Reaching Down the Rabbit Hole (2 page)

“Yeah, he’s better right now, on steroids. This is way better than he’s been in a long time now.”

I turn to him. “Can you tell us a joke, Dennis?”

“Hopefully not,” he replies with little affect. Dennis seems indifferent even when he grins. His head is shaved, and his cranial stubble reveals the dark shadow of a receding hairline. He has a tattoo on his left forearm, in Gothic text, that reads:
SICK
.

“There’s enough rattling around in there so that your thinking seems really good.”

“Yeah.”

“What’s missing? What’s not right in your head?”

Dennis pauses, inhales deeply through his nostrils, thinks about it, and lets it out slowly, but says nothing.

“You have trouble expressing yourself in language, or expressing yourself in emotion?”

He takes his time. “I think it’s in emotion.”

To the girlfriend again: “I understood from the residents that
there’s a conversation going on about hospice. How did you get to that? Was he becoming unmanageable at home?”

“Over the last few weeks, yes, he was.”

“On account of what?”

“Behavior. He wouldn’t listen to me. He kept getting up and opening the fridge, wanting to take his medicine. Then just being a little bit agitated. But as far as stopping treatment, we talked to the oncologist last night. The chemo affected him very badly. The doctor said that there aren’t any drugs left that won’t make his life really terrible. So we’re talking about quality of life at this point, because the tumor is so large, and it’s inoperable.”

I turn to Dennis: “Are you engaged in these conversations?”

“Not that one, but we did talk about treatment a bunch yesterday with Dr. Nadgir.”

“Before,” she says, “he wasn’t on a high dose of steroids, he wasn’t clear at all. But now, on the steroids, this is a complete turnaround.”

“Are your plans staying the same?” I ask her.

“I’m thinking maybe we can bring him home and have hospice come in.”

“Can you behave yourself if you go home?” I say to Dennis.

He grins and shakes his head in a “no” gesture.

“No? So this is some combination of the tumor and his character. I mean, you
are
a character, Dennis, right?”

His grin springs from memory more than feeling. He knows that it’s only a matter of months. The irony is that the location of the tumor has neutralized the part of his brain that would allow him to care. So he
was
a character, but not so much anymore.

What Dennis, his girlfriend, and everybody else on the ward need, more than anything else, is to tell us their stories. Many of them have driven for an hour, two, even three hours to the Hub of the Universe (as Boston styles itself), and they want to be heard. What they hope, what they expect, what they deserve, is that we take the time to listen, because the act of listening is therapeutic in itself. When we do it
right, we learn details that make us better doctors for the next patient. The residents may not get this yet. They are focused on diagnosis and treatment, on technology, on scales, titers, doses, ratios, elevations, and deficiencies. All well and good, I tell them, but don’t forget to listen.

Hannah and the team are huddled around a computer monitor in a cubbyhole near the nurses’ station on the tenth floor. They have made it through medical school and earned their MDs, chosen to go into neurology, and this is now finishing school for them, a prestigious appointment at a top-tier hospital. My role is to look over their shoulders, provide them with someone to model, give them a hard time (or, pedagogically speaking, challenge their assumptions).

As they play at the computer, a patient sits twenty feet away, just beyond a glass door and curtains. He was admitted three hours ago with the sudden onset of a speech difficulty and a complete personality change. The team hasn’t visited him yet. Instead, they are transfixed by the images of his brain up on the screen, like passengers glued to the in-flight movie as they fly over the Grand Canyon at sunrise. It reminds me of an old joke: Two grandmothers meet on the boardwalk, one pushing a baby carriage. “That’s a beautiful granddaughter,” the friend says, and the other replies, “This is nothing, you should see her pictures.”

“Anything?” I ask.

“I don’t think it’s a stroke,” Hannah says. “Maybe there’s a low-grade glioma in the right frontal lobe, a little bit of hypodensity.”

“Would you put a dime on anything now?” I ask her.

“Tumor.”

“What do you think?” I ask Rakesh, a second-year resident.

“I think it could be a combination of tumor and postictal changes,” he replies. “How about you?”

“Me?” I say. “I’ll be glad to hold the money for you.” What I want
is for them to step away from the monitor and into the room, sit by the bed, talk to the patient, and examine the man instead of his pixels.

“Here’s a small side bet,” I add. “I don’t think the enhanced MRI is going to help us. Look, you’ve got the whole package here—the scans and the tests—and you’re still not sure what’s going on. That’s why neurology is the queen of the medical specialties.”

“Not the king?”

“No, it’s the queen because the queen is elegant, and it is the last place in medicine where your personal synthetic intellectual effort is value added, and despite all these gee-whiz gizmos, there really are no tests. You’re going to have to sort this one out at the bedside.”

