Gee, Thanks, Miracle of Modern Medicine: Down Syndrome and Alzheimer's Disease

The caregiving family member is my mother, and the afflicted is my brother.

He's 55 years old now and has been symptomatic of Alzheimer's for five of those years. He's been late-stage Alzheimer's for three or four years now-- probably four, but if only three, he knew the Red Sox won the World Series, and I prefer that illusion.

In the video, Dr. Lai describes the pathology of the condition and its implications for caregiving. Because such persons were already low-functioning developmentally, for example, it can be difficult to diagnose early-onset Alzheimer's and, again because of the pre-existing condition, the progression to late-stage dementia happens sooner. Almost all adults with Down syndrome begin exhibiting symptoms of Alzheimer's at around age 50, though she points out in an article for the ARC Advocate, Spring 2006 (Waltham, MA), that symptoms can begin as early as age 40.

Mr. Guay provides a wealth of tips and practical advice for dealing with the myriad of problems associated with the condition(s), calming an agitated person and even how to avoid accidently agitating the person yourself. "Approach from the front," he advises, for example, because objects in their peripheral vision tend to cause anxiety and fear.

My mother describes the experiences of a family member closely engaged in caregiving.

The video is, as Dr. Moran told me, "Geared to the caregiver, the people on the front line on a day-to-day basis." Her hint of battle or warfare is not inapt. As in all wars, the objective costs are measurably enormous--and feelings typically run immeasurably high. I asked two officials of People, Inc., Mark Duval, the Residential Director, and Nicole Cabral, the Coordinator of Psychological Services, about programs and costs.

Mr. Duval told me that the conventional day-hab model was obviously inadequate for this population that agencies will soon be required to provide for in increasing numbers. Even the typical group home, which has been the foundation for service provision to the developmentally disabled for a generation now, can't be sustained as a solution. Adults with Down syndrome and Alzheimer's not only require close and consistent staff contact to maintain their health and abilities for as long as possible, but the provision of such care in a day-hab or group home context deprives others in those facilities of needed staff contact. Any "new service model" capable of providing humane and effective services, he contended, will have to include some version of a "One to One staff program" probably related to current PCA (Personal Care Attendant) programs.

At the moment, their agency is only providing for one client in need of those special services, but they are already providing services for many adults with Down syndrome now, many of that certain age, so the construction of services and training of personnel is little short of urgent. Ms. Cabral pointed out—it seems obvious once you think of it—that even the real estate involved will have to be specialized. They're hoping to set up a house soon, but it will have to be gutted and the interior rebuilt. Facilities have to be one-story houses without steps inside or out.

Dr. Moran explained that to me. Negotiating stairs is actually a fairly complex task, requiring depth perception, balance, physical coordination and ordered movements. As the Alzheimer's sufferer regresses cognitively, they lose what they once knew. I know I first noticed the definite deterioration in my brother by watching him try to come down a set of stairs I'd watched him easily negotiate for years.

It seems obvious that providing even the minimum in humane care for this population is going to cost a lot of money—public sector money in most cases—and require considerable commitment of time and energy by professional and family caregivers. One of the reasons that this undertaking will be so great is the changes in social attitudes about the mentally retarded and other social issues that occurred over the last generation or two. But those very attitudes are destined to be the source of passionate conflict and those immeasurably high feelings I mentioned earlier.

Those attitudes combined with changes in medical protocols and technology have produced an odd, nearly paradoxical phenomenon I call "The Window." Before the 1950s or so, mental retardation and Down syndrome in particular was regarded as (at best) a tragedy to be resolved as quickly as possible. The tragedy sometimes resolved itself naturally because, according to Dr. Moran, infant mortality ran about 50% in such children. The resolution for most of the remaining children was institutionalization in short order, where they had estimated lifespans of about twenty years. So, death in infancy or after a childhood of institutionalization was the fate of most such persons. Thus, they weren't around to generate strong feelings in the public sphere. They were never discussed in public, and even in private, within families, they were frequently not discussed.

But beginning about 1950 (probably exactly in 1950, as I'll show below) to about 1970, attitudes transformed incrementally and Down syndrome children were more and more kept by their families and raised at home as modern medicine steadily increased both their chances of surviving infancy and their expected lifespan afterwards.

