Health: Does it come in all shapes and sizes?

Last month I sat in on a bioethics session where health providers discussed 2012 media stories with a health ethics angle.

One was about using costly growth hormone in kids who are short (but who are healthy, not growth-hormone deficient and otherwise typical).

In this National Post story, two parents in a custody battle couldn’t agree on whether their healthy son should receive daily growth-hormone shots at a cost of $30,000 a year over many years.

In this case the healthy boy’s predicted adult height without the injections was 5’3.” Growth-hormone treatment might give him an additional 1.5 inches.

One health professional at the bioethics talk questioned whether it was fair to deny a child the opportunity to do something in adulthood that depends on size—like being a firefighter—by restricting growth hormone treatment. What if a healthy but exceptionally short child has dreams of fighting fires?

Because I was once a parent demanding growth hormone for my son Ben—whose short stature stems from a genetic condition—I understand how extreme shortness can be viewed as a physical disability in a world designed for big people.

However, having given my son shots of growth hormone six nights a week during a six-month trial many years ago, I question the treatment of what I now believe is more of a social, than physical, disadvantage.

Can't we promote a vision of health that is more inclusive of different body types?

Can a gain of one or two inches in final height justify daily shots over a decade that cost as much as a mortgage?

There are all kinds of studies showing that tall people make more money and have other advantages in life. This 2004 Journal of Applied Psychology study found that a person can earn an extra $789 per year for every inch above average height they are.

“There's plenty of evidence to suggest that height—particularly in men—does trigger a certain set of very positive, unconscious associations,” notes Malcolm Gladwell in Blink, the bestseller about how we make snap judgments.

Just before Christmas I was driving home and heard a CBC radio interview with bestselling author and New Yorker staff writer Adam Gopnik. I haven’t read any of Gopnik’s eight books but I have read some of his brilliant writing in The New Yorker.

When asked “If you could change one thing about yourself what would it be?” this is how he answered:

“I wish I just had six inches vertically because I am a very short man and unfortunately and truthfully being short is a deeply limiting condition in every sense. I think that if I had been somewhat taller my life would have taken a completely different line. And though people don’t admit it, the sense that the short are fundamentally jumpy, nervous and needy is built into that and the tall man is always the man who is the calming leader.”

For someone who’s achieved remarkable success in his career, Gopnik’s wish to be taller struck me as unimaginably sad. I think he's right about the prejudice against short people. But to go through life in a healthy body feeling that your height prevented you from a certain greatness makes me shudder. What kind of world are we living in?

Instead of being gatekeepers to drugs that are driven by the social stigma of being short, how can we work for a world in which a child’s opportunities don’t depend on height and other attributes of appearance? Wouldn't that be a kind of disease prevention—with huge savings in medical and psychological costs? What can be done to change public attitudes so that we expand our notions of health? Can't wellbeing come in all shapes and sizes?

My son’s trial on growth hormone didn’t work and we took him off it.

But sometimes I imagine how I would have felt if we’d given him shots six nights a week for 10 years, and then had to face up to the fact that he was one or two inches taller than what he'd have been without it. I know I would have been wracked with guilt and the sense that I'd made the wrong choice. At the time I was gung-ho to reduce the limitations a genetic syndrome placed on my son. But it's painful to remember the times my younger daughter would alert my son that I was getting the needle ready and he would run to hide in a different part of the house.

To think that this treatment is gaining use with families of children who are otherwise healthy is troubling.

I think we have a critical role to play in challenging stigma so that kids and adults can live in the world without interventions to make their bodies fit social norms.

Read More

Answering your question on withdrawal of fluids in newborn

On Friday a parent posted this question on the BLOOM blog:

"Is it routine to withdraw fluids and nutrition from infants in the NICU? That is what we were offered for our son in hospital after we removed the ventilator and put a DNR on his chart and waited for him to die. He didn't die. He didn't need a g-tube, he was able to breastfeed and get enough nutrition for himself yet it was offered to us to just stop feeding him orally, give him morphine for the pain and let him go. Is this sort of thing common?"

I contacted Dr. Jonathan Hellmann, who is clinical director of the neonatology unit at the Hospital for Sick Children in Toronto for clarity on how to answer this question.

Dr. Hellmann authored a paper earlier this year about parent perspectives on withdrawing intravenous fluids in newborns with severe neurological injury or disease where the decision to stop life-sustaining medical treatment such as ventilation is made (Withdrawal of artificial nutrition and hydration in the Neonatal Intensive Care Unit: parental perspectives). None of the newborns in the study could safely be fed orally.

