Whose Wishes Should Prevail

Do Not Dodge Moral Anguish

The tragic case of Terri Schiavo in Florida highlights the importance of making our health-care wishes known.

- Bill Nelson

Terri Schiavo shortly before her attack

by Christopher Caldwell

The Vatican, the US president, both houses of the US Congress meeting in emergency session and some of America's most respected philosophers - have mobilised to block a flimsy argument, advanced in a provincial Florida court, that a 41-year-old invalid named Terri Schiavo be denied food and water until she dies. And they have lost. Mrs Schiavo was drifting towards death as this column went to press. Her parents were being prevented by law enforcement officials from giving her food or water. And 63% of Americans, traditionally sceptics about "mercy killing", professed themselves happy with the result. Perhaps we do not know the US electorate as well as we think we do.

Terri Schiavo suffered brain damage after a heart attack in 1990. Her husband won a $1 million malpractice settlement from her doctor shortly thereafter. In 1993, he and his in-laws began to quarrel. They were hoping for a miracle cure; he argued for "death with dignity". Which side is right may depend on how one views Mrs Schiavo's condition.

She cannot speak. But videos posted on her parents' website show her following a balloon around her hospice room with her eyes, and laughing and purring at questions. She gets prescription pain medications during her menstrual periods. She is not comatose, and not on a respirator or other life-support.

The Schiavo family in better days.

Michael Schiavo, who has since fathered two children by a woman he plans to marry, says he remembers a conversation in which his wife told him she would wish to die if disabled. He began petitioning in 1998 to remove the tube through which Ms Schiavo is fed, and has spent much of his lawsuit money on litigation. Judge George Greer, ruling Mrs Schiavo to be in a "persistent vegetative state", has assented 3 times. Each time, legislators intervened to block the process.

Until now. A month ago, Judge Greer set March 18 as the deadline for removing Mrs Schiavo's tube. Florida's Republican legislature was too disorganised to thwart him. Mrs Schiavo's parents had no standing to appeal against the judgment in federal court. So, last weekend, an alarmed US Congress reconvened during its holiday and passed a law giving the parents that standing. Mr Bush flew in from Texas to sign it. Judge Greer and Mr Schiavo had both sought to block new diagnostic tests of Mrs Schiavo, and the bill invited federal courts to revisit her diagnosis. Bill Frist, the heart surgeon who is Senate majority leader, was sure this would involve reinserting her feeding tube, "because Terri would need to live in order for the court to consider the case". Dr Frist misjudged. The federal court chose to let the earlier findings stand. Mrs Schiavo's parents argued that their daughter, a Catholic, was being denied her religious rights by being forced to starve herself. It was not a strong legal argument. But the parents can be forgiven for thinking they could get by with a weak one. Where the "right to life", an American constitutional right for centuries, is pitted against a "right to die", a newfangled and subjective preference (and one that the patient is not on record as having expressed), an institutional bias can be assumed for the former. Besides, if those who favour Mrs Schiavo's death are correct that she can feel nothing, then there is no risk (or harm) in keeping her alive. But if those who favour continued feeding are right, then the moral stakes are very high. Cutting off her food might be an act of torture. Robert George, the Princeton moral philosopher, warned last week against creating the Nazi category of "life unworthy of life".

None of these arguments cuts much ice with the courts or the public. In fact, they were used by Mr Schiavo's side. John Lewis, a Georgia congressman, inveighed: "We are playing with a young woman's life." Mr Schiavo's lawyer said that "it is simply inhumane and barbaric to interrupt her death process". One newspaper described as "Orwellian" the idea that Mrs Schiavo should continue to receive the same care she has received for the past decade-and-a-half. Nancy Pelosi, the House minority leader, expressed pity for Mr Schiavo, who faces, she says, "a devastating decision".

But this is not true. The court has ignored an obvious link between spousal rights and spousal duties. Mrs Schiavo is "warehoused", as her brother puts it. Mr Schiavo has no spousal duties (except to the new family he has formed). But the court has granted him all the spousal rights of a pre-feminist paterfamilias, including the right to dispose of his wife's body, regardless of his own conduct. This granting of ownership rights to a husband is wholly at odds with other trends in family law on, say, domestic violence, abortion or school attendance.

Euthanasia is what Mr Schiavo has proposed for his wife. The case has evolved in such a twisted way because none of his allies dares to speak the word. But it is a tricky case on which to argue for a "right to die" in the first place, because Mrs Schiavo was not, until last week, in imminent danger of dying. She can be made to die only in the way that healthy people can be made to die - by deliberate measures, in this case by preventing her parents and siblings from feeding her. This is not a passive "pulling the plug" on a brain-dead person. It is a Dutch-style act of "mercy killing", minus the moral clarity and the concern for the comfort of the patient.

