Mama and Dad

“They told me I have a choice,” says Peggy Chan. “That I don’t need to keep her. The doctor said they had families who choose to give up their baby because they won’t have any quality of life. I was very mad and I said: ‘Are you crazy? As a mom, you’re not even giving me a chance to try to raise her?’ If I had given her up I would have regretted that decision for the rest of my life.”

Emily lived for six years at Holland Bloorview before her medical condition improved and she was able to move home with her parents. Emily says she likes “all the typical teenager stuff” and wants to go to university to become a child psychologist.

BLOOM: How do you define quality of life?

Emily Chan: It’s living each day to the fullest, being happy. I think everyone deserves to have that chance. The purpose of life is to be happy, to be happy with yourself and what you’ve done and hopefully make a difference somewhere.

BLOOM: What is your life like now?

Emily Chan: I have a great life. I have everything—family, friends, cute guys to look at. Everything is going great in my life. I have pretty good marks at school—an 82 per cent average. I like Facebook and I’m really into (Korean)-pop. I play the guitar and piano and really love doing that. Whenever there’s stress in my life I pick up my guitar and play my worries away. It’s a great stress reliever. I like talking, hanging around, going shopping, going to see movies—all the typical teenager stuff. I love Harry Potter.

BLOOM: What about reading. Do you like those teen romances?

Emily Chan: No, that’s so cliché. I like the deeper, darker stuff. I’m just finishing The Hunger Games.

BLOOM: What are your dreams for the future?

Emily Chan: I want to become a child psychologist. I also want to have a family and drive a Ferrari—don’t we all? But right now I just want to get to university. Living in a hospital for the first six years of my life has given me a broader perspective of things. I got to interact with adults more than the average kid, which made me mature faster. It’s like my brain is 20 when I’m 16. I’ve known a lot of people who had to go through really difficult situations and I’m less quick to judge. I know that even though a person may appear a certain way it’s because of something that’s happened to them in the past. You have to see the person, not just the person they appear to be or how they act. I understand the feeling of being isolated, which will help me understand someone who feels alone for different reasons.

BLOOM: How do you view disability?

Emily Chan: It’s just a part of you. God made you this way for a reason and you have to learn to love yourself. You have to realize that a disability isn’t going to hold you back. My mom always told me that it doesn’t matter how you do something as long as you get it done. If you have a wheelchair it just becomes another part of you. And sometimes you can use it to your advantage—like running over people you hate!

BLOOM: Some kids resist what makes them different.

Emily Chan: You need to embrace it, because you can’t change it. If you want to live a good life you have to be happy and being happy means loving yourself. You do it for the sake of you and your happiness. Everyone has flaws, no one’s perfect. For me it’s my equipment. But I learn to look past it.

BLOOM: How do you see your equipment?

Emily Chan: It’s a part of me, so I learn to love it. If someone rejects my equipment they’re rejecting me because it’s grown on me. If they ask ‘Why do you use that?’ they’re kind of insulting me. I help my mom when she changes my trache and if I need to suction, I’ll do it myself. It makes me feel like I have more control in my life.

BLOOM: What is it like for you to be in a regular high school?

Emily Chan: I feel I’m really lucky because I go to an arts school and there’s a lot of diversity there. They have so many different kinds of people that the students are more accepting. If I went to any other school I think I would be more outstanding, more prominent. Here I’m just part of the diversity.

BLOOM: Did you ever have trouble with other kids at school?

Emily Chan: When I went to my home school for Grade 2, I was the only one in a wheelchair and it was hard. They were always teasing me. For group projects no one wanted to partner with me. These kids had been together since kindergarten and were close knit.

BLOOM: How do you think they viewed your disability?

Emily Chan: Like it was going to hold me back: ‘She’s not normal, she doesn’t fit in with the rest of us, she’s an outcast, she can’t do anything.’

It doesn’t happen much now, but there was a group of girls taunting me at school. When I went past them one said ‘Oh, you almost ran over my foot’ when I was no where near them. I said ‘No, but would you like me to?’ I like to use humour to turn the joke back on people.

BLOOM: Is there advice you’d give kids who struggle to make friends?

