9

The Examined Life

What it takes to survive a motor-neuron death sentence

“I had this guy come by yesterday, this twenty-nine-year-old guy who shows up in the office with his wife. They’re from the Deep South. Mississippi. White guy. Cracker. A slob of a tattooed smoker.”

Elliott was telling a story. It was Saturday morning, and he was telling me a what-the-hell-brings-you-here story. We had run into each other at the hospital on a mutual day off, and we were perched on tall stools at one of the café tables at the entrance to Au Bon Pain, a place which not only fuels the Brigham staff with caffeine, but also supplies the bulk of the empty calories and artery blockers. It is tucked inside the hospital’s main lobby. I should have been home fixing the guy wires on the ham radio antenna on the back of my house, the bane of my neighbors’ existence, but a lifeline for me. Instead I had come in to check on Hans, one of my Parkinson’s patients, in order to adjust his deep brain stimulator, after which I was expected in Nahant, at George and Felicity Kalomiris’s house. I hadn’t seen George in five years.

Elliott was on a roll. “So the guy says, ‘I’m in pain, PAIN! I can’t touch here, I can’t touch there! My whole body … it hurts, and it’s just killing me.’ And the wife is saying, ‘Oh, baby, oh, baby, tell the doctor about that other doctor you saw.’ And they hand me a paper that says ‘Decrum’s disease.’”

I nodded at the reference: a condition with multiple little lipomas, or benign soft tissue tumors, that can be very painful.

“She says, ‘That’s what he has! That’s what you have, baby.’ So they show up here—listen to this—they drive all the way up here from Mississippi, with no place to stay, no money, no insurance, and they tell me, ‘We’re staying in Boston until you solve it.’ Now this is a guy with no intellectual capacity at all—a moron—and he goes on the Internet, and he finds this Decrum’s disease, which of course isn’t Decrum’s disease at all, but Dercum’s disease. ‘I hurt all over, I’ve got Decrum’s disease!’ He’s wailing like a banshee. It was the most disembodied type of nonsense. Blatant. Of course what he’s got is a major psychiatric problem, as well as being a nut.”

This passes for conversation sometimes. We get all kinds of patients, some of whom we help to live, some of whom we help to die, some of whom we can’t help at all. All we can do is listen.

Before Elliott could get any more traction with his tale of woe, a rumpled old guy in a trench coat materialized by his right shoulder, and as if Elliott wasn’t even there, as if he didn’t happen to be right in the middle of a sentence (which he was), said to me:

“Somebody just pointed you out and said, ‘When this man has a friend in need, that’s a man he stays away from. Is that true?’”

“That is true,” I replied automatically. “And do you know that for a fact?”

“No, but somebody pointed you out.”

“And is your name Henny Youngman?” I asked him.

“I used to write for Henny when he lived on Thirty-five East Thirty-fifth Street.” By the looks of him, that was entirely credible. “The first joke I wrote for him: ‘I ate at his house and his wife was the world’s worst cook, and I said, ‘Now I know why you say your prayers after you eat.’”

Very good, I thought, even though it’s a Myron Cohen joke, but good enough that I returned the volley. I couldn’t help myself.

“My secretary just buzzed me and said, ‘Doctor, there’s a man out here who thinks he’s invisible.’ ‘Tell him I can’t see him.’”

Elliott gave me a what-am-I-chopped-liver look, but our new friend ignored him and pressed on.

“I just bought my girlfriend a diamond ring, and she says, ‘Gee, thank you. It reminds me of Arkansas.’ I say, ‘Why Arkansas?’ She says, ‘Little Rock.’ I have another girlfriend, her name is Bubbles, and Bubbles was wearing very tight skirts. They were so tight that I became concerned about her circulation. Then I realized, the tighter the skirts, the more she circulates.”

“You can’t be a local. Are you from New York?” I asked him.

“I live in Framingham. You know what happened, I got a call from one of your colleagues this morning, he said, ‘The check you sent me came back.’ I responded, ‘So what? So did my arthritis.’ My marriages were so horrible, my first marriage, my wife divorced me, my second marriage, my wife won’t.”

The guy was a throwback to a time and place I identify with.

“What’s your name?”

“Sheldon. You call me, we’ll have some fun.”

“Dr. Allan Ropper,” I said, extending my hand. “It’s been a pressure.”

He gave me his number, but like any good comic, he had to have the last laugh.

“You know what happened to me this morning? At three a.m. my neighbors began pounding on my door, POUNDING on my door—would you believe? At three a.m.!? Luckily I was still awake playing my bagpipes. That raises chutzpah to new heights.” And he was gone.