Mr Schiavo's story of his wife's wish to die is slender justification for ending her life.

Why are Americans so eager to believe it? Debbie Wasserman Schultz, a Florida congresswoman, who found fault with her colleagues for taking up the matter at all, probably speaks for the country. "We are not doctors," she said. "We are not bioethicists." Thus an interesting line is crossed. In Ms Schultz's view, not only does the citizenry have no standing to make ethical decisions or moral distinctions - neither do its elected representatives, unless they can claim some kind of ulterior expertise. Americans want the' convenience of euthanasia, but they want to hand over the moral anguish to others. As such, the Schiavo case reveals the deepest American religious conviction - an unshakeable belief in the free lunch.

The writer is a senior editor at The Weekly Standard

Source: Financial Times 26 March 2005

The Unspeakable: Buried Alive in Your Own Skull

by William Saletan

[Excerpt]

[A] young woman [was] devastated by a car crash in England. For 5 months after the accident, tests showed no signs of awareness. Doctors declared her vegetative. Then, scientists put her in a Functional Magnetic Resonance Imaging scanner, which tracks blood flow to different parts of the brain. They asked her to imagine playing tennis and walking through her home. The scan lit up with telltale patterns of language, movement, and navigation indistinguishable from the brains of healthy people. Something was awake [but] without the scanner, no one but her would have known.

How rarely does this happen? Until a decade ago, FMRI didn't even exist. ... By various estimates, 25,000 to 35,000 Americans have been diagnosed as vegetative. How many of them have received FMRI scans? How many would light up? How many are awake in there?

The scientists who studied the English patient report that in the 5 months after her injury, "No elaborated ... 'voluntary' or 'willed' responses, were observed ... no evidence of ... tracking to visual or auditory stimuli, no overt motor responses to command were observed." In short, she was "unresponsive." ...

Then they put her in the machine and gave her the imagination test. Result: "Significant activity was observed" in key brain areas. She managed "to respond to [commands] through her brain activity, rather than through speech or movement. Moreover, her decision to cooperate with the authors by imagining particular tasks when asked to do so represents a clear act of intention." According to the attached analysis, her "patterns of brain activation" suggest "an active mental performance." ...

Now scientists are debating what goes on in the English patient's head. Some call her performance a "decision" others dismiss it as a mere "response." They ask why her body doesn't move, since her motor pathways appear to be preserved. ... she has a "rich mental life" but may not be "conscious." What ... does that mean? Would you pull the plug on a 24-year-old relative with a rich and responsive but unconscious mental life? Go ahead, raise your hand. Or just think about raising it, and we'll record your vote by brain scan.

Most hospitals don't have equipment that can find trapped souls like the English patient. Does the reality of your mental life depend on which hospital you're taken to? Does it depend on which tests your insurance covers or your family can afford? ...

Source: slate.com/id/2149182 12 September 2006

Music Stirred Her Damaged Brain

by S Allen Counter

Judging normal brain function in a neurologically impaired person is never an easy task. The case of Terri Schiavo illustrates this challenge in a dramatic way.

A Boston-area patient I first saw when she was 32 also proved the point. The woman had been born without the portions of her brain associated with thought and awareness - a condition called hydranencephaly that's usually fatal prenatally or in infancy. But she had somehow survived, mainly through good custodial care, including being fed pureed foods by a caretaker all of her life. Her eyes were open wide, and she could move her head from side to side, and make groaning sounds, similar to Terri Schiavo. A quadriplegic, the woman was bedridden or strapped to a wheelchair for several hours each day. She weighed 77 pounds and was 4½ feet tall. She could swallow and breathe on her own.

The neurological evaluation revealed that the woman failed to blink in response to objects rapidly approaching her eye, indicating blindness; she had abnormal reflexes, and no clear sensitivity to touch. A bright light shone on one side of her slightly enlarged head revealed a bright red fluid-filled cranium. A subsequent MRI revealed the absence of cerebral hemispheres, with only small remnants of cerebral tissue, and a small brain stem.

The conclusion by the attending doctors was that this woman was unresponsive to sensory stimuli, devoid of any intellectual function, and in a persistent vegetative state.

To confirm these conclusions, she was referred to me for an objective, noninvasive evaluation of her brain's electrical activity and her response to sensory stimulation, including sound, sight and touch. In summary, I found that, although her eyes were open and moving from side to side with her head, there was no brain response to visual input. Similarly, mild electrical stimulation in the fingers and toes travelled up the spinal cord to the brain stem, but no farther in the absence of the cerebral hemispheres.