However, once medical science succeeded in identifying the genetic defect at the root of Down syndrome and amniocentesis was invented, allowing for testing for Down syndrome in utero, more and more prospective parents with fetuses known to be Down syndrome opted for termination of pregnancy, since, meanwhile, abortion had been decriminalized. According to the recent New York Times article cited above:

Until this year, only pregnant women 35 and older were routinely tested to see if their fetuses had the extra chromosome that causes Down syndrome. As a result many couples were given the diagnosis only at birth. But under a new recommendation from the American College of Obstetricians and Gynecologists, doctors have begun to offer a new, safer screening procedure to all pregnant women, regardless of age.

About 90 percent of pregnant women who are given a Down syndrome diagnosis have chosen to have an abortion.

This means, in sum then, that whereas two generations ago, Down syndrome children were rare creatures, it seems likely that two generations hence, Down syndrome children will be similarly rare for an entirely different set of reasons. So society has a window of about two generations of persons afflicted with Down Syndrome who are relatively common within the social experience of most people.

Part II: A Brief Social History:

Prior to 1950 in almost all instances, and following that until about 1970 in lessening instances, Down syndrome was widely regarded in society at large as a "Shame," both in the sense of "Oh, what a shame" and in the more potent sense of something to be "ashamed of." One set of my in-laws (all dead now) were never told by my aunt that there was a Down syndrome child on her side of the family. Over thirty years she kept the family "secret."

Almost all obstetricians and pediatricians advised strongly against keeping a Down syndrome child at home and supported that advice with recommendations and diagnoses that were both disturbing and frightening. Mothers were told, for example, that bonding was counter-indicated because the baby would probably die soon in any event--so the children were kept from their Mothers. Since my brother was a first born, the doctors told my mother to "put him away" and have another baby as soon as possible to fill the void. If children were already present in a family, parents were told, as in the case of the playwright Arthur Miller, that a Down syndrome child in the home would retard the development of other children in the home, setting up an impossibly tragic conflict for parents between the best interests of two or more children within a family. (See: New York Times 1 Sep 2007, A New Stage for Arthur Miller's Most Private Drama of Fathers and Sons).

But social attitudes began to change on August 26, 1950. That was the day that Roy Rogers and Dale Evan's "Mongoloid" daughter Robin was born. Evans was an incomparably brave woman, for she not only defied the doctors' frightening advice by insisting on bringing her baby home to raise, but she defied the prevailing social convention of Shame by making sure that literally everyone in the country knew that her child was Down syndrome. Sadly, the doctors weren't completely wrong--poor Robin only lived for two days less than two years, yet her life was documented in book written by Evans that remains in print to this day, Angel Unaware by Dale Evans Rogers.

By the time George Will's Down syndrome son Jon was born in 1972, the culture of Shame and Fear had almost entirely faded, although a new complex of social attitudes had not quite established itself. Public sector programs like Special Education had been instituted and medical protocols had advanced to such a degree that Down syndrome children like my brother were growing into young adulthood socialized, educated appropriately and with their families comforted by the possibility of their children's potential for happiness, heath and security being fulfilled.

During the first twenty years or so of Jon's life, a new culture of Celebration and Rights developed--the celebration evident in the continuing stream of heartwarming stories which became a staple of local newspaper human interest and in the critically-acclaimed television series Life Goes On starring a highly-functioning Down syndrome actor, Chris Burke. You yourself feel the celebration as an ordinary person in the ordinary course of an ordinary life witnessing the forthright yet common presence of Down syndrome persons. That Down syndrome person you see smiling at you at the end of a supermarket checkout line never failed to soften whatever resentment you might've felt at the person ahead of you with eleven items in the ten-items-or-less line.

Simultaneously, the Disability Rights movement successfully pressed for legal and civil rights for the developmentally disabled in all fifty states, culminating in the Federal Americans with Disability Act.