“And this is not unusual for you?” Elliott said.

“It’s a curse.”

*   *   *

Felicity and George Kalomiris live in Nahant, a peninsula that juts out into Massachusetts Bay some ten miles north of Boston. The views from their living and dining room windows facing south are striking: the quaint waterfront houses across the street barely obscure, in the middle distance, an expanse of placid ocean that fronts Revere Beach, while off in the distance, the skyline of the city stands unobstructed, including the old Custom House Tower, the financial district centered on State Street, and even, if the telescope is pointing just so, the golden dome of the State House, where George used to work.

Felicity describes their lives as quite normal, and they are normal, or at least “normal-looking” to anyone with the same level of education and accomplishment. Felicity goes off to work each morning at a downtown law firm, their ten-year-old daughter goes off to a private school, and George works from home, mostly on a desktop computer in the living room with the commanding view.

The room itself could be a showroom of fine contemporary furniture. I would not be surprised to see Cindy Crawford stroll through, extolling the style and comfort of the place. Muted earth colors, natural wood floors, textured fabrics, fine millwork, and coordinated window treatments contribute to the inviting, if not becalming, atmosphere. It is a fine house, not merely a nice one, a beautifully situated, tastefully appointed home, undoubtedly the envy of the odd passerby.

“Our lives are so frighteningly normal in this house,” Felicity told me, “that sometimes I have to stop and wonder if it really should be this way. George rarely complains about anything, and our preoccupations are pretty typical—work, family, finances, running the house, connecting with friends.”

What George could complain about, and has every right to, is that over the last ten years he has lost motor control of over 99 percent of his body. His voluntary movements are now restricted to eyeblinks, very slight lip pursings, and a barely perceptible shifting of his left knee. He cannot speak, a machine breathes for him, he cannot clear his throat, scratch an itch, reposition himself in his motorized chair, or even turn his head to face a different direction when the sun coming through the wooden parlor blinds shines directly into his eyes. He can still feel everything—his sensory nerves are intact—and it is unclear if that’s a blessing or a curse. Despite all this, his brain, his thought processes, his intelligence, his creativity, and his yearnings remain undiminished. He is still George, the one-time high school and college basketball star, the local kid made good, the former state director of economic development, the devoted husband and loving father. He is a man who considers himself lucky, the oddity of which dissipates, along with the oddity of Felicity’s remark about the normality of their lives, when you spend some time in their home. George Kalomiris is a very lucky guy, and his home life is indeed frighteningly normal, as long as you can come to terms with the frightening part. George and Felicity have, as has their young daughter, and so have I.

George first came to me ten years ago when I was the head of neurology at St. Elizabeth’s Hospital in Boston. He had just completed construction on a new house, the baby had just arrived, his career was taking off, his wife had landed a great job. Everything was coming together for him. My job was going to be to tell him how it was all going to come apart, and to walk him through the stages. Except that it did not entirely come apart. George is one of the few ALS patients I have treated who made the decision to press on no matter what. It is an agonizing choice either way, or so I thought until I met George.

It had been quite a while since I had last checked in with him. My secretary had arranged the visit for the first Saturday after New Year’s Day. My ICU service was done, there was no pressing business at the hospital, so it was a good time to follow up. The day had begun bizarrely enough, with Elliott being upstaged by Sheldon’s stand-up routine, followed by a testy session with my Parkinson’s patient, and then a calming drive up Route 1A in light traffic. The tide was high as I drove across the narrow causeway that leads to the sheltered hamlet of Nahant, next to the city of Lynn. The deep hue of the ocean met the crisp blue of a cloudless sky in a line so sharp that I kept sneaking glimpses to my left as I drove.

When I got to George’s front porch and rang the bell, I could not help glancing behind me, out over the water at the sheltered beach and the city skyline beyond it. To the left of the front door, through a bay window, I noticed that the parlor had been set up with a hospital bed and a lift. This was George’s bedroom. This was his view.

When I first met him back at St. Elizabeth’s, George Kalomiris looked as if he had stepped out of the pages of Sports Illustrated. He was six feet four inches tall and built like a tight end. His face conveyed his senses of humor, accomplishment, compassion, determination, and also fear. Today, although still recognizable, albeit with much less hair, George has been transformed, and his ability to communicate through a look, a glance, a tilt of his head is almost gone. He now sits in a mechanical chair, his head held up by a strap across his brow, his lips somewhat flaccid.