Hannah has heard this speech before, and has a pretty good idea what it means. It means that if a patient comes in and can’t speak clearly or zones out mentally or can’t feel emotion, there are no standard tests. The symptom has to be reframed in terms of brain function. The only way to accomplish that reframing is through the elegant choreography of the neurological exam, not with scans, but through the painstaking examination of the patient. Every gesture, every movement, every inflection of speech, every reflex, all of these point to the precise location of the problem in the nervous system, and to its cause. The physician’s art is to synthesize the symptoms and signs in a larger framework of the patient and the structure of the nervous system. The tests are merely confirmatory, all the way from carpal tunnel syndrome to brain death.

But for now, the team is still learning about the brain, if not the entire nervous system, observing up close the ways it can malfunction. In time they will figure out how to interact with the person inside the brain, with the brain inside the person. They will find their comfort level with the families—how to give good news, how to give bad news. Someday, they will be the ones who have to tell a patient that nothing will ever be the same.

“You have a brain tumor.”

“You have motor neuron disease.”

“You have Parkinson’s.”

“You’ve just had an irreversible stroke.”

What does it mean to be a doctor to these patients? More importantly, what does it mean to be the patient faced with these seismic problems, and how is a connection made with the physician who embodies the knowledge that can make it better?

This book is about the process and progress of my craft. Like clinical neurology itself, it proceeds on a case-by-case basis. The stories are real, the dialogue mostly verbatim, and although the identities of almost all the patients are disguised, the challenges and issues facing the neurologist in a teaching hospital are on full display.

Where does it begin? It begins with a bowling alley manager who gets confused, with a right fielder who starts spouting gibberish, with an undergraduate who suddenly becomes psychotic, with a salesman who drives around and around a traffic rotary, unable to get off, with a college quarterback who can’t stop calling the same play, with a psychiatric social worker who notices a tremor in her pinkie finger, with an ex-athlete who cannot grasp the pull tabs on his newborn daughter’s diaper, with an Irishman who slips on the ice and cracks his head. They wind up here, on the inpatient ward or in the neuro-intensive care unit, passing through in a parade that never ceases to challenge, astound, and enlighten me.

Where does it end? It doesn’t.

Dr. Vandermeer, one of the last survivors of the generation that trained me, sits up on 10 West with a brain tumor the size of a lemon. Gary the programmer is having electrical leads affixed to his head on 10 East. On 10 North, Dennis of the butterfly glioma, along with his girlfriend, have decided to put off hospice for a few more weeks, and they are preparing to go home. Speed rounds have ended, and I am in the elevator, bearing coffee, on my way to 10 South, where Hannah is waiting, patient list in hand. When she sees me, she grabs the hospital ID on the lanyard around her neck, sweeps it over a wall-mounted
detector, and the doors to the neuro-ward, like all hospital doors, swing open in counterpoint. As we walk through, she exchanges the patient list for the coffee cup I hold out to her, and I ask the question I have asked countless times:

“So what have we got?”

1

Six Improbable Things Before Breakfast

Arrivals, departures, and delays on the ward

On the third of July, a day after a routine colonoscopy, Vincent Talma was playing right field in a company softball game. A short, intense man with thick gray hair and a perpetual scowl, he did not look as though he was having fun, or even capable of having any fun. Whenever a teammate said something amusing or cracked a joke, Vincent would laugh without smiling, as if to say, “funny, funny,
ha, ha
.” When he disagreed with a call by the umpire, he would throw up his arms in disbelief, kick the dirt, and swear under his breath, not for show or for the approbation of his teammates, but out of real anger and disgust. No one called him Vinnie, few called him Vince, and when he stood at the plate, none of his teammates dared to cheer him on by name.

As the game wound into the late innings, Vincent’s behavior began to change, subtly at first, then dramatically. By the time he was dropped off at his house, his wife was startled to see a bemused look on his face, an air of innocence in place of his usual gruffness. He gave one-word answers to her questions, avoided eye contact, and seemed quite unlike himself. He was smiling too much.

“Are you okay?” she asked.

“Sure, fine,” he replied.

“Did you win?”

“Fine, yes.”

“Did something happen?”

“Fine.”

The more she persisted, the more Vincent perseverated.

“Did anything happen at the game?”

“Fine, yeah fine,” he mumbled with a sheepish smile.

She called their primary care physician, who told her to get him to the emergency room immediately.

“Vincent, we need to go,” she said.

“Fine. Okay.” Still smiling.

