Reaching Down the Rabbit Hole (23 page)

“No problem,” they said. In the end, I presented the thalamotomy to Michael as the best option, even though at the time it had become passé among U.S. neurosurgeons.

“This is a good idea,” I told him, “and it doesn’t close the door on anything else. That’s the consensus. I think you’ve got almost nothing to lose.”

“Let’s do it,” he said.

But where? Several surgeons in Boston were doing functional neurosurgery for Parkinson’s disease, but few were doing surgery for tremor. The bigger problem was getting a highly visible patient out of the public eye. I resorted to an old friend, Dr. Bruce Cook, who had helped me on some tough cases. Bruce had admitting privileges at a smaller hospital north of Boston that was affiliated with ours. The hospital had recently installed a $10 million, state-of-the-art neurosurgery suite.

The scene conjured up Dr. Frankenstein’s lab more than an ultra-modern operating theater, not because of the hospital, but because of the novelty of the surgery itself. It was intensely computer-aided. Using anatomical landmarks on an MRI, Bruce guided a test electrode through a tiny plastic tube down into Michael’s right thalamus. He
warmed it up a few degrees, and the tremor, once violent enough at times to knock a glass off the table, stopped in its tracks. That was the sign that the location was correct, that the procedure would work.

There were five of us in the room: Fox, fully conscious and responsive the entire time, Bruce Cook, a surgical assistant, a nurse, and me. Nothing dramatic occurred until the end of the fourth hour, when Bruce said, “Okay, Michael, create a tremor in your left arm for me.”

“I’m trying,” he said.

“Keep trying.”

With evident frustration, Michael said, “I can’t.”

Bruce abruptly stepped back, whipped off his gloves, and said, “We’re done.” The tremor was gone. The next day Michael walked out with a sweatshirt over his head and went back to New York, a new man.

The criticism was predictable, and not long in coming. Journalists who can’t get close to celebrity patients do the next best thing: they pigeonhole a talking head into Monday morning quarterbacking a case of which they have no firsthand knowledge.

“Some in the medical profession are questioning why celebrities who could afford the best of everything would select a small community hospital that sits twenty-five miles up the road from some of the best teaching hospitals in the country.” Thus began the carping in one local paper.

“I found it a little odd,” a local neurosurgeon was quoted as saying.

“My initial response?” said another, “I found it a little disconcerting.” He publicly wondered “whether the thalamotomy is the best procedure for the actor, since he is so young and his disease will likely progress and include other symptoms not alleviated by that particular surgery.”

My answer? Yes, it was the best procedure, and Michael agrees.

“That thalamotomy,” he recently told me, “I don’t think for a second that that thalamotomy was a mistake. It was fantastic. And people say to me, ‘Why don’t you want to have a DBS?’ and I say because on
my best day without it, I’m ten times better than the people who I see who have had the surgery. They may have less tremor and less dyskinesias, but they have this kind of slowness and this whisper and this fragility. So I’m fine, my thalamotomy was great. It couldn’t alter progression on my right side, but it never was intended to.”

Back in my office, I have to explain to Tikvah that she is not a candidate for DBS, or even a thalamotomy. No one does thalamotomies or pallidotomies anymore, but that’s beside the point. She can’t get a stimulator.

“One of the contraindications,” I tell her, “is that you can’t have any cognitive difficulty or hallucinations that are not solely attributable to Sinemet. Now, if you have hallucinations that abate when you reduce the dose, you’re still a candidate. But in your case, because of the confusion and visions, you are not. There are too many bad outcomes with confused people.”

Tikvah’s hallucinations are partly Sinemet-related, are mainly nocturnal, and are a common feature of diseases related to Parkinson’s, such as Lewy-body dementia. She has enough cognitive difficulty that she has begun to struggle with executive organization, sequencing, and planning. That’s what keeps knocking her down day after day, that’s why she has a whiteboard in her kitchen that details her daily routine, that’s why she cannot get the surgery. There is a high likelihood that the surgery would bring on the kind of mental slowness that Michael alluded to. It’s not her memory problem that is so worrisome, rather her inability to organize.

“Who are you?” I ask her. “How do you see yourself?”

