3.1. Locked-in Syndrome

Locked-in syndrome (LIS) typically results from brainstem lesion that fully preserves consciousness and cognitive functioning, but results in almost complete paralysis – often, only eye movements are spared, and these are how patients communicate (although there are cases of total LIS that involve paralysis of eye motility).

Most people unfamiliar with LIS would, when given a brief description and asked, find the prospect of LIS horrifying – perhaps even worse than death. But this is not what many people with LIS say. A recent survey of LIS patients asked them to report on various aspects of their condition, including their perceived quality of life (Bruno et al. 2011). 91 out of 168 invited patients responded, a fact that the experimenters admit may have biased the sample towards higher quality of life ratings. 65 of the surveys could be used for statistical purposes. The results are interesting.

The experimenters asked participants to rate their quality of life on a scale from -5 to +5, with -5 indicating their current level is ‘as bad as the worst period in my life,’ +5 indicating ‘as well as in the best period prior to LIS,’ 0 indicating ‘neither well nor bad,’ and other numbers indicating something in between these options. For reporting purposes experimenters grouped participants into ‘happy’ if they reported in the 0 - 5 range, and ‘unhappy’ if they reported in the -1 - -5 range. According to this grouping, 47 reported happy and 18 unhappy. Moreover, participants were significantly more likely to report happy than unhappy if they were in LIS for longer, had recovered some speech production, reported positively to the prompt ‘I move around my community as I feel necessary,’ reported positively to the prompt ‘I am able to participate in recreational activities (hobbies, crafts, sports, reading, television, games, computers, etc.) as I want to,’ responded positively to the prompt ‘I feel that I can deal with life events as they happen,’ responded an absence of depression or anxiety, and a lower frequency of suicidal thoughts. Bruno et al. summarize these results as follows: ‘Although most chronic locked-in patients self-report severe restrictions in community reintegration, the majority profess good subjective wellbeing . . .’ (1)

In my view it is very important to take these kinds of reports seriously, and it is important to note that a majority reported that their quality of life was ‘neither well nor bad’ or better. But it is equally important to note that this grouping into happy and unhappy is somewhat artificial. A plausible interpretation of the scale given to participants is that many reports below a +5 indicate a reduction in wellbeing due to the injury. Only five participants reported +5, and many of those grouped in the ‘happy’ class reported 0 or +1 (16 by my count: the graph Bruno et al. offer is not clear). This should be coupled with the observation that 13% of participants reported depression, 67% reported moderate or extreme anxiety, 46% reported moderate or extreme pain, 58% did not want resuscitation in case of cardiac arrest (including 49% in the ‘happy’ group), and 32% reported having suicidal thoughts occasionally or often.

These numbers are comparable to another recent study on LIS wellbeing (Rousseau et al. 2015). In that study a cohort of 67 LIS patients was surveyed. 44% reported chronic pain. 55% reported anxiety or mood disorders. 27% reported suicidal thoughts. 67% wanted resuscitation if needed.

3.2. Minimally Conscious State

Minimally Conscious State (MCS) exists on a spectrum of similar conditions, usually due to traumatic brain injury, all of which severely impair consciousness and cognitive functioning. A rough ordering can be imposed in terms of preserved elements of consciousness and cognitive function, with Persistent Vegetative State (PVS) at the low end of the scale, MCS in the middle (MCS is sometimes separated into MCS- and MCS+ (Bruno et al. 2011), and emergence from MCS (eMCS). It must be noted that behavioral differences between these conditions are often very slight: misdiagnosis rates are as high, with misdiagnosis of PVS instead of MCS often estimated at roughly 40%, and misdiagnosis of MCS instead of eMCS occurring frequently as well (though given low sample sizes, it is hard to offer a percentage, see Schnakers et al. 2009).

To give some indication of how the diagnosis is made, consider the deployment of the Coma Recovery Scale-Revised (CRS-R, see Giacino and Kalmar 2005). Administration of the CRS-R involves presenting a patient with stimuli of various sorts. The CRS-R divides the stimuli into an Auditory Function Scale, a Visual Function Scale, a Motor Function Scale, a Verbal Function Scale, a Communication Scale, and an Arousal Scale. Although a patient will be given an overall score from 0 to 23 depending on their individual scores on the subscales, most of the subscales offer sufficient conditions for a diagnosis of MCS: given the distributed and unpredictable effects of traumatic brain injury, the scale incorporates significant redundancy. Failure of all of these will lead to a diagnosis of VS, which is considered permanent if a patient shows or has shown no improvement for 30 days following injury.