At East Shore Hospital an MRI showed an ambiguous blotch on the left frontal lobe of Vincent’s brain, and at the suggestion of one of his sons, a pediatrician, the family requested a transfer to us. He arrived sometime around 10:00 that morning and was brought up to the ward.

A week earlier, Cindy Song, a sophomore at Boston College, had started acting a bit withdrawn. Her roommate was concerned enough to call Cindy’s sister. The first phone call was not too worrisome. “Not a big deal,” the sister said. “She gets that way. Just give her time. She’ll be okay.” The next call could not be taken so lightly.

By morning, Cindy wouldn’t leave her room, and would not or could not tell her roommate why. Alternately anxious and distracted, uncharacteristically morose and sullen, she spent the day in bed. That evening she refused to eat, and her roommate made the second call, this one to Cindy’s mother, a first-generation Korean immigrant. Despite the language difficulty, there was no mistaking the concern in the roommate’s voice. Cindy’s mother took the next commuter train from Framingham, exited at Yawkey Station, took the Green Line out to Chestnut Hill, walked up the steep hill from the terminal, past the
Gothic spire of Gasson Hall, and down the long, winding road to the dorms. When she got to Cindy’s room and sat down in front of her daughter, all she got back was a blank stare focused on the wall behind her. Her daughter’s eyes were wide open and her pupils dilated. She was shivering mildly and sweating all over. Finally, she spoke.

“Mom, they’ve been after me for weeks, creeping in through the cinderblocks, taking my clothes off.”

“What are you talking about, honey?”

“My clothes, my clothes,” she said desperately, “can’t you see them?”

Like all universities, Boston College has a health center that provides minimal services overnight, on holidays, on weekends, and during the summer, relying on referrals to local emergency rooms for anything serious. The after-hours nurse, who was used to such things, assumed that Cindy had been using recreational drugs and was “just flipping out.” Nothing unusual as far as the nurse was concerned, but Cindy’s mother was outraged. Convinced simply from cultural experience that there were no drugs involved, she would not let that stand. Cindy was so jittery and sweaty that the nurse gave in and called an ambulance to take her to the Brookline Hospital emergency room. Once there, Cindy remained agitated, stopped responding to questions, and started thrashing, as though reacting to hallucinated visions. This prompted a round of phone calls to the eight local psychiatric hospitals to see if there was a bed for an acutely psychotic young woman. Such beds are hard to come by, and it took a hard sell by the emergency room doctor to secure the promise of one by the next afternoon, “if you could just hang onto her and give her Haldol in the meantime.”

As daylight broke, Cindy was close to berserk. Her arms had to be restrained with straps, and she was soaking up tranquilizers like a sponge. Finally, the ambulance came to bring her to the psychiatric hospital. After a short interlude of relative calm, the psych nurses became alarmed when Cindy’s jitteriness escalated into full-blown myoclonus—arms and legs flinging up off the bed, her head jerking back violently. Her pupils were huge. If it had been a drug overdose,
they realized, this would have abated by now. Instead, the hallucinations continued, and Cindy was excessively restless and sweaty. I got a call at about 9:30 a.m.

“Is she salivating like she has rabies?” I asked the psychiatry resident.

“Yes, like a dog,” was the reply.

“You’d better send her over.”

By the time Vincent Talma and Cindy Song had settled in at the Brigham, Arwen Cleary had been there for four days. She came by ambulance on the morning of July 1, and was admitted to neurological intensive care from the Emergency Department later that evening. Of the three cases, hers was the least clear-cut, the most troubling, and one that had the potential to become an absolute shambles. According to her medical records, her problems had begun two years earlier, when she showed up at a central Massachusetts hospital with disabling nausea, difficulty walking, and vomiting.

Arwen Cleary had been a professional figure skater as a teenager, had retired from the Ice Capades upon its dissolution in 1995, had then raised three children, gotten divorced, and moved with her two younger children to a ranch house in Leominster, a distant suburb, where she worked part-time at a local health club. Her medical history was unremarkable: once a smoker, she had quit ten years earlier. Her travels had taken her no place more exotic than Bermuda and no more distant than Orlando. Her only hospitalizations to that point had been in maternity wards. She was remarkably fit and in seemingly good cardiovascular health, if judged only by her appearance and vital signs. But shortly after a visit to a chiropractor, she had suffered a vertebral artery dissection, a form of stroke.

Chiropractic neck adjustments are not a common cause of stroke (maybe one in every twenty thousand treatments produces one), but the high rotary force involved, one with just the right vector and amplitude, can strip off the inner layer of a blood vessel, causing it to
tear and collapse into the channel, impeding the flow of arterial blood to the brain. At her local hospital, Ms. Cleary was started on a blood thinner, and after a long inpatient and rehab stay, she recovered her motor skills and balance, and was sent home.

