Reaching Down the Rabbit Hole (5 page)

“She shouldn’t be here on the ward, the ICU would be better,” I said. “She has massive hydrocephalus. She’s not going to able to walk soon.”

“So we essentially cut right to the chase?”

“Right. There’s no time to make this diagnosis. She needs a shunt ASAP.” A shunt is an internal one-way drainage valve that relieves and controls the internal pressure of cerebrospinal fluid.

“Neurosurg needed a place to put her while they figured this out,” Callie said.

“Well I can figure it out for them: send her back to the ICU, have them do a temporary, external shunt, get some cerebrospinal fluid to test, get her walking, make a diagnosis, and
then
do a permanent shunt. We need to prevent her from getting demented and being wheelchair-bound. I know this is meta-level stuff, but we should keep our eye on the ball here.”

I once had a professor who used to say you should never joke with your patients, and you should never return their jocularities. He said it was a bad, bad mistake. I have never followed that advice, although it might be a good rule to apply with the residents. In hindsight, I should have stayed on message. Instead, I asked if Mrs. G had any unusual exposures. “She has a Greek name. Does she work any place special? The last case of this I saw was a guard at the Egyptian room at the Museum of Fine Arts. He got Nocardia from the mummy.” Nocardia is a bacterium, typically found in soil that, if inhaled, can cause a slowly progressing pneumonia. In rare cases, it can cause an inflammation of the meninges, the brain’s protective sac, and lead to hydrocephalus. The museum is just down the street from the hospital. It was an interesting case, but didn’t shed any light on the matter at hand.

“Oh, that’s awesome,” Callie said. “Actually, I don’t know what her exposures are, her mummy exposures.”

As entertaining as this was, my little anecdote diverted the team’s attention from the very point I wanted to drive home: that this woman should be sent back to the ICU or even straight to the operating room.
We needed to keep a close eye on her to make sure that the pressure in her brain did not cause her to crash. But in a setting in which thirty cases are discussed within two hours, no patient gets more than fifteen minutes of fame, making it doubly imperative that we stick to relevant facts. Instead, I took my eye off the ball, and the rest of the team did, too.

When we finally got to see Mrs. G on rounds that morning, I was impressed by her odd combination of mental dullness accompanied by considerable head pain. The bluntness told me that her hydrocephalus was pressing on the frontal lobes from the inside, and winding down the engine that makes the nervous system spin. The pain that caused her to intermittently clutch her head and neck suggested that the pressure inside her skull was not only very high, but rising.

The skull is like a fixed container. If pressures within the skull are unevenly distributed, there is a breaking point at which the entire brain gets squeezed downward like a plunger, compressing the brain stem. This is precisely what happened when Mrs. G blew her pupil later that afternoon, resulting in the code blue. I just happened to be outside the door to her room when the problem came to a head (so to speak).

Once a code blue is called, it takes a minute or two for the team to materialize from the other pods and from the ICU a floor below. In that brief interlude the nursing staff swings into action. Three nurses rushed in to help move the patient from her recliner onto the bed. As we prepared to roll her onto a board for chest compressions, I wiped the sweat off her back so the defibrillation pads would stick, then pushed the triangular mask of the Ambu bag around her mouth and nose.

“She’s going to need mannitol,” I said, and as I came out of the door I ran into Hannah, who seemed genuinely surprised when I told her that Mrs. G had blown a pupil.

“This woman needs mannitol!” I yelled again over to the desk nurse, and Hannah went to help her get it. Mannitol, a drug administered by IV, is a sugar alcohol used to pull fluid out of the brain in
order to reduce the internal pressure. In this case it was just a stopgap. Mrs. G was going to need more than mannitol.

A code is a highly choreographed performance executed in a small space measuring approximately eight by twelve feet. Among the dozen people who rushed into the room in the next few minutes, each one had a specific part to play, much like the musicians in an ensemble. The code leader, a senior medical resident, is nominally in charge, but because Mrs. G was my patient, I took up a position at the head of the bed and “directed” the code while the senior resident “ran” it. In a sense, I appointed myself guest conductor. I had to be there to provide my perspective, because for a code team, whatever’s going on with the brain is secondary. Their primary focus is to restore respiration and circulation—keep her heart and lungs going.