According to the study, in addition to requiring the full support of the parents and the senior medical staff and team, the practice of withdrawing artificial nutrition and hydration requires "the clear demonstration of the inability of an infant to safely tolerate oral feeds (oral feeding was regarded as routine care even in those in whom it took many hours to achieve a satisfactory intake.)"

Given this criteria, I asked Dr. Hellmann whether withholding milk from a baby who is able to breastfeed -- as reported above by the parent on BLOOM -- fell outside the practice of withdrawal of artificial fluids and nutrition.

Dr. Jonathan Hellmann: Your interpretation is correct. Only if the feeding is regarded as medical treatment i.e. requiring tubes, IVs, lines etc. is it ever our practice to possibly raise the issue with parents of a profoundly affected infant. If the child is able to tolerate feeding it is absolutely not acceptable to consider withdrawal of hydration and nutrition. I am extremely concerned that individuals might interpret our findings without every effort being made to feed even small sips, and only when it is unsafe to potentially raise the issue. It is certainly not common practice here and requires very careful consideration in every case.

I hope the parent who posed the question above sees this blog and lets us know if she has more questions.

Read More

Busted

What do you do if you and your partner disagree on how to handle an issue with your child?

Let's say it's an issue that rankles, so one day you bring it up at a meeting with a social worker you're seeing for your child. And the therapist pretty much sides with your partner and even suggests that it would be helpful for you to get therapy (which you've already had lots of). She asks you if you were to let go of the vigilant worrying you do for your son, what would be left? What would be underneath? And she suggests that maybe it would be grief or maybe it would be something else.

If you're me, you sit on it for a while and do nothing.

Then you ask a friend who's seen the same social worker for her child what she thinks. "You're talking to the wrong person if you think I'll talk you out of therapy," she says. "All I do is therapy!"

Then, because you're so stubborn, you convince your partner that a second therapist, a cognitive-behaviour therapist, should weigh in on the matter. You're pretty sure this therapist's opinion will be more in line with your own.

So off you go. The second therapist suggests that this is more to do with you, your relationship and parenting styles. She isn't willing to criticize your partner's approach. She feels the issue is something you together should keep an eye on, but at this point she isn't red-flagging it and she isn't supporting your version of how to handle it (that hubby follow your precise recommendations).

Which brings you all back to the point that maybe there was something to what therapist number one had to say about you. That maybe the 'problem' isn't outside you, but within you, something that you have to take a closer look at.

Meanwhile, hubby is sitting on the couch, fighting heavy eyelids to stay awake and focused on therapist number two. "She never got to the point," he says afterwards. "I didn't really understand why we were there."

Busted, you think.

I guess you can never have too much therapy.

Read More

Flippity flop

This is a piece I wrote four years ago about Ben's unusual ears and the plastic surgery to correct them that wasn't successful. I was reminded of it because last week I attended a Grand Rounds by Jennifer Johannesen, author of No Ordinary Boy, in which she spoke about how challenging it is for parents of children with disabilities to make good medical decisions. That statistics, the assurances of specialists -- which in our case often came in the form of "If this was my child, I would pursue it" -- can't take the place of careful inner enquiry on the part of parents to understand 'why' they're considering the intervention. Who is the surgery being done for? What might the child choose? If gains will just be incremental, does it justify the pain of the procedure and rehab? We're often not prodded to look at the bigger picture in terms of how this surgery fits with everything else going on in our child's and family's life. And how may we feel if the outcome isn't what we hoped?

Flippity flop
By Louise Kinross

"The best and most beautiful things in the world cannot be seen or even touched. They must be felt with the heart." Helen Keller

It stands out from his head. It is cupped, missing the typical indentations and folds, with a bit of extra skin at the top. It has been called a deformity, a lop ear – named after the distinctly floppy ears of the lop rabbit. It is my son Ben's right ear, and it was one of a handful of facial anomalies that caught the attention of the midwife who delivered him.

That impossible-to-ignore ear, which vigorously resisted surgical attempts to tuck it back at the head, represents my own struggle as a mother of a child who looks different.

My child stands out. My child doesn't fit in. My child, by some, is considered deformed and ugly.

Ears. I had never given them a passing glance. Who did?

But this particular ear has led me to think long and hard about beauty. What is it? Is it an objective standard? Is it a certain facial symmetry, as suggested by evolutionary theory, which helps us identify people with good genes and exclude others? Can it be altered by the perceptions of the one viewing, or by the personality or spirit of the one being viewed? Is it subjective and fluid, based on characteristics that bring delight to a particular individual? How is it possible for us to see beauty in people deemed ugly by others? Why can our perception of beauty in a person change over time? How do people who are blind detect beauty in others?

Fourteen years ago, as I bubbled with glee at the birth of my first child, I had never had to consider these things. I saw a blurry little body with a mohawk-like tuft of wet hair being whisked away to be suctioned and waited for them to bring him to me.

