Reaching Down the Rabbit Hole (18 page)

“Better?”

A tentative nod. I asked the nurse to call down to the supply room for a replacement tracheostomy tube. A stunned nursing student took over the bagging. I had to tell her to do it with both hands, and smoothly. I don’t like to be overly solicitous with patients because it can seem gratuitous and end up as anything but reassuring. But I took Louise’s hand, damp with sweat, and held it just firmly enough to let her know she was still connected to something. The look in her eyes gave me a sinking feeling that this experience had clued her into what might be coming her way.

We slipped the new tracheostomy tube in—a messy affair, with mucus dripping out of the opening onto her neck in a frappe of blood mixed with saline, with small bubbles around her trachea. She probably wanted to cough but could not generate the contraction in her diaphragm. The nurse suctioned out the tracheostomy. More blood.

Things settled down for her, but I had missed the first half of my
clinic, putting additional pressure on me to move along. At times like these, I have always felt it best to step back and let the world take care of itself for a few minutes. The idea that I could be in two places at once fell off the table for me decades ago, and I realized that, in the end, it just means being late for dinner. Over ninety minutes had passed since I first went up to see how she was doing. The contrast between her initial groan of breathlessness and now the tapping samba rhythm of the ventilator, the whooshing in and whirring out, was as comforting to me as it was to her. Respirator Zen.

We had reached the next level of her descent, the point of no returning home. I called the respiratory hospital a few times, but waited a week until I visited again, hoping that her husband would be there.

“That must have been a very uncomfortable situation, wasn’t it, when the tracheostomy leaked? This kind of thing happens infrequently. We can handle it if it comes up again.”

A single long blink—yes.

“Has enough time passed that we can talk about what we should do going forward? Are you okay with the way things are?” She signaled her response by looking to her extreme left where her husband stood.

“We had begun to talk about this before Louise got so sick,” he said, while looking out the window. “The kids and I have been over and over it, and I just can’t come to grips with what’s happening. Louise wants this to be over with. She can’t take being stuck in bed with people turning her from side to side and changing her diaper. This business of having to be fed through a tube and breathing through a ventilator is killing her.”

“You do understand that on the ventilator she could be kept alive almost indefinitely, she could see the kids grow, and her mind would be good?”

“Yes, but she’s stuck on not wanting to be a burden to us.”

Her facial muscles could barely mount an expression, but she was obviously tearing up.

“It’s tough for me as a physician to judge anyone’s quality of life, Louise. I owe it to you to make sure you’re comfortable no matter what you choose, and to make sure there’s no pressure to make any decision.” We had been dancing around this for almost a year, more intensely since her respiratory episode of the previous week. Was she going to ask me to ease her way out? Was I going to suggest this as a real possibility to her?

I can only assume it requires a long internal conversation to make a confident decision to die. Louise was not in physical pain, was not suffering from lack of air, and had no bedsores to produce discomfort. But no amount of reassurance from her husband that she was not a burden could possibly ring true. To arrive at a decision to die because of the mental anguish you are causing your loved ones is, to almost any of us, an absurd and anguishing hypothetical, the stuff of made-for-television movies. An Alzheimer’s patient who drives his family and the nursing staff utterly crazy lacks insight to make this decision. Would Louise be selfish if she wanted to end it? Would she be magnanimous? She had latched onto the diagnosis of ALS so rapidly, despite my euphemisms, that I was inclined to think she would take the lead. But again, I looked around and found all eyes on me.

The respiratory hospital is a world unto itself, a kind of purgatory, a place where tomorrow and tomorrow creep in their petty pace. The cheerful and encouraging staff do what they can to mute the peculiar odor of sweat, feces, disinfectant, and hopelessness. Louise’s husband, sitting in a chair with his back to the bed, looking out the window, shifted toward me slowly, staying seated while making the pivot. A depressive move, I thought.

“Doc, she wants it over with.”

“Have you thought about how everyone would feel when you’re gone, Louise?”

A nod, and this time with no hint of tears. She was clear-eyed and clearheaded, as best I could tell.

“Louise, are you depressed?” With all her energy, she took an in-breath
and exceeded the chest movement caused by the ventilator. Her eyes darted around to the plexiglass board resting on a chair. I picked it up and put it at arm’s length between us. She went right to the letter N.

“Do you mean you’re not depressed?”

Another deep inspiration. Now, with a determination that she signaled to me by rapid and emphatic movement of her eyes from one letter to the next, she spelled out:

“N . . . O . . . I . . . A . . . M . . . N . . . O . . . T . . . D . . . E . . . P . . . R . . . E . . . S . . . D”

“Okay then.”

The next day, she spelled out: IVE HAD ENOUGH. And then: NEVER BELIEVED ANYBODY COULD GET THIS SICK.

