Knowing when to stop

When a malignant brain tumor first sprouts in the brain, it arrives unex­pectedly and makes its presence known indirectly. A subtle feeling of weakness might arise, a dimming of peripheral vision, or just a vague sense that something is not quite right. These symptoms are often ignored, attributed to lack of sleep, stress, or maybe age. As evidence mounts, accep­tance does not always follow. Even the unequivocal image that appears on the MRI scan can be shrugged off as a possible misreading or a mistake: "Couldn't it be something else? How do you know it's a tumor?" As a brain tumor surgeon, I often find myself answering such questions up until the very last minute. The morning before surgery, I obtain a new MRI scan, which I load onto a computer and use for intraoperative navigation. I can­not tell you how often I am asked by my patients whether the tumor is still there – as if somehow, it might have magically disappeared in the interval between diagnosis and surgery. 

A few hours later, mid-operation, I am often faced with a decision: how aggressive should I be? In an ideal world, I take out the entire tumor, leaving nothing behind. Although this will not provide a cure for most brain cancers, since microscopic cells invade into the surrounding normal tissue, the more I remove, the longer the patient lives. Unfortunately, many tumors sit extremely close to parts of the brain that are critical for abilities such as movement, navigation, and speech. Others are wrapped around small nerves and blood vessels that, if damaged, can cause double vision, blindness, deafness, or an inability to swallow.

Every case is different, and the scenario changes based on a few factors. How large is the margin of safety? How quickly will the tumor grow? What are the alternatives if I leave a piece behind? Most operations last several hours. Usually, somewhere between hours three and four, just as fatigue begins to set in, the moment of truth arrives when such lingering decisions demand attention. When faced with a fragment of tumor stuck to a nerve, or one resting dangerously near an area known to move some part of the body, I must decide, right then and there, how to proceed.

The patient, on the other hand, is usually unconscious, unable to affect the outcome of this momentous decision. In one version of their future, they are whole, living as if nothing had happened, their operation but a distant memory. In another version, they are paralyzed, or deaf, or maybe blind, their normal lives now a distant memory, replaced with one beset by disabilities. Which future will unfold? It depends entirely on the experience, judgment, and technical abilities of their surgeon, which, if you are lying on my table, ends up being me. Because we brain surgeons must, at some point, balance quality of life with amount of life, sometimes the hardest part of our job is simply knowing when to stop.

When I finally emerge from the operating room and talk to the family after the operation, any fantasy of disappearing tumors also disappears. After my close encounter with the alien invader, the family's first question is usually "What did the biopsy show?" During an operation, we always send at least two pieces of the tumor to the pathologist. The first, called the frozen sec­tion, or just "the frozen" for short, is evaluated immediately and provides rapid information that we use to determine how to proceed with the rest of the surgery. Occasionally our intuition about what we are seeing with our own eyes is wrong. Sometimes a seemingly benign tumor reveals ma­lignant features. Occasionally a feared cancer appears more indolent. While we always hope that what looks like a GBM – short for glioblastoma multiforme, the most common form of brain cancer – is really an infection or perhaps some oddball benign tumor that merely mimics the appearance of a GBM, after years of operating on brains, sadly looks are usually not deceiving. The frozen usually confirms what we already knew. 

And this is where the hard part begins. But this is also where trust is built. I view it as my re­sponsibility to reveal as much as I know, when I know it, and not to hide facts or hard truths. If the frozen is consistent with both my interpretation of the MRI scan and what I encountered during surgery, it's time to let the family know that, despite their inability to see the asteroid in the sky heading toward earth, this cataclysmic planet-destroyer is not just a blip on the radar. It has arrived. It will hit, and the effects will be devastating.

This is when the tone of the conversation shifts from the breezy "The surgery went well!" to the more sobering "But here's what we're dealing with in the days and weeks ahead." And so begins the next stage of the process. The people in the waiting room are often the patient's closest rel­atives and friends, their support group. The sooner they can be involved in their loved one's care, the better. If the tumor is malignant, I let them know that the sum of the evidence is now leaning in that direction. That said, I am also always careful to add that we cannot be 100 percent sure until the final report comes back.

I try to gauge by their expressions their level of understanding of the difficult news I've just imparted. My job is to hold their hands, both literally and figuratively; to answer their questions; and to let them know that we are in this together: I will not be leaving their side. Although the family might ask my advice about how to break the sad news to the patient, since the family will see them in the recovery room before I do, they almost never take on that burden. Instead, I'm the one tasked with having the frank conversation with my patient the next day, after the anesthesia has worn off, and they have rested and are ready to listen and absorb the infor­mation. 

The truth is I've already started preparing them for this moment from their first visit to my office. I've removed almost 10,000 brain tumors and reviewed at least twice as many scans. The diagnosis is almost never a surprise: the MRI tells the story, like a crystal ball that shows the future with crisp, brutal clarity. I know a GBM when I see one. 

