People in a coma or other impaired state still have rights and deserve respect, ethicist says

Host Amber Smith: Upstate Medical University in Syracuse, New York invites you to be The Informed Patient with the podcast that features experts from Central New York's only academic medical center. I'm your host, Amber Smith. Today, we're diving into bioethics and the social, medical and ethical obligations to people with impaired consciousness with philosopher, Dr. Syd Johnson. She's an associate professor of bioethics in humanities at Upstate and an ethics consultant at Upstate University Hospital. And, she's also the author of a new book, "The Ethics of Uncertainty: Entangled Ethical and Epistemic Risks in Disorders of Consciousness." Welcome back to The Informed Patient, Dr. Johnson.

Syd Johnson, PhD: Thank you. It's good to be here.

Host Amber Smith: A lot of your research focuses on ethical issues related to brain injuries and loss of consciousness, so I'd like to start with some definitions. What is brain death?

Syd Johnson, PhD: Brain death is defined as the irreversible loss of brain function. In the U.S. the legal definition of brain death is called whole brain death, and it is the irreversible loss of all functions of the entire brain, including the brain stem. In some other countries, they use a brain stem death standard, which is the loss of function in the brain stem resulting in an irreversible loss of consciousness and the capacity to breathe without assistance. In the U.S. someone who is diagnosed as brain dead is considered legally dead.

No other state besides New Jersey has an exception to that. In New Jersey law, if there's a religious objection to the determination of brain death, then death has to be determined using the more traditional circulatory respiratory or cardiopulmonary criteria. But New Jersey is the only state that has that kind of exception.

In New York State it's considered legal death, and the individual who is declared brain dead can potentially be an organ donor, if that's what they wanted, or medical treatment will be withdrawn from that person.

Host Amber Smith: So what is, by comparison, then, what is consciousness or the disorders of consciousness?

Syd Johnson, PhD: A disorder of consciousness is a state of impaired or diminished consciousness. And there are three main disorders of consciousness. One that most people would be familiar with is coma. In a coma, an individual is completely unresponsive. They're unaware. They don't sleep or wake up, and they don't breathe on their own typically. And this is usually a time-limited condition. And it typically lasts roughly four weeks.

The syndrome that used to be called the "persistent vegetative state" is a state of unresponsiveness in which we think there's no awareness of the self or the external world, but the patient has sleep-wake cycles, including periods of being awake with their eyes open. Recently, there have been changes in the use of terminology, and the term "persistent vegetative state," although it is still commonly used, both by the public and by doctors, is considered outdated and inaccurate. One thing that I discuss in my book is the way that that term "vegetative" has become really stigmatized. And it suggests the kind of subhuman status for patients. They're sometimes even referred to as vegetables, commonly. And I think that term's really objectionable for that reason, right? It has all of these implications of hopelessness and pointlessness, and I think we should stop using it. There have been some efforts to rename that disorder. For example, one proposed name is "unresponsive wakefulness syndrome," which is a mouthful. But it describes the symptoms and doesn't actually imply anything about consciousness.

One really interesting medical and ethical feature of this syndrome is that we've known for decades that there's a really high rate of misdiagnosis. About 40% of these patients are misdiagnosed as unconscious or unaware. In the last decade, we've seen some efforts to rename that category of patients who appear to be unaware, but whose consciousness can be detected using technologies like functional magnetic resonance imaging, or fMRI. And so currently we're calling that condition "cognitive motor dissociation," because there's a disconnect between that patient's external behaviors and what is going on inside their brain or inside their minds.

And then there's a condition called "minimally conscious state," in which a patient shows fluctuating but reproducible signs of at least minimal awareness. And this is a condition that a lot of those misdiagnosed patients might be in. What's important is that we have pretty good evidence that these patients can feel pain and also feel emotions. So we need to be really concerned about how they're treated and make sure that they're not experiencing pain or loneliness or other aversive states.

Host Amber Smith: So all three of these disorders that you described, are they thought to be temporary conditions? I mean, a person is in, say, the coma and comes out of eventually?

