Another Day in the Frontal Lobe (12 page)

Other surgeons argue this makes no sense and is a waste of time. Those surgeons aim only to “debulk” the tumor, with the simple goal of relieving any pressure and allowing some room in case the brain swells from the radiation. They acknowledge that a glioblastoma is not a “surgical disease” in that it can’t be cured with an operation, and the extra effort to get as much out as possible doesn’t confer a significant enough benefit (a few extra weeks, maybe?) over the less aggressive approach.

Some neurosurgeons dedicate their careers to these tumors, looking for alternative strategies to outwit them. Their work has led to some creative options, such as thin wafers of chemotherapy that can be left along the edges of the brain where the tumor was removed. Logic might dictate that this should greatly prolong the time to recurrence, but the results have been less than stellar. Clearly, a breakthrough solution will have to be radically different from the options we have now, and it’s probably not going to involve surgery.

The most difficult decision is faced when the tumor comes back in full force after all treatments have already been exhausted. Do you make a second trip back to the OR? What about a third?

A close family member will often be the first to realize that a tumor has grown back. One woman explained to me how she knew it was time to bring her husband back into our clinic. We had operated on his brain tumor months earlier and he had been functioning relatively well. He was the host of a weekly polka hour on a local radio station. His wife always tuned in. One day in particular, the pauses between songs were too long and he even played the same song twice. She knew it was time, and she was right.

When neurosurgery residents get together in a pack, out of earshot of anyone else, the talk is often blunt. (A common saying such as “this guy is toast” seems unimaginably callous on the page, but it’s said as an acknowledgment of the often harsh nature of reality, as in “life sucks” or “s—t happens.” It’s not meant to demean the individual in any way.) When the discussion turns philosophical, which it can on rare occasions, we wonder, for example, what
we
would do if handed the diagnosis of an imminently fatal brain tumor. Here is a sampling of the types of sentiments you might hear bounced around among the jaded:

“I’d be outta here, man. You wouldn’t see me going through radiation, chemo, more surgery. I wouldn’t prolong things; in and out of the hospital for my last few months; none of that crap. Give me a one-way ticket to Tahiti.”

“I’d quit my job, go out with a bunch of beautiful women, sell my house, and blow through the rest of my cash. You gotta live it up while you can.”

Then there’s the more tempered, slightly less cynical, approach: “I don’t know, I’d probably give radiation and chemo a try, but when it comes back, forget it. Look at Mr. So-and-so…three operations…that’s nuts. We should have closed him with a zipper last time.”

Although the words are irreverent, bold, and definitive, I wonder if the primitive, hardwired survival instinct would kick in and erase all thoughts of the classic “one-way ticket to Tahiti.” Would we keep coming back for more, like so many of our patients, or would we remain true to our strategy of hedonistic nihilism? Would we really be different from anyone else, just because we’ve seen it all before, over and over again?

I met a patient with a glioblastoma who went against our usual expectations. He didn’t use the word “fighter” or the phrase “do everything you can.” The oncology fellow paged me at midnight on a Sunday. The patient had arrived at our hospital that afternoon from a small town a few hours away. His brother drove him. He was advised by his oncologist back home that he should consider undergoing chemotherapy at the university hospital. His glioblastoma was diagnosed three weeks earlier. It was multicentric—somewhat unusual—meaning that you could see more than one focus of tumor on his MRI.

At an outlying hospital, he had already had surgery to have the right-sided tumor debulked. The smaller tumor, on the left, was even more of a problem because it had a large cyst associated with it, near an area involved in speech. The patient had surgery on that side, as well, to have the cyst drained.

According to the patient’s brother, he was originally reluctant to undergo surgery once the needle biopsy had confirmed the diagnosis. He eventually consented to a craniotomy, but decided that once was definitely enough. He knew that some people with a glioblastoma consent to have their head reopened once the tumor reappears, but that would not be for him. He understood that the tumor was incurable and preferred not to prolong the inevitable. His family was in agreement. The problem was, by the time I met the patient, he was not conscious enough to share this opinion. The brother spoke for him.

A scan at our hospital showed that the patient’s cyst had reaccumulated a large amount of fluid from the surrounding tumor since the time it was drained, and must have reached a critical volume just before his admission that night, causing enough pressure to impair consciousness. During the car ride over, the patient’s brother did note that he was a bit sleepy, but chalked it up to poor rest the night before. Within hours of his arrival, he had become sleepier to the point of obtundation: he only opened his eyes if you inflicted pain, he moaned but didn’t speak, and he wouldn’t follow any simple commands.

