Maggie didn't make her trip to Weill Cornell Medical College to participate in studies of patients with severe brain injury. Left partially paralyzed and perhaps even permanently unconscious more than a year after a brainstem stroke during her senior year at Smith College, she was now fighting her third bout of pneumonia in succession. Her mother Nancy called to tell us she had to cancel the visit. Maggie was just too sick and the doctors at Good Samaritan Medical Center in Brockton, Massachusetts, wanted to reinsert a tracheostomy tube to help her breathe.
The procedure is relatively simple and safe. A small incision is placed into the neck just below the Adam's apple and a plastic tube is inserted to create a new airway to the trachea and lungs. Just twenty minutes in the operating room, but Nancy agonized over the decision. As she saw it, it was a step backward. Maggie had a trach placed weeks after her stroke in May 2006. That tube served its purpose and had been removed. Unfortunately, Maggie had not regained a level of consciousness that would help her manage her secretions. Instead of safely swallowing them, they would pool in her mouth and eventually lodge in her lungs, setting up infections.
Nancy worried that by agreeing to the tube she was destining her child to a return to a breathing machine, which could be the next step in a series of medical escalations that were seemingly excessive in light of Maggie's utter lack of responsiveness. But the doctors were pushing hard for the trach tube and a bronchoscopy to take a look at the lungs. Harmless enough, but they would have to put her on a breathing machine to do the bronchoscopy. The pressure was intense. The doctors told her, “she's going to die, not for sure, but this is going to happen if you don't do something.”
Nancy was close to saying no and letting this end peacefully. She had watched her beautiful daughter, the child who had dreamt of becoming a veterinarian, traveled to Spain, and had amazing friends, struck down before her prime. In a photo taken before the stroke her eyes shone brightly full of hope and promise. She was now bald and swollen with eyes that were barely open.
Images of Choice
Before I left the conference, and my visit with Cindy Sykes, she shared one more observation about Heather's care, which really encapsulated the challenge of prognostication, discerning who will do well and who not. This is a critical task for families as they try and make decisions about care. Having a better sense of possible outcomes is necessary to avoid the heartache of disappointment that comes with unfounded optimism or the lost opportunity for recovery that attends unsubstantiated pessimism. Early during Heather's course, Cindy recalled speaking with one of the doctors at the academic medical center where she was treated who described the predicament. In a confessional tone he shared a secret, most outsiders would not suspect: experts aren't very good at prognosticating, even when they make use of neuroimaging techniques. In fact, sometimes the scans can be very misleading. The doctor told Cindy, “We can look at two patients’ scans and one can look very bad and bleak like Heather's, and the other can look very promising, and the two have absolutely opposite outcomes. The one that looked good doesn't do well and the one that looked terrible does very well.”
Cindy's response to the predicament was both generous and illustrative of the challenges these families face. Generous in that she felt that both of “… those two patients should have the same opportunity to show their determination against the odds.” And illustrative of her experience, the challenge of making choices without being adequately informed about how things might turn out. Without the advantage of prognostic knowledge gleaned from science, one had to give the two patients both a chance. It was only fair because their futures were indistinguishable.
But Cindy's caution raised a deeper question of whether or not these families have been deprived of the right to informed consent and refusal. After all, how could they engage in informed decision making without the requisite amount of information they would need to make a choice? All the more so in the face of indeterminacy, that middle group referred to by Dr. Plum in the 1970s, when the outcome is unpredictable.
We Should Be So Lucky
“Indulge me,” I remember asking the research team. It was winter 2003 and we were at a round table in the corner of Weill Cornell's Griffis Faculty Club. Niko, Joe Giacino, the surgeon, and I were finalizing the research protocol that would eventually be approved by the institutional review boards at JFK-Johnson, the Cleveland Clinic, and Weill Cornell.– It would also inform the basis for an application for investigational device exemption (IDE) from the Food and Drug Administration. IDEs are required when a new device is tested or when an established device is proposed for a novel purpose. Our plan was to use a DBS device routinely used to treat Parkinson's disease in our study to address the impairments seen in the minimally conscious state.–
By this time, the details of the protocol were being finalized. We decided to restrict the study to individuals who were MCS secondary to TBI. We agreed that going into patients with a higher level of function put them at risk of incremental harm when exposed to an intervention that was experimental and of unclear benefit. We specified traumatic brain injury over other etiologies because of their more favorable prognosis, as compared to anoxic injury. Inclusion was limited to patients from eighteen to fifty-five years, so as not to have data complicated by either the developmental processes of a younger brain or the degenerative ones of an older one. All subjects had to be past the one-year postinjury mark for study inclusion so as to not confuse the natural process of recovery, which can occur up to a year in TBI, with the facilitated recovery we were trying to prompt using DBS.