Consider, for example, the Auditory Function Scale. It is insufficient for a diagnosis of MCS that the subject to orient head or eyes towards an auditory stimulus on the trials. It is sufficient if, on ¾ of the trials, the patient displays ‘object-related eye or limb movement’ to aurally delivered commands – that is, commands like ‘stick out your tongue,’ or ‘look at [some object],’ or ‘look up at the ceiling.’ On the Visual Function Scale, it is insufficient for a diagnosis of MCS if the patient fixates on a visually presented target for more than 2 seconds. It is sufficient if the patient follows a moving mirror for 45 degrees without loss of fixation for 2 seconds, on 2 out of 4 trials.

The Communication Scale of the CRS-R offers a way to distinguish MCS from eMCS. A patient is diagnosed as in MCS if they offer clearly discernible and accurate yes/no answers to at least 2 out of 6 visually or aurally based questions (e.g., ‘Am I touching my ear right now?’). And a patient is diagnosed as eMCS if they clearly and discernibly answer 6 out of 6 such questions.

Both MCS and eMCS, then, preserve some elements of consciousness and cognitive functioning. But the amount preserved varies by case, and is often extremely limited – a patient can be diagnosed as in MCS even if they score 0 on many of the sub-scales. Neuroscientific studies including MCS and eMCS patients demonstrate that they lack many of the neurofunctional structures necessary for sophisticated information integration, such as the kind of connectivity between the thalamus and frontal cortex that could enable performance of ‘top-down’ cognitive control tasks such as, e.g., counting the number of times a target word is spoken over a 4-second period (Monti et al. 2015). In line with this, most MCS and eMCS patients lack the capacities required for legal competence (e.g., capacities for understanding their condition, appreciating its gravity, developing a view about how their own treatment should proceed, and communicating this view), a fact which often raises difficult questions that family members, doctors, surrogates and in some cases the courts have to answer.

Most patients in MCS and eMCS, then, often cannot report on their quality of life because they do not retain the mental capacities necessary for such reports. Thus, one important way to measure a subject’s quality of life is missing. Nonetheless, it is the working assumption of many doctors and scientists and judges that there is something it is like to be in MCS, and that things can therefore go subjectively better or worse for them. Whether it is overall subjectively good or bad to be in such a condition is generally unknown, although one can find empirically informed speculation in the literature (Wilkinson and Savulescu 2012; Shepherd 2016a).

3.3. Implications for the Mere Difference view

What are the implications of these disabilities for how we understand the relation between disability and wellbeing? Regarding LIS, one has to acknowledge that these wellbeing-relevant reports are much lower than what one would find in the general populace. Indeed, by Bruno et al.’s (2011) own scale, it looks like a vast majority of patients rated their current quality of life as lower than it was before the injury. Some of the wellbeing reduction – or alternatively, some of the reduction in quality of life reports – can certainly be attributed to social conditions, access to adequate care, and so on. But it is not at all clear that the entirety of the reduction is due to social factors. LIS patients often suffer from chronic pain, anxiety and depression, and suicidal thoughts. A high percentage of LIS patients do not want resuscitation. LIS gives many indications of qualifying as a Bad Difference.³

The case is, in my view, even clearer regarding MCS. One might resist this verdict by pointing out that, since MCS patients do not explicitly report on their quality of life, it is possible that at least some MCS patients enjoy a good quality of life. Perhaps their lack of knowledge regarding their own condition prevents them from conceptualizing it as bad, as seems to happen in some cases of LIS.

Even if this is true, however, I doubt one can plausibly maintain that MCS patients in general maintain a quality of life as good as LIS patients. Some of these reasons have to do with injuries and complications associated with the severe brain injury that causes the condition. But the chief reason is the absence of many so-called higher psychological capacities in MCS patients. To be in MCS is to lack normal capacities of attention, reasoning, imagination, memory and cognitive control, to lack access to the range of patterns of thought and streams of consciousness available before the injury, to lack access to the full, rich range of emotional and social experiences available before the injury. With proper care, much of this is available to LIS patients. On most plausible theories of wellbeing – hedonist views, objective list views, desire satisfaction views – access to a range of rich emotional experiences, and capacities to exercise sophisticated agency with respect to one’s inner mental life and with respect to one’s world-involving goals and desires, will be critical for higher levels of wellbeing. Whatever one’s theory of wellbeing, then, it seems to me that in virtue of their disability, prima facie MCS patients are worse off overall than LIS patients.