We rarely stop to think about how much of our persona is created by the forty-three or so facial muscles at our disposal, especially those that encircle our eyes. When we think of eyes, other than their color, we think mainly about their frame: the lids, lashes, and brows; a squint, a glint, an arched brow, a purposeful asymmetry. We speak with our eyes. We read other people’s faces through a myriad of micro-expressions. One of the cruelties of ALS is that it not only forces its victims onto ventilators, thus robbing them of speech, but it eventually neutralizes most of the facial muscles, reducing the expressive palette to a few basic gestures.

It was hard at first to read much in George’s demeanor, though I could tell that he was happy to see me. His lips barely formed the outline of a smile, his eyes evinced the stirrings of joy, his personality still came through.

Next to his wheelchair, in a compact box with tubes that run up to, into, and down through his trachea, George’s ventilator emitted a rhythmic whoosh, not as sinister as Darth Vader’s, yet still disembodied.

We took our seats in the sunroom off the kitchen—Felicity and I across the table from each other, George wheeled to the head of the table by Robert, his home health aide. Robert is one of three Ugandan assistants who trade off attending to George twenty-four hours a day, periodically suctioning out his tracheostomy tube, shifting his limbs, setting up his feedings. Robert seems perfectly suited to the task, the very picture of placidity and competence.

“We’ve been very lucky,” Felicity said. “When George first got his trach, we hired a gentleman from Uganda to live with us and help me care for George, while I also managed a very young child and a demanding full-time job. He was here six-and-a-half days a week. His name was Elijah, and through him we got tapped into this incredible community of caring, hardworking, Ugandan health-care workers.”

Felicity showed none of the care and worry I would expect, or that I had seen in spouses of other ALS sufferers. She is a handsome woman, with a face that is full, intelligent, and very pleasant. She has been through a lot, having survived her own health issues (as she would eventually tell me) over the last five years, but she wears none of this on her face and in her manner. For her, that’s all in the past.

“By the time George got the tracheostomy he was worn out. In hindsight, maybe he shouldn’t have waited. With the wheelchair, it took him falling and getting a concussion before he finally used it. With the feeding tube it took a ghastly amount of weight loss, and then almost choking to death before getting it. With the ventilator, it took a collapsed lung, pneumonia, and an insanely low vital capacity. He was reluctant with each new decision he faced along the way. With no available effective treatment or cure for ALS, he understood that once he sat in that wheelchair, he would never walk again; once he got that feeding tube, he would never taste food or drink again; and of course, once on the ventilator, he would never speak or breathe on his own again. He was really sick for about four months after the trach was put in, and he had a series of hospitalizations for various infections, the last being in May of ’06, when he wound up in the hospital for forty-eight hours with bronchitis. But that was the last time he was in the hospital.”

“Does he get simple things, like flu shots?”

“We see the pulmonologist every six months for a trach change and a respiratory assessment. And we get his pneumonia and flu shots through the Visiting Nurse Association.”

“How often do they come?”

“We have an aide from VNA who comes seven days a week, and a nurse who comes about twice per month. Two people spend about three hours each morning getting him up, bathed, and dressed. It then takes an hour or two to get him back into bed at night. He definitely does best being cared for here at home, away from the hospital.”

The conversation started up where it had left off years before. On its face, this was a social call. But it was just as much a follow-up visit. I came partly because I needed to know how this works, how they make it work, how others could make it work. As I expected, it works very slowly.

“Do you have a plexiglass letter board like we used to use?” I asked.

“No. We should get one. But the computer has been fabulous. He uses it to write letters, and he can e-mail. But it’s a lot of work.”

“Are you controlling the computer with your finger or with your head?”

“With his knee,” she replied. “It’s kind of weird, but he has continued to have almost imperceptible movement in one knee, and in his head.”

“No finger movement?”

“No,” George signaled with blinks.

“Yes” and “no” are a simple matter for George. In order to speak in full sentences, he uses a spelling system that divides the alphabet into five lines. He blinks to confirm the line, and his respondent then recites an alphabetical string until George blinks again.

“How do you know when to stop?” I say. “Do you go through the lines of the letter board, George, and then you stop her at one of the lines, and then she goes across the letters?”

“Yes.”

“George is now texting like a teenager,” Felicity said. “He’s gotten a little bit shorter with his messages. He used to spell out whole words and long sentences, and I would say to him, ‘You don’t need to spell it all out, or use prepositions.’ But it’s hard to break good grammar habits.”

“So he’s bringing you to the line?…”

“Yes. And he’ll blink when I get to the right line. Then I go across to the letter.”

“And you have an equanimity and a patience with it? You are his voice?”

“Yes.”

“And this is the main thing you use with your child?”

