Do No Harm Page 2

  A YEAR OR SO before Patrick's operation, a psychologist asked him if he would take a pill to make his BIID go away, should such a treatment exist. It took a moment for him to reflect and answer: maybe when he had been a lot younger, but not anymore. “This has become the core of who and what I am,” he said.

  This is who I am. Everyone with BIID that I have interviewed or heard about uses some variation on those words to describe their condition. When they envision themselves whole and complete, that image does not include parts of their limbs. “It seems like my body stops mid-thigh of my right leg,” Furth told the makers of a 2000 BBC documentary, Complete Obsession. “The rest is not me.”

  In the same film, the Scottish surgeon Robert Smith tells an interviewer: “I have become convinced over the years that there is a small group of patients who genuinely feel that their body is incomplete with their normal complement of four limbs.”

  It’s difficult for most of us to relate to a notion like this. Your sense of self, like mine, is probably tied to a body that has its entire complement of limbs. I can’t bear the thought of someone taking a scalpel to my thigh. It’s my thigh. I take that sense of ownership for granted. This isn’t the case for BIID sufferers, and it wasn’t the case for David. When I asked him to describe how his leg felt, he said, “It feels like my soul doesn’t extend into it.”

  Neuroscience has shown us over the past decade or so that this sense of ownership over our body parts is strangely malleable, even among normal healthy people. In 1998, cognitive scientists at Carnegie Mellon University in Pittsburgh performed a deceptively simple experiment. They sat subjects down at a table with their left hands resting on a table. A screen prevented the subjects from seeing their hands: instead, a rubber hand was placed in front of the screen. The researchers then used two small paint brushes to stroke both the real hand and the rubber hand at the same time. When questioned later, the subjects said that they eventually felt the brush not on their real hand, but on the rubber hand. More significantly, many said they felt as if the rubber hand was their own.

  The rubber-hand illusion illustrates how the way we identify our body parts is a dynamic process, one that involves constant integration of various senses. Visual and tactile information, along with sensations from joints, tendons and muscles, gives us an internal sense of the relative position of our body parts.

  This feeling is a crucial component of our sense of self: it’s about my body, my thoughts, my actions. It’s only when the process that creates this sense of ownership goes awry, for example when the brain receives conflicting sense information — as in the rubber-hand illusion — that we notice something is amiss.

  And if we can feel as if we own something as inanimate as a rubber hand, can we own something that doesn’t exist? Seemingly, yes. Patients who have lost a limb can sometimes sense its presence, often immediately after surgery and at times even years after the amputation. In 1871, an American physician named Silas Weir Mitchell coined the phrase “phantom limb” for such a sensation. Some patients can even feel pain in their phantom limbs. By the early 1990s, it was established that phantom limbs were an artefact of body representation in the brain gone wrong.

  The idea that our brain creates maps or representations of the body emerged in the 1930s, when Wilder Penfield probed the brains of conscious patients who were undergoing neurosurgery for severe epilepsy. He found that each part of the body’s outer surface has its counterpart on the surface of the cortex: the more sensitive the body part—say, hands and fingers, or the face—the larger the brain area devoted to it. As it turns out, the brain maps far more than just the body’s outer surface. According to neuroscientists, the brain creates maps for everything we perceive, from our bodies (both the external surface and the interior tissues) to attributes of the external world. These maps compose the objects of consciousness.

  The presence of such maps can explain phantom limbs. Though patients have lost a limb, the cortical maps sometimes remain—intact, fragmented or modified—and they can lead to the perception of a limb, with its potential to feel pain. Even people born without limbs can experience phantom arms or legs. In 2000, Brugger wrote about a 44-year-old highly-educated woman, born without forearms and legs, who nonetheless had experienced them as phantom limbs for as long as she could remember. Using functional magnetic resonance imaging and transcranial magnetic stimulation, Brugger’s team verified her subjective experience of phantom limbs and showed that body parts that were absent from birth could still be represented in sensory and motor cortices. “These phantoms of congenitally absent limbs are animation without incarnation,” Brugger told me. “Nothing had ever turned into flesh and bones.” The brain had the maps for the missing body parts, even though the actual limbs had failed to develop.