Both LIS and MCS are Bad Difference disabilities: independently of the effects of social stigmatization they tend to make a person worse off overall. But they differ in important ways. Is there a unifying explanation regarding their relationship to wellbeing? And does it generalize to other disabilities? In the next section I argue in the affirmative. The upshot is a general and tractable normative distinction between classes of disabilities.

4. Control over one’s situation

Here I argue that a central factor in an explanation of the difference between Mere Difference disabilities and Bad Difference disabilities is that the latter significantly⁴ undermine control over one’s situation, while the former do not. Strictly speaking, I offer this control-based explanation as sufficient to explain the asymmetry in a wide range of cases. As features of complex biological beings, disabilities are diverse: we should not expect one factor to offer an exceptionless explanation. (Accordingly, I do not aim to explicate a necessary condition for some disability’s qualifying as a Mere or Bad Difference.) Further, in many individual cases there will be other factors relevant to a full explanation of some singular disability’s relationship to wellbeing. To take one example, a disability that causes chronic pain may be negative with respect to wellbeing both because it undermines control over one’s situation, and because it causes chronic pain. To take another, a disability that significantly impairs one’s memory may well undermine one’s control (by impairing mid-range and long-term planning) while at the same time undermining the significant richness that a fully functioning memory adds to one’s ongoing stream of consciousness. On views of wellbeing that give weight to the value within one’s stream of consciousness, memory impairment will undermine one’s wellbeing in two distinct, though intertwined, ways.

Those qualifications aside, in this section I focus on control over one’s situation. The terminology is my own. As such, I owe readers an explication of it. As an introduction, I note that the notion is similar to the more normatively loaded term autonomy.⁵ I choose not to use that term because I do not want to commit to any particular view of autonomy. It is worth mentioning, however, that on most plausible views of autonomy, central to the possession of autonomy will be possession of psychological capacities that enable processes of effective practical reasoning and decision-making. These are processes that include not only inference making, evidence weighing and intention formation, but general background capacities for knowledge acquisition, the integration of information with one’s goals and values, and so on. Just what psychological capacities enable these kinds of processes will depend on how one decomposes the mind – plausibly some of the perceptual capacities, along with capacities for attention, memory, practical reasoning, imagination, cognitive control, emotional regulation, and perhaps metacognition will play important roles. When decomposed in terms of contributory mental capacities, then, autonomy is a wide-reaching condition. The same thing is true of control over one’s situation.

Why do I refer to one’s situation? I need a notion that is sufficiently general. While control of one’s situation will include control over elements of one’s stream of consciousness (e.g., one’s imagination, one’s ability to ruminate coherently and reason practically and theoretically), it is broader than this. Given the kinds of beings we are, control over one’s situation for beings like us must include abilities to execute intentions, satisfy desires, and accomplish naturally arising goals in the world outside the mind. Roughly, then, possession of control over one’s situation can be understood as possession of capacities – these capacities may not stop at the skull or body, since assistive technology and caregivers may significantly enhance one’s control – that enable one to enhance good experiences and inhibit negative experiences (via, e.g., attention, reappraisal, imagination, targeted planning for the future, inhibitory control), to find and seek beneficial experiences and avoid negative ones, to shape one’s conscious mental life, and to shape one’s broader patterns of action in the world.

I have said control comes in degrees (see Shepherd 2014). Humans are in many ways fragile, and no matter how healthy or how well placed in society, no human adult possesses total control over her situation. Items hijack our attention, motivational levels fluctuate, the body can be difficult to work with, the environment can be uncooperative, and our desires are often wayward or regrettable. But adults display a significant amount of control in many ways. We exhibit significant degrees of freedom and control over what we attend to, over our trains of thought, over a life of imagination, over our responsivity to positive and negative events, over the ways we satisfy intentions, desires, curiosities, and so on.