I sat down with the brother. Was the patient firm in not wanting any further surgery? Yes. What if we could improve his condition, at least temporarily, by redraining the cyst? Well, that would be surgery and he wouldn’t want it. Did he write down any of his wishes in any formal way? No, unfortunately. The brother told me more about the patient. From the way he talked, I could tell that they weren’t just brothers, but were good friends as well.

I learned that the patient was fifty, a biologist who specialized in fish, and was single. He loved the outdoors. He was a triathlete. It pained him to have his life slowly and progressively restricted by his tumor. It had started to affect his vision and strength. Because the tumor on the left was in an area critical for speech, he was experiencing worsening word-finding difficulties.

I explained to his brother that surgery to redrain the cyst would be straightforward, especially given the fact that there was a recent opening in the skull that we could work through. It would probably only take an hour or two, at most. He should wake up rapidly after the fluid was drained. I explained that his impaired consciousness was due to an increase in pressure from the fluid. If we could just drain the cyst, we could relieve the pressure and have him wake up. Otherwise, the end was very near.

The brother kept focusing on the longer term, though. He already understood the facts and wouldn’t let me sugarcoat the situation. He had me acknowledge that the cyst would probably just reaccumulate again, despite repeat surgery, and that both tumors would continue to infiltrate the surrounding brain, even if slowed down by whatever new protocol was being tried at the time.

There was no convincing the brother to have the patient go for more surgery. He knew that the cyst was hastening death and he was okay with it. He knew his brother would accept this. At first, I was reluctant to let nature take its course, but my surgical tendencies softened after getting a clearer picture of this biologist’s view of his own life. I called my attending and told him the story. He agreed to abide by the patient’s and brother’s wishes. We were dealing with an incurable tumor, after all. I went back to speak with the brother, left a long note in the chart, called the oncology fellow, and went to bed.

Early the next morning I received a page from the attending oncologist, yelling on the other end, irate as hell. “How could you just ignore this guy…the cyst? He’s about to die! We were planning to start chemotherapy. You guys are going to take him to the OR. This is ridiculous!” Before calling to chastise me, the oncologist had phoned my attending and persuaded him to take the patient to the operating room. Neither had said more than a few words to the brother, who had slept all night by the patient’s bedside. They gave him no choice.

I decided not to participate in his operation, a conscientious objector. Technically, all went well, the pressure was relieved, and the patient woke right up, with a plastic tube and bag hanging from his scalp to collect ongoing drainage from the cyst.

A few hours later, after the patient was sent from the recovery room to his hospital room, I was paged about him again. This time it was my own attending, the neurosurgeon who performed the operation. He requested that I stop by the patient’s room to remove the drain and send him home. It turns out that after the effects of anesthesia wore off and the patient realized what had happened to him, he wanted out. He was upset that he had undergone another operation and was shocked to feel the plastic tube coming out of his head. He wanted the drain removed and wanted to go home. He had even decided against chemotherapy.

I found him in his room sitting up in a chair. His brother was standing and looking out the window. The patient was already dressed in his jeans and flannel shirt and was ready to go. His duffel bag was packed, by the door. The surgical catheter and drainage bag looked out of place—otherworldly—hanging from the scalp of this rugged outdoorsman. I was surprised he hadn’t tried to remove it himself. I introduced myself and told him that I already felt like I knew him. He, of course, hadn’t remembered meeting me at all.

In my brief interaction with this patient in his awake state, I sensed a strong resolve and a healthy realism rather than depression. I removed the drain and cinched down a single stitch around the exit site to prevent any fluid from leaking from his scalp. He pointed to the IV in his arm and asked whether or not I would mind taking it out, too. This is usually the nurse’s job, but what the heck. He was in a hurry. The IV tubing had been secured to his hairy forearm by a large square sheet of clear sticky tape. “Just rip it off,” he told me. “Better not to prolong the ordeal, if you know what I mean.”

I once gave a talk to a group of undergraduate students who were learning about the brain. My role was to give a neurosurgical perspective. I wanted to have them do some reading beforehand, so I chose an interesting chapter out of a book written by a neurosurgeon about a patient of his with a glioblastoma (
Judith’s Pavilion
by Marc Flitter). I thought it was well written and provocative. I was hoping to stir up debate about the brain and neurosurgical interventions.