The initial office visit, in fact, can be surreal. At this early stage, they are often minimally symptomatic. They are obviously aware that something is growing in their brain—they've sought out my care, after all—and they know that it needs to be removed. Maybe they've done a little research, or they've had a family member with a brain tumor, which may or may not have been similar. They are also often scared and unsure of what lies ahead or what it all means. 

Commonly, they are frequently somewhat oblivious to the gravity of the situation. This is all new to them. But as I listen to their questions, I see things they are not yet capable of seeing, let alone processing. 

I see the mother of three young children who will not make it to their high school graduation. I see the father and sole provider for a family of teenagers with college payments looming who will not be walking his daughter down the aisle. I see the hedge fund manager who is sitting on top of the world, plan­ning his retirement and next lavish vacation, who will soon be closing his fund. He's not only about to lose his long-anticipated opportunity to spend his money but his ability to bathe and feed himself.

Thinking of others' deaths can be debilitating to even the most hardened of us surgeons: giving bad news, seeing families crumple from the oncoming train bearing down on them. As I stare into the void, imagin­ing their future, I want to stand up and scream at the top of my lungs or collapse on the ground in a flood of tears. I do none of this, of course. My job at this moment is to fight this battle with every fiber in my body and shepherd these victims of nature's callous and indifferent design.

I believe in revealing the truth of my patients' prognoses at a slow and deliberate pace. But I also never, ever take away their most powerful weapon: hope. We're not talking about false hope, as in "We're going to beat this thing," but rather, true hope, a concept introduced by Jerome Groopman in his book The Anatomy of Hope: How People Prevail in the Face of Illness. True hope sounds more like "There are a small group of long-term survivors and I'm going to do everything in my power to give you the best chance of being one of them." Or even "Your remaining days with your family can be beautiful―in ways you never imagined during all the days that came before."

So, how does a doctor walk this tightrope between truth and hope?

At the first office visit, I begin by focusing on the upcoming surgery, which is the initial psychological hurdle. Most people fear the unknown, so I take them through each step of the procedure. I describe what will be done to them like a tour guide: "The anesthesiologist will put a mask over your face, and you will go to sleep. You won't feel anything after that. Then I'll make an incision behind your hairline, which you won't see once the little bit of hair I might have to shave grows back. Once the skin is opened, we will re­move a piece of the skull . . ." and so on. The vague and terrifying becomes concrete and simple when presented step by step.

We then discuss the possible complications of the surgery. I generally stick to things that could reasonably (if infrequently) happen, like an infec­tion or a bleed, rather than one‑in‑a‑million calamities like the ventilator breaking or the anesthesiologist having a heart attack. 

If we are operating near the optic nerves, for example, then I must pre­pare them for a possible loss of vision. If we are encroaching on the lan­guage areas, they may wake up with speech difficulties. Basically, any faculty in the vicinity of the surgical corridor is at risk of being impaired. This is the essence of brain surgery: navigating the narrow boundary be­tween causing harm and doing good. 

In my experience, emphasizing the remote possibility of disaster is not helpful. Think of a pilot explaining the odds of the plane crashing as the wheels leave the tarmac: most of us would rather not know this statistic at that particular moment. It just creates unneeded anxiety. As Groopman wrote, "Don't let fear overwhelm hope." At the same time, I will not lie or deliberately hide things from my patients. If the patient wants to know ev­ery possible wrong turn their surgery can take, I'll go there with them. 

At this point, my job is more psychoanalytic than surgical. I read their faces, try to figure out how much they really want to know. If the tumor I'm expecting is a GBM, the brain surgery itself should be the least of their worries. What's more important right now is processing the news of the tumor's existence and preparing them for the rapid and relentless progres­sion of the disease. 

Which is why, at this very first visit, I always introduce the possibility that the tumor could be malignant. Even if I'm often confident it's a GBM, sometimes—rarely—looks cans can be misleading, and my initial suspicions can be mistaken. Hitting a patient over the head this early by telling them that there is a high likelihood that they have a malignant tumor is, in my opin­ion, insensitive to them and to their family, and it does not promote a proper mindset going into surgery.

It's critical for the patient to show up on the day of surgery with both hope and optimism that things will go well. I also tell them that I'll do ev­erything I can to ensure that the procedure unfolds as seamlessly as possible and that I'll be there for them every step of the way, no matter the ultimate diagnosis. I want them to know they have a seasoned veteran as their ally.

So then how do I break bad news to the patient after the surgery? I take my cues from them. I usually start the conversation with a clear presentation of the facts. I may say that the preliminary diagnosis showed what we feared: that the tumor is malignant. I prefer to use the words "we" and "us." I also emphasize whatever positives I can. The good news is that the sur­gery went extremely well and we got out as much tumor as could safely be removed: "Although it's a tough tumor to beat, the surgery puts us in the best place going forward to attack the microscopic disease invariably left behind."