I was astounded, however, that when I turned on a child's music box in the room, I observed that this hydranencephalic patient turned toward the musical device and began to smile and make sounds, as if she were enjoying the experience. I then tested this observation several times and found a consistent response to sound stimulation. When I conducted a test of electrical activity in her brain stem, the portion of the brain that controls bodily functions like breathing, I was surprised to find that the neurons of the brain stem involved with hearing were normal.

Several more advanced electrophysiological brain measures showed that she had normal hearing response waves, reflecting neural activity in the higher brain stem. She was aware at some level of the sounds and people noises in her environment, and responded to these sounds with the appearance of joyfulness.

I immediately brought her other doctors back into the room, where they began to interact with her in a totally different manner, in some cases holding her hand and trying to speak with her, and treating her more like a normally functioning human being. I was so emotionally moved by her struggle for human definition through the single modality of hearing that I went down to a local electronics shop and bought her an audio cassette player, and some modern and classical music.

She continued to appear to enjoy the audio cassette player and her music until her death some years later.

This patient demonstrated the dilemma we face in determining whether people in an apparent persistent vegetative state, who by all objective measure have little or no function in the cerebral hemispheres, have any residual human capacity that would persuade us to sustain their lives, even by artificial means.

Her case was a reminder of how much we do not understand about the brain, and that even people in an apparent vegetative state may have ways of connecting to the world around them.

Dr S Allen Counter is professor of neurology and neurophysiology at Harvard Medical School and the Massachusetts General Hospital

Source: boston.com 29 March 2005 © The New York Times Company

Silent Minds: What Scanning Techniques Are Revealing about Vegetative Patients

by Jerome Groopman

Ten years ago, Adrian Owen, a young British neuroscientist, was working at a brain-imaging centre at Addenbrooke’s Hospital, at the University of Cambridge. He had recently returned from the Montreal Neurological Institute, where he used advanced scanning technology to map areas of the brain, including those involved in recognising human faces, and he was eager to continue his research. The imaging centre was next to the hospital’s neurological intensive-care unit, and Owen heard about a patient there named Kate Bainbridge, a 26-year-old schoolteacher who had become comatose after a flu-like illness, and was eventually diagnosed as being in what neurologists call a vegetative state. Owen decided to scan Bainbridge’s brain. "We were looking for interesting patients to study," he told me. "She was the first vegetative patient I came across."

For 4 months, Bainbridge had not spoken or responded to her family or her doctors, although her eyes were often open and roving. (A person in a coma appears to be asleep and is unaware of even painful stimulation; a person in a vegetative state has periods of wakefulness but shows no awareness of her environment and does not make purposeful movements.) Owen placed Bainbridge in a PET scanner, a machine that records changes in metabolism and blood flow in the brain, and, on a screen in front of her, projected photographs of faces belonging to members of her family, as well as digitally distorted images, in which the faces were unrecognisable. Whenever pictures of Bainbridge’s family flashed on the screen, an area of her brain called the fusiform gyrus, which neuroscientists had identified as playing a central role in face recognition, lit up on the scan. "We were stunned," Owen told me. "The fusiform-gyrus activation in her brain was not simply similar to normal; it was exactly the same as normal volunteers’."

Excited by this result, Owen resolved to try to conduct brain scans of other vegetative patients in the Cambridge area. Since 1997, he has studied several dozen people, though he decided to use speech sounds rather than photographs to stimulate their brains. (Owen was concerned that showing images of faces might not be a reliable way to test recognition, since the eyes of vegetative patients often wander. "We shifted to auditory responses because you can always put a pair of headphones on the person and know that you are transmitting sound," he said.) Three years ago, he began using a functional MRI (fMRI) scanner, which is faster than a PET scanner, capturing changes in blood flow in the brain almost as they occur. The patients’ brains were scanned while they listened to a recording of simple sentences interspersed with meaningless "noise sounds." The scans of some of the patients showed the same response to the sentences as scans of healthy volunteers, but Owen wasn’t sure that the patients had understood the words. "So we went the next step up the cognitive ladder, to look at comprehension," he said.

Psycholinguists have shown that when we hear a noun at the beginning of a sentence we tend to associate the word with its most common meaning. For example, Owen said, most people hearing a sentence that begins, "The shell was..." think of an object typically found at the beach. But if the sentence is completed by the phrase "fired at the tank," the listener quickly corrects himself, a process that is evident on a brain scan. "You can actually see it happening and image it on the scanner," Owen said. "The beautiful thing about the psychological task is that we just do it automatically. When you play ambiguous sentences, areas in the inferior frontal lobe and in the posterior temporal lobe become activated, and these areas are very important for speech comprehension. They show that you understand the meaning of the word: it’s not just about perceiving speech; it’s about decoding. Your brain somehow appreciates that there are two meanings to a word like 'shell.'"