Allow me to apply the key claim of this section – that in virtue of the fact that they significantly undermine control over one’s situation, some disabilities are inherently negative with respect to wellbeing – to both MCS and LIS. It is clear that the brain injury that results in MCS significantly impairs many of the capacities that enable such control. The same thing is true of LIS, although the impact of LIS is more indirect. And it is here that a contrast between LIS and MCS affords an important insight. For, when given access to proper care – and perhaps in societies that do not stigmatize such a condition – LIS patients can enjoy high levels of wellbeing. Notice, in this connection, that 5 out of 65 patients surveyed by Bruno et al. (2011) reported that their wellbeing was as high as it had been in the best period prior to LIS. That’s a low number, but that any LIS patient could report such a number is important. Notice, as well, that the elements that correlated with higher reports of quality of life were in the main those that indicated the LIS patient possessed greater control over her situation. As I discussed above, surveyed LIS patients were significantly more likely to rate their quality of life as higher if they had recovered some speech production, reported positively to the prompt ‘I move around my community as I feel necessary,’ reported positively to the prompt ‘I am able to participate in recreational activities (hobbies, crafts, sports, reading, television, games, computers, etc.) as I want to,’ reported positively to the prompt ‘I feel that I can deal with life events as they happen,’ reported an absence of depression or anxiety, and reported a lower frequency of suicidal thoughts. In addition, Rousseau et al. (2015) report that access to good electronic communication devices and to powered electric wheelchairs made a positive difference. This is some evidence for the view that retention of control is a central factor in enabling a level of wellbeing robust against even severe perturbation.

LIS illustrates two important aspects of control over one’s situation. First, one cannot control one’s situation very well without some degree of control over one’s body. That said, the amount of control over one’s body required for a robust level of wellbeing may seem, to many, surprisingly low. This is because, second, control with respect to one’s inner mental life appears to be weighted more heavily with respect to wellbeing than control with respect to one’s body.⁶ The upshot is a graded spectrum on which control over one’s situation can be impaired by bodily disability or psychological disability (however we wish to make that distinction). But bodily disabilities on their own rarely lead to significant impairments to control, while psychological disabilities more readily lead to such impairments given that many psychological disabilities target capacities central to the possession and exercise of this control.

The central claim of this section, then, enables an explanation of the difference between Bad Difference disabilities like LIS and MCS, and Mere Difference disabilities like deafness and blindness. As David Wasserman notes in comments on this chapter, the resultant typology is similar to that set up by Asch and Wasserman’s (2010) discussion of static disabilities. But there will be important differences. In particular, control over one’s situation may be significantly impaired when one suffers from various static psychological disabilities. Hopefully, then, my appeal to control affords a more illumination explanation of the relevant distinction. In this connection, consider the following list of potential disabilities.

Impairments to limbs due to congenital conditions or injury

Mobility impairments due to spinal cord injury or stroke

Loss of perceptual function, leading to, e.g., blindness, deafness, or deafferentation

Congenital insensitivity to pain

Language aphasias, e.g., Global, Anomic, Wernicke’s, or Broca’s aphasia

Attention Deficit Hyperactivity Disorder (ADHD)

Depression

Bipolar disorder

Personality disorders, e.g., Obsessive Compulsive Disorder, Scrupulosity, Narcissistic Personality Disorder

Learning disabilities, e.g., dyslexia, dyscalculia, or dysgraphia

So-called ‘severe cognitive disabilities’ associated with, e.g., traumatic brain injury, Down’s Syndrome, or dementia

Impulse disorders, e.g., intermittent explosive disorder, kleptomania

Volitional disorders, e.g., abulia, or akinetic mutism

It is possible to organize this list in a rough fashion according to the degree to which items on it undermine control over one’s situation. As already claimed, given adequate care and a non-ableist society, loss of perceptual function and certain mobility impairments undermine this control very little if at all. Other items are much worse, although given the complex interaction of capacities that enable control, various items will undermine control in various ways. That said, however, the notion of control over one’s situation is helpful in assisting reflection on the relation between the items on this list and wellbeing.

Some items on this list fall in something of a gray area. These items might, in very idealized conditions, qualify as Mere Difference disabilities. Congenital insensitivity to pain is often a debilitating condition, and proper care is high-effort and difficult to find. The reason is that pain sensitivity is crucial for navigation of our world. But one could perhaps imagine circumstances in which this disability did not undermine wellbeing. Language aphasias are a very different kind of thing. Some are mild and undermine control very little if at all. Some – e.g., Wernicke’s aphasia, which renders an agent unable to understand written or spoken language – seem to leave control over one’s inner life largely intact, while undermining control over how one interacts with others in most imaginable human societies. Plausibly, this latter kind of aphasia is much worse with respect to wellbeing than the former kind.

Other items on this list seem clearly to be negative with respect to wellbeing, although there will be differences in degree. Intermittent explosive disorder undermines emotional regulation. It is possible to manage the condition such that it’s impact on wellbeing is minimal, but in most cases the condition’s impact seems decidedly negative. Those with intermittent explosive disorder sometimes lose control of their behavior, engaging in violent bouts of rage and aggressive behavior. It is difficult to consider these episodes as merely different ways of behaving. The behavior and the consequences are often very negative.