I next turned to the remaining ethical issues and explained that we had made a good argument for the use of a surrogate decision maker, the legally authorized representative, to consent to the surgery. But, I asked, what happens if a subject got better and could give his or her own consent?
I walked over to the Neuro Unit at New York–Presbyterian Weill Cornell Medical Center on a cold and wet night to see Maggie after speaking with her mother that morning. It was now February 26, 2008, just a few months shy of the second anniversary of her stroke. As I crossed York Avenue, I thought about all that Nancy had shared with me about her daughter and herself. It was an informative interview, but so unbelievably sad. It was the first time I’d heard Maggie's story.
Nancy filled in the gaps of her biography. She was one of those kids who did it all. Academically strong and involved in enough co-curricular activities to keep several students busy. According to The Providence Journal, she was an honor roll student, dorm president, freshman captain of the crew team, and an Ultimate Frisbee player. She had studied abroad and did a semester in Granada, Spain. A classmate recalls Maggie as someone who “was rarely ever sitting still.” She was a whirlwind.
With no shortage of pride, Nancy told me that Smith College had awarded Maggie her bachelor's degree. Because her stroke occurred at the end of her senior year, she already had enough credits, so she was allowed to graduate. During commencement ceremonies, her classmates wore blue ribbons in solidarity with their friend. Stacey Baird, the president of the Smith class of 2006 and student speaker, dedicated her commencement address to Maggie.
By all accounts Maggie was an exceptional individual. Her friends mourning their early loss wrote essays and poems, all of which indicate the profundity of what a basilar artery stroke can do to injure the brainstem and shatter a promising life. At Middleboro Skilled Care Center, her friends put up one thousand origami paper cranes in her room, which according to Japanese legend promotes the return to health.
Maggie did not get better in time, and like so many others was dispatched to a skilled nursing facility. For her mother, Nancy, it was as much a strategic decision to accept the transfer as a practical one. She was still new to brain injury and being her daughter's advocate. She did not know she could have appealed the denial for more time on the grounds of equity, “… there were lots of people that were there longer and I could have fought for insurance.” But she did not pursue an appeal because she was frustrated by the quality of communication with families, or more specifically profoundly upset by one event. In the context of the Worthen's narrative what upset her seems almost trivial. But in the psyche of Nancy as a mother, it loomed large.
When Maggie was found to be colonized with Methicillin-resistant Staphylococcus Aureus (MRSA), a virulent bacteria that is resistant to most antibiotics, she needed to be isolated from other patients until cleared of the infection. Consequently, Maggie was moved out of her room to another floor where she could have a private room. This is common medical practice.
The only problem was that Nancy did not know about the transfer. When she called up to Maggie's old floor to check in, she was gone and the staff on the next shift did not know where she had been sent. For a brief time, Nancy lost track of her daughter.
It was a little thing in the scope of all that had happened and Maggie was soon found. But this degree of insensitivity was especially upsetting to Nancy. Her daughter had been moved as if her connection to her family did not matter, either to Maggie or to Nancy. But it did.
She had been transported without advance warning: “they didn't tell me so I'd come in and one time I called on one floor and they didn't know who she was, the floor she'd been on. And I'd been in that day and they didn't tell me she was moving.” It was incredibly frustrating, “I'd just had it with the sort of organizational structure of the hospital.”
Stephen Carrier's point is very well taken. Unlike other areas in medicine where terms are more accessible, brain injury remains shrouded in mystery and is obscure to most. One mother of a child who sustained brain injury, herself a physician, confessed that neither she nor the child's father, who was also a doctor, had experience with brain injury. Even they were befuddled by the diagnostic categories. Indeed they were at a disadvantage because the treating physicians “assumed a lot more than we knew, and we didn't.”
Terms like coma, brain death, and persistent vegetative state are used with families with the assumption that they are understood. But in our experience, surrogate decision makers are unprepared for this technical language. The nomenclature is foreign and sometime oxymoronic. How can someone be brain dead and still alive? How is a coma different than the vegetative state? Why is a vegetative state patient unaware when their eyes are open and darting about? Such questions confound daily reality and our usual operating assumptions when dealing with each other.