After introducing myself to the class I asked for their comments on the reading. The hand of a young cynic shot up. His comment went something like: “I don’t know why anyone would want to have a career like that. I wouldn’t choose it in a million years. Too depressing!” There was no argument, no debate, from the rest of the class. I hadn’t anticipated quite so strong a visceral reaction. It made me realize that I had become almost too accustomed to death and devastation. It’s a part of the job, and you can’t cry over every patient. But is there a price to pay in getting “used to it”?

I have to admit that I sometimes wish I had become something like a handbag designer or a cookbook author (maybe not those exact things, but something along those lines). Those careers wouldn’t involve the handing out of fatal diagnoses. I could enjoy a job with a little less gravity to it. My day-to-day would be more carefree, more creative, less serious.

I’ve even had other surgeons from different specialties divulge to me that they had, as medical students, strongly considered going into neurosurgery because of the brain and the neat technical aspects. “Too depressing” is almost always the reason they give for having decided against it.

So how can I save myself here? How can I justify tolerating this seemingly depressing job? (I am not a pure altruist and I don’t think anyone is. The desire to “help humanity” can certainly go far, and that is still a primary reason, but there’s got to be something in it for the helper.) Believe it or not, I have experienced at least one personal upside to seeing so much go so wrong. It sounds a bit hackneyed, but I have to admit that I have developed a distinct appreciation for everyday life. For many people, this requires some sort of personal near-death experience. You hear about these revelations all the time, about how someone didn’t appreciate their life until they almost lost it.

I’ve been fortunate enough to borrow from everyone else’s experience. I’ve seen people in every state of neurological decline and I’ve seen death, over and over again. And this makes me feel lucky about life, every day.

As I think about this, I have to admit that my appreciation for the everyday has become a well-entrenched part of me now. I probably don’t need the constant reinforcement.

ELEVEN

Disturbing Deviations

I remember when I discovered that the lungs are actually beautiful, as internal organs can be, if you look at them with the right eye. I had seen real lungs before, the lungs of my cadaver in medical school, but those lungs were not the least bit attractive. They were inert, preserved, and cold. They were the lungs of a dead person who—I presumed—had probably been a smoker. I wasn’t impressed.

Then, later in medical school, I scrubbed in on the open-heart case of a young girl with a heart defect. She was an otherwise perfectly normal girl except for this small defect and, luckily, a straightforward operation would be able to fix it. Although this was not my first time as witness to the human heart in action—I caught a glimpse of a routine bypass procedure when my dad took me on a tour of the ORs in his hospital once—it was the first time I was able to get a good look at living, breathing, human lungs. You might assume that the heart would upstage the lungs, but I’d say they’re equally impressive.

The girl’s lungs were pristine, pink, and glistening, and I could see them inflate in sync with the mechanical breaths of the ventilator. I was amazed. I could appreciate these lungs just as easily and as naturally as anyone can appreciate the skin of a baby. It’s difficult to accurately describe the organ’s look and delicate, airy, crepe-like feel. There is no other tissue or material in nature even remotely similar.

Midway through the case I had a sickening thought: What if this girl becomes a smoker? I imagined her, years later, as a college student with a cigarette, deliberately inhaling all the ingredients necessary for coaxing DNA into cancerous mutations, inhaling them directly into those thousands of fresh alveolar air sacs. I would be as devastated as her parents, maybe even more. If only the chest cavity were transparent, the beauty of the lungs would be exposed to everyone, and young women might be compelled to treat them as well as they treat their skin.

I think people enjoy their careers more if they find something that they work with to be visually appealing, like mathematicians who work with fractals or marine biologists who study jellyfish. Part of what I like about neurosurgery may seem superficial: I like the way the brain looks. The brain is, by far, the most complex and interesting organ of the human body (take note, guys, if you thought otherwise) and a feast for those who appreciate architectural detail. (It’s not a gray blob by any means, if you take a good look. Actually, it’s not even gray.) Unlike my revelation about the lungs, though, I can’t remember when I first began to appreciate the brain. The brain is appealing not only in real life, but also as a line drawing in textbooks and as an image on scans—so much complexity packaged so neatly in a relatively small space.