So by the time Michael Berube's Down syndrome son Jamie was born in the early 1990's, the social, medical and legal environment surrounding those with developmental disabilities had been almost entirely reversed from what it had been barely a generation earlier. Berube, a Professor of Literature at Penn State, also wrote a book about his child, Life As We Know It: A Father, a Family, and an Exceptional Child. In addition to the painful personal narrative, the book lays out his literary theory of Disability Studies.

On one level, Berube's book is about as far from Dale Evans' book as it's possible to imagine and still place both objects in the category of "book," yet, on the other hand both are animated by the same emotive force, hanging from the same narrative frame. She was a provincial who left school to elope out of Uvalde Texas at age 14, a devoted and undoubting Fundamentalist Christian and conservative Republican; he, a New York City born, Ivy League educated liberal/left cosmopolitan. Nevertheless: They loved their Down syndrome child, they hurt, and they found solace in writing of their love and pain. Together, they perfectly frame the social history of Down Syndrome over the last 50 years.

Part III: An Essay:

Growth in a field thought barren is no less than thrilling, the redemptive reward for the planter infinite. Dale Evans explains in the brief first person introduction (the text proper is written from Robin's point of view) that:

It has been said that tragedy and sorrow never leave us where they find us. In this instance, both Roy and I are grateful to God for the privilege of learning some great lessons of truth through His tiny messenger, Robin Elizabeth Rogers.

And the point is emphasized by Norman Vincent Peale (The Power of Positive Thinking) in the introduction to Angel Unaware:

Where most babies die and leave their mother crushed, Robin put on immortality and her mother found the very joy of God in what otherwise might have been an overwhelming tragedy. [emphasis added]

Here, with the declarations of joy, gratitude and privilege, we see the foundation of the culture of celebration. And yet . . . despite its obvious appeal to our more beneficent impulses and the morally superior demand for humane treatment of the developmentally disabled, there is something discordant and dissonant. It's all well and good to celebrate Evans's courage and strength of spirit, but what about "most" mothers who Peale himself says would be crushed by such events? Do we leave them abandoned and unconsidered because their lives aren't the kind of lives likely to be celebrated?

Even if Peale's language is hyperbole, if the experience isn't crushing for most, it's not fun. One of the mothers of a Down syndrome child quoted in the New York Times article on pre-natal testing says:

"If someone had told me Sam would still be in diapers at age 5 — ugh — I probably would have died. . . . Living through it, not such a big deal. Because you don't give birth to a 5-year-old, you grow with and love this kid for five years."

Which reminds me of a story. My brother was born. My mother changed his diapers for a year-and-a-half. Then the twins were born. She changed ours until we were toilet-trained at the normal age. Still she changed my brother's. Then my younger sister was born three years after the twins. Then my Mother changed her diapers. Still she changed my brother's. Changing diapers on the same child for five years is a Grind, especially when it seems possible that the diaper-changing will be perpetual. Every one of those tiny triumphs documented in those heartwarming local newspaper stories or on Berube's blog represents months or even years of grinding stagnation and failure. Not everyone is capable of withstanding that grind.

Tilling the seemingly barren field is always hard work, even when growth unexpectedly occurs--how demoralizing and dispiriting it must be to plant that field so diligently and to see little or nothing sprout.

I don't mean to sound bitter. I do indeed understand the rewards of those tiny triumphs. What I resent, I suppose, is romanticizing by the purveyors of the heart-warming sentiment, as if that's all there was to it. When you share a smile with the Down syndrome cart-collector at the supermarket and then step spritely to your car, you have no idea of the energy and effort that had been invested to give you that moment of touching grace. There was 23 hours and 59 minutes of grinding behind that minute of the day you were given. Deciding that that minute is worth the 23:59 is a value judgment, a trade off, that not everyone is willing or able to make.

The presumption that one Down syndrome family's experience will be as positive and rewarding as the next is simply unwarranted. In Tolstoy's construction, the part of the experience that's happy is the same for all of us; the part that is unhappy is unique to each of us. And the unhappy aspects don't make very interesting or uplifting stories. The predominant subtext in the current culture of Celebration and Rights is to generalize precisely those aspects of experience that don't need to be generalized or that have been repeatedly generalized already and to leave unstated those specific aspects of experience that do not easily fit the new attitude.

