Coma and vegetative states are confounding for loved ones of brain-injured patients. Dr. Joseph Fins, Chief of Medical Ethics at Weill Cornell Medicine, explains the sobering discovery of hidden consciousness in coma patients and shares a painfully fascinating patient story.
Dr. Stieg: We're back again with Dr. Joseph Fins, Chief of Medical Ethics at Weill Cornell Medicine / New York Presbyterian Hospital. I now want to get into the area of your particular expertise, which is brain injury and altered conscious states to see where medical ethics plays such a dominant role in that whole subject. Can you briefly describe for us what are the guidelines now for brain injury?
Dr. Fins: Right, so the guidelines have just changed in 2018 and one of the changes was in how we characterize brain injury. So let me, let me just go back a little bit and just define some of the categories so we know what we're talking about. Coma is an eyes closed state of unresponsiveness and it's sort of like stunned brain. You're unresponsive, your eyes are closed. Then the first thing that happens when somebody recovers, is that the brainstem, the lower part of the brain that's just above the spinal cord recovers and your eyes are open but they're not seeing eyes. You're unaware, they're kind of roving. You have sleep wake cycles. You might even have a startle reflex and that's the the vegetative state. If it lasts for more than than a month, it's described as persistent and if it lasts for three months after anoxic brain injury, it's permanent and after a year after traumatic brain injury it's permanent and that was sort of where we were until September of 2018 where the the American College of Rehabilitation Medicine, the American Academy of Neurology and the National Institute of Health Institute on Disability and Recovery reformulated the guidelines and they changed the permanent vegetative state to the chronic vegetative state, which means what they said was that looking back at all that data that about 20% of people who were thought to be permanent actually may recover out of the vegetative state and recovery is into the next level up, which is called the minimally conscious state and a minimally conscious state was a brain state.
Dr. Fins: That was described in 2002 and these people often appear vegetative and but they have episodic and intermittent evidence of awareness of their selves, of others or the environment. So they might reach for a cup, they might look up when you come into the room, they might respond to their name. The problem is these behaviors don't always manifest themselves. So you know, if you say," Hey Phil, you know like look up, look up when the doctor comes in." You may not look up, but that didn't mean you didn't look up, you know, three hours ago when the family saw it. But the doctors who come in to see you in the nursing home, which is where these people end up, we'll just discount the family reports is denial or wishful thinking. But the biology of it is that is episodic and intermittent. So here's a number that will give you goosebumps.
Dr. Fins: 41% of people in nursing homes who have had traumatic brain injury who were thought to be in the vegetative state when carefully studied with a bedside exam called the coma recovery scale. 41% of them are actually minimally conscious. So what that means is that we're missing conscious entities four out of 10 times. That also means, and this, this is the thing that you know, gives me goosebumps, is that the vegetative brain cannot perceive pain. We know that from neuroimaging studies, minimally conscious patients do. So that means that four out of 10 times we treat people thinking they cannot perceive pain. So I've heard of stories in the work that I've done speaking to families with loved ones who have a severe brain injury of people actually having procedures done with them to them without anesthesia because it was presumed that they were vegetative and it was presumed because they were vegetative they couldn't feel pain when in fact they were minimally conscious and could perhaps be able to feel pain. Everybody who's listening now knows about this patient in the Hacienda Nursing Home in Arizona, which was a woman who just gave birth and they only discovered she was pregnant when she gave birth so that reflects the neglect of the patient.
Dr. Fins: But she was moaning, so she was experiencing it. So she couldn't have been vegetative, which means she felt pain. And you know what's even more tragic? If she could feel the pain of childbirth, she might've also felt the pain of being sexually assaulted, which is— and she couldn't call out in distress.
Dr. Stieg: A little bit later I want to get into the complexity of their understanding, meaning the emotional aspects. She might also have experienced, aside from the pain of being molested, the pain of birth, but to what degree can we assess their emotion. But I want to stay focused on the 41% is a pretty startling number. So what is, from a patient's family's perspective, what is the doctor's responsibility that they can say, have you done X, Y, and Z?
Dr. Fins: I think, you know, families don't necessarily know enough to know that something it should be happening is happening. I think it's an issue of professional competence and that's one of the things that the, the guidelines, the American Academy of Neurology, ACRM, the NIH, had talked about, you know, physicians knowing about this stuff and adhering to guidelines. That was, those guidelines were based on an evidence-based review, which took five years from these, these three major entities. But I think we have to, we have to understand not only the kind of the knowledge deficit, but why people have allowed that knowledge deficit to continue. I mean if, if people were getting a neurosurgical procedure wrong 40% of the time, you know, when there was, no one had to do it, you'd ask yourself what's, what's the, where's the lesion here, you'd be on the front page of the Times, and why isn't this on the front page of the Times?
