A Hopeful Departure

Maggie was one of the lucky ones. She was ready to leave the hospital and she got a chance to go to a brain rehabilitation program. So with tracheostomy and stomach tubes in place, Maggie was discharged from the hospital and transferred to Spaulding Rehabilitation Hospital in Boston. Spaulding, a Harvard affiliate, is one of the nation's leading programs for brain rehabilitation, and Nancy was hopeful that Maggie might qualify for transfer. Not everyone gets into such rarefied places. They were reserved for those families who knew of their existence and had injuries that might be amenable to whatever rehab they could offer.

At the end of her acute hospitalization, her clinical staff thought that Maggie might be in a minimally conscious state (MCS). (Nancy later recalled that was when she first heard of that diagnostic phrase.) Spaulding had a special program for patients who were in MCS. It was not certain, Nancy recalled, “… but they tested her because they decided that there was a possibility that she would have some consciousness.” Spaulding accepted her into their eight-week program.

Nancy was genuinely happy because the transfer provided a glimmer of hope. It meant that the doctors were wondering if she was not actually vegetative. It was a small opening to a bigger future, but hopeful nonetheless. That eight-week acceptance brought joy because it conveyed the sense that the doctors thought that some degree of recovery was still possible. As Nancy told us, “… It was a hopeful thing just for them to accept her into the minimally conscious” program because it meant that the doctors either knew or suspected that she was minimally conscious as opposed to vegetative. Nancy had learned by now that was a difference that would make a difference.

Good to Go

Not all patients are ready or stable enough to leave the hospital and fewer get to go to a rehabilitation center, ending up instead in a nursing home. As Burt Brody, whose wife Jean collapsed in his arms with a brain aneurysm, began to understand placement options he put it this way, “To learn the terms acute and subacute are almost contradictory to what they really are.

A Consensus on PVS

Beyond all the media hype, and faux explications invoking all manner of pseudo-science, how could a patient, like Terry, diagnosed as vegetative for so long, recover so dramatically? There were emerging prognostic rules about such things, and Terry had seemingly broken all of them, regaining consciousness so late in the game. No one came out of the vegetative state after nineteen years. It was impossible, and not what the experts had agreed upon back in 1994 when a definitive set of consensus papers were published in the New England Journal of Medicine by leading representatives of clinical societies concerned with brain states.

Under the banner of a “Multi-Society Task Force,” these expert neurologists, physiatrists, neurosurgeons, and others laid out such parameters about the vegetative state. A central feature of their deliberations was the recognition that brain injuries were not inherently fixed and immutable, but sometimes brain states could, over time, lead to the recovery of consciousness.

They agreed that when a vegetative state continues beyond thirty days, it is described as persistent and that permanence sets in three months after anoxic injury (severe oxygen deprivation) and twelve months after the insult resulted from trauma. Presciently, the authors of the document worried about being too definitive about predictions, accounting for the possibility of both error and ignorance of our future state of knowledge. To their credit, they hedged just a bit on the question of permanence, making it a more probabilistic statement than an outright certainty. In a brilliant distinction they observed that:

In retrospect, one reason Terry could have breached these categories was diagnostic error. Bryan Jennett, the co-originator of the vegetative state with Dr. Plum, later put it this way, “Some alleged late recoveries might in fact have been late discoveries of earlier recovery.”

Nancy's Hopes

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.

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.

Voice Lessons

Was Nancy comforted that Maggie had begun to communicate? Less than I thought she would be. I had expected that she would be thrilled that Maggie had reached this longed-for elusive milestone. But in fact, Nancy was incredibly frustrated, even alarmed that Maggie had access to communication as infrequently as she did.

It seemed that Maggie's ability to communicate, as intermittent as it, was most dependent upon the reciprocal skill of a speech therapist who visits Middleboro three times a week. According to Nancy, she “is the only one who communicates with her.”

So was it comforting that at least it happened on those occasions?

Nancy tells us, “I mean it's comforting to know that it can happen, but kind of frightening to know that it only happens a few times a week when the speech therapist is with her. …” And then, “… not regularly, partly because Margaret hasn't been consistent …” in her pattern of communication. Sometimes the therapist shows up and Maggie won't communicate. Other times when she might, the therapist is not there.

Imagine the scenario, first you learn that your daughter is conscious and not vegetative, that she can understand and sometimes communicate. Then you discover that those precious opportunities are limited to but a few hours a week. And in those other moments, you suspect that she might be able to communicate, if given the opportunity. But until that happens, she is silenced.

Nancy is frustrated and she believes that Maggie is as well. She also thinks that her daughter is angry and depressed too. Nancy told us, “So I mean I'm frustrated … how can I really be happy about what's happening with Margaret when it's so limited. …” Like many patients with disorders of consciousness, Maggie finds herself wholly dependent upon others and/or machinery to regain her voice, to have the ability to communicate with others. To express a thought, tell us she is in pain or hungry, or tell someone that she loves them – all of that self-expression is dependent upon the resources necessary to pay a speech therapist (or support a run in the scanner).

