November 6, 2006

Helping families care for the helpless

Bioethicists who work in health care are frequently called upon to make difficult decisions in often less than desirable situations. Thankfully, the steady introduction of new technologies provide ethicists, health practitioners and families with a variety of options. The trick these days is to choose the most desirable course of action. But the fact that most new technologies and the manner in which they are applied often appear shocking and radical at the outset makes ethical decisions even more difficult.

Take the recent case of a severely disabled 6-year old girl and her parents' decision to administer her estrogen treatments to deliberately stunt her growth. According to her parents, the reason for wanting the girl to remain small have to do with their ability to care for her on an ongoing basis. The fear is that she will grow too large such that the parents will no longer be able to care for the girl and will have to be institutionalized. The girl was also given a hysterectomy.

At first blush, this sounds quite bizarre and even a bit extreme. My own initial reaction was negative; any time I hear about constraints (or what might even be considered mutilation) being deliberately imposed upon someone my alarm bells go off. But after deeper analysis, I have come to conclude that I agree with the parents and their medical advisers.

Before I get into my reasoning it’s important that that the girl’s condition be described for proper context. She is 6-years old and suffers from severe combined neurologic and cognitive impairment; she is physically disabled and will permanently have the mental capacity of an infant. There is currently no known treatment to help alleviate this. The girl can respond to her parents and siblings by vocalizing and smiling in response to care and affection. She is described as being an “integral, and much loved, member of the family."

That said, her parents recently came to the conclusion that her continued growth would soon start to pose a problem. Children with combined neurologic and cognitive impairment are utterly dependent on their caregivers much like an infant is – they cannot bathe, dress and transport themselves without assistance. In these cases, the responsibility almost always falls on the parents. Over time, as the child grows into an adult, these challenges increase.

Given these factors, and considering the loving and healthy household that the girl currently finds herself in, the question must be asked: Will the estrogen doses and hysterectomy reduce the quality of life for this girl? One could make a strong case that, by virtue of the fact that her parents will still able to care for her, that her quality of life will in fact increase (or remain consistent) relative to the kind of care she might hope to receive by her parents should she not receive these treatments, or considering the introduction of a 3rd party and her potential response and adjustment to it.

Moreover, this girl is severely disabled enough such that her self-awareness is virtually negligible. She is barely engaged with her outer and inner worlds. Nonetheless, like an infant, she requires care such that she remains comfortable and finds herself in a loving environment. Ultimately, however, the estrogen doses are not so much for the girl as they are for the parents. Aside from the enhanced care that she will receive from her parents as a result of the treatments, the doses will have no bearing whatsoever on the girl given her permanent condition.

A colleague of mine noted that there are some potential abuse issues that need to be addressed. For example, is the resource-crunched medical establishment more inclined to have parents take the burden of care? Is this a way of passing the buck? Is the hysterectomy really necessary?

These are valid concerns, but I think they can all be addressed.

First, I believe the wishes of the parents are what is driving this decision and not the demands of the medical institution. In this case, it appears to me that the needs and desires of the parents are being met.

As for the hysterectomy, I have to question the value of keeping this girl fertile. If the concern has something to do with the girl’s dignity being violated, then I have to protest by arguing that the girl lacks the cognitive capacity to experience any sense of indignity. Nor do I believe this is somehow demeaning or undignified to humanity in general; the treatments will endow her with a body that more closely matches her cognitive state – both in terms of her physical size and bodily functioning. The estrogen treatment is not what is grotesque here. Rather, it is the prospect of having a full-grown and fertile woman endowed with the mind of a baby.

Stepping back a bit, it’s obvious that this is an undesirable situation to begin with. I have to wonder if this case qualifies as a so-called ‘wrongful birth.’ On a related note, the UK's Royal College of Obstetricians and Gynaecology are now urging health regulators to consider allowing the "active euthanasia" of severely disabled newborn babies (notably when babies are born with such debilitating and painful conditions as severe spina bifida). "A very disabled child can mean a disabled family," notes the College, "We would like the working party to think more radically about non-resuscitation, withdrawal of treatment decisions, the best interests test, and active euthanasia, as they are ways of widening the management options available to the sickest of newborns."

