Living with Trisomy 13, Part 1: Stereotype Propagation and the Illusion of Choice

[This is the third post in a series highlighting a public dialogue held at the University of Alberta on October 23rd, 2008, titled The Modern Pursuit of Human Perfection: Defining Who is Worthy of Life. The dialogue was sponsored by the What Sorts Network, in conjunction with the Canadian Association for Community Living and the Alberta Association for Community Living. This series will bring forward the videos made of this event twice a week, roughly every Wednesday and Saturday. For further context, please see the introductory post in the series, which can be found here; we'll string together all posts in this series when we have most / all of them up.]

In the following video clip (with transcript) Sam Sansalone begins to share his experiences fighting to save his daughter, Katya, who was born with Full Trisomy 13 (a condition where the child has an extra chromosome 13, for more information see www.livingwithtrisomy13.org). I think you will find this story interesting for two points that Sam shares. First, there is the issue of medical personnel intentionally withholding information about the quality of life that children with Trisomy 13 can expect; an act of deliberately propagating a stereotype. The exact reason for sharing this behaviour is unclear but it is likely at best a form of misplaced paternalism and at worst a set-up for point two.

Point two is Sam’s experience with the illusion of choice that can so often exist in our society, whether inside the medical community or not. In such situations people are initially presented with the opportunity to make their own choice about a difficult decision (and typically information to sway them to a particular side, the tie-in to point one). If the choice falls inline with what is expected then all is well, there may be a few tears and some whispered “I know it’s hard, but you’ve done the right thing”s, but life goes on. BUT if the choice falls outside of what is expected, then you are clearly not in your right mind, your response is rejected, and authority for the decision is assumed by whomever gave you the illusion of choice in the first place.

When the bioethics community steps in, well, watch and see… 

Part 2 of Sam’s story can be found HERE.

Transcript:

Sam Sansalone: My daughter’s name is Katya Sansalone. She’s now 7, which is extraordinary because that apparently puts her in the position of being the oldest one alive in the country with her condition, which is full trisomy13. We don’t know that for sure, but two hospitals believe that. And I was really moved by Wendy’s story, second time I’m hearing it now, and you know there really are similar themes in a lot of these stories. I think in our case, as she mentioned, it was about making the right decision and fighting a system that largely just does not want to give access to medical resources for these children who are developmentally disabled or have prospects of being profoundly developmentally disabled. Katya is an extraordinary little girl. She’s certainly developmentally disabled, but she’s one of the happiest people you’ll ever meet. She experiences joy and gives joy to people around her; strangers even. She’s a real example of joie de vivre. And, the way I see it, she’s just another kid and the problem that she experiences is that she’s not allowed to be viewed that way and treated that way. Going now to the issue of where the big ethical challenges were with her, it was pretty much right from the beginning. Once they had a diagnosis for her, they made a determination without telling us that this was someone whose life should not be saved. What they told us in the beginning, however, was we had a choice to make. She had a heart condition, which was common for kids with this chromosomal defect, that she would die from if she didn’t have a certain kind of lifesaving cardiac surgery that is now routine – tetralogy of fallot repair – but they couldn’t recommend it because of quality of life issues. And we didn’t know much about trisomy 13 or trisomy13; my wife’s a physician but in a completely different specialty and just remembered very little about it from medical school, so we were relying on what we were being told by the physicians and her colleagues, some of whom, you know, she would have brushed shoulders with in that hospital. And what we were being told turned out to be false. And it wasn’t by coincidence or by error. It was intentional. We were told that the range of outcomes for these kids was that: virtually no life expectancy, within months they die, that any that do survive – very rare ones that do survive – beyond that don’t have a life that we consider of quality, that they used examples of kids and people who go through lives not smiling, not recognizing people, not knowing their family, not interacting socially. And the bizarre thing about this is that there was research 7 years before that, we later found out, that completely dispelled this myth. And yet they were propagating it on an active basis, in a way that would have led to a decision that would have killed our daughter, by withholding of vitally needed medical interventions. That was just part of it. So they, when we found that out, which was by very interesting means, there was one of the resident neonatologists, I remember he had a clipboard and I remember the study because the title was bold, and when I saw that he had this study on his clipboard and went to look at it – and he referred to it saying “look, we’ve told you about the natural history of this, it’s dismal,” he made it sound like there was no hope, there was no point in choosing to give her cardiac surgery, so when I went to look at it, he literally turned the clipboard away from me, withholding information that would have been vital. I got that study from the author himself, a doctor in Utah who is one of the eminent researchers in this area of medicine, after doing some research. I communicated with Dr. Utah and he faxed those very studies right to the neonatology department to my attention – went right past the people who were trying to prevent me from seeing this, and what that document revealed was a study – now well-established; it was about 7 years old – that it’s not true, it’s all myth, the range of outcomes is very wide. The ones that survive infancy do have quality of life that’s defined by any reasonable ethical measures. And once I got that information, and realized that these are happy kids if they survive infancy, there was no question in my mind that she deserved to have and should have the lifesaving cardiac surgery. Why would I deny Katya a medical intervention that was needed if I wouldn’t deny Gemma, my older daughter, or Caleb, my son – they’re not developmentally delayed – why would I discriminate against her? It made no ethical sense whatsoever to me. And at that point, when they realized that we were deciding in that direction, all of a sudden it wasn’t our choice anymore. So, first we had the choice: ‘it’s going to be a very difficult choice and we’d hate to be in your position, but this is what we suggest,’ and then when we make the decision it’s: ‘well, you don’t actually have a choice after all.’ So we had to fight them and it took about three weeks of debate. We involved the bioethics community – or they actually initiated the involvement of the bioethics community within the hospital, and then within the hospital they went to the broader Ontario bioethics community and at one of the meetings they had with a good number of attendees they had an informal vote after a discussion about this case and they all voted unanimously with us. So as a result of that, that really gave the impetus for us to force the hospital to give her this lifesaving cardiac surgery, which had never been done before in Canada – strangely. There were plenty of opportunities to do it, but apparently no parent had at that point been aware, and we could easily see why: the wool is pulled over the eyes of the parents and they’re encouraged to basically dispose of these kids that are going to be burdens to the system. That’s sort of the overt side. There was a covert side that’s more sinister, I think, than that. Because at least this was an open debate and people taking what I think are absurd positions, but at least there was a discussion about it and a resolution. What was happening behind the scenes was worse.

