S1 Episode 53: Conscious and Trapped Inside

From drug cocktails to deep brain stimulation, there is new hope for brain-injured patients with “locked-in syndrome” in minimally conscious states. Dr. Nicholas Schiff, Professor of Neuroscience at the Feil Family Brain and Mind Research Institute at Weill Cornell Medicine, tells the dramatic stories of patient reawakenings from comas lasting many years.

Dr. Phil Stieg: Today, I have the pleasure of being with Dr. Nicholas Schiff. Dr. Schiff is Professor of Neurology and Neuroscience in the Feil Family Brain and Mind Research Institute at Weill Cornell Medical College. In this segment, we’re going to be talking about the newest diagnostic techniques used to assess altered mental states, and then talk about hope, what we’re doing to treat patients that have these altered states. So let’s take a specific example of one of your intriguing cases, Maggie Worthen. Tell us about that.

Dr. Nicholas Schiff: So, so Maggie Worthen is a public figure, there’s a very nice and beautiful book written by my colleague, Joe Fins called Rights Come to Mind that tells her story. There’s also a Scientific American article that describes her history, but Maggie came to us after having been thought to be in a vegetative state for two years after a stroke, her mother brought her into the research studies we were doing at Cornell. And when we do our studies, we bring people in for several days and examine them and do different tests. And the first day that we brought Maggie in, we couldn’t identify any evidence of awareness—that was true for the full day. And then the next day, when she was more alert, we discovered for the first time that she had one movement that showed unambiguously, that she was aware. We asked her to look down and with one eye, she was able to look down from that point on, we then were able to do a whole series of tests with her.

Dr. Schiff: And we brought her back to do what’s called functional magnetic resonance imaging magnetic resonance imaging is MRI. Everybody who’s been in a hospital or watches TV probably knows what an MRI is. It’s a machine that can take pictures inside body tissue. When you use it in the brain, there are a lot of different things you can do. Functional MRI means that you use the MRI machine to study oxygen in the brain. And when oxygen changes its level, it’s associated with the activity of nerve cells doing work. So many investigators before us had discovered that if you imagine doing a task like playing a sport in your head, if you or I were to do this, there’s a very reliable signal that comes up in a particular location in the brain. Colleagues of ours in England demonstrated for the first time in early 2006, that you could use this technique to look at patients who appeared to be vegetative or minimally conscious to see if they had higher level consciousness that you couldn’t see.

Dr. Schiff: We did this test for Maggie, except that we had her specifically swim because she was a swimmer. The results were totally unambiguous. She did the task. She had very, very clear results, just like a healthy control. And the thing that remember about this, I think most people don’t appreciate it as it’s an awfully hard thing to do. If you take a stopwatch and time yourself for 30 seconds. Imagine yourself swimming. Imagine yourself swimming for 30 seconds and then say, stop. You have to do it seven more times and complete each of those accurately to meet the threshold, to generate the little colored picture that you see in the newspaper. That is why this is a very convincing test. If you pass it. Not passing, it doesn’t necessarily mean you’re not conscious because that’s a very high bar.

Dr. Stieg: I’d like to move on to the concept of hope and what you are now doing now that you are able to determine that people are in a minimally conscious state or a locked in state and how you can help change their lives. And one thing is this concept of deep brain stimulation or DBS. What is it? And where are you going with it?

Dr. Schiff: Okay, I’m going to take that question as a whole start with at the outset, because I think this is the whole point that we can develop therapies and we can actually turn the hope of understanding into something practical to do. And I think there are sort of two major issues. One is we have to identify people who are hiding the level of high-level consciousness at the bedside, find ways of identifying them and giving them a chance to try to communicate. That’s one line of work. The other line of work is that we have a lot of patients who can talk, who can interact to some degree, but can’t do it reliably. And we can work to try to restore their function. The organizing approach that that I’ve taken has been around a concept of a particular role of a circuit called the meso circuit and the deep brain stimulation work has really been centered on this idea.

Dr. Schiff: Deep brain stimulation is a technique in which essentially you put electrodes just like cardiac pacemakers inside of small structures in the brain. This has been in use since the 1960s, but really came into sort of a Renaissance in the late 1990s with the advent of deep brain stimulation for Parkinson’s disease. The work we began over 20 years ago and demonstrated the first proof of concept in 2007 for was to put deep brain stimulating electrodes in the central thalamus, a particular region of the brain in the minimally conscious state. We found in 2007, that we were able to restore consciousness in the form of spoken language, consistent interaction and recovery of organized goal-directed movements in a patient who had remained in the minimally conscious state for six years. Prior to that surgery, that demonstration proof has led to the full, sort of, development of thinking about the circuit, the network, and how deep brain stimulation, along with medications, might eventually be developed into a systematic therapy.