One would hope that future medical technologies will help severely disabled children overcome their debilitating impairments – but the reality of the situation is that such treatments are quite a ways off. This girl is alive in the here-and-now and as such should be given the appropriate level of care. And of equal importance, the family's needs have to be considered as well. In this particular case, it will be through the estrogen treatments that the parents will be able to make the best of a difficult situation.

4 comments:

AnneC said...

Well, personally I find it tremendously disturbing that people are making such dramatic assumptions about this girl's capacity for self-awareness. Additionally, I don't think it's at all appropriate for doctors (or even parents) to assume anything about a person's developmental trajectory when they are only six years old.

Imagine being that girl and knowing what was going on but not being able to communicate your impressions or feelings of the situation...knowing all the while people are making decisions for you and that they are violating your bodily autonomy for their own convenience. It could be that the girl will really never know the difference, but the fact is undeniable that that she might -- and the fact that present means of establishing self-awareness and developmental potential in people with disabilities are dismally backward and inaccurate.

I also don't think any of us has any right to say which kinds of brains should go with which bodies. You might assume someone has "the mind of a baby" when they're an adult, but how can you really know that?

Also, this sort of thing sets a terrible precedent. First we've got purposeful growth-stunting...will we have forced amputations next? Will disabled children have their arms and legs cut off so they won't run away or grab things? Will they be purposely malnourished at a young age, since that can also permanently stunt growth?

Back to hormones, though...hormones are responsible for a lot of signalling processes in the body, including things that affect brain development and such. People go through a lot of cognitive changes in adolescence -- by forcing hormones on a small child, you could be denying them the opportunity to develop in ways that nobody could have anticipated.

The idea that ANY of us can really judge another person's cognitive or emotional capacities, particularly just through reading an article about them, is rather ridiculous. As far as radical new technologies go, one thing I would really like to see is a better means of determining self-awareness in people without easily-interpreted communication systems. That is something the world is sorely lacking.

And one place to start is probably that of talking to communicative people with disabilities, who are often much better at empathizing (and communicating) with people deemed "unreachable" than are nondisabled people. THAT might sound like a radical idea too, though!

George said...

On 11/7/06, AnneC wrote:
> Well, personally I find it tremendously disturbing that people are making
> such dramatic assumptions about this girl's capacity for self-awareness.
> Additionally, I don't think it's at all appropriate for doctors (or even
> parents) to assume anything about a person's developmental trajectory when
> they are only six years old.

> Imagine being that girl and knowing what was going on but not being able to
> communicate your impressions or feelings of the situation...knowing all the
> while people are making decisions for you and that they are violating your
> bodily autonomy for their own convenience. It could be that the girl will
> really never know the difference, but the fact is undeniable that that she
> might -- and the fact that present means of establishing self-awareness and
> developmental potential in people with disabilities are dismally backward
> and inaccurate.

I think you're making some fairly special claims about this girl's cognitive state and potential. You say "the fact is undeniable" that she *might* have such awareness. Aside from this being a 'begging the question' argument, on what do you base this? Are you seeing any research that reveals otherwise?

I happen to have some personal experience with this as my mother is a home health care provider. I've seen these individuals first hand, and the suggestion that they have some kind of complex internal world is a stretch. My mom is handling one case with a grown woman with severe cognitive impairment. She has adequate physical capacities but her mind is that of a toddler's. She can walk around, but mindlessly follows anyone who will lead her. She cannot feed herself even though she has the motor skills to do so; her own mother has to feed her by hand and has found that it's best if she place the food directly into her mouth by hand. She can grunt out the first 3 or 4 letters of the alphabet, but that's about it. It's not a case where she simply cannot express herself or her needs; she simply lacks the cognitive capacity for such mental processes and actions.

Now, this particular woman is doing reasonably well in the physical department, but the 6-year old also suffers from a neurologic disorder. Her physical disabilities, combined with her cognitive disorder, make her a far more severe case.

As for making 'assumptions' about the girl's developmental trajectory, I don't think anyone is making any assumptions at all. Have you heard of any case where a child born with such a severe neurologic and cognitive disorder has 'pulled out if it' and had any kind of developmental trajectory to speak of? I have not -- and when the doctors claim that such conditions are permanent, I don't think for a minute that they're saying it lightly.