Owen eventually identified two vegetative patients whose brains showed the same activity in response to ambiguous sentences as the brains of healthy volunteers. He also took brain scans of healthy physicians, who were presented with the ambiguous sentences while under general anæsthesia. Owen found that, as the effects of the anæsthesia increased, the physicians showed less activity in the brain regions associated with comprehension. "That, of course, is in keeping with our personal experience of consciousness, which is that as you sort of drift into sleep you understand less and less of what is around you," he said. (An article about this experiment appears this week in PNAS, the journal of the National Academy of Sciences.)

Owen’s final experiment was the most ambitious: a test to determine whether vegetative patients who seemed able to comprehend speech could also perform a complex mental task on command. He decided to ask them to imagine playing tennis. ("We chose sports, and tried to find one that involved a lot of upper-body movements and not too much running around," he said.) First, he took brain scans of 34 healthy volunteers who were instructed to picture themselves playing the game for at least 30 seconds. Their brains showed activity in a region of the cerebrum that would be stimulated in an actual match. "This was an extremely robust activation, and it wasn’t difficult to tell whether somebody was imagining tennis or not," Owen said. He then repeated the experiment using one of the vegetative patients, a woman who had been severely injured in a car accident. The woman had to be able to hear and understand Owen’s instructions, retrieve a memory of tennis - including a conception of forehand and backhand and how the ball and the racquet meet - and focus her attention for at least 30 seconds. To Owen’s astonishment, she passed the test. "Lo and behold, she produced a beautiful activation, indistinguishable from those of the group of normal volunteers," he said. (Another vegetative patient, a man in his 20s, also passed the test, though Owen, having learned that the man was a soccer fan, asked him to imagine playing that sport instead of tennis.)

In September, 2006, Owen, along with Martin Coleman, a neuroscientist at Addenbrooke’s, and 4 other researchers, published an article about the tennis experiment in Science and ignited a vigorous debate. In letters to the journal, some neurologists argued that the woman must have been misdiagnosed - a claim that Owen disputed. "She fulfilled all of the internationally agreed-upon criteria, and there wasn’t anything that she did that would lead anybody to say she wasn’t vegetative," he told me. "Now, naturally, in hindsight she wasn’t vegetative; she was actually conscious. It’s a very interesting issue, because it means that she was in fact misdiagnosed, but not misdiagnosed in the sense that somebody made an error. Clearly, she is consciously aware of things around her. So something is missing in the diagnostic criteria."

For decades, doctors assumed that patients who have been diagnosed as vegetative lack any capacity for conscious thought. Most are previously healthy people who suffered a traumatic brain injury, or oxygen deprivation after a heart attack or stroke, and have been regarded more or less as zombies: patients whose bodies continue to function - sometimes for decades - but whose minds are incapable of willed activity. (The term "vegetative" was proposed in 1972, by Bryan Jennett, a neurosurgeon, and Fred Plum, a neurologist, who chose it based on a definition in the O.E.D: "an organic body capable of growth and development but devoid of sensation and thought.") In the occasional newspaper stories about someone who suddenly recovered consciousness after spending years in a vegetative state, the event was invariably described as a medically inexplicable "miracle." The Mohonk Report, a paper prepared by a group of experts in brain injury and presented to Congress last year, cited estimates suggesting that there are approximately 35,000 Americans in a vegetative state and another 280,000 in a minimally conscious state - a less severe condition, in which patients show erratic evidence of deliberate behaviour, such as responding to a simple command or focussing on a person or an object for a sustained period. Because insurers typically won’t pay for rehabilitation, on the assumption that such patients are unlikely to improve, most are given little in the way of therapy. "These people with brain trauma are out of our view," Joseph Fins, an internist and medical ethicist at Weill Cornell Medical College, in Manhattan, and a member of the Mohonk group, told me. "We ignore them, and we sequester them in places where we can’t see them, usually in nursing homes."

According to several American and British studies completed in the late 90's, patients suffering from what is known as "disorders of consciousness" are misdiagnosed between 15 and 43% of the time. Physicians, who have traditionally relied on bedside evaluations to make diagnoses, sometimes misinterpret patients’ behaviour, mistaking smiling, grunting, grimacing, crying, or moaning as evidence of consciousness. A neuroscientist showed me a video on the Internet of Terri Schiavo, the Florida woman who spent 15 years in what most doctors agree was a vegetative state - tests revealed almost no activity in her cortex - and whose death, in 2005, provoked fierce debate over the rights of severely brain-damaged patients. (Schiavo died after the Supreme Court rejected her parents’ appeal of a judge’s decision approving her husband’s request that her feeding tube be removed. An autopsy showed extensive brain damage.) In the video, a man’s voice can be heard praising Schiavo for opening her eyes in response to his instructions, and the neuroscientist told me that he was impressed until he muted the sound. "With the sound off, it is clear that her movements are random," the neuroscientist said. "But, with the voice-over, it is easy to make a misdiagnosis." (The prognosis for patients such as Schiavo, who suffered brain damage owing to oxygen deprivation following cardiac arrest, is much worse than for those who suffer brain damage as the result of a head injury.)