She replies, “I’m a wife, I’m a mother, I’m an observer of mankind. But what I am—just like you—you’re a doctor, and I’m a psychiatric social worker who’s seen a lot of whitewater go under the bridge. I’ve seen people out on the ledge, I’ve seen people die, I’ve seen people not wanting to die. That’s who I am.”

Though she was forced to retire after thirty years as a therapist, she still views herself and others through the lens of her profession.

“I’m in two support groups,” she reminds me, “one for PD and the other for children of Holocaust survivors. Both are about survival. The woman who brought me into the PD group was a remarkable woman, an artist, very successful, very strong, very well known in Manhattan, a force to be reckoned with. She could go to a store on the Upper East Side, and come back with a new member for the group. She didn’t survive. She killed herself. I have two insights on that, one professional and one personal. It’s an awful thing either way. She had a rough time of it, more so than the rest of us. Some people might say maybe they were going to kill themselves, and then not do it. But she was a determined soul, she looked it up online, she measured the drugs out, and there was suicide in her family, so it wasn’t a foreign concept.”

“Was she very disabled?” I ask.

“Yes. She was the most disabled person in the group, although we didn’t notice that because she was so competent and so talented and so friendly.”

“From your professional experience, do you think she got profoundly depressed, or was it a conscious effort to find a way out?”

“A conscious effort, yes. She was very angry, very irate at the disease, what it had done to her, how badly her family had responded. She was an artist and did really good work. All they did was criticize her. That’s how she viewed it. The group as a whole got very upset with her family, which I think ruined it for everyone. Then one day she was gone.” She goes silent for a moment, then resumes.

“Personally, in my bones, I’ve increasingly felt, partly from my parents’ experiences, partly from my upbringing in Israel, that the Holocaust is very much a part of how I interact with this disease. Maybe it’s a less explicit analogy for others with PD. The sense of imprisonment, the sense that you’re not going to get out of it, that the disease is in charge. You can do certain things, you can get along, go out to a movie, but in the end, the disease will take over and you will
be tired, and it will wear you down. The suffering, the not knowing what you will end up with. No relief. Who’s going to take care of you? Who’s going to want to take care of you when you are in that deformed condition? We talk about this in my support group. Thank God for the group.”

Lenny K sent Michael J. Fox to me most likely because he looks at me as the last Mohican. He knew I would take a detailed history and try to tailor a treatment to Fox’s needs, and stick with him. That’s my value added. There are people who may think I’m the world’s expert on Guillain-Barré syndrome, people who may think I’m the world’s expert on concussion and head trauma, and people who may think I’m the world’s expert on Parkinson’s disease, mainly because I’m an expert in none of those, but I’m a good doctor for all of them. Michael didn’t need the world’s leading Parkinson’s expert. That guy is required only for a case that no one else can figure out. Everybody else out on the street with Parkinson’s simply needs a good neurologist. Michael just needed someone good enough, and he got me.

Michael starred in a new show that premiered in the fall of 2013, twenty-two years after his initial diagnosis. His foundation has raised a quarter of a billion dollars for Parkinson’s research. He is no longer my patient, but he checks in from time to time.

“I took up golf in my forties, with Parkinson’s,” he recently told me, “so that’s optimistic beyond anything I could ever do. What I’ve had a good time with is watching able-bodied golfers agonize over missed shots and putts, and they get so angry, and they attach shame to it, and they say, ‘I’m sorry, I’m so sorry I missed that,’ and I laugh and shank it into the woods, and I say, ‘What’s my handicap? Isn’t it obvious? It’s not a number, it’s what I deal with.’ And I feel so bad for these people who attach shame and attach failure to a game. It’s their life, but I refuse to do it, I refuse to apologize for my Parkinson’s. If I manipulate it or cover it up, it’s not out of shame, but it’s about being able to do what I do to the best of my ability, and accomplish what I
need to accomplish. And I recognize what it has cost me, I know the loss.”

“What do you mean? What does it cost you?”

“I can’t run, I had to stop training for the marathon, I couldn’t do that and other things, so I applied it to writing a book. In a large way, you were responsible for me looking at it empirically, and looking at it in the nonemotional way that I really needed to do, to separate all of the feelings of fear and all of the feelings of concern and shame and guilt, the idea that I must have done something to deserve this, and then: ‘What am I putting my family through?’ You helped me put all of that stuff aside, so I could ask myself, ‘What is it I am dealing with? What is happening in my brain? Why is it happening? Can I stop it from happening? Can I, now that it’s out there, exercise my privileges in the wider world to move forward toward a cure.’ All of that came from our meeting.”