Dr. Fins: So the reason for that, and it's sort of how we've thought about severe brain injury in the context of right to die. If I could just veer a little bit into that history, the right to die was established in the context of severe brain injury, in fact in the vegetative state. So going back to the Karen Ann Quinlan case in 1976 is young woman who had presumptive drug overdose, was in a vegetative state and her parents who were strict Catholics actually wanted to remove her ventilator. He was asked by, by Judge Hughes, who was a chief judge of the New Jersey Supreme Court, himself also a Catholic, to testify on Karen Ann Quinlan's brain state. And he testified that she was in the vegetative state and based on being in the vegetative state, Judge Hughes said, that was the moral warrant to allow the family to remove the ventilator and allow her to die.
Dr. Fins: Now of course she didn't die because she was in a vegetative state. She had an intact brainstem. And unlike a brain dead person who didn't have a brainstem, she was able to breathe on her own. She lived for a number of years thereafter. But what happened over the years is that we've acquainted the futility of the, of the vegetative state and over-generalize it to people who look like they're vegetative but they're not. And those are the 40%, the minimally conscious. We presume they're never going to get better. And you, and you take care of people with brain injury all the time. Right? So are you a nihilist or are you a hopeful neurosurgeon? You have saved people and change their, their trajectories because you've done operations on them. So you know that it's always catastrophic when you have a brain injury. You've seen recoveries. But this goes back to history.
Dr. Fins: It's a split between Hippocrates and Galen. And so, so what Galen actually did is he took like little snails and he compressed them and he saw changes in their levels of arousal as it were. And it was like an experimental thing. He saw the injured brain healed. And so the notion is that brains can heal. So this notion of like is it always futile and overgeneralized because of the vegetative state and the establishment of the right to die. And now I think we have to preserve that right to die, which comes out of Quinlan and in these other cases. But we have to affirm the right to care for people who are conscious, who actually have some possibility. And then if we look at that permanent vegetative state category, right, and now it's chronic and 20% of them are not permanent, then even permanent is not permanent for some segment of the population.
Dr. Stieg: Back in the 70s when I was getting my PhD, the fundamental thought was that you were born with the neurons you were born with. And you didn't have plasticity and you couldn't regenerate. Obviously that's wrong. And so when a patient presented with a deficit that looked permanent, the thought process was those neurons are dead and you're, you're not going to have a chance to get better. We know that's wrong now. So as a doctor, what tests can they do at the bedside or to to help differentiate between the persistent vegetative state and a minimally conscious state?
Dr. Fins: Right, so, right, it would be so persistent is still early. So it's a time course and it'd be like the permanent vegetative state in the minimally conscious. The best test is, is a bedside exam called the coma recovery scale revised. And it's a, it's a neuropsychological task with multiple domains and it's, it's a way of doing bedside exams and was interesting is you have to do it five times over, over a period of time to get an adequate sample because if these behaviors are episodic and intermittent, if you get people when they're down, they don't manifest and we give them credit for their best performance. And so that's, that's the best test to actually, then there's tests using fMRI and functional connectivity and some emerging work with EEG as well. But it's really an aggregation of these different tests based on the, the clinical exam, the history.
Dr. Fins: So we know that people that have traumatic brain injury have a far more higher likelihood to be in one of these higher states than people who've had an anoxic, like a cardiac arrest kind of global insult versus a structural insult. So when people hear about the 20% of people who are no longer permanent but it's chronic and maybe they're going to get better, they assume that it's all, it's all plasticity. But let me go through who those folks are. Some of them are going to be people who have plasticity and by plasticity, it's not necessarily new neurons, but it's a new axonal connections — white matter connections between remaining neurons. So they've actually rewired their brain and my colleagues in Science Translational Medicine a couple of years ago, did a 54 month longitudinal study of Maggie Worthen whose name I can share, who was the protagonist with her mother Nancy in my book Rights Come to Mind.
Dr. Fins: She was a Smith College student who had a brainstem stroke that bled up into her thalamus and she came to us and people thought she was vegetative and we showed that she was minimally conscious, but over a 54 month period, she regained functional connectivity in Broca's area, the speech area of her brain, and across the hemispheres. That allowed her using her one remaining eye that was able to move to have a 90% accuracy in communication. So that correlated with functional improvement. Then you have people who are actually in a, in a, in a state of non-behavioral MCs, the circuitry is intact, but they're not manifesting it behaviorally, but they have the circuitry. The one of the key differences between the vegetative and minimally conscious states is that the vegetative state — imagine if, the interstate highway system in the United States, it's been all bombed out and you can't get here from, from there, right, because there's no system versus the system is intact.