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6 thoughts on “Living with Trisomy 13, Part 1: Stereotype Propagation and the Illusion of Choice

  1. If you go to the Living with Trisomy 13 website linked in the post, you can also find Barbara Farlow‘s reflections on her daughter Annie’s death right here. We hope to have some information the continuing investigation of that death at a later date, including something on the recent Ontario Human Rights hearing on this case.

    See also Dick Sobsey’s previous post on this at What Sorts, written in June just after the Canadian Pediatric Society’s journal, Pediatrics and Child Health, published Farlow’s commentary, over here.

  2. Hello!

    I have a 22-year-old-daughter with a Translocation Trisomy which consisits of part of the long (q) arm of her 13th chromosome and part of the long (q) arm of her 15th chromosome. Rebecca is a source of great joy in our lives and is a joyful person all around. Rebecca was born in a very large city hospital in the USA with several anomalies consistent with a syndrome – mishapen skull, “funny-sounding” heart, an extra toe, large “strawberry” hemangiomas, low-set ears, clenched fists, etc. She was not; however, diagnosed at birth with a syndrome, and I had refused the AFP test during pregnancy. As a result, all of her anomalies were addressed with needed tests, treatments, therapies, and/or surgeries. The LORD addressed the heart problem with healing!

    Currently, Rebecca is ambulatory and verbal, reads on about a 5th-grade (or better) level, writes very well, calculates, tells time, and is comfortable microwaving foods, making sandwiches, cereal, and other simple meal preparation and clean-up. She loves church and very much enjoys attending a twice-weekly social group for higher-functioning young adults with special needs, as well as bowling and playing baseball on special needs’ teams.

    Her story is posted at http://www.livingwithtrisomy13.org along with the stories of many beautiful children and adults with various forms of Trisomy 13, including many with Full Trisomy 13. The oldest person on the site with Full Trisomy 13 will soon be 27-years-old , and the oldest person with Partial Trisomy 13 is 49-years-old. There are also individuals with Mosaic Trisomy 13. The site has a Treasured Memories Album for children and adults who had Trisomy 13 and have passed away.

    All of these children are beloved by their families. They recognize family members and most have attained further milestones with the love and commitment of family and, often, therapies of various kinds. I always tell people Rebecca’s latest choice of her favorite praise and worship song, because the choices never cease to amaze me and are a reflection of her heart. The most currently shared favorite goes like this: “LORD, I offer my life to You. Everything I’ve been through; use it for Your glory. LORD, I offer my days to You, lifting my praise to You as a pleasing sacrifice. LORD, I offer You my life.”

  3. Pingback: Living with Trisomy 13, Part 2: The Monitor « What Sorts of People

  4. Pingback: The Modern Pursuit of Human Perfection: The Full Story « What Sorts of People

  5. Pingback: Chromosomal microarray analysis, newgenics, and Annie Farlow « What Sorts of People

  6. Pingback: Modern Pursuit of Human Perfection talks: now captioned « What Sorts of People

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