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This chapter is from the book

Silent witness

Nick Chisholm was a gifted 23-year-old athlete—in love with sports and life—when he took a devastating hit on a New Zealand rugby field. His vision went blurry and he felt sick immediately, but he chalked his symptoms up to a minor concussion. When he came off the field, he asked his coach to put him back in after a mere ten minutes' rest—but then he collapsed and paramedics rushed him to the hospital.

Three days later, it was still unclear what had happened to Nick, but he seemed to be recovering well. The doctors were going to let him go home. Then he nearly collapsed again in the hospital shower, and for the next six days suffered a series of seizures that ultimately left him paralyzed and unable to speak. A battery of tests—which felt like "all the tests known to man,"27 as Nick reports it—revealed that he had suffered several strokes due to a dissection of his vertebral arteries. One of the strokes was so massive that it effectively annihilated his brain stem, severing the connections between his higher brain and the rest of his body.

After that horrific accident in 2000, Nick was diagnosed with locked-in syndrome (LIS), an extremely rare condition in which a person's conscious mental life and senses remain intact, but in which he is unable to move or to use his body to communicate (except by shifting his eyes or by blinking). LIS can result from a sudden injury like Nick's, or it can be due to progressive degeneration of motor neurons in the devastating neurological condition amyotrophic lateral sclerosis (ALS).

It took some time—an excruciating stretch for Nick—before he was properly diagnosed. The doctors didn't know that he was aware and listening when they told his mother that he would probably die, and then offered her the option of withdrawing life support. It was Nick's family that guessed that he wasn't comatose at all, but instead was fully conscious and able to follow every word that was said. When asked by TV New Zealand several years later to share the most frightening thing he heard during that time, Nick did not hesitate to answer that it was the doctors speaking of turning off his life support machine.28

"Nick's mother and his girlfriend pleaded with the medical staff to realize that he was aware of what was happening,"29 reports medical ethicist Grant Gillett, who co-wrote a piece with Chisholm for the British medical journal BMJ. "When the clinicians appreciated that the diagnosis was locked-in syndrome, the climate of care changed."

Misdiagnosis of LIS is tragically common, as it is often mistaken for the vegetative state or even coma. As happened with Nick, more than half of LIS cases are identified by family members rather than by physicians or nurses, because family members tend naturally to be more attuned to the patient's needs and to read signs of a conscious presence. A 2002 survey of 44 LIS patients found that diagnosis took an average of two-and-a-half months,30 and in some harrowing cases, it took as long as six years. Even after family members catch on, they may still have trouble convincing the attending physicians that their loved one is fully conscious.

In recent years, brain scans have shed terrifying light on the acute experience of LIS—what it is like to realize that you are conscious inside an unresponsive body without the means to communicate your experience to others. PET scans have shown that glucose metabolism in the brain's higher regions does not differ significantly in patients with LIS as compared to healthy age-matched controls, supporting the conclusion that their injury is restricted to physical paralysis, and that these patients can, as Laureys and colleagues report, "recover an entirely intact intellectual capacity."31 PET images have also revealed a telling neural signature in acute LIS patients, one that differs strikingly from healthy controls: The amygdala—a primitive part of the brain linked to primal emotions like fear and anxiety—was hyperactive in acute, but not in chronic, LIS patients.

"It is difficult to make judgments about patients' thoughts and feelings when they awake from their coma in a motionless shell," say Laureys and colleagues, but in light of the evidently normal metabolism in higher regions of the brain, these researchers hypothesize that the pronounced amygdala activity in conscious patients who had not yet learned to communicate with the outside world "relates to the terrifying situation of an intact awareness in a sensitive being, experiencing frustration, stress, and anguish, locked in an immobile body." They conclude from this preliminary imaging evidence that medical professionals caring for patients with LIS must adjust their bedside behavior to address states of extreme emotional distress.

We can only imagine, Gillett notes, "the sheer awfulness of hearing others discuss turning off one's life support."32 Even after medical professionals knew that Nick was fully conscious and aware of everything unfolding in the room, Gillett says that Nick still "heard things said about his prognosis and his life that paid little regard to what he might have been thinking." Nick reports that his case manager for New Zealand's Accident Compensation Corporation said in his presence that even if he did live, he wouldn't want to anyway, and a specialist told him to get used to the wheelchair, because he would be in one for the rest of his life. In his second year in the hospital, another specialist told him that whatever gains he had made to date were the only improvements he'd ever see.