A passerby could be forgiven for mistaking a code blue for an assault. A junior resident was kneeling over the bed, stiff-arming the patient’s breast bone to force blood through the heart chambers. On the third pump, I heard the audible crunch of Mrs. G’s ribs cracking, a sign that the resident was doing the chest compressions forcefully enough. That was her job. My job was to tell the code leader that the brain was not secondary in this case, that its internal pressure was pushing the brain stem down onto itself, causing the nerve cells that control breathing and heart rate to shut down, and that the only way to resuscitate her would be to reverse that vertical displacement.

At two minutes into the code, the mannitol was being infused, but I lost her pulse at both the carotid and radial arteries, and asked one of the nurses to call down to the neuro-ICU and get a neurosurgery resident with a ventricular tray right away. Hannah, who had placed the intravenous line that delivered the mannitol, would later admit to me what was going through her mind at the time: nothing. She was completely nonplussed. For weeks afterward she agonized over whether, had I not been there, she could have composed herself enough to figure out why the patient had crashed and what needed to be done about it. What Mrs. G needed, literally, was a hole in the head. She needed to
have a tube threaded into her brain through which the excess fluid could be drained from her over-pressurized ventricles. The procedure, called an EVD, or external ventricular drain, is done with a hand drill—the eggbeater—which cuts a hole in the skull, through which a straw-like tube is inserted. Both of these items were brought up on a tray by the neurosurgeon, a young Chinese American woman who stood just over five feet tall.

Meanwhile, the code leader took charge and told everyone to quiet down. As one nurse injected epinephrine intravenously, another was kneeling on the bed and pumping Mrs. G’s chest. Floor nurses swarmed like ants around poor old Mrs. Newlin, the ninety-year-old with explosive headaches who shared the room, and whisked her away to another pod. The room was filling up with people, and the floor was now littered with about six inches of trash. There were syringe covers, a dozen plastic bags, little cardboard containers underfoot, and four-by-four gauze pads everywhere. The team had administered two shocks through the defibrillator pads, and Mrs. G had briefly regained a pulse and blood pressure, but then lost them again. A third shock was administered.

While the neurosurgeon got set up, I stepped out of the room to talk to Nick, Mrs. G’s husband, ushering him to the far wall of the nurses’ station, though still in sight of the commotion. He was clearly stunned. I wanted to let him know that I thought we’d be able to bring her back. I asked him if he was doing alright, but his answer was perfunctory. A social worker came by to stay with him.

Back in the room, a surgical assistant shaved and scrubbed a large patch on the right side of Mrs. G’s scalp. Hair fell to the floor in clumps, and the area glowed orange with iodine disinfectant. The neurosurgical resident quickly donned a mask, gown, and gloves, and with hands aloft she deftly pirouetted so that I could wind and tie the paper belt on her gown. She poured another half bottle of iodine on the patient’s scalp, took out a tape measure, and began to mark Mrs. G’s head with a sterile pen. From the bridge of the nose she drew a line up the scalp,
measured five centimeters to the side, and made a quick large X. The scalpel came out of its scabbard, and a one-inch linear incision appeared from nowhere down to the skull. She then inserted a small retractor, took the eggbeater drill out of the kit, and pressed down on the back of it. As she turned the handle, bone chips started coming up the drill bit. Ten turns in, and the drill stuck.

Later, Hannah went around saying that I ran the code, but I hadn’t. I made some midcourse corrections for the medical resident, and I was cheerleading the neurosurgeon. When the drill got stuck I was about to put on a gown and gloves to give her a hand, but she persisted. Seeing a diminutive Chinese American woman lean in like a rugby player in a scrum, and push with her whole body weight against the skull of a fragile woman was quite a sight. It was getting hot, and I was sweating. The neurosurgical resident was sweating more. She braced herself for one last push and the drill broke through the cranium. I told her that she didn’t need to make a perfect trajectory with the catheter since the patient’s ventricles were huge, and she could probably hit them from almost any angle. “Don’t tunnel it under the skin,” I told her, “we don’t have time, just stick it right in.” But she was in her zone, and she dutifully dragged the ventricular tubing under the scalp as she had been taught, as if we had all the time in the world. Suddenly, a fountain of spinal fluid spurted in a jet past my left ear. I could hear it whizzing by. Everyone at the head of the bed looked incredulously at the wet splash running down the wall behind me. In less than five seconds, Mrs. G’s pulse returned, and she started breathing on her own.