Dr. Schiff: One of the breaks on that, and one of the challenges has been, that this is a problem that exceeds small individual investigations. We need infrastructure to access patients like this and commitments to doing it. And honestly not a lot of people have gotten behind this problem. It’s a serious problem. And the general approach has implications beyond minimally conscious state across all structural brain injuries, going to the problem of people trapped in their heads. That problem gives me the most worry. I have a picture of five of the 15 people we just randomly found like this in our own studies on my wall. And I put it up on the wall to remind me every day that we haven’t really helped them. We found out that they’re there. We know that they’re trapped in their head and we’ve tried things. And even for Maggie Worthen who got one way communication with a very nicely designed closed circuit camera that allowed her to capture this one downward eye movement to do yes, no questions, And she got better and better at it.

Dr. Schiff: And when she did that, we found, and we published a paper two years ago that her brain was rewiring. That was showing large-scale plasticity associated just with being able to recommunicate again with the outside world, but she never established a two way communication. We have examples of some patients who stayed in this cognitive motor association state, where they have this high level cognition, but they can’t respond who then, on their own, after years, recover gestural communication or speech. And they look the same as all these other patients that we have who haven’t yet emerged. The, the question I ask myself every day is, “What’s it better to believe? Is it better to believe that the people who got out and started talking and show that they were high-level conscious and could be tested on normal intelligence tests are different than the 10 or 15 other people who look the same on the fMRI studies and the EEG studies that we have, or is it better to believe that those other people are trapped in a brain that doesn’t have motor control, but need a way out.

Dr. Schiff: And I think that even if the answer is, it turns out that only the people who have emerged and could show us that they’re there, were the ones who could, it should be a burden on us to try to see if we can get the other people out and fail.

Dr. Stieg: Just a brief question about this deep brain stimulation—is it durable?

Dr. Schiff: Kind of like a, it’s like gilding the lily, right? You know, yes. It’s durable. I’m speaking to the chairman of neurosurgery. Deep brain stimulators are placed in many, many patients around the world for many conditions and have performed extremely well, just like cardiac pacemakers.

Dr. Stieg: There are different kinds of electrodes we can put in a patient’s brain. Thinking about the person with Lou Gehrig’s disease or the quadriplegic, the person who can’t move any extremities. There are now ways to bypass that kind of activity, at least communicate through computers.

Dr. Schiff: So in fact, with colleagues, uh, we’re exploring the, the potential for developing the use of these brain computer interfaces in this population of patients who are severely brain injured. And I think that is going to be a very important line of work. And that’s one of the main directions toward new therapeutics that we’re trying to take.

Dr. Schiff: Don Herbert, who was a firefighter in upstate New York, who ran into a burning building and was on the top floor when the building collapsed. And he had a traumatic brain injury, but he also lost oxygen. He had started the process of recovery. He was in coma, vegetative state, minimally conscious state. He was starting to be moved, you know, and walked and ambulated. And then he regressed and went back into what was probably a very low level, minimally conscious state. And his family went, took him to doctors and they said, well, you know, there’s nothing to do. You can see this guy just looks like he can’t do anything. And for 10 years, he stayed that way until an enterprising new physician in a rehab center or a convalescent center where he was at decided to put them on a cocktail of three medications, something called L-DOPA, which is a medication used for Parkinson’s disease. And two drugs used for ADHD, one called Celexa and one called Strattera. And in about four weeks, the family got a call that Don Herbert was awake!

Dr. Schiff: I remember getting the call. The thing that was so remarkable was that when he woke up and this is 10 years, he was fully present. He was blind, and everybody who came to see him that day, he recognized them by voice. But he now, he was able to recognize all of them, but he was shocked and dismayed that he had been away for 10 years. And he talked about it and he agonized about it that like, that’s the most dramatic example I’ve ever seen in my career or something like this. That’s your Rip van Winkle. People ask you all the time. Is it like Rip van Winkle? That one is Rip van Winkle. And, you know, we got to avoid all of this. We got to do better for Terry Wallaces and we gotta do better for Don Herberts. And that’s basically what the game has been for me since I got into this one.

Dr. Stieg: Hopefully we can.

Dr. Schiff: I think we can. I know we can. We’ve actually shown we can, this is proof. The proof of concepts out there, the generalized ability and getting people behind it is not, that’s more than just the interest investigators.

Dr. Stieg: So let me ask you a future question. You’re still young. Where do you want to be in ten years?