Syd Johnson, PhD: So back in the 1970s, when the persistent vegetative state was first named and defined, it was thought to be a permanent or chronic condition that people did not recover from. By the early 2000s we understood that there was this other category of patients in the minimally conscious state, some of whom had recovered from the so-called vegetative state. So we started to get more and more information about these patients that some of them actually did recover consciousness to a greater or lesser degree. So there is the potential for patients to recover consciousness, but some patients, to the best of our knowledge, can remain chronically in either that unresponsive wakefulness syndrome or in the minimally conscious state.

Host Amber Smith: Why did you go with the title, "Ethics of Uncertainty" for your book? What does the uncertain refer to?

Syd Johnson, PhD: I was concerned about two kinds of uncertainty that we see in disorders of consciousness. We also see those in other areas of medicine, but this was the one that I sort of used as my test case. And the two kinds of uncertainty, I think, are very much entangled and difficult to tease apart. And one is epistemic, or uncertainty related to our knowledge. And that's the medical and scientific uncertainty about disorders of consciousness and the patients. We don't know exactly what consciousness is, and we don't know how the brain actually gives rise to consciousness. That's one type of uncertainty that we have, and we also do this fairly bad job of diagnosing when someone is unconscious, that 40% rate of misdiagnosis is a pretty big problem -- especially when we start making decisions about those patients based on a misdiagnosis. And those can include decisions to withdraw life sustaining treatment, because we think someone is unconscious and will never recover consciousness.

So the other kind of uncertainty is ethical uncertainty. When we make decisions about what to do for a patient, we're making decisions with ethical implications. In many cases, those are life or death decisions. But if our reason for making a particular decision is that we have a diagnosis of unconsciousness, then we ought to be really certain that the diagnosis is right, because the treatment choices that we make are going to be very consequential. From the early days of disorders of consciousness, there was a sense of certainty, both about the diagnosis and the hopelessness of these patients, and also about the ethical question of what we should do. We saw really early on, legal precedent set about whether it was legally acceptable to withdraw life sustaining treatment from these patients. The Karen Ann Quinlan case in the United States was in the early 1970s. And just a couple of years after, the persistent vegetative state was named and defined in the medical literature. So there's this persistent belief that there's just no good reason to keep someone alive in a state of impaired consciousness. And these attitudes persist today.

Even though today, we know a lot more about these disorders and we know that we still don't know a lot about these disorders. And we don't know a lot about these patients. So there's a saying about disorders of consciousness that no two patients are alike. The complexity of the brain, the uniqueness of different injuries to the brain and to different areas of the brain, make every patient, in a sense, unique. If we can't be sure we're right about the diagnosis and the prognosis, then we can't be sure that we're right about the ethical prescription either. So, one thing I worry about is this sense that there is some universal right answer about what we should do for, or about these patients. I don't think there is.

Host Amber Smith: Well, your book is getting great reviews. I saw on Amazon, someone described it as "a passionate and conceptually deep effort to open up new ways of thinking about and taking care of people with dramatic brain injuries." In general, what are we doing now in treating people with brain injuries that you believe we could do better?

Syd Johnson, PhD: The possibility that someone with a serious brain injury will not recover, or will not recover in the way that they would want -- they might be disabled, they might have impaired consciousness -- that drives a lot of decisions that are made in the immediate aftermath of injury. So the majority of deaths after a serious brain injury occur after life-sustaining treatment is withdrawn. And that often happens within the first 72 hours after an injury. There's a lot we don't know about that patient and their potential for recovery in the first 72 hours. But what's driving those decisions to withdraw treatment is fear. It's a fear that the patient will survive, but not in a condition that they would have wanted or would find acceptable. And we can only prevent that from happening by deciding that they won't survive. The problem is this results in a self fulfilling prophecy. It looks like a lot of people die after serious brain injuries because lots of people do die, but many of those people die when they die because treatment is withdrawn.

Some of those patients would die no matter how hard we tried to save them, but some of them would have survived if we had continued to treat them. So, one thing that happens is that our data about these patients is skewed. So it looks like most people die after a serious brain injury, even though many of those deaths occur because it was decided that they would die. That's the self fulfilling prophecy. So one thing we're not doing is giving people a chance to improve and recover. We're giving up too soon, I think, in many of those cases and acting too soon in order to avoid a bad outcome that might or might not happen much later. In fact, despite improvements in neuro intensive care and in our ability to save lives, the percentage of patients dying after treatment is withdrawn has actually been increasing.