  When confronted with BIID, Brugger saw parallels to what the 44-year-old woman experienced. “There must be the converse, which is an incarnation without animation,” he said. “And this is BIID.” The body had developed fully, but somehow its representation in the brain was incomplete. The maps for a part of a limb or limbs were compromised.

  Recent studies have borne out this idea. Neuroscientists are particularly interested in the right superior parietal lobule (SPL), a brain region thought to be vital to the construction of body maps.

  Brugger has found that this area is thinner in those with BIID, and others have shown that it may be functioning differently in those with the condition. In 2008, Paul McGeoch and V.S. Ramachandran of the University of California, San Diego, mapped the activity in the brains of four BIID patients.

  The researchers tapped the feet of the control subjects and watched the SPL light up. But the BIID patients were different: the right SPL showed reduced activity when the disowned foot was tapped, only lighting up normally when the tap was on the other foot.

  “What we argue is that in these people something has gone wrong in the development, either congenitally or in the early development, of this part of the brain,” McGeoch told me. “This limb is not adequately represented. They find themselves in a state of conflict, a state of mismatch that they can see and feel.”

  There are almost certainly other brain regions involved. Last year, scientists reviewed a number of “body-ownership” experiments, including the rubber hand illusion, and identified a network of brain regions that integrates sensory data related to maps of our body, its immediate surroundings and the movement of our body parts. These regions, they suggest, are responsible for what they call the “body-matrix”—a sense of our physical body and the immediate space around it. The network maintains the integrity of the body-matrix, and reacts to anything that threatens its integrity and stability.

  Intriguingly, the physical differences in the brains of BIID patients that Brugger identified include changes in nearly all the parts of this network. Could BIID result from alterations to this body-matrix network? Brugger’s team thinks so.

  It’s crucial to emphasise that these findings are correlations—they don’t address causality. Could a lifetime of thinking about amputating a leg lead to these brain changes? Or were these brain differences driving the desire? These studies cannot yet answer such questions.

  Then there is the issue of how body states and body-matrix networks translate into a sense of self. And, for BIID patients, how a skewed body map leads to the desire for amputation.

  “‘Owning’ your body, its sensations, and its various parts is fundamental to the feeling of being someone,” the philosopher Thomas Metzinger has written. He argues that our brain creates a phenomenal self-model (PSM), and the content of the PSM is our ego, our identity as subjectively experienced. If something is in the PSM, it belongs to me. If it’s not, then it’s not me. The rubber hand experiment works because it modifies the PSM: the brain replaces our real hand with the rubber hand, which is now embedded in the self-model. And since anything in the PSM has the subjective property of mineness, we feel as if the rubber hand belongs to us. In BIID, it’s likely that a
limb or some other body part is misrepresented or underrepresented in the PSM. Lacking the property of mineness, it is disowned.

  Therein lies a clue to why someone with BIID might want to amputate a limb that doesn’t feel like it belongs. My self—as defined by the content of the PSM—is not just my subjective identity; it also the basis for the boundary between what’s mine and everything else. “It’s a tool and a weapon," said Metzinger, when we spoke on the telephone. "It’s something that evolved to constantly preserve and sustain and defend the integrity of the overall organism, and that includes drawing a line between me and not-me, on very many different functional levels. If there is a misrepresentation in the brain that tells you this is not your limb, it follows that phenomenologically this will be a permanently alarming situation.”

  The debate rages on over whether amputation is ethical. In the meantime, BIID sufferers often take treatment into their own hands.

  §

  Visceral negative reactions are common when people first hear about voluntary amputations. Twelve years ago, when media attention to the condition was at its height, bioethicist Arthur Caplan, then of the University of Pennsylvania, called it “absolute, utter lunacy to go along with a request to maim somebody.”