“Yes. She’s very adept, incredibly so. She stores it all in her head and rarely writes down the letters he is spelling out. My daughter read five of the seven Harry Potter books in the second grade. They’re five-hundred-page novels, on average. She’s an amazing reader, and I tell people it’s because at the age of three she would come home from nursery school, and George would type: ‘Hello, Sophie, how was your day?’ Then he would hit SPEAK, so she would see what he was typing, then hear it. When she was five she learned the entire line system as a surprise for his fiftieth birthday.”

It was time to get down to the business I came for.

“We had conversations when George was getting worse about what to do,” I began. “There was the nodal conversation. You may be aware that while it’s not exceptional to decide to keep living with ALS, it’s not the normal decision, either. Most people throw in the towel. A lot of them are quite a bit older.”

“We’ve known many ALS patients in the last ten years.”

“I can imagine.”

“We’ve met many through this journey that haven’t chosen our path.”

“I was acutely aware of the fact that George was young, in his thirties, and that you had a young child, you had a life to live. He had certain notions of what was important and what wasn’t, what he wanted to do for himself, and what he wanted to do for his family. I’m sure you had those conversations at great length. So I’m just curious, what was that like?”

“What was it like to have those conversations?” Felicity looked at George, who rolled his eyes. “I can say a few things I remember about that. As soon as it was clear that he had ALS, George started doing the research, looking around the corner, looking the bull in the eyes, and didn’t back away from trying to understand what that meant, and what was coming for him. And very early on, long before he was even in a wheelchair, he made a very clear and informed decision that he wanted to have the trach and go on a ventilator. I can recall one conversation in particular, perhaps the first on this subject. We were out on a drive, and he told me, ‘I want to go on a ventilator when the time comes.’ I responded that he would have my full support in whatever decision he made, and that I was glad to know that now, because we both knew there would be a lot of planning. My understanding at the time from talking to you and reading about ALS myself, was that George would lose his physical body, but his mind and senses would remain intact. I remember telling George that I fell in love with him because of his soul, and that I knew that his soul, his essence, his spirit—what defined him as a man—was going to remain intact whatever happened to him physically…”

She turned to face George. “Do you remember this conversation? We were on our way to Salem Hospital to see our first neurologist.”

“1-A-B … 1-A-B-C-D-E … 3-K-L-M-N … 4-P-Q-R-S-T … 3-K-L-M-N-O … 3-K-L-M-N … yes.” Benton.

“So it was after we had seen you, and we had a pretty good inkling of what was going on, and we were on our way back to see Dr. Benton. We were in the car talking about what it means to get to a point where you are no longer physically able to move, and you are locked into your body. Is all hope lost, and are you no longer worth anything? Are you no longer a human being? Are you not really alive at that point? George’s philosophy, then and now, is that hope is never lost, although the things he hopes for may have changed with time. He tells me often that life is a daily gift, always worth living, notwithstanding the physical limitations of ALS.”

When Felicity and George first visited my office together, they told me the story of the pull tabs. George was unable to grasp the baby’s diaper pull tabs between his finger and his thumb, and he had said to Felicity, “Something’s wrong here.”

“That was October 2002, the diaper thing,” Felicity reminded me. “I told him, ‘Nice try, now go in there and change the diaper.’ But over the winter there were other things. It would become increasingly troubling, but not enough to make us think something was wrong. I would ask, ‘Did you pinch a nerve?’ because George was incredibly athletic. And I remember we went to an event in January 2003, a black-tie event, and George couldn’t get his cuff links on. His thumbs were weak. And at that point I finally said, ‘You really should go see somebody.’”

“U … 3-K … 2-F-G-H-I … 1-A-B-C … 3-K.” You kicked? “3-K-L-M…” Me.

“Oh, yeah, we were roughhousing on the couch with Sophie, and I kicked George in the thumb by mistake, and the thumb went right out. It popped out, because, though we did not know it at the time, all of the muscle was gone.”

With seemingly infinite patience, Felicity picked up on George’s subtle signals when he had something to say, and led him through the letter system. Most of his interjections were brief, but at times, I had to repress my natural urge to butt in as she painstakingly spelled out one of George’s longer thoughts, a process which involved anticipation, a lot of word-filling guesses, and a good memory. As she ticked through the alphabetical system, all the time keeping her eyes on George’s eyes and lips, her right hand scrawled letter sequences on a lined pad of paper that gradually filled with wayward, clipped words and sentences, cross outs, and doodles. She did all of this without a hint of frustration.