From the Brigham tower, facing north, the view from the tenth floor runs down along Brookline Avenue toward Fenway Park and the famous CITGO sign less than a mile away. During the 1970s and 80s, Dwight Evans patrolled the outfield at Fenway. To most Red Sox fans, Evans was the greatest right fielder of all time, and much of that regard
is due to a play he made in Game 6 of the 1975 World Series. In Boston lore, it has come to be known simply as “The Catch.”

It was the top of the eleventh, one out, Ken Griffey on first, with Joe Morgan stepping to the plate. Evans remembers thinking through everything that might happen. “Good fielders do that,” he said later. “That’s how great plays are made.” Morgan hit a long fly ball to right, a potential disaster. As Evans recalled it, “I turned towards the line because normally a ball turns toward the line too. Well, this particular ball didn’t turn. This particular ball stayed straight, and you ask any player, when you lose a ball, that’s a scary situation. No one was more surprised than me. I jumped, and my glove went behind my head, and the ball landed in my glove. I’m glad, thank God, I caught the ball, because if not, it goes in the stands. To me it was the most important catch I’d made. It wasn’t the best catch, it was an awkward catch, but there was a reason it was so awkward, because I did lose the ball for that split second.”

After the catch, Evans’s throw to first base was twenty feet wide to the left, requiring an alert play by the shortstop and the first baseman to double up the base runner. No one remembers the errors Evans made that season, or the strikeouts. They remember the great moments. Very few people get a chance to make a catch like that.

Would Mrs. G have died had I gone home early? During any code there is a laundry list. Most codes deal with catastrophic but common medical problems like heart attacks, pulmonary embolus, and aortic rupture. The code team would have gone through the list in order to address those possibilities. The code leader would eventually have realized that it was none of the above, that it was a neurological problem. Hannah knew that. She would have connected the dots. Whether she would have had the middle-management ability to pull everything together by ordering people around and saying, “Get the neurosurgeon up here, get the mannitol in,” that would have been a test, but
she would have done it, she would have made the catch. All I did was bridge the gap, maybe speed it along.

The next morning at about 11:00, I went down to the ICU to gauge the aftermath of what I thought might be a terrible situation. Elliott was there.

“I hear you missed the game,” he said. “Too bad. They pulled it out in extra innings.”

“So did we,” I replied.

Mrs. G had developed some complications from the CPR, notably a sharp pain in her chest with every breath. She had no idea how her ribs got cracked. But there she was, sitting in an easy chair eating breakfast, with a ventricular drain coming out of her head and snaking down an IV pole to a reservoir. She was wide-awake, and I asked her if she knew who I was. She said, “No, I’ve never seen you before.”

“Well,” I replied, “I’ve seen
you
before. And it’s very nice to see you again.”

3

The State of Confusion

Two characters in search of a neurologist

Within a span of five years Gordon Steever’s life had been reduced to a half-mile radius of a bowling alley in Dorchester, Massachusetts, where, until recently, he had worked as the day manager. He lived five blocks south of the lanes, his ex-wife lived four blocks north, he could walk to the superette three blocks east or to his favorite diner just down the street, and on rare occasions, if absolutely necessary, to the Fields Corner subway station. At the age of sixty-seven, his routine kept him within a purlieus that had enclosed almost the entirety of his life. In other words, Gordon was a townie. He didn’t own a car. He lived alone in a two-room flat above a beauty salon. He still owned an analog television. He listened to the Celtics and Red Sox on a Zenith transistor radio. He went to mass only on Easter and Christmas Eve, but still avoided meat on Fridays. He rarely saw his grown children.

On the evening of May 26, Gordon was feeling increasingly agitated. Earlier in the day he had tried and failed to fill out the paperwork required to collect unemployment checks. He had been let go from his job at the bowling alley two weeks earlier, not so much for
erratic behavior, something the owner could live with, but for not showing up, which the owner would not tolerate. As his mood darkened, Gordon walked through streets that he had known all of his life, streets that suddenly seemed oddly unfamiliar. Finding himself on Gibson Street, without recognizing it for the first time in sixty years, he walked through the front door of the Boston Police Department District C-11 Headquarters, and started haranguing the desk sergeant.

“I can’t take it anymore,” he said.

“Can’t take what?”

“A lot of the kids there, a lot of the kids. A lot of people, not around here now, but not like that one.”

“What’s your name, fella?”