Doctors can also miss signs of consciousness in vegetative patients, according to the British and American studies. Ten months after Owen and his colleagues completed the tennis experiment with the vegetative woman, she was brought back to the imaging center and placed in an MRI machine. "We were absolutely dismayed, because we scanned her and there was nothing," Owen recalled. The team tested the woman again the next day. This time, in response to a command to play tennis, her brain showed normal activity in the regions that mediate arm movements. Owen now repeats scans for each patient, conducting them twice a day for 3 days. Patients with brain injuries have "seriously impaired attention capabilities and their levels of general arousal are likely to be shot," he said. Recent research by Owen and other neuroscientists may eventually help make diagnoses more accurate, but it is not yet clear how the new brain-scan data will affect the medical understanding of consciousness. As Owen put it, "The thought of coma, vegetative state, and other disorders of consciousness troubles us all, because it awakens the old terror of being buried alive. Can any of these patients think, feel, or understand those around them? And, if so, what does this tell us about the nature of consciousness itself?"

Owen’s article in Science was accompanied by an editorial by Lionel Naccache, a neurologist at the Hôpital Pitié-Salpêtrière, in Paris, who called the results of the tennis experiment spectacular. "Despite the patient’s very poor behavioural status, the fMRI findings indicate the existence of a rich mental life, including auditory language processing and the ability to perform mental imagery tasks," Naccache wrote. Yet he cautioned against drawing general conclusions about vegetative patients from a single case, and asked, "If this patient is actually conscious, why wouldn’t she be able to engage in intentional motor acts, given that she had not suffered functional or structural lesion of the motor pathways?" Prompted by questions like this, Naccache and several of his colleagues are conducting brain-imaging experiments with the goal of identifying objective indicators of consciousness, and thus enabling doctors to better evaluate patients who are unable to communicate their awareness of themselves or their environment.

We assimilate information unconsciously all the time; at any given moment, we process thousands of stimuli, of which we pay attention to only a few. As you read this sentence, you may not be aware of the birds singing in the back yard, but your brain has analysed the sound and concluded that it poses no threat to you. In the past several decades, scientists have uncovered particularly dramatic examples of unconscious processing. In the early 70s, researchers at MIT studied 4 patients who had experienced trauma to an area of the brain involved in vision and had been found to have a condition that was later called "blindsight." These patients’ eyes functioned normally, but they did not perceive much of what was in their field of vision. When the researchers flashed a light at the patients and asked them to describe what they saw, the patients reported that they had seen nothing. Yet the researchers noticed that their eyes often located the source of the light. In a second experiment, a blindsight patient was shown pictures of faces displaying happiness, sadness, anger, and fear. The patient said that he could not see the faces, yet he was frequently able to correctly identify the emotions. The researchers concluded that, despite the patient’s injuries, pathways in his brain had been preserved which allowed him to process at least some visual data, even though he wasn’t consciously aware of doing so.

In the early 1900's, the Austrian neurologist Hermann Zingerle described patients who, because of tumours or other abnormalities of the parietal lobe on the right side of the brain, ignored the left side of the body and objects in the left field of vision. (The right side of the brain controls awareness of the left side of the body.) For example, some of these patients would shave only the right side of their faces, since they were unaware of their left cheeks. In the 1980s, researchers determined that patients who had the syndrome - now called "neglect" - could process some objects in the left field of vision. In one experiment, a patient was shown two pictures of a house. The images were identical except that, in one, flames were emerging from a window on the left side of the façade. The patient said that she couldn’t see any difference between the images, but, when she was asked which house she would want to occupy, she almost always chose the one that was not on fire. "This is more complex than blindsight, because it means that the patient was unconsciously able to interpret and understand the symbolic meaning of the pictures," Naccache said. "It is a powerful experiment to demonstrate that unconscious perception and unconscious cognition can reach upper levels of the brain."

From these and other recent experiments, including his own, Naccache and his research team are developing a working medical definition of consciousness. "When we are conscious, the key property is our ability to report to ourselves or to others the content of the representation - as when I say, for example, ‘I am perceiving a flower,’ or the fact that I am conscious of speaking with you now on the telephone," Naccache told me. "You have patients who are conscious, or who are able to make reports, but you can prove that some stimuli escaped their conscious reports, as in the case of blindsight or neglect. You can study the neural fate of these representations by showing that, even if the stimuli were not reported by the subject, they were still processed in the brain." He added that, in the case of Owen’s vegetative patient who imagined playing tennis, it’s impossible to know whether she reported the event to herself - which would suggest that she is capable of conscious thought - or whether, as in the case of the blindsight and neglect patients, she had no subjective awareness of the experience. However, Naccache believes that consciousness also requires an ability to sustain a representation over time, which Owen’s patient clearly was able to do. "In assessing apparently vegetative patients who are unable to speak, and thus report, the direction of research should be to look for sustained representation," he said. "If we can prove by neuroimaging techniques that this person is able to actively maintain a given representation during tens of seconds, it provides strong evidence of conscious processing."