Dr. Schiff: 10 years. I want to see systematic clinical studies of deep brain stimulation for people with structural brain injury. And I want to see screening of all patients at risk for being trapped in their heads and a graduated program of trying to see who we can help get out. I’m starting on it now with my colleagues and hopefully you and others here. And that’s where I want us to go. 10 years from now. I’m sure we can do that.

Dr. Stieg: Have you thought about the cost of that?

Dr. Schiff: It would save so much money. I’d love to get a healthcare economist to come in right now and figure that out.

Dr. Stieg: So the rehab and—

Dr. Schiff: Well, let me give you an example. We didn’t have the forethought to figure this one out, but our first patient, the patient that we published in 2007, who had the minimally conscious state after traumatic brain injury was beat up, assaulted, stayed in a nursing home for six years. We brought him into the trial and he was stimulated, was able to start to move, you know, do things with his arm to work, like, pick up a cup or, you know, show you all a functional movement. He could speak. He could talk before that. He couldn’t do any of that. Well in those six years, he also learned that the other thing that we found out in that trial that we learned was that after six years of being fed by a tube in his stomach, one of the therapists working with him notice that he wasn’t pooling the secretions in the back of his throat and was swallowing every morning.

Dr. Schiff: So she thought, well, maybe we should start to feed him. Within five months he was chewing and swallowing three meals a day within 15 minutes. Now, here are the hidden costs for traumatic brain injury. For six years, he had bedsores and aspiration pneumonias and medical problems he couldn’t make anybody aware of, almost regularly on clockwork every few months from when he got the deep brain stimulators for the next six years that he lived. And this is in Dr. Fins’ book. He didn’t have another aspiration pneumonia during that initial time. And he moved. So he didn’t get bed sores. And when he was in pain, he could tell us, “This hurts.” Okay. You tell me that that’s not going to save money. And it turns out every now and then I get approached by somebody from the reinsurance companies, right? You know, the reinsurance companies are, they’re the people who pay the big societal costs that underwrite all of the major, major economic problems. And I learned from them that about 12% of the re-insurance budget is long-term care for people who basically had brain injury. So I don’t think this is a problem of adding money. I think this is, this is recouping costs and adding benefits by reconnecting people with their families. I am certain, this is going to save money. So I’m happy to answer that question.

Dr. Stieg: And I’m happy I asked it!

Dr. Schiff: If somebody’s listening and they’ve got a family member that’s in one of these states, is there a website or somewhere where they can go? You said, one of the comments you made earlier was you got to educate yourself. If you’re the family member, where are they go to educate themselves.

Dr. Schiff: So, so I think this is, I mean, if there were a resource for families and patients, this is, this is another issue for this whole area of medicine. Everybody’s vulnerable to this. Therefore nobody is, and you understand this from neurosurgery and traumatic brain injury: most problems of this scale have advocacy and groups and families. And everybody’s devastated when this happens and nobody thinks it’s going to happen to them. So there’s no prospective organization. It’s not like I had a family member who had this. And so I’m involved in this for the future of my grandchildren. It could affect everybody. It effects nobody. So there’s very little out there, but I think that’s going to change over time. I wish I could tell the people who are listening to this. Okay. Here’s where you go. And they’ll, they’ll solve all this for you.

Dr. Schiff: Unfortunately, I think what ends up happening is that you go and you try to find people who you can talk to who are knowledgeable about this. It’s not hard to find people who’ve had the same experience, but not everybody can go to the web and access it and read, you know, read it and get information. So I think what I found has been most effective for most caregivers and most families is that there’s usually one doctor in their sphere of physicians who really cares and is smart. And they may not be a neurologist and they may be a foot doc. They might be whatever they are, but they’re the person who is willing to talk to them think, and then think of a friend or a colleague who they can put them in touch with you work through your social network and you try to educate yourself. And if it’s past your ability to do it, you go and you get, you get sort of a buddy and that buddy could be a nurse or somebody in your social sphere who is a little bit more tuned in the medical system. And you try and take notes and you write it down and you, and you document and you learn. And for, I mean, we should be doing a lot better than that. And believe me, I understand that, but at least I’m telling you what I’ve learned.

Dr. Stieg: Well, I’m hopeful that somebody listening to this might take up the mantle and say that we want to put together a website. Just listening to you describe the panoply of different altered states is complex and for the lay person to understand this is hard. Dr. Nicholas Schiff, thank you so much for spending time with me. It’s been fascinating talking with you.

Dr. Schiff: Been great to be here. Thanks Phil, appreciate it.