I then tell them they will likely need radiation and chemotherapy—the standard of care in treating GBMs—and that we will find them the most experienced neuro-oncologist to help coordinate the next stage of this pro­cess. While our neuro-oncologists at Cornell are some of the best in the world, patients often want second opinions, so I let them know we will help them get their records together to send them wherever they like. Patients often express a fear of telling you they want a second opinion, as if they're cheating on their spouse or insulting a relative. You never want anyone looking back as the end approaches and feeling that they didn't do every­thing in their power to find the right treatment, didn't explore all the options, or left a stone unturned.

I have witnessed only a handful of medical miracles in my career: tu­mors that miraculously shrank without any treatment, long-term survivors of fatal diseases. What's the explanation? We don't know. But these cases do provide some room for hope. The patients I have treated who are still alive five, ten, or even fifteen years after a GBM diagnosis are a rare re­minder that my degree and years of experience only go so far. What makes these long- term survivors so special? What did they do to beat the odds? Were they in some incredible new trial of a groundbreaking drug, or did they eat nothing but kale and quinoa and exercise every day? Nope. They were each treated the same way we treat everyone, and they ate whatever they wanted.

Which brings us to clinical trials. With easy access to the internet, patients often hear about new treatments and tri­als for GBM, but the quantity of material out there can be overwhelming. It's nearly impossible for the layperson to sift through the dozens of ongoing and future trials and know which, if any, are appropriate; which are somewhat reasonable, perhaps; or which are too dangerous, with little likelihood of suc­cess. My general advice to my patients is that they get no more than three opinions and then go with their gut. Find a trustworthy neuro-oncologist and follow their advice. I also remind them that, were there a magic bullet to cure GBMs, we'd all know about it and already be prescribing it. 

The odds of success for any new trial therapy, given the track record of clinical trials for GBM, are disappointingly low. All too often I hear from patients who wish they'd spent less time pursuing ineffective treatments and more time enjoying their remaining days with loved ones. That said, I do encourage my patients to enroll in clinical trials if they sound reason­able, don't require too much travel, don't drain too many resources, and don't take away too much time from family and friends. Clinical trials are the only way medicine advances, the only way we will eventually defeat this disease.

Although hope remains a critical component of any cancer care plan, we must remember that there is always a human being at the epicenter of the oncologic universe. Doctors make recommendations. We don't have the final say.  Every now and then, when I find myself forgetting this most critical of lessons, I am reminded of Eugene Desmond O'Kelly, the former CEO of one of the largest accounting firms in the world. One morning only a few years into his tenure, O'Kelly's wife noticed a slight drooping in her husband's right cheek. An MRI revealed a multifocal GBM on the left side of his brain, meaning it had already spread to several different locations and was just starting to impact his functions: speech, motor, and visual. While trying to decide how to proceed, O'Kelly sought the counsel of two neurosurgeons. The first, at Memorial Sloan Kettering, recommended a debulking surgery. He would perform a craniotomy but take out only a part of the tumor, since it had invaded O'Kelly's brain to such an extent that it could not be removed entirely without causing significant harm. Recovery after such a debulking would take at least six weeks.

A day later, O'Kelly showed up in my office. After listening closely to his story and unraveling his priorities, I recommended a biopsy, noting that the risk of removing it far outweighed any possible benefit, as it would not increase his life expectancy. Taking a tiny sample via a biopsy, on the other hand, would be safer and sufficient to make the diagnosis. Furthermore, the biopsy would not put any of his faculties in jeopardy and would allow him to begin radiation and chemotherapy sooner, which might extend and maximize the quality of his remaining life. 

Given these two options, O'Kelly chose the biopsy. Then he made another choice that not many other patients make. He refused chemotherapy. He didn't want whatever time he had left to be consumed, either emotionally or physically, by the debilitating ravages of the treatment.

O'Kelly lived for one hundred more days, and during that time he wrote his New York Times bestselling book Chasing Daylight: How my Forthcoming Death Transformed My Life. In this inspirational account of his final weeks, he described, with both clarity and sensitivity, how he translated the win-at-all-costs mentality of a CEO into the language of dying with dignity, optimism, love, and grace. He strove for presence. He found closure in each of his relationships and created perfect moments with his friends. This calculation—to forgo aggressive treatment—allowed him to take control of his disease and to die on his own terms.

Sometimes it's not the doctor but the patient who knows when to stop.

Theodore H. Schwartz is a neurosurgeon at Weill Cornell Medicine in New York. This essay is adapted from his new book, "Gray Matters: A Biography of Brain Surgery," published by OneWorld on 5 September, hardback £25..