Nick begged to differ. "What do they really know? They only know what they read in textbooks," he says, and he characterizes most of his specialists and doctors as "so extremely negative."

In cases of locked-in syndrome, clinicians naturally make mistakes all the time about what a patient is thinking or feeling, Gillett says, because it is a state of consciousness that simply defies our ability to know how it feels to be the other person. "We must make special efforts to reach past the locked-in syndrome barrier and allow the patient to connect with us," he argues. No matter how closely Nick's condition resembles other states devoid of awareness, he must be treated like a whole person, capable of experiencing a complete range of thoughts, feelings, and emotions.

Rather than experiencing cognitive deficits, Nick feels that some of his senses—sight and hearing in particular—have actually been enhanced by his situation. Because of the natural tendencies of people surrounding LIS patients to dwell on everything that has been lost, it is all too easy, Gillett observes, to become blinded to the "ongoing work of 'self remaking' that someone like Nick is doing." To hear Nick speak through his brother (who has learned to interpret for him), it is obvious that despite severe disabilities, Nick is as mentally quick as ever—and that however we choose to define personhood, he hasn't lost it. Before his accident, he thrived on social interaction and on cracking wicked jokes; if anything, those aspects of his mental life have become more nourishing to him than ever.

"I don't think I could've made it this far without the support of my friends, carers, and family," Nick says. After his return from the hospital, Nick was able to regularly attend his old rugby team's games with the help of a good friend, and his injury did not get in the way of his pulling outrageous stunts. (Nick, his brother, and several buddies landed on You Tube, for example, for showing up naked to the polls on election day.) And it is not just family and old friends who keep Nick going socially. "I have met a lot of people since my accident," he says. "Some have become friends; some have become great friends."

By all accounts, Nick's recovery has been remarkable. During his two years in the hospital, he had to exert an enormous amount of effort to make a single sound, but in 2005 he wrote that "I can now say heaps of words, count, pronounce about four carers' names relatively clearly, sometimes stringing some words together when lying down and relaxed." Since this account in BMJ, Nick has learned to use a walker for short distances and even to use certain weight machines with assistance, defying specialists who told him that he would never recover any physical skills. (He remembers one specialist who told him that he'd never move or talk again. When Nick returned home from his long hospital stay, the specialist examined him and was taken aback by Nick's progress. He apologized to Nick for his early predictions. "I gave him the finger," Nick reports.)

His recovery has been extraordinary, but not atypical for LIS patients receiving an intense and consistent level of care. A 2003 study followed 14 patients with locked-in syndrome in three rehabilitation centers for periods ranging from five months to six years, and found that early and intensive rehabilitation treatment significantly improved outcomes and reduced mortality rates.33 Laureys and colleagues have reported that in a study of 95 patients with LIS,34 92 percent recovered some ability to move their heads, and half recovered limited speech production (single intelligible words). Some patients recovered the ability to make small movements in fingers, hands, or arms (65 percent), and three-quarters recovered the ability to make small motions in their legs or feet.

Will to live

Even for someone like Nick, gifted with a fighting spirit and keen sense of humor, there are the inevitable moments when life feels unbearable. Before the accident, reports one of his best friends, he had "muscles on his muscles, he was happy as hell, he had a beautiful girlfriend. He couldn't have been any better."35 Now Nick says it can be a lonely existence, and he believes that dating and romantic relationships are out of the question. "It would be great," his brother translates, "but able-bodied people struggle getting dates. So I'm stuffed."

He talks of the utter humiliation of his condition—times when he loses control of his bowels in public places, for example. "Believe me, when you're 30 it's totally degrading," he says. "And nowhere more so than in the public gym, in front of people. It definitely changes my mood extremely quickly when it happens, as you can imagine."36 He admits that he has often thought of suicide, especially alone in his bed at night. Sometimes he wishes that he had died in the ambulance on the way to the hospital—it would have been less frustrating that way—but even if he wanted to commit suicide now, he couldn't do it. "It's physically impossible," he notes—though he has discussed the topic with the people closest to him.