“… O-n-e t-o t-w-o m-o-n-t-h-s? Yes. W-e-n-t t-o h-a-n-d s-u-r-g-e-o-n. Right. One or two months went by … a-n-d i-t n-e-v-e-r … h-e-a-l-e-d. It never healed. He’s just saying that it never healed. He ended up seeing an ortho guy who was a friend of his, who had done his knees, and he was the one who observed the bilateral loss of muscle tissue, and sent him to see the neurologist right away. At the time, George was still an avid basketball player. We had just moved into this house. We moved in the summer of 2002, had the baby two weeks later, and then a year later, unfortunately, we met you. It was a fast-paced series of events that moved us from a state of incredible joy to one of profound disbelief and sadness.”

Back then George had a powerful athletic body. Scattered around the living room were pictures of him from that era. The muscles were now gone.

“Where did you meet George?”

“George and I met in the late ’80s. I was working in the same office building as he was. It was love at first sight, and I stalked him for a while. But then I went off to law school, George got engaged, and I got seriously involved with someone else. But we stayed in touch, our friendship deepened, and eventually we both came to realize we were meant to be together. We’ve known each other for a long time.”

“George, do you think that religious or cultural background played a role in the decision to stay here at home on the vent?”

“L-o-v-e f-o-r l-i-f-e … a-n-d … f-a-m-i-l-y.”

“If there has to be a yes or no answer to that question, I would have to say no,” Felicity added.

“Did having an athletic background give you an edge, George?”

“M-a-r-t-i-a-l a-r-t-s m-o-r-e s-o…”

“George was a brown belt—judo. At the time the symptoms started he was doing aikido.”

“Did the martial arts training help with your decision to go on the vent, or did it make it easier to manage the vent?”

“T-h-e w-i-l-l t-o c-a-r-r-y o-n … w-i-t-h p-u-r-p-o-s-e.”

“He’s saying he had reason and purpose to stick around.”

“In retrospect,” I asked, “did you have a realistic expectation of what it would be like to be here today?”

“It’s hard to say. We had no expectations because it was so unknown. It would be as if someone had said to us: ‘Felicity and George, we want you to go live on the surface of the moon for five years,’ and after we lived there for five years, they said, ‘Was it what you had expected?’ I would have to respond, ‘I wouldn’t have known what to expect.’”

“George, could you have envisioned it, or do you have Felicity’s perspective that it was going to be what it was going to be?”

“W-e w-e-r-e g-o-i-n-g t-o … a-d-a-p-t … n-o m-a-t-t-e-r w-h-a-t.”

“There was very little anguish about what it would be,” Felicity added. “In my estimation, it takes a very special person, one with great conviction and patience, to be able to say: ‘This is what I want. If it turns out to be a disaster, it’s going to be a disaster, and that’s it.’”

“It takes a lot of courage,” I added.

“It takes incredible courage. George is the bravest human being I will ever meet, but I don’t think George has ever patted himself on the back about this. There are people around us who’ve never been able to come to terms with this: friends and family members who love him very much have had an incredibly difficult time mustering up the effort to tackle his mode of communication. And many found the tubes, the vent, the total paralysis, the seemingly complete incapacitation of a vital human being, too difficult to witness, and they eventually faded away. But those friends that were able to look into the ugly face of ALS and not turn away came to realize that the essence of George had survived this calamity, and for that they have been blessed with the ongoing gift of his love, his humor, his friendship, and an inspiration for life that comes from being around him. I know it sounds irrational, but for George and me, and maybe in part because of our daughter, we have endeavored to live our lives normally. I tell people all the time, we are a pretty normal couple. George is an amazing father and an incredibly supportive husband and friend. There’s just a few things in our lives that are a little different, like, you know, like that he is ninety-nine percent paralyzed and totally dependent on others.”

“I assume that financially it’s a burden, but that there are also resources available to you.”

“Yes, it has been a financial burden. But we were also very blessed. We were at a time in our lives, just before he got sick, when we were preparing to have a child, and were taking on a significant mortgage, so we were planning for the future, financially speaking. He had health insurance…”

“And disability insurance?”

“And long-term care. And it was really very fortuitous that we had pursued all of that. It’s very difficult financially to live like this, and we are incredibly lucky that I have a good job, we’re lucky that we have been able to keep this house. You can’t leave George by himself; he requires twenty-four-seven care. It’s a big issue for people facing these situations to come to terms with the economic impact.”

“If I hear you right,” I said, “you would have made that first leap anyway, because that was based on character, and then figured it out later. But you’ve been lucky. You told me all these things early on, and I thought, if anybody could do this, it would be you. I don’t recall, was I encouraging and supportive, or did I try to discourage you?”

“No, you did not try to discourage us. Do you remember these conversations, George?”