But before I could hold him for the first time, the midwife returned and put her hand on my arm. "He has some unusual features," she said.

When he was finally passed to me, wrapped up in a blanket with a little stocking on his head, he did look different: his face was swollen, his nose a bit squashed, his chin was retracted and his ears stood out, one smaller than the other and fully cupped. His lips were almost non-existent.

I was concerned, but I wasn't panicked. My body was tingling with joy, endorphins coursing through it like the wind shaking the leaves of a poplar tree.

The hospital pediatrician arrived, unswaddled Ben and looked at him disapprovingly. "He has anti-mongoloid eyes, low-set ears and a bit of a hare lip," he said. He was obviously annoyed at being called out at that time of night and he didn't like what he saw. He said our son had "something like a cleft palate" and muttered under his breath: "the timing wasn't right."

An earlier amniocentesis on Ben had come back normal, so it was a surprise when the doctor said Ben had a constellation of symptoms that looked like a chromosome problem. At exactly one-hour-old, the doctor said these were soft signs for mental retardation, though he didn't know what he was dealing with.

We returned home with Ben in a haze of shock and a black hole of fear.

What did Ben's appearance mean? Would he be mentally retarded as the doctor intimated? How would others react to him? What would we tell them? How do I control what others think (in the early days I naively thought I could)?

"Couldn't we still be a happy family?" I asked my husband on day three.

On day four we saw a geneticist. She said Ben had a greater than 50 per cent chance of having a rare genetic condition associated with "abnormal facies."

But I had already started to see him differently. Outside of the clinical setting and scrutiny, my boy was growing beautiful before my very eyes. Here's how I described it:

2 weeks and a day
You have very delicate features – beautiful blue eyes with extravagantly long lashes, well-defined and delicate eyebrows, a round face with plump cheeks and tiny little lips. You have very light, fair skin.

3 months
You are becoming more beautiful every day. Your eyes are drop-dead gorgeous – big big, blue, long, long lashes.

5 months
Your face has changed a lot. Your eyebrows are growing in just like your Dad's – very thick. You have the most gorgeous smile – an all-out, ear-to-ear grin with dimples. You are very masculine-looking. Your body is a miniature of your Dad's.

6 months
It seems like a miracle that you are here. I like to sit and wonder at you – where you came from, how you came to us, how you are so perfectly formed.

How was it possible that I could view Ben as "perfectly formed" when medical experts described his face as "a complex picture with multiple congenital anomalies?"

I have come to the conclusion that beauty is something we choose to see.

When I was immersed in the everyday tasks of loving and caring for Ben, he was so much more than his unusual features: he was a cuddly ball of heart, giggles, interests and charms, whose chest rose and fell at precisely twice the rate of mine.

When I gazed into Ben's eyes it was like looking into a complete world. Ben mirrored back my joy and acceptance. In each others eyes, we were whole. In each other's eyes, we were enough.

And so it was that I could be emotionally broadsided when, instead of delighting in my sweet prince of a boy, others placed his so-called defects front and centre.

When Ben was almost a year he was diagnosed with torticollis; the muscles had developed too strongly on one side of his neck, causing him to sit with his head in a perpetual tilt. As we introduced ourselves to the therapist assessing him, I explained that Ben had a rare genetic condition. "I can SEE that," she chortled.

Ten days later, as Ben lay on an examining table charming a handful of medical students, the surgeon who was to bring down his testicles pranced into the room and demanded angrily: "What is WRONG with his head?"

Medical records show that emergency staff often noted Ben's facial appearance before his pressing medical problems. When he arrived by ambulance with bronchiolitis he was the "settled baby with unusual facies." A trip back to emergency with breathing problems two weeks later yielded this description: "Very unusual-looking baby who is active and happy."

Specialists who were consulted on other parts of Ben's body couldn’t seem to resist documenting that he had "dysmorphic" – malformed, misshapen or underdeveloped – features. I remember the first time I raced to the dictionary to look the word up, heart thumping at this new and horrible name that had been ascribed to my son. My sister-in-law, a physician, explained that doctors had a code name for kids like Ben: FLK or "funny-looking kid."

When we wrote my uncle about Ben's syndrome he wrote back: "We could see in the photo that something wasn't right."

The genetics counsellor assured me that I had every chance of having a "perfect" baby next time.

The implication that my son was imperfect, flawed, deformed could send me reeling with rage and grief for days.

After opening my uncle's letter, I phoned my friend Candace. I asked her a question I had not dared verbalize – except to my husband – for over a year: "Does Ben look different to you?" I didn't want an honest answer, but this time she gave it to me: "He doesn't look different to me because I'm used to him, but he probably looks different to other people."