Tikvah is still visited regularly by Dick Tracy and Jimmie Walker, and her form of the disease is different enough from Michael’s that I can hold out only the slimmest hope of a surgical solution. After our appointment ends, she finds her way back to the reception area, and I drop by the conference room to hear a visiting speaker, Mike Okun, from the University of Florida, who has come to the Brigham to give a talk about deep brain stimulation. His concluding statement strikes a familiar chord: “After twenty years of DBS, it is time to stop thinking about which [surgical] target is best, and ask which symptom the patient needs fixed. We have to move from disease treatment to symptom treatment.”

The remark takes me back to the future, back to my recommendation for Michael’s surgery: treat the symptom, not the disease; treat the patient, not the disease. That’s the mantra for Parkinson’s patients—patch them up and send them back out there, and be waiting when they come back at the end of the next round.

That’s me in the corner, still waiting for my Ferrari.

11

For the Want of a Nail

A hard-knock lesson on the way to the morgue

I was in the kitchen preparing a snack when my cell phone rang. It was the radiology department. “Your patient, Mr. Connaway, has a spinal epidural abscess,” the neuroradiologist said. “It’s massive.”

This confirmed my worst fear. Connaway was going to be a quadriplegic at best (the correct term is
tetraplegic
), or dead within days. And the thought lodged in my brain:
Had I killed him?
But he wasn’t dead yet. Maybe there was still time to save him.

From my house it is about a twenty-minute drive to the hospital in afternoon traffic. I didn’t have twenty minutes, so I sailed through two stop signs and one red light before I caught a break: an idling Boston patrol car on Commonwealth Avenue. I pulled alongside, rolled down the window and said, “Look, I’m a doctor at the Brigham. I know this is unusual, but I have to get to the hospital NOW. Like ten minutes ago.” And he said, “No sweat, man. Follow me.” With the siren wailing, we zipped through the evening rush hour traffic, defying red lights, down a back way I didn’t even know existed. Less than ten minutes later I abandoned my car at valet parking, ran up to radiology,
took a look at the MRI, then ran up to the ICU. Harry Connaway and his wife were there, just as I had left them two hours before. Harry was in terrible shape, had been ill served by a dozen practitioners, including me, and had little hope for a recovery, or even survival. But the existence of a chance, however slight, meant that I had to do everything I could for him.

Four days earlier, Connaway had been playing tennis. He was pretty good at it, and played regularly. He lived in an expensive house in an exclusive suburb. Recently retired, he was reaping the rewards of four decades of very stressful work as an investment banker. He was conscientious about his health, took a daily cholesterol drug, and flew through his annual medical exams. He was understandably baffled when a progressive numbness took hold in his legs, and then spread to his arms and hands over the course of a couple of days. His wife called 911, and Harry was brought to the emergency room at a local community hospital.

His most visible symptoms—generalized weakness of the arms and legs that, by the time he arrived at the hospital, made it impossible for him to stand, accompanied by loss of reflexes—pointed to a problem in the peripheral nervous system, most likely the result of Guillain-Barré syndrome, a treatable form of paralysis due to an inflammation of the nerves. It can cause a rapidly progressive weakness, and the disease is right in my wheelhouse: serious but manageable if caught in time. Joseph Heller had it, as did Andy Griffith. Both men recovered.

Harry Connaway’s legs were weak, but not paralyzed. His bladder wasn’t quite right, but it was functioning. Having settled on this diagnosis as the most probable one, his doctors were preparing to treat him when he suddenly became very unstable. His blood pressure and heart rate began to bounce between dangerously low and absurdly high. On day three, when he lost bladder function, they threw in the towel. “This is beyond us,” they said. “Let’s send him to a bigger place.”

I got the call from Harry’s physician early that evening, and said, “Sure, send him over. It sounds like it could be Guillain-Barré. We’ll treat him tomorrow morning.” I saw no reason to do it in the middle of the night. It wasn’t justified. A few hours delay in starting the IV drip would not make a difference.