Knowing what the normal brain should look like, it’s also quite easy to have a visceral reaction to one that doesn’t look quite right. When I see a brain that deviates significantly from the norm, it can be jarring and even disturbing, despite the fact that I’ve developed a healthy degree of clinical detachment that I can deploy as needed. This is especially true when the brain is the brain of a child. In a fair world, all children would be born with beautiful, architecturally complete brains. But, given the randomness and indifference of nature, sometimes a brain just doesn’t develop all the way, and we’re left in the uncomfortable position of having to decide what to do.

The most abnormal brain I’ve ever seen belonged to an infant whose external appearance was frighteningly normal. His cute baby look was what made for such a sticky situation: the parents, relatives, and nurses saw a cuddly little boy who sucked on a bottle, cooed, kicked his legs, and even smiled. A basic and somewhat crude underlying fact, though, is that any infant’s sucking, cooing, kicking, and smiling functions (or reflexes) require only the most rudimentary, nonthinking, parts of the human brain, the parts that are roughly similar to those found in much less sophisticated creatures, like reptiles. The overwhelming problem with this infant was that those primitive reptilian parts were the only parts that he had. The more highly evolved regions of the brain—the large cerebral hemispheres—the parts that allow us to develop conscious thought, experience emotion, and communicate with each other (in short, the parts that make us human), were completely absent. A cold, clinical, and involuntary question flashed through my mind on seeing this baby’s scan: Is he really “human”? I kept the question to myself.

This condition, hydrancephaly, is rare. (And not to be confused with the more common hydrocephalus, or “water on the brain,” which is easily treated and often compatible with a normal or nearly normal life.) Hearty survival is not expected in hydrancephaly, but it’s hard to predict duration of survival, especially as we’ve become so expert at anticipating and intervening in the earliest hints of bodily breakdown, staving off what would otherwise be an early death. An infant who would promptly expire in less wealthy societies can be exquisitely maintained in ours by advanced technology, an abundant supply of skilled enthusiastic doctors, nurses, and therapists, and exacting parents. Such high-tech medical care is—normally—considered to be one of the key advantages of a wealthy and civilized society.

When I walked into the infant’s hospital room for the first time, the lights were off and the shades down, in the middle of the day. The pediatric neurology team was crowded around the crib. The senior neurologist was holding a flashlight up against the baby’s head, and all the residents and medical students stared at the resulting spectacle: a round, pinkish, glowing orb. Because most of the skull was filled with fluid and not brain, and a baby’s scalp and skull are normally relatively thin, the light was able to pass right through, lighting up the head in an eerie display. This “transillumination” diagnostic technique is a very old and simple one, completely antiquated and unnecessary in this age of high-quality CT and MRI scans. Nonetheless, most doctors can’t resist the temptation to try something that they’ve only read about in textbooks, and most doctors will never have the opportunity to examine an infant with hydrancephaly (especially given the ubiquity of prenatal ultrasound), so I probably would have placed the flashlight against the infant’s head, too, if I hadn’t happened to have caught the spectacle secondhand.

Here’s why I was summoned to see this baby. His head was starting to grow faster than it should, out of proportion to the growth of his body. The normal cerebrospinal fluid created by the brain (the fluid filling up almost the entire intracranial cavity in this case) was not being absorbed properly. This was leading to an excessive accumulation of fluid. Because an infant’s skull is somewhat malleable, such an unchecked accumulation leads to enlargement of the head. If left untreated, the head can become enormous, the child’s skinny neck unable to lift it or move it, eventually leading to a downward spiral of pressure sores, full-thickness scalp breakdown, infection, sepsis, and death.

Scalp breakdown, of course, could be staved off for a while by keeping the child in the hospital, having the nurses adhere to a strict regimen of changing the head position at frequent and regular intervals, and by using creative padding techniques, similar to the fastidious care required to keep quadriplegic patients healthy and alive. Or, such care could be undertaken at home by hiring around-the-clock nurses. However, if the family were of more modest means, the task would probably be left to a nonworking parent, predictably the mother, who would then be at risk for mental breakdown within her confining and demanding world.

Another option, seemingly more humane, would be to implant a special shunting device into the baby’s head so that fluid could be diverted directly from inside the head, through a tube tunneled underneath the skin of the neck and chest, all the way to the belly (a ventriculoperitoneal shunt). This would halt the excessive head growth and prevent scalp breakdown.