> I also don't think any of us has any right to say which kinds of brains
> should go with which bodies. You might assume someone has "the mind of a
> baby" when they're an adult, but how can you really know that?

In this case, I am satisfactorily convinced that a) this girl's condition is severe and permanent enough such that changes to her body will have no physical or cognitive bearing on her current or future condition, which means that b) any effort to help the family take care of her will be to the benefit of both the girl and the family.

> Also, this sort of thing sets a terrible precedent. First we've got
> purposeful growth-stunting...will we have forced amputations next? Will
> disabled children have their arms and legs cut off so they won't run away or
> grab things? Will they be purposely malnourished at a young age, since that
> can also permanently stunt growth?

This is a slippery slope argument and I'm surprised that you resorted to it. Your examples are so extreme, unwarranted and out of context that I'm not going to bother responding to them.

> Back to hormones, though...hormones are responsible for a lot of signalling
> processes in the body, including things that affect brain development and
> such. People go through a lot of cognitive changes in adolescence -- by
> forcing hormones on a small child, you could be denying them the opportunity
> to develop in ways that nobody could have anticipated.

Again, you're going to have to show actual examples in which this has happened to individuals suffering from similar conditions.

> The idea that ANY of us can really judge another person's cognitive or
> emotional capacities, particularly just through reading an article about
> them, is rather ridiculous. As far as radical new technologies go, one thing
> I would really like to see is a better means of determining self-awareness
> in people without easily-interpreted communication systems. That is
> something the world is sorely lacking.

I agree that we should develop technologies to help us better grasp the internal state of people's minds. This would be particular helpful for people in comas. fMRIs are going a long way to help in this process. But you're making a strange claim that I am making an assumption just by reading an article. I'm not sure I understand. My post was about the decision made by the parents and the team assessing this girl and the conclusions that they have reached. This obviously wasn't my decision, I am merely reflecting my agreement with their decision. I'm sure their assessment of this girl and any potential she might carry was as thorough as possible.

> And one place to start is probably that of talking to communicative people
> with disabilities, who are often much better at empathizing (and
> communicating) with people deemed "unreachable" than are nondisabled people.
> THAT might sound like a radical idea too, though!

It sounds like the parents and the girl's siblings are doing this. As the article noted, she responds by smiling much like an infant does. I'm also sure the assessment team tries to engage the girl at a communicative level, perhaps even utilizing non-verbal communication. I don't think there's anything radical about this and why you feel no one has tried to reach her in this way.

Cheers,
George

AnneC said...

OK, now for a hopefully comprehensive response...

I think you're making some fairly special claims about this girl's cognitive state and potential. You say "the fact is undeniable" that she *might* have such awareness. Aside from this being a 'begging the question' argument, on what do you base this? Are you seeing any research that reveals otherwise?

I don't think I'm making any special claims at all, but rather that I'm perhaps starting from a different default assumption than you are. I don't see any reason to assume someone lacks a particular degree of self-awareness, provided they're not dead or anencephalic. The six-year-old girl whose situation prompted this discussion is described as a person who "...responds to her parents and two healthy siblings — vocalizing and smiling in response to care and affection."

If a person can smile in response to care and affection from family members, that at least indicates she recognizes these family members and can interpret their actions to an extent.

I've seen these individuals first hand, and the suggestion that they have some kind of complex internal world is a stretch.

Why is it a stretch? I'd like to know how, in general, you assess the complexity of another person's internal world. It seems like you're basing this assessment mainly on external behavior and the presence or absence of self-care skills.

It is fallacious to suggest that walking, talking, and feeding onesself are precursor skills to the ability to have a busy inner life. It is, of course, assumed frequently that development is a linear progression through a particular set of steps, and that a "missing" step means that the rest of the person's development collapses into some kind of void, but that's not the way it works in real people.

My mom is handling one case with a grown woman with severe cognitive impairment ... It's not a case where she simply cannot express herself or her needs; she simply lacks the cognitive capacity for such mental processes and actions.