“Gordon.”

“Where do you live?”

“Dorchester.”

“Okay, Gordon, what’s your address?”

“I can’t remember what the address was.”

“How about
your
address, where your house is?”

“Well they kind of changed a little bit, because they’ve . . . they’re not quite as good as . . . by next week I think it’d be really perfect, people would be able to understand what these things are.”

“What things?”

“You don’t have no idea, you haven’t even thought of it, or anything else. Every person in this world, unless they have had health problems, they’re done. It’s true! You should come down here. You sit there and look. The intelligent person would be a person just sitting there and looking. Just like that.”

To the sergeant’s trained eye Gordon did not look drunk. Nor did he look sick or delirious or drugged. What he did look like was a very confused, wiry guy in his late sixties, bowlegged, with a bristly mustache, short gray hair on a balding pate covered with peeling skin. Maybe he’d been a seaman, not Navy, more likely Merchant Marine.
The guy had seen better days, and now appeared to be consumed by resentment over some perceived slight. He wasn’t threatening, but, as the sergeant later told the EMT, “He’s either got a hair up his ass or something’s really wrong with his head. Most likely both.”

After the EMTs dropped him at the nearest hospital, Gordon became paranoid and agitated, so much so that they could not get him into the MRI machine. Another ambulance then took him to the psychiatric ward of the Faulkner Hospital, where it was determined that Gordon’s problem might not be psychiatric. They wanted him checked out first by a neurologist. So Gordon got to take one more ride.

Three weeks later, on a cloudless June day just after lunchtime, Walter Maskart was driving his wife to a doctor’s appointment when he missed his exit off a traffic rotary in Braintree, and ended up heading east instead of west. Had he taken the exit MapQuest had directed him to, he would have arrived at the medical associates in plenty of time. The office building was located just off the rotary. Instead, Wally drove through East Braintree, then South Weymouth, and eventually down to Bridgewater before getting his bearings and heading back north on Route 3. He had left his house at 1:00 for a 2:00 appointment, allowing himself plenty of time. He did not arrive until 4:00 p.m., and then got it into his head that he had been driving for five hours.

The receptionist told Wally that he would have to come back the next day, but he insisted that she go explain to the doctor what had just happened, that he had lost track of things, that something was wrong with him. The doctor, an oncologist who was treating Wally’s wife for lung cancer, came out and talked briefly to Wally in order to see whether he was oriented: did he know who he was, where he was, what day it was, did he know how to get home? Satisfied that Wally was indeed “oriented times three,” he told him to return the next day. As the couple walked back to the car, Wally’s wife started to cry, not for herself, but for her husband.

At the age of seventy-eight, Wally had reached a breaking point. His wife’s chances of beating the cancer were merely fair, his daughter’s marriage was falling apart, and his business—a party supply store—had just failed. He now had to run a household, care for his wife, cook the meals, manage his stock portfolio, all while dealing with his own health issues: diabetes, hypertension, COPD, obstructive sleep apnea, and congestive heart failure. The last straw that sent him to a local hospital, not for his wife but for himself, was the confusion. After a complete medical examination revealed the respiratory and cardiac problems, it was decided that Wally would need the resources of a larger facility. In addition to his confusion, it seemed, Wally would occasionally stare into space and blink in clusters. It was thought that he might be having small seizures, so he was sent to us, and we fitted him with a twenty-four-hour EEG monitor.

Wally Maskart was placed in a room next to Gordon Steever, two doors down from Mrs. Gyftopoulos. Wally and Gordon, although in the throes of acute confusional states, were a study in contrasts. Wally engaged the world, while Gordon hid from it. Wally witnessed Mrs. Gyftopoulos’s code blue and wrote about it in his journal, while Gordon kept to his room and stared at the floor. They had a few things in common. The first, which was speculative, was that Gordon had been Wally’s daughter’s seventh-grade basketball coach. The second, which was unfortunate, was that both of their spouses were battling cancer. The third, which was definite, was that I had no idea what was wrong with either one of them. The fourth, which was obvious to even the most casual observer, was that something was very, very wrong with both of them. We couldn’t rule out the possibility that their confusion was not a neurological phenomenon, but a psychiatric one. We also couldn’t rule out the possibility that in both cases it was life-threatening.