So Harry Connaway was wheeled through our doors at 11:45 p.m. on the third day of his crisis. Stanley, the first-year resident who was handling admissions, called me at home to say that he had just examined a sixty-eight-year-old man who had lost his reflexes. He said nothing about sensory levels, a standard neurological exam finding that tests for numbness along the spine. A specific transition point from normal sensation to numbness is a characteristic indicator for spinal cord problems. Stanley did mention a fever and high white count, but I downplayed those facts in my mind.

“Keep him supported,” I said. “We’ll see him in the morning.”

I can’t blame Stanley. He got almost every detail right. The problem was my rigid thinking. I should have asked for more detail, but I was stuck on a snap diagnosis, and because I did not ask, the rest of the night passed with no action taken.

Harry Connaway’s fortunes started spiraling downward upon his arrival at his local hospital, where the radiologist missed something on his MRI. In his defense, it wasn’t something obvious. Harry had a rare condition. Still, for want of the correct reading, three days were lost. Upon his arrival at the Brigham, that mistake was compounded by me, by my failure to ask the right questions immediately, leading to more errors of omission. In Harry’s case, I latched onto an explanation that happens to be a specialty of mine: Guillain-Barré syndrome. As it turned out, my patient would have been better served by a fox than by a hedgehog.

The interdependency that is built into a modern hospital, our reliance on checklists, hierarchies, consultations, and each other, protects us individually from taking all of the blame when things go wrong. We can and do make mistakes, but in theory someone is there
to catch and correct them. In practice, the system of checks and balances can fail at every level, and a low probability event, a succession of unlikely failures, can be set into motion. In the case of Harry Connaway, a cascade of errors of omission began to fall like dominoes.

I saw Connaway the morning after his admission to the Brigham at around 6:30 a.m. I was serving as the attending in the neuro-ICU. My first impression, based on the man and his chart, was that Harry was the equivalent of me in another profession: hardworking, committed, active, with a wide circle of friends. At some point the similarities ended. Even in his weakened state, crammed into a hospital bed, I could tell that Harry Connaway was a tall, strapping man, the kind who projects physical vitality. His face had the confident and commanding aspect of a hawk. I still didn’t know about his sensory level. Because we were making speed rounds, we weren’t yet collecting the kind of detailed data that we normally do on the ICU. Too many things were going on at once. Our paramount goal was to check on all of the patients in the unit just to make sure they would live through the next few hours. When the patient count rises, as it had on that morning, rounding can get disorganized. It would take us three hours to get back to Harry for a more thorough exam. We were assuming his condition was urgent, but not critical. I was still working with a diagnosis of Guillain-Barré.

Stanley, who had admitted Harry, was the only one of us who had examined him carefully. He was the member of the team who knew him best. At 9:30 that morning he had to sign out because he had reached his mandated eighty-hour limit for the week. That was unfortunate. The one person who embodied most of the detail in Harry’s case was leaving. At the end of speed rounds, he gave the team his handoff and went home. Distilled in that handoff were Connaway’s records from the community hospital. These included an MRI of his lower back, recorded as normal and dutifully passed on as such to us. I have no idea why they had ordered a lumbar MRI at all. A scan
can occasionally be helpful in confirming a diagnosis of Guillain-Barré, but it is not a standard test. When Joelle, the senior ICU resident, looked up the scan in the patient notes, she repeated to me that the scan was normal. I didn’t know whether she had seen the film itself or was merely reading the summary, and I didn’t ask. Although she was nominally in charge, I said, “Let me examine the guy, I know a lot about Guillain-Barré, and I’m always interested to see variations.” Already I was misleading myself.

By the dictates of the scientific method, we are obliged to seek evidence to disprove our assumptions rather than verify them. If you have a theory, you are not supposed to fixate only on the evidence supporting it. Admittedly, you will rarely see Sherlock do this on
Masterpiece Mystery!
The Holmesian thrill lies in instantly seizing on the right explanation from the barest facts. What Holmes should be doing, and what Dr. Watson usually suggests, is exploring other possible explanations. Instead of assuming that he’s always right, Watson might say, “Just this once, consider the possibility that you might be wrong.” Without realizing it, I, too, had thrown the scientific method out the window.