Some people with developmental disabilities have difficulty initiating and prioritizing tasks, and planning motor activities. This only tells you that they have trouble with initiating and prioritizing tasks and planning motor activities -- it doesn't tell you what they are thinking, how often they are thinking, what they are thinking about, etc.

I don't think anyone should hold unrealistic expectations about what activities people can engage in; obviously, if someone cannot feed themselves, they are going to need assistance with that task, and it is no indignity or insult to help them.

But behaviors are not brain states. Unfortunately, behaviors and observable skill sets are often the only empirical means by which a person can be assessed (such as would be necessary in order to secure services) -- but remember that this is a limitation of present human powers of observation, not incontrovertible evidence that there's "nothing going on" in these people's heads.

Have you heard of any case where a child born with such a severe neurologic and cognitive disorder has 'pulled out if it' and had any kind of developmental trajectory to speak of?

Since I don't know the exact diagnosis of the six-year-old in question here, I can't say whether I have heard of anyone with her particular difficulties who ended up somehow demonstrating a high degree of self-awareness.

But I have heard of people considered "severe", "profound", etc., in various developmental realms -- until they learn to type or otherwise communicate. I could start naming names here, and I noted a few in my e-mail, but I think what we really need here is a study.

I've got a theory that "outliers" aren't so uncommon as they may seem; it has more to do with particular sets of environmental and interpersonal variables that these people end up encountering in their lives.

The very existence of even one person who looks like an "outlier" -- someone who has an appearance many associate with "no higher cognitive functioning" yet who reveals, through some means, a very strong sense of self awareness -- is an unignorable and significant data point.

Significant on the order of a person surviving a cryonic suspension -- perhaps you could think of it as a "proof of concept". The proof exists, and in my mind, that proof necessitates an overhaul of attitudes toward and perceptions of people with severe disabilities.

Again, this doesn't mean expecting that someone who cannot use a fork is going to win a Nobel Prize, but it DOES mean accepting that being able to use a fork has nothing to do with being able to feel or think. Why not default to treating people like people, regardless of their demonstrated skill set? Are people afraid of looking "silly" or something?

I would rather err on the side of "looking silly" than on the side of assuming anyone is a nonperson based on their behavior. All I can judge based on their behavior is the set of practical skills they can reliably demonstrate, but a set of practical skills isn't a reliable indicator of anyone's sense of self (unless you can point me to some research in this area that could convince me otherwise).

This is a slippery slope argument and I'm surprised that you resorted to it. Your examples are so extreme, unwarranted and out of context that I'm not going to bother responding to them.

I'm failing to see how making a valid comparison (all measures I cited, including hormonal treatment and hysterectomy, represent drastic physical interventions that could be employed with the intent of making the receiving individual "easier to care for".) is extreme or unwarranted.

Yes, it is a slippery-slope argument -- but slippery slopes do exist in real life, though one must be very careful about invoking them. But you're probably right that amputations and such will not follow the kind of hormonal treatment being discussed, simply because of the "yuck factor" a lot of people associate with amputation and physical deformity or appearance of asymmetry or "incompleteness". Hormonal treatment and hysterectomy are "invisible" to the observer, save for the stature of the person on the receiving end, but small people aren't generally considered pathological for the mere fact of their smallness.

This doesn't mean it's an open and shut case, though; I don't think anyone can read a story about a girl being stunted with hormones and not ponder it for at least a while afterward, due to the stickiness of the issues it raises.

I agree that we should develop technologies to help us better grasp the internal state of people's minds. This would be particular helpful for people in comas. fMRIs are going a long way to help in this process.

Yes, and I am very supportive of this research. We're definitely in agreement on this point.

But you're making a strange claim that I am making an assumption just by reading an article. I'm not sure I understand. My post was about the decision made by the parents and the team assessing this girl and the conclusions that they have reached. This obviously wasn't my decision, I am merely reflecting my agreement with their decision. I'm sure their assessment of this girl and any potential she might carry was as thorough as possible.

Ah. Well, I'm not so quick to assume that the assessment was either thorough or carried out by competent individuals. I have no fear of questioning authority, including medical authority, and including "teams of experts".

The stakes are too high for me to just accept that doctors, right now, have the ability to assess a live person-with-a-brain's ultimate potential when the person in question is only six years old.