One aspect of the Down syndrome family narratives I've read, from the items in a local paper to the books, is how the families or authors almost invariably employ those experiences and construct those narratives to vindicate and validate pre-existing worldviews--this writer most definitely not excepted. I remember during the heated debates on the Terri Schiavo case one of my interlocutors brought me up short with the pointed rejoinder that just because my brother was one step away from Schiavo's condition didn't mean I owned the issue (and I expect there will be those who make a similar and not altogether unjustified claim about this very work).

Still, is it not notable that Dale Evans situates Robin in support of her view of Christianity, that Michael Berube situates Jamie within Literary Theory associated with Disability Studies and that George Will situates Jon as a powerful argument against Roe v. Wade?

The temptation seems to be all but irresistible, although perhaps necessary as a coping mechanism, but do we not lose something of the Down syndrome person as an individual when we use our experiences of them as a buttress for a worldview? When the Disability Rights laws were being passed in the 1990s one social worker excitedly informed my mother that my brother was now eligible to vote, and urged her to have him registered. The underlying generalization was that the developmentally disabled were citizens too, and should have all the rights citizens have.

Are you kidding me? No way my brother registers to vote. Philosophically, of course, he shouldn't vote because he is incapable of the kind of abstract thought required of a knowledgeable citizen. Granted, I'd love to have had an extra vote for Bill Clinton that year, which I could've had by simply taking my brother to the polls, having him clog the lines as he signed in, then telling him "Bill Clinton is the good guy, Bob Dole is the bad guy," but that wouldn't have been very ethical. But practically speaking, registering him to vote was a ridiculous idea.

He would register to vote. He'd get on the list of those subject to be called to jury duty. He'd get called. My mother would fill out some form declaring he's incompetent to serve. Some judge or bailiff would decide it's just someone trying to avoid jury duty. My mother would have to waste a day driving my brother to Dedham to present him in person for disqualification. It's not like that all hadn't happened before when some fool at the Pentagon tried to draft him to go to Vietnam. The well-intentioned social worker, at the end of the day, while celebrating the right to vote, was in all truth probably simply adding to the Grind.

Do I think the developmentally disabled should be allowed to vote? Actually, I don't know, or rather, I think it should be decided case-by-case, but in any event, it's not important. I'd rather my brother have the right to services that allow him comfort, happiness and security than that he be given a right he can never really understand or utilize as intended. Is mainstreaming and inclusion a good idea or a bad idea? Again: I don't know. It has to be case-by-case. Those who would or will benefit should be mainstreamed, those who wouldn't or won't should not be.

When I read this passage in the "Prenatal Test" article, I guess I must have shuddered:

[A] growing group of parents is seeking to insert their own positive perspectives into a decision often dominated by daunting medical statistics and doctors who feel obligated to describe the difficulties of life with a disabled child. . . . .

The parent evangelists are driven by a deep-seated fear for their children's well-being in a world where there are fewer people like them. [emphasis added]

The deep-seated fear, I get. The skepticism of the medical profession, I get. The evangelism, I don't. And that evangelism is implicit or explicit in almost all the family narratives that reach public notice. Will, for instance, says:

Jon, a sweet-tempered man, was born the year before Roe v. Wade inaugurated this era of the casual destruction of pre-born babies. And he was born just as prenatal genetic tests were becoming routine. Since then, it has become routine to abort babies like Jon because they are like Jon. Without this combination of diagnostic advances and moral regression, there would be more people like Jon, and the world would be a sweeter place.

Will also says in his column that life-expectancy for Down syndrome has risen to 56, although the Times article claims 49. But it really doesn't make any difference because those extra 7 years will simply be a rapid descent into late-stage Alzheimer's anyway. Isn't modern medicine "sweet"? Okay, that did sound bitter, and I meant it this time.

Will offers a general proposition, that the world is a sweeter place because his son Jon is in it. I'm sorely tempted to say that that is untrue simply because of the indirect influence Jon has on Will's polemics, but in truth, I don't know whether the proposition is true because I don't know Jon. Is the world a better place because it includes a population of persons with Down syndrome? As a general proposition, that remains an open question. I don't know.

But.

I do know that my spirit is enriched and my own life is better for having lived it with my brother.