I noted that Harry could barely move his face, and assumed it meant that his symptoms couldn’t just be a problem at the neck or in the spinal cord. Also, his limb tone was gone. He was floppy and had no reflexes, both of which are highly characteristic of Guillain-Barré. Third, he did not have Babinski signs, a toe reflex which is probably the most typical feature of compression of the spinal cord. So I said, “Okay, that all fits together. It’s GBS. Let’s get the electrical nerve conduction tests and see how bad it is.” I told Joelle that I wanted it done that afternoon. An alternate theory, the one that I was overlooking, was that some of the examination findings could have been caused by the medications Connaway had been given. They could easily have wiped out his facial tone and muscle tone completely.

At midday, a team showed up to run the electrical nerve tests. The technologist called me an hour later and said, “It wasn’t easy to do
because he was in the unit, and there was a lot of electrical noise and people coming and going, but the bottom line is he has no excitable nerves. When I tried to stimulate a nerve in his limb, I didn’t get any response. He probably has really bad Guillain-Barré because the nerves are shot.”

It all seemed to fit. The technologist was merely echoing my own thoughts: a case of
axonal
Guillain-Barré, in which the nerve fibers (or axons) are wiped out by an immune reaction. The senior EMG physician called me with the same conclusion.

Deductive reasoning, as opposed to inductive reasoning, is what detectives and diagnosticians should be doing. Deduction works from general facts toward specific conclusions. If the “facts” really are facts, the conclusions have to be true. But induction—a quicker and much more practical method of reasoning that everyone uses every day—can lead to errors. That’s because an inductive process infers a conclusion, but doesn’t prove it. If the nerve conduction tests are normal, for example, the patient cannot have Guillain-Barré. But the inverse of that statement—that an abnormal test (like Harry’s)
must
be Guillain-Barré—does not logically follow. My initial error was excessive dependence on induction due to time pressures, as well as hubris. In practice, neither Sherlock Holmes nor I have the luxury of time to indulge in the true deductive method, and when the clock is ticking we have to rely on intuition. But sometimes intuition can let you down.

When Harry’s wife showed up early that afternoon, I finally got a chance to hear a firsthand account of the onset of the paralysis. The next fifteen minutes represented the height of clinical skill: a mental juggling act that involved listening with an informed ear while classifying everything relevant, filtering out everything irrelevant, and creating a hypothesis to test against the examination findings. The time had come to apply the synthesizing, value-added process that would bring me back to the scientific method. The challenge was that my source of information was an increasingly and justifiably frantic
spouse who was picking up on the urgency of the situation through my facial expressions.

“Well, you know,” she said, “he was doing alright, then he had some trouble walking, then he felt feverish and just terrible, and he couldn’t urinate. Then he suddenly got a very bad pain between his shoulder blades, and it got worse and worse over the day. Then the pain went away.”

The acute pain, while not typical, could possibly fit with Guillain-Barré. I know because I had written the paper on it. It’s called the
coup de poignard
, or stroke of the dagger. But the fever and bladder issue didn’t fit. That was highly suspicious. At this point it occurred to me that something wasn’t right.

“Let’s start over,” I said to Joelle.

“Why?”

“Something’s off here. In fact, let’s look at those films.” It was now 3:00 in the afternoon. The other residents on the team demurred, saying, “But they were normal.”

“Have you seen them?” I asked.

“No.”

“Well then let’s have a look, because if the nerve roots are enhancing, that’s got to be GBS, and we could maybe put this to bed.”

On Harry’s MRI everything looked completely normal, if not pristine. The nerve roots were enhancing a bit. But at the very top edge of the scan, I noticed a small white ball, a mere eighth of an inch in diameter, which seemed to be pressing on the bottom of the spinal cord. (Most people don’t understand that the spinal cord doesn’t go all the way down the spinal column, but stops in the upper back.)

I said, “What’s that?”

Joelle replied, “It’s just an artifact,” by which she meant a by-product of the imaging process, like a dead bug in the lens.

“I don’t think so. That looks real. I think it’s an epidural abscess.”

Only now, at 3:30, did it occur to us that Harry Connaway’s problem might be far more dire than we originally thought. I immediately
took the MRI down to one of our radiologists, and said, “I want you to find your chief and reread this. I’m not going to tell you what’s there. Just reread it and call me.”