Homecoming

Perhaps to buffer against untoward communication, and to ease the shock of appreciating the degree to which her own child was injured, Nancy was met at the hospital by a couple of girlfriends upon her return from London. Her ex-husband had already left after a long night to get some rest.

Nancy knew Maggie had had a stroke and an operation to remove a clot but neither appreciated the gravity of her situation nor the details of the surgery. As a “really good social worker” and doctor explained, Maggie's stroke was a consequence of a blood clot in the basilar artery in the back of her head just above the spine. It is a crucial vessel that supplies blood to the all-important brainstem, whose essential functions are responsible for reflexes and breathing and all the sorts of things that make someone awake.

The clinical team that met with Nancy were initially circumspect about Maggie's prognosis. At the outset she was being treated for brain swelling that “could kill her.” Maggie overcame these early hurdles and the threat to her survival subsided. And as the risk of brain swelling faded, the question of whether she would live or die was replaced by the harder question of the kind of life she might have.

Maggie was at a pivotal junction in her care. She was leaving the acute care heroics of emergency rooms, neurosurgery, and high-tech interventions and transiting into the realm of decisions that would most likely destine her to a life in chronic care, of nursing homes and dependency. Several days after her stroke and into this period of relative stabilization, Nancy began to ask the doctors caring for Maggie about their views of her future. One of the doctors urged her to forgo the pivotal decision to put in her first tracheostomy and feeding tubes that would help her breathe and eat. He told Nancy that, “I needed to look at the quality of life that she would have and what she would want.” He also brought up the question of resource allocation and “they talked about even the resources that would be used.”

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.

Finding Placement

Finding placement for a MCS patient who is severely disabled but still conscious can be difficult for social workers. Paradoxically, it is sometimes easier to find a bed if the patient is vegetative and not in MCS. The “better” diagnosis would require more specialized placement where additional elements of care such as cognitive rehabilitation and physical therapy would be available. The more gravely injured patient, ironically, places less burden on overtaxed facilities.

In one instance, I heard of a case in which a patient with traumatic brain injury, Heather Sykes, was alleged to have been deliberately misdiagnosed, or at least mislabeled as vegetative, in order to facilitate discharge to chronic care. According to her mother, Cynthia (Cindy) Sykes's retelling of her story, she was discharged from the hospital diagnosed as being in the permanent vegetative state, even though Cindy did not believe it.

I met Cindy when we were both speakers at a regional conference on TBI in the fall of 2007. She and an advanced practice nurse partnered up to tell Heather's story. It was a compelling account consistent in many ways with what I had learned from the families I had been interviewing at Cornell. As with Terry Wallis, and so many other families we have interviewed, Cindy thought she saw purposeful behaviors that would give Heather an MCS and not a vegetative diagnosis.

And that is where our experiences diverged because Cindy alleged that her daughter's misdiagnosis was more than the all too common error of omission where an MCS patient is mistaken as vegetative. Instead, she asserted that her ongoing vegetative diagnosis had became one of commission, a deliberate attempt to misrepresent her condition, and chronic care needs, in order to facilitate Heather's nursing home placement. If true, it would be an act that some might construe as diagnostic fraud orchestrated to deal with a chronic care system unprepared to accept seriously disabled but conscious patients. It is a sobering and sad story that I share with the generous permission of Cindy Sykes.

A Bad Wreck

Terry Wallis and his friend Chubb Moore were driving their pickup truck in the Ozarks during the early morning of July 13, 1984. There had been drinking and there was a crash. No one knows who was at the wheel, but as Terry's mother Angilee Wallis told us, it was a bad wreck, “one of the boys was thrown from the car and the other was hanging out the window.” They were taken to Harrison Hospital and then medevaced to St. John's Hospital in Springfield, Missouri.

During the four-hour car ride to Springfield, Angilee did not know if when she arrived she would find her son alive, “… they just told us that they were really sick boys and they didn't know if they'd be alive when we got there or not. And they did tell us … that he had a head injury, but at the time they had Terry and Chubb confused … they didn't have wallets on them or I don't know exactly what it was.”

Angilee took it that Terry was doing better than Chubb and that Terry's friend was the one with the head injury. She recalls, “… on the way to Springfield I was sitting in the back seat with Chubb's mother and I thought, I couldn't imagine Terry laying even in bed, I couldn't imagine him even being in the hospital because he was always huntin’, and fishin’, and mechanicin’, just an outdoor person. And I was feeling really bad for Chubb's mother because they were talking about how bad he was … I was feeling, actually feeling guilty because I was so happy Terry wasn't hurt as bad as Chubb was. Even though they said both of them was hurt really bad.”

When the families arrived at the hospital it soon became apparent that the boys had been mixed up. Terry was more grievously injured. Angilee was not angered by the error, she was grateful that Terry was still alive, but not yet registering the significance of his having sustained a brain injury.

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?