Note, though that I'm not making positive claims about the existence of any particular level of self-awareness, I'm just questioning the validity and accuracy of "assessments" commonly used, and I think these are very important things to question.

Again, the existence of "outliers" is proof of concept that even the most supposedly-thorough doctors can be wrong. Realize that what is happening here is the permanent experimental alteration of a child's morphology for the convenience of the parents (and for society, which won't need to provide as extensive a set of resources to support a smaller person).

I'm not saying I know exactly what should be done instead, but in some ways this sounds like a reverse version of the Peter Singer argument that you yourself dismissed as rather silly a while back (the one about parents possibly selecting for taller and taller children, resulting in a race of resource-depleting giants).

At any rate, you should know by now that I'm not a bioconservative of any stripe, nor do I argue on the basis of naturalistic or religious fallacies.

One thing that makes these kinds of discussions so challenging is the fact that everyone on every side thinks their position is based on the best possible set of ethics and scientific principles -- I realize that the parents in these situations don't have "weird agendas" and I also realize they love their children, and I realize that caretakers need care too, but I also think that good intentions do not a good decision necessarily make.

You've actually given me a bit of an idea for a possible article/project in the future (once I finish a few of my current projects) -- I'm now very curious about the science of self-awareness and how it can be evaluated respectfully in both people and machines (I'm already envisioning plenty of debate over the supposed awareness of the first AI...)

George said...

Anne: you make some strong cases for being cautious, which I'll grant you. I'd like to bring the conversation back full circle, however, to discuss the issue at hand, which is not necessarily the degree of awareness that this girl has, but the application of estrogen and the hysterectomy.

As I stated to you in our e-mail correspondence, my feeling is that you're overstating the impact of these treatments on the girl. I think when the pro's and con's are brought out, the benefits exceed the risks and potential constraints.

How will being small impact this girl who is neurologically damaged to begin with and cannot move on her own? I would argue that this is a non-issue as far as the girl is concerned.

What about the hysterectomy? I think it's fair and reasonable to say that this girl, if she remained fertile, would not have any kind of sex life to speak of. This would also prevent the girl from becoming pregnant should she be raped (an extreme possibility, I know, but I thought I'd mention it here). A strong case could be made that any kind of sexual relationship this girl has is tantamount to abuse. So again, this is a non-issue as far as the girl is concerned, and may actually prove to be a benefit.

Will the hormonal differences have any kind of measurable impact on the girl's cognition and sense of awareness? And if so, would these differences be detrimental? My opinion is that the impact will be trivial. Moreover, if the changes are measurable in some way, I'm inclined to think that the impacts will not increase the girl's suffering. I'm sensing this is where you and I diverge on the issue.

Finally, a cost-benefit analysis like this has to take the family into consideration and their ability to care for the girl on an ongoing basis. I believe the girl's level of care will increase as a result of these treatments, and to me that is the ultimate reason for advocating these treatments. Further, the family's quality of life will remain fairly constant, leading to their ongoing ability to care for the girl. This is an important factor that needs to be affirmed.

Essentially, what I'm saying is that you may be right about the girl's awareness (but I highly doubt it), but that does not impact on the need to ensure that her care remains at a high level of quality and that her suffering not increase.

As a thought experiment, let's assume for a moment that the girl has a fairly complex internal life and that the family abandons the estrogen treatments and hysterectomy. She soon grows to the size of an adult and the family cannot transport and care for her like they used to. The family starts to become increasingly frustrated, which impacts on their relationship with the girl. She soon becomes institutionalized and has to deal with the trauma of a new environment and new caregivers, many of which may not be up to the same standards as her family members. The woman eventually becomes deeply depressed and is utterly unable to express her concerns.

To summarize, the estrogen and hysterectomy do not impact on the girl in any meaningful way, and do not increase her degree of suffering. They do, however, increase her quality of life by ensuring that she can continue to be cared for by her family and avoid institutionalization. At the same time, the members of the family have their lives enhanced by knowing that they can continue to care for the girl well into the future and are not at any risk of dangerous or overwhelming hardships -- including the need to institutionalize the girl.

This is the right decision.