Reaching Down the Rabbit Hole (17 page)

Anyone diagnosed with ALS has a series of gut-wrenching decisions to make. After the shock has worn off, once the capacity for rational
thought returns, many small choices and a few large ones come along in relatively rapid succession. When will I need a wheelchair? Will I accept a feeding tube? Will I go on a ventilator machine?

The pace of the disease’s progression varies from person to person, but the end result does not: eventual loss of all motor function with the exception of eye movement and blinking, and possibly some lip movement. Because the tongue can go relatively early, speech does too. The fasciculating muscles begin to atrophy. The ability to swallow is soon compromised. The disease is most certainly fatal if left to run its course. Yet it leaves the brain and the sensory system intact. The patient can feel everything and move almost nothing, eventually coming an eyelash away from being completely locked in. The ultimate question facing the ALS patient is whether to be or not to be. Quite simply, will I do whatever it takes to stay alive, in what ever condition, or will I let the disease take over? In practice, the question is much more direct: Are you willing to have a tracheostomy tube inserted and be placed on a ventilator? If so, you will no longer be able to talk, but you will be able to breathe. The majority of patients opt not to do this. Initially, Louise Nagle made it very clear: no ventilator, no feeding tube. But the decisions we make in relative calm and those we make under duress are not always the same.

In the fall of 2002, George Kalomiris had everything to look forward to: a lovely wife, a new daughter (just three months old), a new house in Nahant with commanding views of Revere Beach and the Boston skyline, a budding career in state government. George himself had been living a dream. A basketball star in high school, he had been recruited by several Division I colleges, but chose to go to Tufts for the academics, to star on the basketball team, and to stay close to his Greek immigrant parents as their dutiful only child. After graduation, he played ball professionally in Greece for seven years. A dominating
power forward at six feet four inches, he played “above the rim.” Upon his return he entered local politics, and eventually landed a job as the Director of Economic Development for the Commonwealth of Massachusetts. After marrying and building his dream house, he was poised to reap the rewards of years of hard work.

George and Felicity Kalomiris are not quite sure when the first signs of ALS appeared. George thinks it might have been in early 2000 when he went quail hunting with some friends in Rhode Island, and found his shotgun to be unusually heavy. Or it might have been when he was changing the baby and had trouble with the pull tabs on the disposable diapers. At the time, Felicity thought he was just trying to shirk his duty.

He can recall the twitches, but everyone has twitches, and these seemed to correspond to a recent return to the weight room after a long hiatus. Nothing unusual there: just work through it, he thought. But two months later he sprained his thumb merely by knocking it against a table. It wouldn’t heal. When a doctor finally looked at it, he noticed atrophy in the surrounding muscle tissue. Only then did George notice it himself: hollows and indentations where there had once been pads of muscle. That’s when he came to see me.

George Kalomiris considers himself lucky. He is not in denial. He has good reasons to think so, and these become clearer when you meet him. He is still alive a decade after his ALS diagnosis. His body may be confined to a wheelchair, but his mind is not. George had planned wisely. He had signed up for long-term disability insurance when Felicity became pregnant. He had gone to a financial adviser who gave him the kind of advice that is given to all new parents, but unlike most new parents, George took it. With his own insurance plus Medicare, he could afford to pay a rotating team of at-home caregivers. His disease, compared to most (Louise Nagle’s, for instance) was of the slow-progressing variety. But none of those things mattered. They did not figure into his decision to live as long as he possibly
could, in any condition that allowed him to watch his daughter grow up, that allowed him to deepen his relationship with his wife, that allowed him to sustain the friendship of the people he loved.

“There really was no decision,” Felicity explained. “There was never any doubt or thought of how we would pay for it or how we would manage.”

Whenever a new case of ALS comes along, I think of George and wonder how he’s doing. It had been five years since our last visit, but I knew he was still active, if only on the Internet. I didn’t know how his home life was going, but I decided to find out, partly for selfish reasons. We all succumb to something eventually, but few of us get to decide when to cede control of our lives. George hadn’t ceded yet, and probably never would.

Fast-forward four months. After a long interlude of relative stability and calm, Louise Nagle arrived in our emergency room in the very same straits that had previously sent her to the local hospital. This time she was breathing at about twenty-nine times a minute rather than eight, she was sweaty all over, and could barely get three words out without gulping for air. This was the real thing. Her diaphragm, one of the largest muscles in the body, was running out of gas.

At her previous office visit we had discussed, but only in general terms, what measures she would want us to take if her breathing worsened and began to fail. She and her husband had previously decided that she would not go on a breathing machine or have a tube stuck down her throat. They had discussed this at my suggestion, and now came back with their final decision. It was not the one I expected, although I should have.

“I just . . . I just . . . don’t want to die now.”

“Louise, you look extremely uncomfortable,” I said, “and I would like to put a breathing tube in because you’re working so hard that you’ll soon fatigue and be unable to breathe. You need to understand
that you will then be on a breathing machine, and it may be difficult to get you off it. And you won’t be able to talk because of the tube in your throat.”

“Okay. Okay. Do it quick.”

She was now angry and agitated, unable to find a comfortable position in the bed with the sheets twisted up around her. After a quick blast of the midazolam to sedate her, the tube went in easily. By the time she woke up from the drug, the ventilator was working for her and she was wide-eyed and comfortable, relieved, gripping my hand.