Emily Chan: I tried to approach kids and be friends with them but it didn’t work out that great. I did become close to one girl and she stuck by my side. In Grade 7 and 8, I found myself developing a skill of not letting people take advantage of me. I can stand up to people and fight back, instead of being tread upon. Something that helped was there was a class where students with physical disabilities could go at recess or lunch and I became very close to those people. They were friends I could talk to and fall back on, and I think that kept me going. I learned not to let things get to me. To look past a hurtful word and feel sorry for that person because they have to bully someone just to boost their self-esteem.

BLOOM: What advice would you give parents of children with disabilities?

Emily Chan: Don’t give up on your kid—no matter how grim the situation might seem. Always stay positive. You have to put in the time and the effort. Kids need their parents to give them love and support. Nurses and doctors will have sympathy, but it’s not the same as a mother’s hug that gives you that warm feeling. Every kid needs that. What got me out of Holland Bloorview was the constant pushing and love and support of my parents. They got me the treatment and the help I needed to thrive. My mom had a drive to bring out my potential and I think every parent should have that. In my opinion, many parents don’t have that devotion anymore.

BLOOM: Is the condition you have usually progressive?

A Pediatrics study of 322 parents who chose not to terminate children with trisomy 13 and 18 finds that while most were initially told their child was 'incompatible with life' and would suffer, 97 per cent later described their child as happy, despite severe disabilities, and enriching their families, even when their lives were short.

A good summary appears in this piece in Science Daily:

Children with trisomy 13 or 18, who are for the most part severely disabled and have a very short life expectancy, and their families lead a life that is happy and rewarding overall, contrary to the usually gloomy predictions made by the medical community at the time of diagnosis, according to a study of parents who are members of support groups published July 23 in Pediatrics.

And here on Medline Plus.

The study was co-authored by two neonatologists and parent Barb Farlow (in photo with daughter Annie, who had trisomy 13, above).

A blog by Montreal neonatologist Dr. Keith Barrington comments on the study and offers these suggestions to health professionals working with families:

1. Don’t say that this is ‘incompatible with life’ or ‘lethal;' anyone can go on the internet and find very quickly that you lied to them.

2. Don’t say that if they survive ‘they will live a life of suffering.' Parents do think that their child had more pain than others, but they also had many positive times, and their overall evaluation was positive.

3. Human beings are not vegetables. These children are conscious and interact, even if at very limited levels. Carrots don’t.

4. Don’t predict marital disharmony, or family breakdown. You can’t see the future, there is no evidence at all that this occurs more when a family has a baby with severe impairments, and indeed in this admittedly biased sample the divorce rate was far lower than the US or Canadian average.

4. Families find meaning in the lives of their children. Whether those lives are unimpaired or lived with severe impairments. Whether they are very short or not.

5. Don’t suggest that the child is replaceable. Sometimes parents will bring up the idea that they can have another child, that is fine if they do so, but for you to suggest it really shows that you think this child is worthless.

6. Don’t say that there is nothing you can do for them. There is a lot you can do. Empathy and a positive attitude can be a great help. Finding resources, respite care, enabling appropriate medical care, these are all things that you can do for them.

7. Be very explicit about medical decision making, come to an agreement about the limits of medical interventions (if you can’t, then find them another doctor who can); and be open to changing the plan as time goes on.

8. Refer to the child by name if there is a name. (Annie has often recounted to me that when she sees parents antenatally with a serious diagnosis she will ask them what the baby’s name is. They often become teary and tell her it is the first time anyone has recognized their fetus as being a real potential human being...)

9. Above all recognize that these babies are human beings who will be loved, who will be cared for, who will leave a positive mark on their families, and who deserve respect.

I contacted Dr. Larry Fenton, a pediatric palliative care doctor in South Dakota who had an invited comment published in the American Journal of Medical Genetics this month. He said he would "be honoured" to see it reprinted here.

We have talked before about quality-of-life measures that are based on how "most people" would rate life with a severe disability with life without: Is life with disability half as good as life without?