Surrogates of patients with brain injury have no reason to have become familiar with the complex language that describes their loved one's condition after severe injury. It is not part of routine experience. Yet, clinical terms like coma are used by clinicians with the expectation that they are understood, a potential error in the wake of the Schiavo case in which familiarity with these terms could be mistaken for actual understanding.
Consider Lucy Busby*, whose child, Sharon*, was in the vegetative state. Asked about their understanding of the vegetative state, she responded, “… it was in our minds quite a bit probably because I think it was 2005, that whole incident with Terri Schiavo.” They “followed” that case when Schiavo had been described in the press as being in a variety of conditions, “… in either quote ‘vegetative state’ or ‘minimally conscious state,’ or ‘coma,’ – all those terms were being thrown around back then … so we were pretty familiar with what that was all about.”
By now the scene is familiar and the story predictable. An expectant family is in my office at Weill Cornell Medical College sharing the story about how a son or daughter, husband or wife, mother or father sustained a brain injury. They have come to Cornell to participate in scientific studies designed to understand how the brain recovers from disorders of consciousness, serious conditions like the vegetative and minimally conscious states. Their goals are modest. They want to know if their loved one is aware and if they will get better, be able to understand, speak, and love again.
When they arrive, most are worn out. They are like refugees, having been cast aside by an indifferent health care system that provided brilliant emergent care only to abandon them thereafter. Irrespective of differences in race, ethnicity, class, or state of origin, a stereotypic pattern of neglect emerges.
Although each case is unique, the overall story becomes rather predictable. Families face a pervasive nihilism with practitioners assuming a static notion of brain injury. Despite stunning scientific evidence to the contrary, the prevailing view in the clinic is that all brain injuries are immutable. From this perspective, it is preordained that the injured brain cannot recover and that the humane course is to pursue palliative care, to let nature take its course.
At the bedside, this translates into early – some might say premature – decisions to withhold or withdraw life-sustaining therapies, writing do-not-resuscitate orders or removing of ventilators, even before patients have had a chance to declare themselves. More worrisome have been reports of families urged to turn their loved ones into organ donors before their prognosis is clear.
Most families do choose a palliative course, knowing that even under the most optimistic of scenarios their loved ones will need ongoing medical care and years of rehabilitation. Theirs is a reasonable choice, and perhaps a logical one, appreciating the considerable challenges, when early optimism and the promise of recovery do not get translated into desired outcomes. For these families, death is preferable to survival and what some would describe as an even worse outcome, a diminished or extinguished ability to interact with others.
Tempus Fugit and MCS
During the summer of 2010, I presented a lecture at the Third International Conference on Disorders of Consciousness at the Campus di Baronissi Facolta de Medicina e Cirugia just outside of Salerno, itself an important seat of medical knowledge in medieval Italy. The conference took place about an hour's drive from the spectacular Greco-Roman ruins of Paestum, the Latinized name given to a settlement named for the sea-god Poseidon. Now several kilometers inland, Paestum was fittingly once on the Mediterranean. The passage of millennia has landlocked the ruin and deprived the modern tourist of glimmering seascape vistas that the ancients once enjoyed. Today, the visitor can only imagine that the sea lies just beyond the next archaeological mound.
The conference's proximity to Paestum was fitting because the most famous of its findings is the ancient Tomb of the Diver. On a series of white slabs, from which his coffin is made, the tomb depicts the passage of a young man to the afterlife. En route, he attends a symposium, embarks on a procession, and – most remarkably – dives naked into an awaiting pool from the Pillars of Hercules (see Figure 3).
The image captures the diver in mid-flight suspended in a moment. Despite the image's flat relief, its ancient artisan depicts time as a fourth dimension. By freezing the young man between life and death, he is “forever overhead” as the archaeologists S. DiGregorio and M. T. Granese note. DiGregorio and Granese describe the diver's portrayal as “the unmeasureable interval between and unrepeatable before and unimaginable after.”
An Eternal Present
When I heard their account of the diver, I was reminded of Augustine's conception of an “eternal present” in which the deity is similarly suspended in all time. Like the diver's moment, this too is an “unmeasureable interval” because it is an infinite convergence of past, present, and future.