Dr. Fins: Maybe there's some detours here and there, but there's no traffic. So you can't, there's no way to get from New York to Seattle, but if you had a car, you could get there. So with activation, with deep brain stimulation, which is an investigational work that we did, um, drugs or rehabilitation, you can actually put commerce and traffic on the highways that remain. But they're just, they're just underpopulated. So that's another way. And then you have the additional paradox. You put the traffic on the highway system and you only get to the level of non-behavioral. So you show it on the scan, you see the functional connectivity. So those studies, are you referring to? You say to somebody, imagine playing tennis. You imagine walking through your house, imagine a dis-aggregating words like creaking your neck or a Riverbed Creek and you light up, you know, your motor or your spacial walking through your house or linguistic areas in your head. But you can't talk, you can't move and you can't move your arms or anything like that. So, so that's another, so those, those are the people who are in that 20%. So it's, it's complicated. It's not just everybody's rewired their brain, although that's, that's a big, that is a part of it.
Dr. Stieg: You mentioned drugs — Mementine, I actually studied that drug for the treatment of stroke years ago. I also thought about Ritalin, something like that, that might stimulate or provoke the brain to be a little bit more active. Where are we in that field?
Dr. Fins: So, so the bottom line is the thalamus is to the brain. What Hartsfield Airport in Atlanta is to Delta Airlines. It's the hub. You can't get there from here if you don't go through Atlanta and you know all the planes can be flying around, but you're not going to make your connection if you don't go through Atlanta. So the brain, as you know better than than me, is organized into lamino cortical tracks, and it's a loop. Now the thalamus also looks down into the brainstem. It connects the, the, the issue of arousal with connectivity. So you can't be conscious, I mean, we'll put aside dreams for a second. You can't be conscious unless you're awake and aroused, right? So you need the input from the reticular activating system down in the brainstem to provide that. But that's not enough because all you have is your brainstem when you're in the vegetative state. Your eyes are open but you're not aware. Then the thalamus, which is the primitive brain, has projections all the way up to the cortex, all the way through the cortex. So one of the theories is that we need to have wide activation of the cortex and, and sometimes when you have a brain injury, you, you block output from the cortex. So the work that we did with deep brain stimulation is, is a direct glutamineric drive to filamic output. Some of the other drugs that have been used actually take the brakes off the basal ganglia that inhibit thalamic output into the cortex. And what this does is provides that connectivity. So I think that there are some, Amantadine is a drug, Ambien is another drug that works in a small percentage of people. Tragically with Ambien, it's kind of like, you know, Oliver and L-DOPA, that, you know, initially it works and then people's brains act like normal brains. They get, it's a sleeping pill again.
Dr. Stieg: I want to dig a little bit deeper. You mentioned her earlier, Maggie Worthen and the, the beautiful story about her kind of waking up. The question I had as I was reading in on any, I couldn't get the flavor for how awake was she? Could you tell that she had a true emotional appreciation from many aspects of her life or was it really simple?
Dr. Fins: You know, Maggie was, was a young woman during her finals week at Smith College. She was done with all her exams, passed all her courses and her roommates heard her rattling around in her dorm room. They tried to get in. She had fallen, she had had a seizure. She was wedged up against the door. One of her classmates crawled out the window, came in through the the window to get her and they turned out she had had this brainstem injury and bleed and up into her, into her thalamus — and was really brain injured. And was for many months in the vegetative stage, came to CASBI here and we assessed her as part of a research protocol and based on concern that her neurologist in Boston that maybe she wasn't vegetative and that was the question you've asked — and we found that she was in the minimally, minimally conscious state.
Dr. Fins: I remember that evening vividly. It was in the first pages of the book that I, that I, when I wrote my book, I had been interviewing her mom who has given me permission to tell this story and we have IRB approval and HIPAA permissions. I think it's important to say that as a bioethicist. And she was telling me the story of Nancy's life and her recovery and her and her travails, like thinking that Nancy was in there but not knowing for sure, you know, she, you know, she was almost like her friends and her community in Rhode Island didn't believe her. Why do you, why do you think that? Is it just denial, wishful thinking. So she comes to New York and while Maggie is being scanned at the hospital and, and I'm, I'm, I'm doing this interview and getting the sequence and I said to Nancy that morning and that afternoon, I said, what would make it all worthwhile for you and your daughter? If she's well, if she knew you know who I was and that I was there and that would, that would mean everything that would make it all worthwhile for her.
Dr. Fins: I had been dealing with that during the day and I go over to the hospital, she was on the sixth floor here, down the hall. And, uh, my colleague and my good friend, Dr. Nico Schiff was there and we went in to do the exam and Nico was at the bedside. And normally you'd take a pen and you'd ask the patient, you know, and she could only look down yes and no with her left eye, you know, is it looked down for yes. If this is a pen, okay. He didn't do that. And instead he pointed to Nancy, the mom, who was at the end of the bed. And I'm on the other side of the bed by the foot. And, and she said, if that's your mom looked down and there was a pause and then there was this big swoop down of her left eye for yes.