  More than a decade later, there is still argument in the pages of academic journals about the ethics of voluntary amputations. Is it analogous to other body-modifying cosmetic surgeries, such as breast reduction? Some bioethicists say no, since amputation entails a permanent disability. Others point out that cosmetic surgery can also be disabling, as when breast reduction results in the inability to breastfeed. Or should BIID be compared to anorexia nervosa as the best, if somewhat imperfect, analogy, because both involve body-image discrepancies? According to this line of argument, amputations should be denied just as anorexics are sometimes fed against their will. The retort to this is that anorexics are clearly delusional about their bodies, as objective measures can show their body weight to be dangerously low. There is no such objective measure of a BIID patient’s internal feeling of bodily mismatch.

  The debate continues, partly because BIID is not a medically recognised disorder. There’s also a lack of data about how voluntary amputations affect the lives of patients. Yet David’s surgeon, an orthopaedic specialist, has made up his mind.

  Dr Lee — which is not his real name — is in his mid-40s, friendly, with an easy laugh. He seems at peace with his secret practice. When a BIID patient first approached him four years ago, he had had his doubts, so he researched the condition as thoroughly as he could and communicated with the patient for several months before deciding to do the amputation. He knew he would be risking his medical license. A religious man, he and his wife even prayed on it, eventually putting some of the onus of the decision on higher powers. “God, if you think this is not right, then put some hindrance,” he remembers thinking. “I don’t know what it is, but put some hindrance.” So far things have gone smoothly, and he’s taking that as divine sanction.

  Dr Lee is convinced that what he does is ethical. He has no doubt that BIID patients are suffering deeply. On the question of whether to amputate to relieve their pain, he invokes the WHO’s definition of health: a state of complete physical, mental and social well-being and not merely the absence of disease or infirmity. As far as he can tell, people with BIID are not healthy, there is no non-surgical cure in sight, and no evidence that psychotherapy helps. Michael First, in his 2005 survey of 52 BIID patients, reported that 65 percent of them had seen psychotherapists, but it had no effect on their desire for amputation (though it’s also true that half of them did not tell their psychotherapists about such desires).

  Of course, there’s also the question of whether BIID sufferers are psychotic or delusional. Again, the scientists who have studied these individuals say that they are neither. Dr Lee insisted that his patients have not been psychotic. On the contrary, he said, many of them were high-functioning individuals, including a pilot, an architect and a doctor. And for Dr Lee the proof is in the near-instant change he has observed in his patients after the surgery, which contrasts strongly with those who have to undergo involuntary amputations because of, say, a car accident. Involuntary amputations are traumatic to even the strongest of people, and they can become severely depressed as a result. “Then you have these BIID people who crutch unbelievably after the first day after surgery.”

  Paul McGeoch, who has studied his fair share of BIID patients, has the same opinion. “They are universally happy. I have never heard of one who is not pleased to have a limb amputated,” he said. But as convinced as Dr Lee seemed, the surgeon repeatedly stressed: “I’ll stop the moment I get my first patient who feels remorseful about the surgery. So far, none have.”

  If BIID were ever to be legitimised, and voluntary amputations to become legal, Dr Lee knows that his clandestine program would end. “I’d be so glad if ever that happened. I won’t have to deal with the tension anymore,” he said. “Right now, I’m torn between the tension of doing the surgery, and the tension of helping them.” Then, in a momentary lapse of caution, he admitted that he would miss the surgeries: “Maybe that’s the weirdo in me.”

  I asked if he would miss the income, which amounts to about $20,000 per operation. The answer was an emphatic no. He said he made the same amount doing legal surgeries for foreign health tourists and that he had a flourishing local practice. He pointed out that his fee covered everything: hospital costs, payments to his fellow surgeons, even some meals and sightseeing for his patients. “You are not paying for the surgery. You are paying for all the risks involved,” he said. “You have to keep everybody happy. We are not talking peanuts here. If this gets out, we all lose our licenses.” He said it was a risk he’s willing to take, as long as his patients are happy.