A Syndrome without a Name
The persistent vegetative state (PVS) was first described – and given its name – in 1972 in the prestigious British medical journal, The Lancet, by the Scottish neurosurgeon Bryan Jennett, known for creating the Glasgow Coma Scale, and the American neurologist Fred Plum, who less than a decade earlier had described the Locked-in-State with his longtime colleague Jerome B. Posner.
Dr. Plum was the revered chairman of neurology at the then New York Hospital-Cornell Medical Center where he was my teacher and eventual colleague. He was an astute observer of detail who had the ability to synthesize information across the neurosciences through beautifully executed prose.
One example of his extraordinary fluency was the paper on PVS he wrote with Dr. Jennett. In the most parsimonious of phrases, Jennett and Plum, described the vegetative state as a state of “wakefulness without awareness.” By that, they meant to describe the paradoxical state of what Plum would also describe as “wakeful unresponsiveness,” a state of unconsciousness in which the eyes are open but there is no awareness of self, others, or the environment. It is paradoxical because we arise out of our nightly slumber by opening our eyes and taking in the world. We express ourselves with our eyes. They are the portal to awareness, communication, and humanity community. That is, except for in the vegetative state when the patient is awake but the eyes are unaware and undiscerning. They move randomly about the room with neither intent nor direction like an unmanned sail boat on a gusty day. These usually sentient orbs are now propelled by the primal forces of an intact brainstem that charts no course but simply keeps the body afloat, directing autonomic function like our heart beat, breathing, and sleep-wake cycles.
According to his mother, Corinth Pecco, Greg was a sharp dresser who liked nice things and always had a good job. He had a number of girlfriends and was a sweet guy. He never got into trouble and did not like to fight. But that, unfortunately, made him “an easy catch” for local hoodlums who viciously attacked him. Greg, whose nickname was Freedom, was thirty-eight, unmarried, and with a young daughter when he was assaulted.
As his mother told us, his attackers “got high and they went out and riding around and they saw Freedom and they know what type of person he is.” And she continues with resignation, “They knew he wasn't going to fight.”
When Corinth arrived at the hospital, Greg's father told her how badly their son was beaten. “His brain was smashed in” and he was not expected to live. The situation was so bad that it was initially thought he was brain dead when he arrived in the emergency room of a local hospital. According to Corinth, “… they said, ‘DOA.’ That he was like dead … brain dead.”
It took her aback because she hadn't imagined Greg's injury was serious. She remembers that when she first got the call from the hospital, everything “was going well, I mean nothing like this happened before this happened, so I paid it no mind. Believe it or not … I got my nails done. I went to the hairdresser and got my hair done. Then [I thought] I'll stop by the hospital ‘cause Greg has never been in any problem, never had any problems with him, so you wouldn't think of anything.”
The medical record confirms his dire condition. The assault had resulted in a closed head injury to the right frontal lobe, which caused bilateral subdural hematomas, or blood collections. These collections, in turn, caused increased pressure within the brain and significant degrees of herniation, the actual expulsion of the brain out of the skull through its large opening at its base where the spinal cord connects to the brain. He had lost his light reflex in his right pupil, a sign that he had herniated and his status was grim, just above brain death.
When Lyndon Johnson nominated Thurgood Marshall to the Supreme Court as its first black justice, Marshall thanked the president for being in command of history. He told LBJ, “You didn't wait for the times. You made them.” Robert Caro, Johnson's biographer, feels that Marshall's gratitude to the president was not for his elevation to the court but rather for Johnson's forward-looking stance on American civil rights.
To date, we have been waiting for the times to change before we change our attitudes and practices toward those who are minimally conscious. Ours is a moment of history that calls to collectively take control. We need to make the times our own and respond to what interdisciplinary study in the neurosciences and the medical humanities is teaching us about the experiences of those in a minimally conscious state. It is time that rights come to mind.
To do this we must build upon the disability rights movement with its deep debt to its predecessor movements and affirm consciousness as a right that must be recognized, respected, and enabled. It will foster productive science and rectify societal deficiencies that remain untenable, if not inhumane. The goal is a nascent social movement that will place the needs of these patients and families on the legislative agenda, so that in a bipartisan fashion they can be fully protected under the law and receive the care and support that they need.
To begin we need our generation of Freedom Riders, those who won't wait for the times but make them their own. These advocates will join in the long march of a new civil and disability rights struggle. Like their predecessors they will be motivated by a sense of what is right and the need to rectify injustice. But their journey will be different.
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