I arranged to have her transferred back to the hospital under the pretense of worsening respiratory failure. It was a complete canard since she had barely any respiration to fail. The residents were puzzled about why I had admitted an ALS patient to the acute neurology ward, but I took Joelle, the senior ICU resident, aside and filled her in. Some day she would find herself in the same position. Yes, there seemed to be nothing acutely wrong with this patient. She was here because her neurologist was going to fulfill his promise to her and her family, and see her illness through to the end.

The medical capability to keep someone going, while not limitless, is long. We could give a leukemia patient ten bone marrow transplants. We could try experimental chemotherapy. We could keep giving them platelet transfusions. A similar array of extreme measures is available to the ALS patient, but none of them changes the disease. So the question becomes: If you deny a patient
everything
that medicine has to offer, are you assisting in her death?

In a hypothetical case of assisted suicide, the patient is saying: “I don’t want to live. Give me something.” But in a case like Louise Nagle’s, the patient is saying: “Do you have to keep treating me to the nth degree?” We don’t, but when we don’t, Death leans in. Before that happens, a delicate conversation has to occur.

“We have to face the reality that without treatment you will die. Do you want me to make it less difficult, even if it accelerates things?”

Any physician in a hospital setting faces this scenario all too often, and is obliged to negotiate with Death through the dying patient. And it is not uncommon for the patient, after a period of consideration and reflection, to say, “Yes. Please. Let’s stop this.”

What happens next is both ugly and peaceful. There is no way to delegate the act of easing someone out. All the chart orders in the world can’t capture the weight of the medications that will be slowly dripped into the vein. In the modern era, I am still not free to be the agent of someone’s demise. It’s not in me to do the deed anyway, and it would not matter one way or the other if the next ballot question on physician-assisted suicide passes or is defeated. What Louise and her husband had decided, since she was entitled to her autonomy and was fully aware of the consequences, and according to my psychiatry colleagues she was not depressed, was that she would have the tracheostomy tube removed. That was her right. In order to prevent the horrific air hunger that would result, I would give her small, then increasing doses of morphine before and after the tube came out.

Louise Nagle’s diaphragm still functioned, but not enough to keep her alive. The morphine would make her groggy, but it would also make her breathing easier. I wanted to get her through a window where she would be awake enough to speak, but wouldn’t suffer. If I managed to take her to there and then removed the breathing tube, nothing would obstruct the flow of air through her larynx. She could utter a few words while her breath held out. She could say her final words to her husband.

I hadn’t raised this point before, hadn’t forewarned her, and by the look in her eyes I could tell that she was shocked. She hadn’t prepared a speech, but then, how could anyone prepare such a speech? “You have a few breaths left,” I might have said, “one more breath with which to say what you have to say before lapsing into unconsciousness
forever.” Too melodramatic, perhaps not fair, but the time had come to remove the tube.

I stood by the bed, my sport coat slung over the bedside table. Louise’s husband sat in a chair on the other side of the bed with his hands resting on the sheets, clasped prayer style, his forehead almost touching the mattress in the triangle of his arms. I couldn’t see whether his eyes were open or closed. The sheets were messy, creased, a bit sweaty. A smoky light from the ward penetrated the room and lent it an air of gloom. I could hear the people in the hall, their murmurs, their squeaky soles, their dinner trays, all in discord with the finality of death. Henry Nagle, immobilized, became part of the furniture.

Louise looked up at me, unblinking, as I nodded the go-ahead, then she blinked her consent and closed her eyes. I dialed up the morphine to make her sleepy, and I waited. Her eyelids fluttered.

“Are you awake, Louise?”

She stirred and nodded.

“Are you okay? Are you getting enough air?”

Another nod.

“Okay, what I’m going to do is take the tube out, and you may be able to speak, to whisper a few sentences. But I’m not going to allow you to become uncomfortable. If it looks like you’re breathing too hard or laboring, I’m going to turn it up, and you might not have that opportunity.”

She was arousable, sleepy but arousable. The ventilator indicated that she was pulling a trace of air as I disconnected it from the tube and cut the cuff reservoir. I could hear a stirring of air moving over her vocal cords. I suctioned around the tube with a catheter so that she wouldn’t drown in her own secretions, and then slowly pulled the tube out.

You can tell when someone is about to die. Their vessels constrict as blood pressure starts to fall, and the kneecaps get a little bluish.
Louise made a few furtive coughs, almost like huffing. Then I heard a noise, a hiss, and she whispered, “I . . . I . . . love you. Good-bye.”

Henry hadn’t moved.

When I got back to the office, I said to my secretary, “Get hold of Felicity Kalomiris and set up a visit with her and George. Please.”

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.