To take a different kind of case, one condition on this list – abulia, which in extreme form is classified as akinetic mutism – targets the ground floor of control over one’s situation, namely, one’s motivation to perform actions at all. Abulia in general is often classified as an absence of willpower, a lack of initiative, or the presence of apathy. With appropriate external prompting, some of those with akinetic mutism are capable of sophisticated actions, such as playing card games, going to and using the restroom, reading and remembering items read. But without external stimulation they tend to do nothing at all, lying unresponsive for hours on end. As Colin Klein comments, ‘The primary deficit in AM patients seems to lie in forming intentions. AM patients present with a picture of profound apathy. It appears that they do not act because they do not want to act. That negation should be read with wide scope: the patients have no intentions whatsoever, rather than an intention not to act’ (2017, 35). Such a disability may not lead to objectively negative experiences. But it seems clearly negative with respect to one’s wellbeing insofar as it takes away all the normal patterns of thought and action that make one’s life go well. Moreover, to reiterate, the way it does so is by undermining motivational capacities central to one’s ability to control one’s own situation.

At the extreme end we find conditions that target one’s control to more significant degrees. Depression and bipolar disorder, for example, both in different ways target motivational, attentional, and volitional capacities, rendering stable patterns of thought and action difficult.

It appears, then, that a control-based explanation of the difference between negative and neutral disabilities can generalize to a diverse range of disabilities. Is significant control over one’s situation sufficient to explain the difference in every case? I doubt it – as I said above, disabilities are multifaceted features of complex biological beings. I doubt that thinking in terms of necessary and sufficient conditions is fruitful in this context. But it is nonetheless worth noting how difficult it is to find cases that qualify as a Bad Difference in the presence of control (cases of long-standing chronic pain being the only obvious one), and cases that qualify as a Mere Difference in the absence of control. In comments on this chapter, David Wasserman offers an interesting proposal regarding this latter class. He asks us to imagine ‘a condition that filled most waking moments with ecstatic but uncontrollable reveries, or a psychosis that, unlike schizophrenia, was characterized by warm, friendly, supportive voices – a kind of internal cheerleading squad.’ Such conditions seem – on some views of wellbeing – to qualify as Mere Differences (or better) while undermining control over one’s situation. But there are a few important observations we should make about such conditions. First, they only qualify as a Mere Difference on what I would regard as implausible views of wellbeing. It is a crude hedonism that cares about the tokening of ecstatic experiences to the exclusion of the wide range of rich, complex, positive experiences we typically undergo. If the ecstatic incidents were so frequent as to significantly undermine control, the subject would be blocked from many of these rich positive experiences. Second, in order to qualify as a Bad Difference, we only need a strong tendency in a disability to undermine wellbeing. This is consistent with some cases bucking the general trend. And it is plausible that any biologically plausible version of one of these conditions would, in many cases, present subjects with difficulties and problems, precisely because these conditions take control away from the subject, rendering her at the mercy of her condition. Some individuals could get lucky and find a good match between positive symptoms of the condition and an environment that allowed these symptoms to flourish in the absence of side effects, even as the disability on the whole qualifies as a Bad Difference. With that said, I do not wish to deny that counterexamples to my proposed explanation could be found. But given that I do not wish to frame my explanation in terms of necessary and sufficient conditions, it is important that these counterexamples run against a general and strong trend. It is the general and strong trend linking control over one’s situation and wellbeing that indicates the kind of control’s explanatory significance.

I would not claim that control over one’s situation can by itself regiment the relationships between the very diverse class of all disabilities and wellbeing. Clearly control over one’s situation is a high-level classificatory notion, and unhelpful in explaining many of the details that render specific disabilities and conditions what they are. But at a high level, the notion is fit for purpose. My aim here has been to render plausible, and worthy of further consideration, a view on which some disabilities are mere differences with respect to wellbeing, and other disabilities are bad differences. In order to do that, I needed to develop some explanation of the distinction. The discussion above indicates that control over one’s situation is critically important for maintenance of the type and level of wellbeing human adults typically enjoy. People with control over their situations possess a level of wellbeing that is robust against the kind of perturbations these disabilities create. Thus, the notion forms a key part of an explanation for why wellbeing is robust against some disabilities, but not others.