"Yeah, we've talked about it," his brother says. "Nick has said things along the lines of, 'I don't want to live 40 or 50 years in this chair, in this cocoon.' And I can understand that. Who am I to say, in my situation, that he should live his life like that?"37 But even if it were possible to end his life—alone or with help—Nick says it wouldn't matter. "I'm just glad to still be alive—most of the time anyway," he says. "I only live for hope of recovery now."38

Despite the common misperception that LIS patients—given the option—would choose to die, Nick's determination to live fits with the majority sentiment in patients with his condition. "Healthy individuals and medical professionals sometimes assume that the quality of life of an LIS patient is so poor that it is not worth living,"39 say Laureys and colleagues. "On the contrary, chronic LIS patients typically self-report meaningful quality of life and their demand for euthanasia is surprisingly infrequent." Experts in LIS are concerned that uninformed physicians might provide less aggressive medical treatment than might be warranted with particular patients, despite studies showing decreased mortality and improved quality of life with early diagnosis and treatment. Physicians less familiar with the experiences and wishes of LIS patients might even influence the family toward removal of life support without ensuring that everything reasonable has been done to reveal the patient's preferences.

Regardless of what care locked-in patients ultimately request, Laureys and colleagues stress that their autonomy should be considered paramount. "Patients suffering from LIS should not be denied the right to die—and to die with dignity—but also, and more importantly, they should not be denied the right to live—and to live with dignity and the best possible revalidation, and pain and symptom management."

For his part, Nick doesn't want it left up to anyone else to decide whether his life is worth living. When asked by TV New Zealand in 2007 if he considers himself happy, Nick said, "Absolutely. Coming from where I've been, this is absolutely fantastic. I'm probably happier than most able-bodied people."40

Speaking without a voice

Nick's tool to communicate with the outside world is a large transparent board with the letters of the alphabet spread across the surface. He spells out each letter of his sentences painstakingly with his eyes, and the person "listening" on the other side of the board must guess which letter Nick is staring at. This goes on until he has spelled out entire sentences—a process Nick describes as "extremely laborious."41 For a person like Nick—to whom horsing around with his friends is so important—the inability to banter can be excruciating. It is "very difficult (almost impossible)," he says, "to express yourself or be sarcastic."

Speech devices like Nick's board require assistance from other people to transmit anything verbally—including thoughts and feelings, calls for help, and requests for information—a frustrating reality that only compounds the utter dependence of LIS patients upon others. Cases like Nick's, Gillett says, require that we care enough to "rebuild the tools of communication (through interactive technology and massive personal commitment) so that he can begin to live again among us, albeit with severe disabilities."

State-of-the-art patient-computer interfaces, such as infrared eye sensors coupled to on-screen keyboards, are improving the lives of many LIS patients and their caregivers. These devices allow LIS patients the freedom to perform simple, everyday actions the rest of us take for granted, such as turning lights and appliances on and off, communicating by phone or fax, surfing the Web, sending e-mails, and using word processors and speech synthesizers. These devices cost thousands of dollars—sometimes a prohibitive sum for patients whose insurance won't pay for them—but for those patients who can manage the cost, these devices provide unprecedented freedom.

Patients like Nick—with the ability to consciously control their eye movements—would clearly benefit from the independence such devices afford, but what of patients like the young English woman, who showed signs of consciousness without the ability to control her eyes? For some patients, computer interfaces relying on eye control won't take communication technology far enough. Researchers working with disorders of consciousness welcome the prospect of direct mind-computer interfaces based on fMRI or other functional neuroimaging that could, in effect, read patients' thoughts and emotions, allowing them an active voice in their treatment and in critical life-extending—or life-ending—decisions.

"The beauty of medical and communication-technological progress for patients with severe neurological conditions is that it makes them more and more like the rest of us,"42 Laureys and fellow researchers with the Coma Science Group observed in a recent editorial. "As a corollary, we caregivers not only owe them the same respect in terms of their autonomy as all other patients, but we also have to rise to so far seldom attained levels of clarity in matters of life and death."

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