Host Amber Smith: This is Upstate's The informed Patient podcast. I'm your host, Amber Smith, talking with Dr. Syd Johnson. She's a philosopher from Upstate's Center for Bioethics and Humanities. She's an ethics consultant at Upstate University Hospital. And, she's the author of a new book called "The Ethics of Uncertainty."

Can you give us a synopsis of the recent neuroscientific discoveries that have taught us more about the disorders of consciousness?

Syd Johnson, PhD: Sure. One of those that I mentioned is the use of neuroimaging that looks at whether patients' brains might be capable of responding to stimuli or to commands. So fMRI has been used for this. Some studies have shown that patients are able to respond to verbal commands by actually modulating or controlling their brain activity. So they might be asked to perform an act of imagination or a mental task like, "imagine you're playing a game of tennis." And the fMRI can show us that the areas of the brain that would normally be activated by that kind of mental task are also activated in these patients, even though the patient's outward behavior shows no indication that they are capable of responding to our questions.

So this tells us a few things, right? It tells us for one that the patient heard the command and understood the command they were given and also was able to perform the mental task. And all of those things require, so far as we know, that the patient be conscious and aware. But there are also lots of reasons why someone might not pass that kind of test, and they're the same reasons that they might fail a bedside behavioral exam as well. Their brain injury might have damaged the parts of their brain needed to understand language, for example, and then they wouldn't be able to perform the task. So that's one example of a sort of revolution that has taken place in the last couple of decades, really in the 2000s, in our ability to detect consciousness in a subset of patients for whom we cannot see it from their outward behavior.

Host Amber Smith: Are researchers looking for ways to pull patients out of unresponsiveness, or the old persistent vegetative state? Is there research being done for how to sort of break the spell?

Syd Johnson, PhD: Yeah, there has been a renewed interest in these patients within this century, as we've started to get these hints that there might be more going on with these patients than we had previously thought. And so one thing that we have pretty good evidence for is that patients have better recoveries when they have access to high quality and dedicated neuro rehabilitation. Unfortunately, rehabilitation of that type is not widely available. It's very specialized. It can be very expensive, and it's simply not accessible to a lot of people. There have been some experiments with some drugs, with technologies like deep brain stimulation, to see if they can help. And they do seem to be helpful for a subset of patients. The drug zolpidem, which is the sleeping pill Ambien has a kind of paradoxical effect. In about 10% of patients with disorders of consciousness, instead of putting them to sleep, it wakes them up, and it makes them more alert. The effect is temporary. It lasts as long as the drug is in their system, but it has been shown to be fairly dramatic in a number of these patients. So, because we have had this renewed interest in these patients and helping them, there are now ongoing efforts to try to figure out ways of detecting earlier on which patients might respond best to treatment, and also figuring out ways of helping them.

Host Amber Smith: Are there criteria that doctors use to determine whether someone's going to survive or whether it's time to pull the plug, so to speak?

Syd Johnson, PhD: The role of doctors, in my view, is to provide surrogates with the information that they need to make those decisions for the patients. Most people don't know that there's a 40% misdiagnosis rate in disorders of consciousness or that because life sustaining treatment is withdrawn so frequently following serious brain injuries that we don't actually have accurate data on how many patients could survive and recover if we continue to treat them. There's a lot of uncertainty about these patients. And of course, uncertainty is something that can be very difficult to acknowledge and to explain. I think, though, that there is both a moral and an epistemic obligation on the part of doctors and other medical staff to acknowledge that uncertainty, to understand the reasons for that uncertainty and to inform surrogates of that uncertainty so that they can make the best decisions they can, given that uncertainty and also make decisions that align with the values and preferences of the patient. But I think we too often fail to really communicate and acknowledge that level of uncertainty that exists with this group of patients, and that's a real disservice to their surrogates and to the patients, as well.

Host Amber Smith: I'm curious about how an ethicist helps a loved one or a surrogate decide what to do, whether it's time to pull the plug or provide other types of care to someone. What kinds of questions can we be asking about particular situations that will help us do the right thing?