Callie, the second-year resident with the idiosyncratic word selection, presented Wally’s workup in the conference room on the morning of Mrs. G’s code: “Mr. Maskart is a seventy-eight-year-old right-handed
man who was recently admitted for episodes of confusion. He has a history of coronary disease, the usual slew of hypertension, hypo-adrenal whatever, diabetes, obstructive sleep apnea. He’s presenting again after persistent episodes of confusion: just wacko, per the family. He’s been getting lost while driving, he’s been having episodes of what he describes as d
é
j
à
vu. His son says he seems to be a little more confused in the mornings. He seems at times to be unable to work the television remote. He’s really into toy trains . . .”

“Model railroading,” I corrected.

“. . . whatever, but it seems that recently he bought the wrong kind of train things, which his family says is just not like him.”

“What things?”

Callie looked through her notes. “A tinplate locomotive, whatever that is.”

“Continue.”

“He was admitted to medicine [the medical ward] on the twenty-fifth. He had three days of continued déjà vu, increased sleepiness, and increased confusion. Then he was readmitted and sent here. The other thing that’s complicated this is that he seems to be playing around with his medications at home.”

“What do you mean?”

“He has psoriatic arthritis and is on prednisone. He seems to be getting his meds confused. Apparently he likes to tinker with them, and he might be taking his wife’s medications. She has cancer.”

“Has he had a tox screen?”

“I truly hope so.”

The presentation took longer than most because the history was clogged with a full battery of tests. To use another of Callie’s favorite expressions: Wally had been worked up “up the wazoo.” Many of the results were still pending, and some would take weeks to come back. In the meantime, I suggested getting even more esoteric and expensive tests. As Elliott never tires of telling me: “Hey, it’s other people’s
money. Don’t start believing it’s our responsibility to reduce healthcare costs.” To which he was quick to add: “Not for quotation, by the way. I can just see
that
on the front page of the
Globe
.”

It may sound trite to say that
confusion
is the most confusing syndrome in medicine, but it is. A confused person behaves in a way so foreign to common experience that it can be unnerving, even for professionals. It is an alternate state of being. Portrayals of confusion in popular culture—the town drunk, for example—may look funny, but in the case of a truly confused person, the sight of someone who can’t find his own mind can be overwhelming.

As a neurological entity, confusion is up for grabs. Any young researcher hoping to make a name for herself might consider starting a large-scale study of it. Or not. It could easily eat up a budding career. An understanding of confusion has yet to be operationalized in the way that stroke or neuropathy or Parkinson’s disease have been. It is not, technically, a disease, but a syndrome, a collection of problems. Clinically, confusion is defined as a loss of the usual clarity, coherence, and speed of thinking, but this description, while accurate as far as it goes, captures only a snapshot of confused behavior. In some patients, blood tests will reveal a metabolic cause that can be addressed: low levels of blood sugar, for example, or high levels of carbon dioxide. But as with many neurological conditions, there are no definitive tests for confusion. We have to rely on the clinical exam, the patient’s history, the story as given by family or neighbors, and any little clues we can unearth. A tinplate locomotive, for example.

On my first visit with Wally Maskart, he seemed fairly lucid, if not completely with it. After introducing myself, I got right down to business.

“You’re a model train guy, right? HO gauge?”

“No. O gauge.”

“Good. I’ve got to come see your setup someday. I have N scale and HO. A lot of scale buildings, too.”

“So do I.”

Wally was sitting up in the hospital bed. His face was ruddy, his gray hair plastered down and tousled by the recently removed EEG leads. He was a hunched, flushed, somewhat pudgy fellow, constantly short of breath, and thus incapable of speaking with ease or completing a sentence without pausing to inhale some of the oxygen coursing through the tubes under his nose. He had the appearance of a guy who was once physically active, if not robust, and was now deflated.

“What brought you to the hospital?” Hannah asked him, and he proceeded to tell the story of getting lost for five hours.

“So that’s where I screwed up, getting all confused again,” he concluded.

“This driving for five hours, you’d never done that before?”

“No. I knew where the place was. I’d been there before.”

“So where
did
you go?” she asked.

“I can tell you exactly what happened. I looked up MapQuest. On MapQuest there’s a big circle, a rotary, and you enter the circle, and MapQuest says bear right, and then bear right again. But what I actually did is take the
second
right and
then
bear right. So I missed the building, which was right there, and got three towns away.”

“Three towns? And that’s the only time that’s happened?” Hannah asked.