All went well for two years, until she returned to the hospital with sudden right facial drooping and difficulty finding words, sure signs of another stroke, but this time a stroke of a very different kind. A portion of one of the language centers of her brain had been deprived of its blood supply. Her speech was now noticeably impaired. Within a few days, she showed signs of improvement, and was again discharged on a blood thinner.

Ten weeks later, to her infinite frustration, it happened yet again, and she arrived at the same hospital in the middle of the night with another language problem, this time even more pronounced, as well as right arm weakness. The scans now showed that several other blood vessels had been stopped up, causing a scattering of new strokes. At that point her doctors became even more worried. Why would this be happening in someone so young? But they could locate neither a cause nor a source. They subjected her to exhaustive tests, the usual suspects for stroke were rounded up, an echocardiogram was ordered, and she was given a portable heart monitor. Everything came back normal. It was decided that the previous chiropractic stroke (the dissection) was unrelated to her current problem. Among the staff, the consensus was: “We’re going to need a bigger boat.” So they sent her to us.

There is an old joke among stand-up comics that goes: “Dying is easy, comedy is hard.” If we were as inner-directed as comedians, we neurologists might say, “Trauma is easy, neurology is hard.” Every one of our patients has, in effect, fallen into a hole, and it’s our job is to get them out again.

In
Alice’s Adventures in Wonderland
, Alice jumps into a rabbit
hole and finds herself in a bizarre realm in which nothing is what it seems, where everything bears little relation to the outside world. It is a place where, as the Red Queen mentions to Alice, it helps to believe six impossible things before breakfast. Unlike the Queen, I have no need to believe six impossible things before breakfast because I know that, on any given day, I will be confronted by at least six improbable things before lunch: a smiling man whose speech difficulties seemed to have been brought on by a colonoscopy, a thrashing young woman whose psychosis seemed to come out of nowhere, a figure skater with a slow-fuse time bomb in her body that was knocking off her faculties one by one. The first of these, I should note, was indeed impossible, and I didn’t believe it for a second, but the next two were quite possible, and by the end of the morning, I would encounter at least three more improbabilities: a woman who could only be cured by a hole in the head, a case of amnesia brought on by sex, and a man who was adamant that I was two very different doctors.

We treat people with seemingly implausible ailments all of the time. Each day they show up in a predictable parade of signs, symptoms, and diseases: an embolus, a glioma, a hydrocephalus; a bleed, a seizure, a hemiplegia. That’s how the residents refer to the cases, as in: “Let’s go see the basilar thrombosis on 10 East.” When viewed in terms of actual patients, however, no day is quite like any other. After the bedside visit, the thrombosis suddenly has a name, the glioma has a wife and children, the hydrocephalus writes a column for a well-known business journal. Our coed suffering from psychosis turned out to be a Rhodes Scholarship candidate, the case of multiple strokes became a charming woman who had competed in the Junior Olympics, and the man for whom a smile was a troubling symptom owned a personal empire of six Verizon wireless stores.

“Good morning, Mr. Talma,” Hannah said, “do you remember me?”

“Yes, good, good, fine,” Vincent replied. He was sitting up in bed, watching television with a smile of bemused innocence. Vincent Talma
was a picture of contentment. His room on the tenth floor of the hospital tower commanded an outstanding view of Fort Hill Park in Boston’s Roxbury section, but Vincent took no notice. Along with twenty-nine of our other patients, he had been waiting for a visit from the neurology team on their morning speed rounds.

Hannah was in charge. Her service, the culmination of three years as a neurological resident, had started a week before I came on board. A “service” involves running the neurology inpatient ward, admitting and discharging the patients, and directing a team consisting of three junior residents, two medical students, and a physician’s assistant—a cohort that could barely squeeze into Vincent’s curtained-off half of the room.

My colleagues and I had some doubts about Hannah when she first came to the program three years earlier. The most superficial of these doubts focused on her style of dress. In a profession where sartorial flair is an unexpected and somewhat suspect concept, Hannah’s clogs, leggings, and wraps seemed needlessly exotic, and sowed uneasiness among the Dockers, Skechers, and scrubs crowd. Perhaps even more alienating was the fact that Hannah did not drive a car, and instead rode her bike from her apartment in Boston’s North End to the Brigham, usually well before the sun rose or long after it had set, in any kind of weather short of a blizzard. Such stoicism flew in the face of the unhealthy lifestyle adopted by most of the residents and teaching faculty, who tend to favor pastries over granola, Coke over water, and elevators over stairs.