Naccache has recently incorporated a third neurological feature into his definition of consciousness: broadcasting. In a person who is conscious, he explained, information entering the brain is processed in a few areas and then distributed - or broadcast - to many others. "It’s as though there is a kind of ignition in the brain, and then information is made available to a very rich number of regions," Naccache told me. "And that makes sense, that the information is initially represented locally and then made available to a vast network, because the person has this ability to maintain the representation within the network for a long time."

In 2005, Naccache conducted an experiment whose outcome suggested the importance of broadcasting as a marker of consciousness. First, he and his research team presented a series of words to three epileptic patients, who had had electrodes implanted temporarily in various brain regions, in an effort to locate the source of their seizures. The electrodes enabled doctors to record the activity in a given region. Some of the words, such as "blood" and "rape," were chosen for their negative emotional connotations. The rest of the words, which included "chair" and "house," were considered neutral. Each word was shown to the patients for 29 milliseconds and then replaced with an image of a geometric figure, such as a rectangle. The patients reported seeing only the geometric figures. However, Naccache’s team discovered that in each patient the amygdala, a brain structure that is associated with strong negative emotions, such as fear, displayed much more activity in response to the negative words than to the neutral words.

"The picture we have now is that, unconsciously, many areas of the brain can process information, and that unconscious representation can be very abstract and very rich - much more than neuroscientists thought some decades ago," Naccache said. "But now we can begin to identify some limits of unconscious cognition. The activation picked up by the electrodes is not only evanescent but restricted to the amygdala and a few other regions, without broadcasting and amplification through the brain." Owen’s tennis-playing patient may have been broadcasting information during the experiment, Naccache said, though he added that he is uncertain whether her diagnosis should be upgraded from vegetative to minimally conscious. Moreover, he said, brain-scan research cannot yet tell us much about such a patient’s prospects for improvement.

The JFK Johnson Rehabilitation Institute, in Edison, New Jersey, is among the world’s largest centers for the treatment of brain injuries and one of the few places where patients suffering from disorders of consciousness participate in research studies and receive innovative therapy. In 2002, Joseph Giacino, a neuropsychologist at the institute, was the co-chair of the Aspen Work Group - which was made up of experts in brain injury - and helped formulate the criteria for diagnosing a minimally conscious state. "I think the rehabilitation field was ahead of the curve in understanding that there were subpopulations of patients who were not in a coma, were not in a vegetative state, but really were not conscious, at least in the way we think about normal consciousness," Giacino told me. "In the medical literature, these patients were lumped together with everybody else."

The techniques that Giacino uses to diagnose patients require no sophisticated technology. He recalled making rounds at the institute with two eminent neurologists and stopping at the bedside of a woman who had had a brain hæmorrhage. The neurologists examined the woman, who lay with her eyes half closed and did not respond to the doctors’ commands. The neurologists concluded that she was in a vegetative state. "So I sort of sheepishly said, ‘Let me show you what happens when we stimulate her,’" Giacino recalled. He had been using a technique called "deep-pressure stimulation," which involves squeezing a patient’s muscles with force and precision. Giacino started with the woman’s face and worked his way down to her toes, pinching her muscles between his fingers. As he explained, the nerve endings of the muscles send impulses to the brain stem, which relays them to other brain structures and rouses the patient to consciousness. "I did a cycle of deep-pressure stimulation, and within a minute or so she was talking to us," Giacino said. "The neurologists were flabbergasted." The woman was able to say her name and her husband’s name, and answer simple questions, such as "Is there a cup at your bedside?" After a few minutes, however, she became unresponsive again.

The woman had what Giacino calls a "drive disorder," in which a patient is unable to speak, move, or, possibly, think unless physically stimulated - by touch. Doctors believe that such disorders are caused by damage to the limbic lobes or to other parts of the brain that trigger and sustain behavioural responses. Some patients with drive disorders respond to drugs that increase brain levels of dopamine, a neurotransmitter that is associated with arousal. "Imagine if the woman were in a nursing home," Giacino said. "Somebody would stop by for 3 minutes, check her bedpan, and present simple commands like ‘Squeeze my hand,’ ‘Close your eyes,’ and ‘Open your mouth.’ She is not going to do any of those things, but she clearly had a significant amount of preserved function. It had to be harnessed externally." At JFK Johnson, patients with drive disorders receive behavioural and drug therapy. (Some patients improve, but prospects for recovery are largely determined by the extent and nature of the damage to the drive system.)