“V-e-r-y w-e-l-l. R-e-c-o-m-m-e-n-d-e-d v-e-n-t. T-a-l-k-e-d a-b-o-u-t l-i-v-e-i-n a-i-d-e … t-a-l-k t-o V-N-A … g-e-t a-n a-i-d-e.”

“I don’t remember you saying anything that was negative. To the contrary, my recollection is that you clearly and nonjudgmentally discussed the choices we would face, and some implications of our decisions.”

“Have you met other ALS patients through the fund-raising?”

“Yes, and this gets to your question of living with purpose. George is very busy, and has been for the last ten years. He gets up early and is engaged every day. When you no longer have a reason to get out of bed, that’s when you’re going to take a long look at the worth of your life. We got involved with an organization, with a group called the Angel Fund, and through that organization we’ve met other people afflicted with ALS, or otherwise affected by the disease one way or another.”

“U … t-o-l-d … a-b-o-u-t … a-n-g-e-l.” George interjected. “Oh!” Felicity interpreted, “You told us about the Angel Fund.”

“Yes, I told you about that.”

“That’s been a way for us to raise money and awareness about the disease. George and I also sit on the board of an organization called Prize4Life. It’s an organization that seeks to increase discovery in the field of ALS by funding prizes for people doing innovative and impactful research. We’ve met people through these organizations, but we’re not tapped into a specific support network. We haven’t had time for that. You had a big impact on George. One thing you said that has always stuck with both of us, when you were pretty sure this was ALS, was ‘Go home and live. Don’t go home and die.’ That may be, from your perspective, just a normal thing you would say as a physician, but from our perspective, being given this kind of death sentence, it was very important advice. We took it very literally and it has saved us both in many respects. It’s sad that not every physician thinks like you. We found that there’s a whole generation of physicians coming up today that don’t share your philosophy. They are being taught that you have to balance economics and all these other factors with the practice of medicine, which clouds their ability to effectively focus on what the patient wants and needs.”

“It comes back to a rudimentary aspect of medicine,” I said, “which is that the physician is the best person to advocate for that patient at that time and for their needs. So somebody wants to die and refuses to be like this? My role is to be their doctor. Somebody wants to keep plugging along in every way possible? My role is to help them plug along. And yet you and George are not judgmental about other people who decide to throw in the towel?”

“No. On this issue of making choices, George’s view is that there is no right or wrong road. There is only the road you choose. And although many may have gone down that road before, it will always seem uncharted and untrodden to the newly diagnosed person. Some choose hospice, some do not. Some elect to have a tracheostomy and go on a ventilator, many do not. Some decide to seek experimental and unproven treatments, and many do not. George feels strongly that these choices shouldn’t be judged or criticized. We are occasionally asked to talk with families that are considering vents. We try to be as helpful and informative as we can, but we understand the gravity of these decisions and avoid interjecting our personal views. George also participates in chat rooms with other patients, but…”

“… n-o-t a-s o-f-t-e-n … a-s I u-s-e-d t-o.”

“George, do you have complete control over the computer independently?” I asked.

“M-o-r-e o-r l-e-s-s.”

“He just needs someone to turn it on. He doesn’t read much online, even though he was an avid reader. He doesn’t read books anymore, but he’s got a pretty impressive entertainment system. I don’t know what people afflicted with diseases like this would do without this technology. He can e-mail friends, he writes letters to our daughter, he can listen to music, download music, and do basically any project you can do with a computer. A few years ago, he composed two songs, and at one point he uploaded hundreds of our old photographs with help from aides, feeding photos into the scanner, digitizing and editing, one photo at a time.”

“S-h-o-p…”

“And he likes to shop on Amazon, that’s another thing. He’s a shopaholic. I used to bitch at him: ‘Look at this credit card bill! Why did you buy all this stuff?’ But then I wised up. Now I say, ‘I need this or that, can you go online today and get it for me?’ It’s great, because I hate to shop, and I don’t have time, and he does all the shopping. The computer keeps him connected. My daughter and I leave here at quarter to seven every morning and don’t come home until seven o’clock at night. So he’s here by himself, with the aide and our dog, twelve, fourteen hours a day, and he’ll often spend a decent amount of that time on the computer.”

“In the end,” I added, “you have to make your own decision about whether to continue. But what I tell other patients is that the decision is not irrevocable. It’s not easy, but it’s not irrevocable. You can change your mind.”

“E-x-c-e-p-t i-f y-o-u d-e-c-i-d-e n-o-t t-o g-o o-n a v-e-n-t.”

“Very funny, George.”