Syd Johnson, PhD: For surrogates or proxies, these are never going to be easy questions for them to deal with. We're often dealing with these problems in a situation of crisis, a situation that is very emotionally difficult for loved ones who are dealing with a serious injury to someone that they care about. What a surrogate should be doing, what their moral and their legal obligation is to do, is to make the decision that the patient would want them to make for them. If the patient could decide themselves what they wanted to be done, that is what we want the surrogate to do for that patient in terms of their decisions about what kind of treatment the patient would get, and whether it's time to stop treatment for that patient.

If that surrogate knows the patient well enough and understands their values, understands their beliefs, their religious beliefs, if they had any, and has talked to that person about their preferences and wishes, then they can make decisions that are probably pretty close to what the patient would decide themselves. And of course there's no single right answer, and there's no perfection possible in these kinds of non-ideal circumstances. People have to do the best that they can.

Host Amber Smith: In general though, what are our social and medical and ethical obligations to people with impaired consciousness?

Syd Johnson, PhD: Well, people with impaired consciousness are still people, and we have the same obligations to them that we have to anyone else who is facing a serious injury or a serious illness, to know as much as we can, to get the best and most accurate information that we can about their condition, about their prognosis. Doctors, I think, can play a really big role here in dispelling myths and misconceptions about these patients. And I think doctors also need to dispel the power of those myths and misconceptions in themselves and the way that those, their own beliefs about these patients might influence what they tell their surrogates and what they personally believe about what should happen. We have to acknowledge again, that there is a great deal we don't know about what's going to happen in someone's future, but we still need, I think, to treat them as persons who have rights and who are entitled to medical treatment and who are entitled to respect for their own values and their own autonomy and their own preferences.

Host Amber Smith: Do you think it's different if the person is medically frail or elderly?

Syd Johnson, PhD: When someone is elderly or has confronted a chronic illness, for example, in their life, that can certainly affect their ability to recover and their ability to survive from a serious injury. It may also affect their values. By which I mean, if someone is already near the end of their life or has the understanding that, their life is going to end and has accepted that, they may not want a lot of medical interventions, and they may not want to be in an ICU at the end of their life. They might've told their friends and their families and their loved ones, "if something happens to me, I'm done, and that's okay." But someone else, for their very own reasons, might've said, "I want you to do everything possible to keep me alive as long as possible."

I consulted on a case a while back involving an elderly man who had a serious stroke. He was not expected to recover. He was minimally responsive. But he had told the doctors when he came to the hospital, and he also told his son who was his proxy, that he wanted to be kept alive as long as possible. And that's what his son decided to do, was to continue treatment, which is entirely appropriate. The surrogate should be making decisions that align with the patient's desires and beliefs.

Host Amber Smith: What about when the person with impaired consciousness is a child? I imagine that's gotta be more challenging.

Syd Johnson, PhD: Parents who are facing a serious injury or illness in a child are always confronted with extremely emotionally fraught and difficult decisions where they have a genuine and heartfelt interest in that child continuing to live. And because children, unlike adults, have not always had a chance to formulate their own values, or to make their own choices, or to think about mortality and think about how they want their lives to go, parents don't have that to rely on, in thinking about what would be best for this child. So they often have to make decisions that consider what are in the best interests of that child, without knowing what the future might hold for that child. So again, there's no single right answer that applies across the board to every patient. And we tend to trust parents to make these kinds of important decisions for their children and about their children's lives. And I think that's the appropriate way to approach it -- even if we think that some of those decisions might be inappropriate at times, or perhaps we think people are having trouble letting go when we think they should be letting go. I think we need to trust family members to do what is best, all things considered, for that patient.

Host Amber Smith: Do you see that cases are approached differently if the person has a disability? Are those cases treated differently, do you think?