"How easy it is to assume we know what a good quality of life is for anyone other than ourselves," Dr. Fenton writes. See below. Louise

Trisomy 13 and 18 and quality of life: Treading ‘‘softly’’

Lawrence J. Fenton
Pediatric Palliative Care Sanford Children’s Hospital, Sanford School of Medicine, University of South Dakota, Sioux Falls, South Dakota
July 2011

Not too long ago I was privileged to speak at an annual international conference of the Support Organization For Trisomy 18, 13 and Related Disorders (SOFT). I learned much more than I taught. There were over 200 families there. Approximately half of the families were bereaved parents of a child with Trisomy 13 or 18. But then there was the other half, the half with surviving children with a trisomy. The oldest child I met was a young lady with trisomy 18 who was celebrating her 30th birthday. She was beautiful. Well dressed and impeccably groomed. She was non-ambulatory, nonverbal, and had a gastrostomy. But she surely knew her parents, she smiled at them and touched them with affection. There were many others with a wide distribution of ages. I met 16-year-old Karah and her siblings 11-year-old Olivia and 9-year-old Spencer. Karah’s smile as she hugs her siblings is contagious. Olivia had written a wonderful tribute to Karah in which she wished that others could see Karah’s heart in the same way she did. She loved her deeply and wanted others to do the same. I met a family with an adorable 3-year-old with trisomy 13. She was walking and squealing and smiling and relating to her parents. Must be a mosaic I thought but then her physician mom told me that chromosomes were done at Boston Children’s and she was a full trisomy. I saw a room full of families with children with trisomies and yes, they had profound disabilities. But the room was filled with smiles and laughter and all of the sounds of people enjoying one another. A room full of longterm survivors. After nearly 40 years as a neonatologist and now several years as a pediatric palliative care physician, I considered long-term trisomy survivors a statistical rarity. Forget the statistics and the rarity. This was a room full of families having fun with their children. Each one was different, each with a distinct personality, each with the dignity of full personhood. I do not want to minimize the struggles these families go through. One mom said that most were just inches away from losing it completely on many days and that sometimes it was just hard to get out of bed to face changing a diaper on her 20-year-old. But, these parents would not have it any other way.

I learned very quickly that the three words these families never wanted to hear from physicians and others who provide care were ‘‘Quality of Life.’’ I was somewhat taken aback because as a palliative care physician I frequently use these words to describe what we do in terms of helping families cope, helping children deal with pain and other symptoms so that life may be as good as it can be up to and including the time of dying. Good palliative care is much more about living than dying. Apparently for these families, the phrase ‘‘quality of life’’ had been used by the medical establishment in judgmental ways perhaps in times of giving advice with regard to treatment or just general commentary. A remark soon after birth that sounds like, ‘‘I’m afraid your child won’t have a very good quality of life’’ may seem innocent but it is not. How easy it is to assume we know what a good quality of life is for anyone other than ourselves. We assess the burdens of care, the impact on siblings, the impact on parental relationships, the impact on finances and the utilization of resources. We perform ethical analyses assuming we truly understand how to apply beneficence or maleficence to a child with trisomy 18 or 13 or any other disorder in which there may be profound disability. As if the child can tell us what he or she is feeling. But we can do our best to assess and treat pain and discomfort. Smiles and laughter need no score pad. We know what they mean. The advice we give may often be centered around our personal notions about quality of life. As a profession we are far from all being on the same page with such advice as was recently well described by Janvier et al. [2011] in an ethical case discussion about a child with trisomy 18. They point out the absence of data on outcome of procedures done on children with trisomy 18.

I write this not to advocate for any particular point of view except to advocate for a willingness to do whatever it takes, however long it takes, however many consults and team members it takes to fully inform and understand the goals, values and aspirations parents may have for their children. Once we have done that we need to honor the parents by helping them achieve those goals whether it is comfort care alone, a full court press or something in between. Regardless of the choices of the family, they deserve our respect, our affirmation, our support, and part of our own humanity as we try to do our part to make their lives and the lives of their children as good as can be. Is not the privilege of doing that exactly why we are here?

REFERENCE
Janvier A, Okah F, Farlow B, Lantos JD. 2011. An Infant with Trisomy 18 and a Ventricular Septal Defect. Pediatrics 127:1–6.

I'm back from holiday and delighted to share with you a guest blog from Jennifer Graf Groneberg (photo above, with her twins Avery and Bennett), author of Road Map to Holland: How I Found My Way Through My Son’s First Two Years with Down Syndrome, a memoir that provides practical insight and emotional support to parents of children with special needs.