“I think,” Felicity continued, “that it is very relevant to this issue of decision-making that when George got sick, George and I were very much in love with each other, and in a strong place in our lives. We had been married for five years, we were starting out, and we were committed to each other, to our marriage, to starting a family together. It’s very personal to your circumstances. We’ve seen families that have fallen apart—one spouse comes down with ALS, and the other gets up and leaves, or stays but lives in a state of ambivalence. It’s hard to imagine, but I try not to be judgmental. The strain of such a devastating illness on caregivers is almost unfathomable. We know families dealing with ALS who have no money, no insurance, no support. We’ve met quite a few ALS families, people who started on this journey before us, and people who started on this journey after us, and the decision to go on a vent is part of the bigger picture of where you are in your life, who’s in your life to support you, what kind of financial resources you have, who you are.”

“What is your estimate of the proportion of people who have elected to go on a vent? A fifth?”

“M-a-n-y o-n-l-i-n-e t-r-y-i-n-g t-o d-e-c-i-d-e. M-a-n-y o-n-l-i-n-e o-n v-e-n-t-s, o-r d-e-a-d, w-h-o d-e-c-i-d-e-d a-g-a-i-n-s-t.”

“The one person that stands out in my mind who was ambivalent about it was your friend Arnie,” I said.

“When George was first diagnosed,” Felicity recalled, “Arnie had had the disease for ten years. We met him at an Angel Fund event, and he was holding a bottle of beer. He said, ‘I’m a slow progressor.’ And George said, ‘That’s my buzzword, too. I’m a slow progressor.’ We had great hope after that meeting because Arnie was doing so well after a decade of the disease. But two years later, I could see that Arnie was progressing rapidly, and it was heartbreaking. Arnie subsequently had a respiratory crisis at home, went into Mass General, and then got vented.”

I remembered the case all too well. Arnie’s respiratory crisis was one for which he and his wife were completely unprepared, and the lack of oxygen caused brain damage. “That was a disaster,” I told Felicity and George. “I was in the room. It was a very painful night.”

“I-f t-h-e-r-e w-a-s … f-o-r-m-u-l-a … i-t-s t-o t-r-y t-o s-t-a-y … o-n-e s-t-e-p a-h-e-a-d … i-n … t-e-r-m-s o-f … y-o-u-r d-e-c-i-s-i-o-n.”

“So, yes, we know people on vents, but my impression is that it’s very uncommon.”

“To what extent do you avoid living in the future?” I asked.

“I’m really curious as to what George’s answer to that is. I don’t know about you, George, but for the last ten years, I’ve lived absolutely one hundred percent in the present. I had breast cancer four years ago, and thyroid cancer three years ago, and George is dealing with this. I think that our lives have leveled out, our priorities are crystal clear. Our chief priorities are maintaining our moral and spiritual compass, our health and well-being, and that of our daughter, and maintaining my livelihood. But I don’t worry a lot about what is going to happen a year from now, or twenty-four months from now, or forty-eight months from now. Will we be okay at that point, or are there things that we’re supposed to be doing now to make sure that we’ll be okay? I don’t know. I try to live in the here and now because that’s what we have. I don’t worry about the future. I don’t know about George…”

“I d-o-n-t h-a-v-e … p-r-o-b-l-e-m l-o-o-k-i-n-g a-h-e-a-d … p-l-a-n-n-i-n-g.”

“I go to work every day, Sophie goes to school every day. We live as normal a life as is possible. Sometimes I say to George, ‘Shouldn’t we be wallowing in despair, shouldn’t we be anxious and depressed, something other than we are?’ I think our daughter has a lot to do with the choices we make and the positive and hopeful attitude we try to bring to this situation. George reminds me often that our role is to give Sophie balloons, not anchors. We like to believe that getting up and living every day, making our lives as normal as possible, having routines in the house, boldly staring down the dragons, and getting on with the business of making life as good as we can gives her balloons, not anchors.”

“From the outside,” I said, “you’re aware that nobody would identify this as normal. But sitting here, it doesn’t seem abnormal.”

“Maybe what’s abnormal is the fact that we’ve been able to survive.”

“You’re doing more than surviving. You even maintain a sense of intimacy as a couple, and many couples can’t manage that. You’re not living parallel lives, you’re living it together.”

“One hundred percent. Well, maybe ninety percent. Sometimes he could kill me. George can yell at you without saying a word, and make you feel that you’ve been yelled at. It’s his expression … he’ll click his teeth. If something’s not happening right, he’ll click his teeth hard. He still has expressions, he can look angry; he can make it clear that he’s very upset. Anyone would recognize it as an angry-appearing face.”

“Does he have the equanimity he had before?”

