Reaching Down the Rabbit Hole (20 page)

“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 in dependently?” 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.”