Thank you Jennifer for this beautiful reminder that we are families first and foremost, and sometimes the focus on disability, difference and rehab has to take a back seat.


I'd like to know how other parents close their own metaphorical "gates" when they need a breather from the intensity of special-needs parenting. Louise


My own private gate
By Jennifer Graf Groneberg


I live at the end of a twisty gravel road in the mountains of Montana in a simple house, just two bedrooms, originally built by a man and his wife as a retirement home. They also built a gate across the driveway. It’s a thick wire strung between two steel posts sunk deep in the ground. When you pull it tight and hook it into place it feels final: gate closed.


We’d lived in this house for almost five years before I felt compelled to use the gate. It was the fall that the twins came home from the Neonatal Intensive Care Unit; the first fall we’d been living with the news that our middle son Avery had Down syndrome. I was tired, then, so tired. I didn’t want to explain anything anymore to anyone, I didn’t want to put on a brave face, or not put on a brave face. I wanted time to get to know my new family, time away from the well-wishers, time out. I put up the gate.


Just the act of stretching the wire across the driveway made me feel powerful. I had some say here. I could close the doors, if I needed to. We could have a break, now and then. I could say no. It was incredibly freeing, and I haven’t forgotten that feeling. The gate, and the lesson of the gate, have stayed with me.


When I am feeling low, or overwhelmed, or simply out of answers, I put up the gate. I play with my children, I bake cookies, I listen to music. We read books. We dance. We wear our pajamas all day. I sometimes cancel our therapy appointments. I say the kids are sick, or that our car has a sudden, unexplainable flat. I feel a bit guilty about these lies, but what I get back is worth it to me—space. A place to reconnect with my family. A place to stop being the mother of a child with Down syndrome, and simply be Mom. It’s so easy, with the gate up. Our family behaves as a family. Everything is natural. I sometimes forget that there is any trouble here at all.


But of course life moves on, as it must. The gate comes down. We go out into the world. We resume our activities. I reschedule all the appointments. I carry on as the mother of a child with Down syndrome. It’s okay. Most days, I am fine. Most days, I can bring that feeling of normalcy out into the world, and try to swing things a bit more toward acceptance. It’s what I do; it’s what we all do.


But when I am not feeling up to it, I know what comes next. I pull the wire tight and start canceling things, until it feels like time to get back into the fray. My own private gate, temporarily up while I’m under repair.

Every time I see an article written by bioethicist Peter Singer, my chest gets a knot in it.

Last weekend, his piece Why we must ration health care appeared in the New York Times Magazine, along with this description: A utilitarian philosopher's argument for placing a dollar value on human life.

The article is about why health care, as a finite resource, needs to be rationed in the U.S. It's about setting limits on what we're prepared to spend to save a human life or to reduce pain, so we get value for our money. Okay, that I get.

In order to compare treatments, we need a common unit to measure their benefits and Singer uses the “quality-adjusted life year (QALY).”

The QALY is a composite of the number of years saved by a procedure or drug (for example, saving the life of a teenager is equivalent to saving the lives of 14 85-year-olds, he says, because a teenager could live for 70 more years vs. the 85-year-old living for five) and the quality of those extra years. The idea, according to Pinning down the money value of a person's life, is that “a year in perfect health is worth more – both to the patient and to society – than a year spent in pain, depression or a wheelchair.”

Singer doesn’t clearly define what he means by quality of life, but implies it is synonymous with "an active life," and is diminished by medical conditions that limit function.

In order to compare how we might allocate resources, Singer suggests we consider how “most people” would rate life with a severe disability like quadriplegia, with life without. Would the average person have a hard time deciding whether they’d prefer five years of able-bodied life or 10 years with paralysis? If so, he says, what they're really saying is that life with quadriplegia is half as good as nondisabled life.

In this scenario, the QALY would rate a procedure that saves the lives of nondisabled people at twice the value of one that extends, for the same time, the lives of quadriplegics.

Looked at in a different way, the value of saving one able-bodied life would be equal to restoring function in two people with quadriplegia.

I have problems with this on two fronts.

First, the premise that reduced physical ability always translates into a less satisfying or happy life flies in the face of facts. A number of studies show that people with quadriplegia rate their quality of life as average or above average, and that they value their lives highly.