“Is this okay? Is this what you want for the moment?” She gave me a quick but definite nod.

Like other patients who have ALS, Louise Nagle had full command of her faculties, if not a kind of hyperawareness. Crises always focus the mind, so much so in her case that she had completely rethought the breathing tube, decided that in fact it was now quite okay because it was not an irrevocable decision. It was okay because it would afford her the opportunity to assess her situation in calmer and controlled circumstances, so that she would not be forced to make the big decisions in the midst of a crisis, or even in a theoretical framework that afforded her only the idea of dying, and not a taste of what it really felt like. I told her several times that she had control over her destiny, that we would not coerce her into a course of action or inaction, but would support her in any direction she decided to take. I was very specific that at any time she wished we could take the tube out and sedate her so that her air hunger was suppressed. I was clear that this would mean imminent death while under deep sedation. I did not back away from offering to guide her, and as our conversations progressed, I told her what other patients had done. I told her about George, my lone holdout. And whether my personal judgment of him vacillated between bravery or selfishness, I had to concede this much, that his determination gave me a perspective on the other choice an ALS patient can make, the choice that few do make, or can even bear to make—to be rather than not to be.

George never had a moment’s hesitation, and it was not in him to rescind his decision. But he was very clear that he did not judge those who chose the other path. He had come to know many such people, had befriended them, and had helped them with their choice. Louise Nagle was now facing that decision. Not immediately, but soon. All ALS sufferers face a kind of Hobson’s choice. They will die. The only question is when and how. How much suffering are they willing to bear? How much incapacity? How much of a burden are they willing to become?

A few days on the breathing tube stretched into two weeks, sufficient time for the presence of the tube to soften the rings of Louise’s trachea, creating even more problems. The tube would have to be replaced by a tracheostomy that, while more comfortable, would still prevent her from speaking—no air would pass through her vocal cords and mouth—and it would commit her to a respirator day and night for the foreseeable future. The alternative was to take the breathing tube out under controlled circumstances while she was sedated, and allow her to die. It was time for another conversation with the whole family.

“You understand, Louise, the tracheostomy is another step toward prolonged support using a ventilator machine?”

A positive nod in response.

“It involves an operation on the front of your neck that is brief. You will be under anesthesia. Just as with the tracheal tube, this step is not irreversible. My goal is to make sure you’re comfortable both physically and psychologically at every point.”

Her husband’s only question was: “It won’t be done by a student, will it?”

“It will be done by a surgical resident under the supervision of a staff member, and that will be fine.”

“So, someone who has barely done it before?”

“Not exactly,” I said, with intentional ambiguity. The procedure is routine enough that it rarely requires a senior surgeon.

The resident inserted the tracheostomy the next morning. By week’s end we were able to get Louise into a nearby chronic care respiratory hospital so that I could stop by periodically, and it would be a short ride to our emergency room if there was an acute problem. Things seemed calm enough when I went to visit her. She was virtually motionless in bed, but could mouth some words and signal with her eyes. I brought in a large clear Plexiglas board that had the alphabet and a few short words written on it. I showed Louise how I could see what letter or word she was looking at from the other side. Communication became a silent telegraph of terse answers projected through Plexiglas.

“Louise, are you comfortable?”

She scanned to “N” on the board. Now began the laborious process of determining the source of that “no.” This was not the time to walk away and ask the nurses to figure it out.

“Are you in pain?” Again, her eyes diverted to N.

“Are you getting enough air?”

To N again.

Air hunger is one of the most uncomfortable symptoms known to man or woman. Struggling to breathe, the awareness of breathing, contemplating the difficulty of each breath—something that a healthy, resting person never has to think about—is the feeling of dying. It probably contributes to the imminent sense of death (
angor animi
) of a panic attack.

Louise had had this experience before, out of anxiety. This time it was caused by the shift from a breathing tube in her mouth to the new tracheostomy in her neck. Given the small diameter of the new tube, it must have felt like breathing through a straw. I took a quick look at the control dashboard of the ventilator. The rate and volume looked good, but a small alarm on the panel intermittently blinked
LOW EXHALED VOLUME
. This is one of the most vexing problems in ventilator management, because it means that air is being lost somewhere between the time it is pushed into the lungs and when it comes back out.
Most often the loss occurs on the way in, either into the chest or somewhere around the cuff that holds the tube in place in her trachea.

I grabbed the tiny bag of the air reservoir that hangs down from the tracheostomy tube like a piece of spaghetti. It was too easily compressible, indicating that the cuff around the tube wasn’t inflated. Grabbing a syringe, I slammed a few extra milliliters of air into the cuff. She began to sweat all over and tremble a bit. I saw that her heart rate had gone up from around 92 bpm to 135 bpm. She was struggling. Her eyes widened and her pupils dilated. There were beads of sweat on her forehead. The additional air I injected had not expanded the cuff. I grabbed a compressible Ambu bag, disconnected the ventilator, and put the coupler of the bag right onto her tracheostomy. By squeezing the black bag slowly and firmly, I could move enough air in and out to expand her lungs at a comfortable pace. Her heart rate settled down below 100, and her eyes no longer looked as if they were going to pop out of their sockets. I heard the French horn rumble of air escaping upward through her trachea and around the leaky cuff.