Dr. Fins: And everybody was like, wow, you know, and why Nico didn't do the pen thing, and did the mom thing after my conference, because we had not had a chance to debrief that day. And then Nancy comes over and just like comes over and just, just landed herself on my shoulder and, and started crying. And I didn't know if those were tears of joy or sorrow, like the joy of like, "Oh my God, my daughter knows that, that I'm here and I'm her mom." Which would answer your question that she's the in there in some meaningful way. She knows who she is, she knows who her mom is, or it's like, "Oh my God, look what's happened to my daughter. And she's in there and she knows what's going on." I just, all I did was hold on tight and then it turned out they were tears of joy.
Dr. Fins: You know, whenever I give a talk, I get this question. It's like, "Can they suffer?" And once they're able to talk reliably about what they're experiencing, they're no longer in the minimally conscious state. They've now gone to the MCS-E or minimally conscious state emerged where they can reliably communicate. So we don't really know, but, but sometimes we can infer. So at one point, Nancy was concerned later in the book, you know, that Maggie was suffering and maybe she should with, you know, withdraw care. By this point, she's done on a ventilator or anything. She's just, it's about feeding, you know, food and water. And I, and she might've had a tracheostomy tube that she sometimes needed, but it was like a decision about what are the goals of care. And she was deliberating. And I'll tell you, I'm so grateful to Mrs. Werther, Nancy, for spending so much time talking with me because when everybody says, you know, should these people live or die, the answer is, it's a nuanced question. You know, and it's really this, this, this odyssey, these people took together. So, but Nancy was very concerned about her daughter's wellbeing and whether they're there, you know, she was suffering and she was beginning to speculate, maybe she was, but then she realized that her right leg or her left leg was, was uncomfortably placed over her left shin of her other leg. And once that leg was moved, she didn't seem to be in much distress. The danger here was to over-interpret in either direction. My concern is that we need to strive, I mean everybody says, well, I wouldn't want to live that way, right? I would never want to live this way. And this was our, it goes back to our first podcast together and that's about patient self-determination. Like as if we could determine what happens to us and would I want to live this way?
Dr. Fins: I don't think so. Right? But what we are trying to do is doctors and you do this as a neurosurgeon every day. Nobody wants to have a brain injury, nobody. And it's not like you'd say, well you know, nobody wants to be this way. Your job is to meet people where they are and to make it better. So this is where she is and this is where she was. I mean she did pass away sadly several years ago. This is where she was. And what we tried to do was meet her where she was and where she was, was that she was unequivocally conscious, unequivocally aware of who her mother was, could perceive pain. And I think the holy grail of our work is to try to restore functional communication to these people so they can tell us if they're suffering. Phil, there's a saying in the disability rights community, nothing about us without us, nothing about us without us. Like, like, like we got to ask them. And the idea is—
Dr. Stieg: Did they, did anybody ask her, "Maggie, are you suffering?" Did she look down?
Dr. Fins: You know, it's, it's not a reliable, it wasn't always reliable. And that begs the question of like, how do we determine if you have enough judgment? And I've actually written about what I call mosaic decision making. Like one of the challenges is how do we bring people into the conversation. And the whole idea, the whole idea is to give them voice, right? If we give them voice prematurely before they've had the ability to understand the risks and benefits and alternative, they're not able to defend their interests. But how do we not exclude them as historically people with disabilities have been excluded from conversation. So I came up with this metaphor of a mosaic decision making process. So if people, you know, think about a mosaic, there are little pieces of glass or ceramics or something and each one is a little shard of nothing, right?
Dr. Fins: If just a little piece. But if you put them together and then you step away, the image reveals itself. Okay, that is a, that isn't, that is a picture of something. So the, the pieces of this decision making mosaic as people regain their capacity is, you know, what the patient once said, okay, that's like sort of their advanced directive, what they're saying now what the surrogate says the mom in this case, what their doctor is saying. And then I added to that what a patient advocate would say. So the mother of another person who had brain injury and in the aggregate together they kind of create an assemblance, an approximation of what the, what might be the right decision. We're not, we're not only going with what the patient said, but the patient's voice is part of that picture. The problem is that we're talking about, covert consciousness — people who we thought were vegetative who were not has been elucidated by science, in other words words with neuroimaging and our testing and everything, we now know there's a problem that heretofore we were sort of naive about. We didn't know, the good news — that's the bad news. The good news is that science is also part of the solution. There are drugs that are devices that perhaps might give these people the ability to come back, but now that we know that these folks are there, now that we know that we have this problem, covert consciousness, now that we know that they're segregated, we can no longer look away.
Dr. Stieg: Dr. Joseph Fins, the chief medical ethicist here at Weill Cornell Medicine. Thanks so much for being with us.
Dr. Fins: Thank you so very much. Really enjoyed it.