This is consistent with research in the world of child disability. For example, a 2007 study of 500 European children with cerebral palsy published in The Lancet found that eight-to-12-year-olds rated their quality of life no differently than children without disabilities. A similar 2007 study of 200 Canadian teenagers with cerebral palsy found participants' self-reported quality of life was not linked to their abilities or degree of disability.

“Is it surprising that subjective quality of life of children with cerebral palsy is similar to that of the general population?” The Lancet researchers asked. “Possibly, from the perspective of a non-disabled adult imagining what it would be like to be disabled, but probably not from the perspective of a child whose sense of self from birth incorporates their impairment and who embraces growth, development and living with the same excitement as most children.”

This leads to my second problem. Instead of relying on science to measure real quality of life, the QALY comes up with a score based on surveys of how "most people" predict they'd experience quadriplegia – without having lived it.

We know from recent "happiness" research that people are notoriously bad at predicting what will make them happy. In Stumbling on Happiness, Harvard psychologist Daniel Gilbert describes common blind spots in how we imagine the future. These include a lack of empathy that would allow us to imagine an experience different than our own and a tendency to overestimate the negative impact of an event and underestimate our resilience. This helps explain a study that showed sighted people will pay more to avoid going blind than blind people will pay to regain their sight. It also explains why most people assume they couldn’t be paralyzed and happy, even though surveys of people with quadriplegia show the opposite.

If someone had told me before my son was born that he would never speak, I would have assumed this was incompatible with a good life. Speaking is one of my great loves. “My son can’t handle it,” I would have thought, and, perhaps, more tellingly, “I can’t handle it.”

Imagine my surprise the first time I sat in a room with people who communicated effortlessly and richly with only their hands. There was great beauty in that silence, beauty I never would have experienced if I'd remained trapped in my narrow mindset. And because – before he learned sign language – I was forced to look deep into my son’s eyes to read what he was thinking, I discovered how much I missed in my old life, when I had to stuff every silence with words, because I was too afraid of what I'd find if I didn’t. Disability can stretch us.

Gilbert, in Stumbling on Happiness, shows that people are remarkably good at making the best of bad situations – by changing their views of the world in order to feel better about the world they find themselves in.

But for some reason we lack this insight into ourselves, he says, and don’t factor it into our predictions: we don’t believe we can adapt to something life-changing.

Given that many people with disabilities rate their quality of life highly, and given what science tells us about the mistakes we make when trying to imagine what will make us happy, why would we rely on the uninformed judgments of the average person to assess the value of a disabled person’s life?

And even if a happy life was determined solely by physical ability, does that mean the value or worth of a person’s life is diminished?

Should twice as many medical dollars be expended to save the life of an elite athlete over that of a couch potato?

What about a child with above-average physical function but a developmental disability?

Does reduced cognitive function lessen the value of a person’s life in Singer’s model?

If we asked “most people” whether living with a low IQ was as “good” as life with average intelligence, what would they say?

I bet “most people” haven’t had a close relationship with someone with a developmental disability – so they wouldn’t have direct experience to go on. Instead, they’d fall back on popular stereotypes. Singer himself equates human dignity with intellectual capacity in A convenient truth, an earlier article in the Times about the Ashley treatment. “... I find 3-month-old babies adorable, but not dignified. Nor do I believe that getting bigger and older, while remaining at the same mental level, would do anything to change that.”

Does this mean we should put fewer dollars into life-saving treatment for people with developmental disabilities?

People with disabilities face so many misconceptions about their experience. The QALY measure only perpetuates them by relying on a simplistic measure of “perfect” health. Being paralyzed or using a wheelchair doesn’t preclude being healthy or living a full life.

Physical function may contribute to a good life, but it’s only one of a myriad of factors. What about social conditions that can marginalize people? Perhaps Singer would like to survey people on whether they'd prefer to live five years white or 10 years black in the U.S.; five years rich or 10 years poor; five years as a man or 10 years as a woman.

Can a person's value be determined by race, income or gender, anymore than it can be determined by physical ability?

The QALY keeps things clean and simple and easy to calculate for economists by reducing quality of life to physical function – at the expense of people with disabilities.

I think Singer’s recipe for health-care rationing has sinister implications.