What to do? On the one hand, how can you allow this baby’s head to continue growing larger and larger? On the other hand, how can you justify performing an invasive operation on a child with no future and only rudimentary brain structures? Is the goal really to prolong survival, to stretch out the emotional turmoil, for as long as possible? The child will certainly never be able to thank you for it, as he has no prospects for conscious awareness. The parents might thank you, but would their gratitude be misguided? And, because we have to think this way, too, is this a sensible way to allocate health care dollars?

During a neurosurgical residency, when a situation is so disturbing as to defy normal conversation, black humor is a natural, although admittedly juvenile, substitute. It’s not directed at the patient or family (we’re not cruel, really) but more at the awkward situation. The darkest of our black humor, while it would horrify any eavesdropper, is sure to lighten the situation and allow us to keep working, especially when we’re overly exhausted and ready to snap. So, for example: What kind of want ad could this poor kid respond to when he grows up? “Looking for an individual who can reliably suck, cry, and kick his legs. Ability to smile also desirable.” This type of banter, tossed around privately on rounds, is just another—albeit pathetic—way of saying, “Why the heck are we doing this?”

Strange, but true to the frenetic existence of a resident, I never found out what happened to this cute-on-the-outside little baby. I was involved in the initial consult but not the final decision. My four-month block on pediatric neurosurgery ended soon afterward and I was back to taking care of adults with architecturally complete brains who, among other things, decided against wearing a seat belt. In retrospect, I should have inquired as to what happened, but there were too many other pressing concerns, too many other strange cases to fill the day and night, and a constant stream of ever-renewing black humor to sweep me along, to keep me going.

During my time on the pediatric neurosurgery service of a busy academic center, I saw a wide spectrum of abnormal brain development. I also became facile with the complex names ascribed to each unfortunate anomaly. Many of the names have an unpleasant sound to them. I always felt awful for the parents who not only had to come to terms with the reality of their child’s brain, but also had to learn, and repeat, the medical terms that went along with it: schizencephaly, pachygyria, holoprosencephaly, tuberous sclerosis (with “tubers” in the brain, for God’s sake). There are so many names, a name for every conceivable anomaly or combination, many of which we may never see, that I had to create a large stack of flash cards in order to remember them all, at least temporarily, for my board exams. There is even a textbook of malformations that has been referred to—again, in our most juvenile but harmless way—as the “Little Shop of Horrors.”

As residents, we would quiz each other with our homemade flash cards before our board exams. These were questions you’ll never see on
Jeopardy!
(Resident #1, holding the card: “Port wine stain…seizures…railroad track calcifications…mental retardation…c’mon.” Resident #2, with the answer: “Uhh…Sturge-Weber syndrome! Yeah!”) For certain disorders, I didn’t need a flash card because I had met at least one patient who exemplified all the features, and that was sufficient to fix it in my memory.

For example, a father brought his baby in, somewhat reluctantly, for consultation with one of the pediatric neurosurgeons. I sat in on the consultation. The infant had Apert syndrome, a congenital condition that includes multiple craniofacial abnormalities as well as syndactyly (fusion of the fingers), which are often surgically corrected, to the best of a surgical team’s ability, when the baby is old enough and large enough to tolerate surgery and the resulting blood loss. In this condition, the sutures, or natural fissures, between different bones of the skull (such as between the frontal bones and parietal bones) fuse too early during growth of the head, leading to a misshapen cranium such as one that is abnormally broad or with an abnormally bulging forehead. Because of smaller than average orbits, the eyeballs appear to bulge. In addition, the “midface” from the bottom of the eyes to the upper jaw appears sunken in.

The father admitted that he and his wife had been shocked by their child’s appearance at first, but then, over the ensuing weeks and months, had gotten quite used to it. They loved him, of course, as they would love any baby of theirs. Now, in fact, they had become so accustomed to his features that they felt he looked perfectly acceptable. They hated the thought of putting him through surgery. “He looks okay to me!” the father told the neurosurgeon, and then asked him, “What do you think?”

The next minute that passed was about as silent and as uncomfortable as a minute could be. The neurosurgeon had been rendered temporarily speechless, trying to figure out how to, at once, impart both honesty and compassion in his answer to an unexpectedly thorny question. I felt for everyone in the room—the father, the child, and the surgeon, in that order. After much discussion, the father was eventually convinced of the logic behind surgery, not only for significant cosmetic and social reasons, but also to allow for more room in the skull, so as to accommodate further brain growth. And with that, the father gave in.