are wide open, and Amy tries to get to the gym or out with friends as often as she can. “That helps—a lot.”

It’s a Spartan existence, but all the bases are covered. There is peace in the valley and all the ledgers are balanced.

Daddy’s Home

Cameron and Sydney don’t know what ALS is. They just know Daddy talks funny and that his arms and legs don’t work. These are things they accept, like an assignment at school.

They have observed that people treat their daddy differently than other people, and to them that makes him special. At school, the maintenance staff built him a ramp so that he could visit his daughters’ classroom. The girls like to tell people “that’s my daddy’s ramp.”

They also like his toys—the motorized chair and the electric lift that carries him from his bed to the bathroom, Daddy’s “zip line,” they call it. Daddy can’t get in the pool anymore, so he watches them swim. And sometimes he gives them rides on his wheelchair—one sitting on the footrest, one standing on the back.

Their daddy is home when they return from school, and sometimes he lets them dress him up and feed him ice cream—although feeding him can be a little frustrating, because he can’t use his hands to help.
While he still could, Keith kept a journal, writing to the girls every day and burning CDs of his favorite music for them to listen to after he’s gone. But these days he mostly just watches, silent, from his perch by the fireplace, the family golden retriever, Mattingly, at his feet, as the girls play.

The Endgame

One of Larry’s favorite rituals is wrapping Keith’s face with a hot towel, tilting his chin back and gently and carefully shaving him. It’s a manly service but also an opportunity to look into his son’s grateful eyes and commit every line on his face to memory—especially Keith’s smile, which has been a beacon for so many. And though Keith now takes all his sustenance in liquid form through a stomach tube, Larry the comforter feeds his son’s starving taste buds rich tomato soup and spicy chicken nuggets cut into tiny pieces so he won’t choke on them.

Nancy and Larry would come every day if Keith didn’t limit their visits.

“He wants us to have our own lives,” Larry says.

Before Keith’s illness they had struggled, like a lot of retired couples, to find purpose and had settled into a routine of sniping at each other just to get the blood flowing. In such an environment, a difference of opinion over the order of spices in a spice rack could touch off a cold war that lasted for days. But caring for Keith has brought Larry and Nancy closer than they’ve been in years.

It has also brought Keith and his father closer.

“When I’d come down before to visit, I’d come down to find my little boy. We’d throw the football around, and maybe he’d have a few projects for me, and that was our relationship. I think, coming down here [now], I’ve learned what a beautiful person he is. It’s a person I didn’t know.”

So has he told Keith any of this?

“No. I’m not that kind of person. I like helping him. I’m his companion. I’m his handyman.”
Keith has already signed paperwork saying he doesn’t want to be kept alive on a ventilator and, statistically, that means that sometime in the next few months his lungs will stop working and he will suffocate. Sure, the doctors could be wrong—theoretical physicist Stephen Hawking has survived with ALS for more than 40 years without a ventilator. But the odds are against that happening, and Keith knows it. ALS has taken everything from him; he doesn’t want it taking any more from Amy, his parents and the girls.

Keith slumps in his wheelchair like a rag doll.The pictures scrolling in the digital frame near him take him back to better times, before ALS consumed his life and Amy’s with it. The trip to California was nice, and Rome was extraordinary. But if he could have a day back it wouldn’t be spent aboard a ship or fulfilling a dying wish. He would want an ordinary day as a healthy Keith James, husband and father.

“It would start off with waking up and retrieving the newspaper with my dog from the driveway,” he writes. “I love the feel of the paper as opposed to online. I’d grab a Diet Coke for me and a Dr. Pepper for Amy and read the paper in bed.

“I’d wake up the kids one by one without startling them, watch television and snuggle on the couch. We’d go in the pool as a family. There’d be music playing. I’d go to lunch with my wife, play in the yard with my kids, teach them how to properly play soccer and basketball, ride bikes, work in the yard with Amy, and go to dinner as a family. We’d have movie night on the couch. Then I’d tuck the kids into bed, read them a story and finish up by myself listening to music in my office.”