Since 2002, the institute has been experimenting with using brain scans to assist with diagnoses. Giacino cited the case of a male patient whose condition had been diagnosed as vegetative but who appeared to have strong emotional responses to people around him. "If a nurse came in to do his care, it looked like he was screaming silently," Giacino recalled. "His mouth would be wide open, and he had an agonized, contorted face, like the one in Edvard Munch’s painting The Scream. The expression would occur if there was a lot of noise around him, or if he was being physically handled, but then his mother would come into the room, lower the lights, talk with him in a soothing voice, and it would just go away." When doctors scanned the man’s brain, they discovered that portions of the right hemisphere involved in emotional processing were intact. (Other parts of the right hemisphere were damaged.) "This shows you how treacherous diagnostic assessment can be," Giacino said. "One can retain one piece of a network but be disconnected from other structures and other networks, so that there is almost no subjective awareness associated with this complex behaviour. I’ve seen other patients with other behaviours that seem to be outside the scope of a vegetative state. Then you image them and you find out some circuits are still relatively preserved, while most of the rest of the brain is not."

However, brain-scan technology has also helped doctors identify one patient at JFK Johnson as a candidate for an experimental therapy. The patient, a 38-year-old man who suffered a head injury and had been living in a nursing home for 6 years, arrived at the institute in 2004. He appeared to be minimally conscious; he occasionally mouthed single words when prompted, but he was unable to respond reliably to simple questions, or to chew and swallow. (He had a feeding tube.) In 2001, PET and fMRI scans had been taken of the man’s brain, and, according to Giacino, one of many researchers involved in the case, "the findings were totally unexpected. The PET scan showed little metabolic activity, but the fMRI scan showed that the region of the cortex involved in processing language functioned in a fairly normal way." The researchers speculated that, because of damage to the man’s frontal lobe, thalamus, and brain stem - areas involved in regulating arousal - the nerve signals in his brain were muted. As Nicholas Schiff, a neurologist at Weill Cornell Medical College who led the study of the man’s brain, put it, "It’s as if a radio were turned to such a low volume that you couldn’t hear the music distinctly." He added, "The scans confirmed our expectation that this patient had a greater capacity for language than he demonstrated."

In August, Schiff, Giacino, Joseph Fins, and Ali Rezai, a neurosurgeon at the Cleveland Clinic, along with 12 other researchers, published an article about the case in Nature. The researchers described implanting electrodes in the man’s thalamus, which, by stimulating the brain tissue, had enabled him to regain considerable physical and mental function. "Deep brain stimulation can promote significant late functional recovery from severe traumatic brain injury," they wrote. When the electrodes were turned on in the man’s thalamus, his speech improved, his movements became more fluid, and he was able to chew and swallow. When the researchers turned off the electrical stimulation, the man soon relapsed. He is now being given regular doses of electrical stimulation and is able to speak in short sentences and to chew and swallow. The researchers concluded that the case "challenges the existing practice of early treatment discontinuation" for minimally conscious patients who show some "interactive behaviours."

Few vegetative or minimally conscious patients ever recover fully, and many are unlikely to improve. (Some neurologists estimate that an adult who has been vegetative for 6 months following a traumatic brain injury has only a 20% chance of regaining consciousness.) For the past 3 years, Schiff and Fins have been studying the brain of Terry Wallis, a 43-year-old man in rural Arkansas who had been the subject of national news stories in 2003, when it was reported that he had begun to speak after spending 19 years in a nursing home, in a minimally conscious state. Schiff and Fins contacted Wallis’s family and offered to help him obtain medical care during his recovery, and to use brain scans to document his progress. In 1984, Wallis, a 19-year-old truck mechanic, had been in a car accident and sustained a severe brain injury; he was also paralysed. Wallis’s father had asked the nursing home to arrange an evaluation of his son by a neurologist, but was told that such an assessment was too expensive and, in any case, would not be useful. In 2003, when Wallis began to speak, he received 12 weeks of physical therapy, which was covered by Medicaid, but the Arkansas Department of Health and Human Services rejected his request for further treatment, concluding that he had not made sufficient progress. One day, in 2005, Fins, who had contacted Wallis’s congressman to solicit his help in obtaining additional medical care for Wallis, asked Mrs Wallis for her son’s Social Security number. "I was on the phone, and Mrs Wallis said to Terry, ‘What’s your Social Security number?’ " Fins recalled. "He gives his number, and I write it down. And I said, ‘Mrs Wallis, was that Terry?’ And she said, ‘Yup. The first time he told us his Social Security, we thought he was wrong. But we looked it up, and he was right.’ "