“One hundred fifty percent. I find that to be completely remarkable. It’s part of the frustration of living with ALS that people can’t get beyond the harsh physical effects and recognize that the person that they knew for twenty-five or thirty years is very much unaltered.”

“C-a-l-l m-e c-r-a-z-y b-u-t l-i-f-e i-s g-o-o-d l-i-f-e i-s g-r-e-a-t.”

“One last thing,” I said. “I just had a patient, a woman who I diagnosed with ALS. She was hospitalized and had all kinds of problems. She initially got a trach, but she didn’t want to persist. She didn’t want the trach to begin with, and I think I may have cornered her into it. It’s the flip side of what you’re talking about. She died in the ICU. She chose to die, and I didn’t stand in her way.”

“So she had the trach taken out?”

“Yes, I took the trach out at her request, at the family’s request. She was able to speak for a few minutes for the first time in a while. But just a few minutes.”

“You did all that?”

“I did what she needed done,” I said.

“Is that comforting to you in a way?”

“Tough. Very tough. The two extremes in neurological practice, as I see it, are the result of the incredible damage that can be done to the brain and spinal cord—dehumanizing effects. One extreme is to save life at any cost; the other is to participate in ending somebody’s life in order to reduce their suffering. You’d think the two extremes can be reconciled, but they can’t. You have to live with both. What are you going to do? It’s not my life. It’s her life. I can’t give or take it away from her. Are you a little bummed out that I would be part of this?”

“No. Not at all. You didn’t take her life away from her. You didn’t knock her off. Isn’t the point you’re making that it was her choice, and you were just the implementer of her wish?”

“All I could do was make her suffer more,” I replied, “because of an ideological perspective that had nothing to do with the individual patient.”

“No. That doesn’t trouble me at all. George and I are not fixated on a notion of: ‘Live, live, live, no matter what.’ We just want people to be able to make their own choices.”

*   *   *

I left, and drove back across the causeway as dusk approached, back to my wife, my house, my dog, my ham radio pals, my life, and Sheldon.

I ran into Sheldon a week later when he came back to the Brigham for a follow-up visit for his wife, who was in bad shape. Her heart was failing. Making matters even worse, his daughter was an inpatient at the Brigham due to a stroke that led to Dejerine-Roussy syndrome, a debilitating combination of aching, burning, jabbing pain that is so life-altering that it frequently leads to depression and psychiatric problems. Sheldon could only stand and watch as his daughter suffered. I had arranged to meet him at Au Bon Pain again.

“You can’t make gold out of lead,” he said to me, “and I’ve been trying for twenty-five years. But it just doesn’t work. Everybody has problems, we all have struggles in life. It should be struggles maybe at first, and the rest of our lives should be lasting delight.”

“This is in relation to your daughter?”

He nodded, then shrugged. No jokes.

“Listen, Sheldon. I have two questions. One is: Why did you come over to me the other day here at the hospital? The second is: What’s with the comedy for you? I am a guy who’s got a lot of comedic material. I grew up in a house where everybody was telling jokes in different languages and drinking schnapps until two o’clock in the morning. That was the crowd, smoking cigarettes and cigars, my uncles giving me cigars when I was nine years old. So while I don’t wear it on my sleeve, I’m always interested in people who have been in the comedy-writing business. Not the flashy stuff, but just what it’s about for you. But first, why did you come over to me that day, how did you know?”

“Well, when I walked over to you, when I came in, I had just gone through a terrible session with my daughter upstairs, and you look like a nice person. So I’m a people person, and I stop to tell a joke. I don’t think it’s inappropriate for most people, and I don’t tell pornographic stories. Anyway, I came over to you because you look like a nice person.”

“But do you do that all the time?”

“No. I came over because I was so overwhelmed with emotion. I just wanted to let it go. I digressed from what was happening in my life, and I went into a new life.”

“And you really wrote for Youngman?”

“Henny was a dear friend of mine, rest his soul. I ate in his home innumerable times. He paid me a dollar a year so I could say I wrote jokes for Henny.”

“You know,” I said, “as a kid I was a waiter in the Catskills, and I saw all those guys, and I picked up their timing.”

“Where did you wait?”

“Tamarack Lodge. I was at Grossinger’s for one summer. So how did you get into writing jokes?”

“Just natural. I never write anything down. I’m blessed with a photographic memory. Some of it is creative, but some is material that I hear. I may embellish, I may change and so forth. But we can’t endure without humor.”

“That’s my feeling,” I said, “but as you well know, not everybody shares that. I think life is too serious to be taken entirely too seriously.”

“This is true,” Sheldon replied.