Syd Johnson, PhD: I think that there tends to be some bias about what quality of life is like for disabled persons, or how valuable the life of the disabled person might be. Now, of course, disabled persons are still persons, and they have the same rights, including the right to medical treatment and the right to have their autonomy respected and their values and interests protected that every other person and every other patient has. Persons with disorders of consciousness are impaired, and they can have a number of cognitive and physical disabilities as well as a result of their injuries. But what guides our decisions about them should be the same as it is for anyone else. If someone comes to us in a coma or in an unresponsive wakefulness syndrome and has a prior disability -- perhaps they were a wheelchair user, perhaps they had a cognitive disability, I mean, there's a wide range of possibilities here -- we still need to treat that patient as we would any other patient, taking into consideration what was important to them.

Host Amber Smith: For someone who becomes impaired without having chosen a surrogate, or maybe they don't have children or parents to speak for them, how do bioethicist help determine what that person's wishes or desires would have been?

Syd Johnson, PhD: So as a bioethicist, I don't usually know the patient, and I don't know what their values are or what they cared about. Situations in which we have a patient who is unrepresented, has no family or no close confidantes that we know of in order to make their decisions, those are extremely difficult. And the law in New York State, at least, certainly does have some provisions built into it, to protect those patients and protect their interests, but also to allow doctors -- and this is a situation that we often call two-physician consent, where two doctors are in agreement about the patient's diagnosis and prognosis about how long they might have to live, about whether or not they are permanently unconscious. Those are the kinds of considerations we can can use in making decisions about what happens to those patients. But those are extremely difficult circumstances for decision-making. In some cases, those might also be patients who are entitled to have a court appointed guardian to make decisions on their behalf.

Host Amber Smith: I wonder, do you start with an assumption that everyone values life and would want to live, or do you try to empathize and learn about the person and their background and who they are and what they might have wanted?

Syd Johnson, PhD: That's extremely difficult to do when you don't know a person. Now, of course, young people get into accidents and suffer injuries, and they tend not to have advanced planning, formal advanced planning documents, for example, like an older person might. So their surrogates, whoever they are, are going to have to try to figure out what was important to that patient and what they would want that patient to do for them. I don't know the patient, so I'm not in a position to say what they wanted or valued. And that's a very individual thing. So generally speaking, we think that life has value for most people, but life has value for us, depending on what's involved in living that life, right? For some of us, a life of unconsciousness would be not a good life. For some of us, though, just the value of being alive might override any other considerations and be worth continuing to live. So it's a very individual matter. And , there's certainly, as I have said, no single right answer that we can apply to all people.

Host Amber Smith: Any last words of advice for someone who's living in an uncertain situation, perhaps with a loved one who has got impaired consciousness?

Syd Johnson, PhD: I think it's important that surrogates and proxies understand that it's entirely appropriate to take an all-things-considered approach to decision-making and to keep in the forefront what's good for this patient and what this patient would care about. One of the things that you sometimes encounter in these contexts is that the family decided to continue treatment for this patient, to fight for this patient, to give them every opportunity to recover and found after a number of years, perhaps, that the patient's recovery was fairly limited. Perhaps they were left in a chronic minimally conscious state, where they weren't able to do the things that they cared about in life, weren't able to go to football games or play with their children or their grandchildren, or go to their jobs, if their jobs had a lot of meaning for them. And those caregivers and those family members will at some point confront this decision about whether or not they think this is the life that that person would have wanted to live. And again, a very individual choice. The circumstances in which families and surrogates find themselves can vary a lot. Their health insurance certainly is going to play a role in what they can do for this patient, the social support that they might have available to them. They may belong to a tight-knit community that offers them lots of support, or they may not. And our society, generally speaking, does not support these patients very well. And this is true of brain injured patients in general, regardless of the level at which they end up. So these are extremely difficult decisions for caregivers and for family members to make and they have to make the best decisions that they can under the circumstances that they find themselves in. They will never be perfect decisions, and they will never be easy.

Host Amber Smith: I appreciate you making time for this interview, Dr. Johnson.

Syd Johnson, PhD: Thanks a lot.

Host Amber Smith: My guest has been Dr. Syd Johnson. She's an associate professor of bioethics and humanities at Upstate. The Informed Patient is a podcast covering health, science and medicine brought to you by Upstate Medical University in Syracuse, New York. Find our archive of previous [email protected]/Informed I'm your host, Amber Smith, thanking you for listening.