Fins was astonished. Not only has Wallis recovered memories from his life before the accident but, Fins said, "he is picking up American culture. He now knows the song ‘Bad boys, bad boys, what are you gonna do.’ Why is that important? It’s important because that song didn’t exist in 1984, so Terry is laying down new memories. It shows sustained improvement." In 2006, Schiff arranged for Wallis to be taken to Weill Cornell Medical College, where he examined his brain using a sophisticated technique called diffusion tensor imaging, which assesses the number and health of axons, long fibres that transmit nerve impulses from one brain cell to another. The scans suggested that the axons in Wallis’s brain were growing and forming new connections - a finding that contradicts the long-standing assumption that a damaged brain is incapable of healing after such a lengthy period. "We need to do longitudinal studies, to see if these kinds of changes are accruing over time, whether they happen frequently or infrequently, and what their association with the patient’s level of function is," Schiff told me. In some cases, he speculated, the brain may sometimes be able to bypass an injured area and devise novel ways of connecting axons. Still, he went on, much about Wallis’s recovery - and the neurological developments that are driving it - remains a mystery. "After 19 years, Terry spoke a few words, but within 72 hours he recovered fluent, expressive, and receptive language," Schiff said.

Kate Bainbridge, the first vegetative patient that Adrian Owen studied in Cambridge, has also made considerable progress, recovering the use of her arms, and much of her mental function, although she is unable to walk. She still has difficulty talking, and uses a letter board to communicate with people who are not used to her speech. "Most scans show what is wrong with your brain, which doctors need to know," Bainbridge wrote to me in an e-mail. "But Adrian Owen’s scans show what is working. I say they found parts of my brain were working. It really scares me to think what might have happened to me if I had not had the scans. They show people it was worth carrying on even though my body was unresponsive."

Source: newyorker.com 15 October 2007

Whose Wishes Should Prevail?

Some people feel that decisions regarding human death must consider God’s wishes, though God’s exact desires may sometimes be in dispute. Other people disbelieve the existence of an Omnipotent Personal Creator. This group must use different criteria than "God’s will" in determining the best course of action to follow when patients, finding life unbearable, turn to their doctors and plead for death. The scene modern man dreads is to find himself in a hospital, desperately ill and alone, by his side a respirator supplementing his breathing, tubes in his nose. As death approaches, he has lost control of life. Should he be kept alive if, without intervention, he would surely die? To properly answer this question, one must first ask: Is the man’s life unbearable? Can no improvement be expected? Is there no doubt as to his desires? If all questions can be answered in the affirmative, then the patient should be allowed to die.

The easiest way to know a person finds life unbearable is to hear him say it repeatedly over time. Often, extreme pain causes a desire for death. Total paralysis motivates others to beg to be allowed to die. Some patients even ask their doctors to speed death along. Should doctors go so far as to occasionally be ministers, not of cures, but of easeful death? For terminal patients, public opinion overwhelmingly sides with withdrawal of all "invasive" and "extraordinary" treatment, but most stop short of approving active assistance to help patients achieve death because of the huge potential for abuse.

Another important consideration for a decision maker is whether the hopeless and/or unbearable condition of the patient’s life is permanent. If a person in extreme discomfort begs for death but there is a real chance for at least some relief in the future, it is best to delay. A few years ago, two friends were lost, starving, and freezing in a remote wilderness. One asked the other to shoot him to avoid what looked to be a sure, slow, pain-filled death. Ever the friend, his companion complied. The sound of the gunshot brought rescuers. The survivor was convicted of manslaughter and sent to prison. So first ask, "Have all options been tried?" Sometimes new medicines bring perhaps not a cure, but at least sufficient relief.

Last, has the patient filed an advance directive? If not, is he lucid? Otherwise, can anyone say on behalf of a patient unable to speak for himself that his life is not worth living? It seems better to err on the side of caution! In Martin Scorsese’s movie Bringing Out the Dead, a paramedic imagined he heard a comatose man beg to be allowed to die, so the paramedic interrupted the man’s life support system, resulting in the death of the patient. Imagining someone wishes for death should be insufficient grounds for terminating life by anyone’s standards! For those who cannot speak, the basis of life-and-death decisions should be the balance of burdens and benefits to the patient of a certain course of treatment.

Developed countries have hidden death away. Most deaths occur in hospitals, behind closed doors. Yet society has a legitimate interest in death. Not many years ago, the whole village, not just a dying man's family, gathered around to give him strength. If societies can no longer agree on the meaning or purpose of death, they can still provide a framework to allay the fears of the dying about what will happen in what is left for them of this world. Each case should be decided on clearly-defined merits. A dying man deserves this much.