  THE MORNING THAT DAVID was scheduled for surgery, I went to meet him and Patrick in their hotel suite. We had flown thousands of miles to be there, in a crowded Asian city. Outside the hotel, the weather was hot and muggy, the traffic heavy. Luxury cars and jalopies jostled for street space alongside buses and two-wheelers. Diesel fumes stung my nostrils. A fetid stream wound its way between high-end hotels and office buildings. Inside the hotel, the wood-panelled suite was air-conditioned, hushed.

  I had spent the night thinking about David’s surgery, and all I had felt was anxiety. I imagined the fear that David must be experiencing: fear of surgery, fear of confrontations with family and friends, fear of disability. But that morning David himself showed no such emotions. He said he had moved beyond those worries. Instead, he fretted about the paperwork. Whom should he put down as emergency contacts? Should he divulge their addresses and phone numbers? Patrick suggested putting down the wrong numbers; maybe change a digit or two. “You’ll have to get used to lying,” he said.

  Questions kept occurring to me. I asked David if he had been evaluated by a psychiatrist. Usually, I knew, Patrick recommended someone for surgery only after a psychiatrist confirmed that he or she was suffering from BIID. David said no. Patrick had used his own judgement in recommending him to the surgeon, saying that he sees himself in David—the same agony, the same mental torture. Plus, David couldn’t afford a psychiatric evaluation. He had to scrape and scrounge and go deeply into debt to come up with the $25,000 needed to cover the surgeon’s fees, the airfare and ten days of hotel accommodation for two.

  Dr Lee had agreed to the surgery based on Patrick’s recommendation. The two had been working together ever since they met via the BIID network about four years ago.

  David was thankful for Dr Lee’s help. “As you know,” he told me in the hotel room. “I was in a DIY mode, where I was going to hurt myself.” Suddenly David started sobbing. Patrick consoled him; David apologised. “Every time I talk about hurting myself, it makes me cry,” he said. David again expressed certainty that if the surgery didn’t go through, he would attempt cutting his leg off himself. “I can’t go on any longer.”

  The surgeon picked us up in
the early afternoon. Given that David’s procedure would require subterfuge to get past hospital staff and nurses, Dr Lee appeared surprisingly calm. “Have to be,” he replied when I asked him later about his demeanor. “Cannot show the patient that I’m nervous.” He drove us to his house, ushered us into the living room and asked David to sit down.

  Dr Lee laid out the plan: he would admit David to hospital, saying he needed surgery for a vascular disorder. The unwitting staff would prepare the patient for an ordinary operation — and then, under the surgical lights, Dr Lee would say that the leg needed removing and conduct the amputation. Inside, the anaesthesiologist and other surgeons would be in on the plan; the nurses would not know.

  Dr Lee laid an old garment on the floor, and set David’s foot on it. Working swiftly, he bandaged the foot, ankle, and calf as a precaution. It wouldn’t do to have curious hospital staff see that the foot was healthy. He wrote the admitting order on his prescription pad and instructed David in the sequence of symptoms he should tell the hospital admissions staff he had endured over the past few days: pain, followed by some cramping, and eventually numbness. The diagnosis that these implied would give Dr Lee the option of amputating during surgery, a judgment that could not be questioned by anyone who wasn’t in the operating room.

  We drove to a small hospital on the outskirts of the city. The high-rise hotels gave way to low-slung buildings and occasional homes with makeshift tin roofs alongside unpaved muddy alleys. The hospital itself was on a major road lined with an odd assortment of shops: a butcher, a pawn shop, an electronics repair outfit and a hairdresser who promised safe and effective hair straightening.

  Dr Lee was not on the staff at this hospital; like many doctors in private practice he had surgical privileges at a number of different hospitals. He dropped us off outside. David, now on crutches, would have to get past the hospital staff. Would they buy his story? We walked into the emergency room. It was a simple affair. Ten iron-frame beds and mattresses covered with spotless sheets were separated by thick curtains. This was not a high-tech, first-world ER, but it was clean and functional.