I hung up the phone in a fit of sobs. I had floated on the illusion that Ben's differences were subtle and perhaps not noticeable to others. But more than that, I had simply stopped seeing them.

Our pediatrician had always been exuberantly positive about Ben – thank goodness. He even made comments about how Ben's facial features had normalized since he saw him at a few days old. Many friends, trying to be diplomatic and supportive, said they didn't notice anything. Of course I slurped those comments in like a draught of cold beer on a hot day and they reinforced my way of seeing Ben.

In my eyes his differences dissolved because I saw his face and being as a whole. But in others' eyes the odd features superseded the boy. In their eyes he was different, an oddity, something "wrong."

Now when I was out in the world, I would suddenly see this boy with the syndrome, the boy whose every feature had been scrutinized and found lacking. It was like I'd been fitted with a new pair of glasses: instead of bringing into focus his merry, dimpled-grin and piercingly beautiful eyes, all I could see were the stark clinical anomalies described in the medical texts: large bulbous nose; downslanted eyes, long philtrum, receeding chin, thin lips, sparse hair, outstanding ears. This new child was a stranger to me.

I began to fixate on the symmetry of normal faces – a certain space between the eyes, and between the nose and the mouth. Ears that were mirrors of each other, tucked tight against the head and identically placed under the short, little-boy hair cut that was so popular at the time.

Sometimes I would catch myself looking at other children's features and just longing for the same for Ben. "I hate these feelings," I wrote. "It isn't so much that I want this for Ben (that I can't accept him for how he is) but my fears that without a normal or conventional appearance others will not accept him and love him." Sometimes I felt bitter. Sometimes I would take photos of Ben's face and hold them upside down to examine the defects more objectively.

Strangers at the park attributed Ben's facial features and decreased facial movement to a negative mood. "Is he mad?" they would say, looking into the wagon at my passenger. "Is he always like that?"

"No, he's not mad!"

One day, while Ben played at the sandbox, a toddler came, stood in front of him and began to chant: "monkey man, monkey man."

"He wasn't being mean, just factual," I wrote. "I was not in one of those educational moods, and didn't say anything. The child seemed too young to understand an explanation and I didn't feel like saying: 'Yes, Ben's ears do look like a monkey's.' Doesn't this become comical or sad after a while?"

I didn't have experience with people who looked different growing up, yet as a child the question of how appearance could predict a life of happiness or sorrow puzzled me. "What would you do if you fell in love with Frankenstein?" I asked my best friend, Cathy. "I wouldn't fall in love with Frankenstein," Cathy said as she bounced back from a handstand on the street curb.

"But what if you DID?"

I can recall a moment of disturbing recognition when I looked at some photos I had taken of Ben as a newborn, but a couple of years later. I could now see the differences in a way I had not been able to at the time.

Something similar happened when I sat behind Ben at the hairdresser. The mirrors didn't correct his differences like my eyes did – they left them naked and punctuated.

While I would gladly have traded places with Ben for the 15 surgeries he endured (his ear surgery the only cosmetic one), I didn't know if I would have the courage to be so strikingly different in a world that places such value on appearance and conformity.

Over time, we learned that some of the features we had loved as Ben's – such as his extraordinarily long eyelashes and bushy eyebrows – were part of the syndrome.

I had read about parents of children with Down syndrome who had their slightly upturned eyes "corrected" with plastic surgery. This felt like a horribly intrusive and wrong intervention. Why put the child under the knife in order to fit arbitrary social norms? But I could understand why a parent would be led down that path. Down syndrome is so common that the facial features associated with it are an immediate marker of stigma.

One of Ben's first caregivers – a young Polish woman – was horrified when I said we'd considered getting Ben's ears pinned back when he was older. "His ears let people know there's something different," Margaret said.

"That's exactly what's so painful and unjust," I said. "No one wants their child judged in a split second based on appearance."

Ben's ears had always been most remarkable and since he was born we'd been told there was a simple operation called an otoplasty to pin them back.

We visited a plastic surgeon at a renowned children's hospital to find out more.

Dr. S was a young man with Hollywood looks followed by a small entourage of plastic-surgery students. He was kind and friendly and took his time looking intently at Ben's face.

Naively, I expected him to see what I saw. How could Dr. S see anything I hadn't already seen in my boy, who was now 3?

But he did.

"Ben could benefit from a bit of cartilage to create a tip at the end of his nose, which is somewhat flat," he said. "This can easily be done when he has the ear surgery." I had not seen the flatness of his nose – except in the minutes following his birth, when he had the typical squashed newborn look.

Pinning back his ears was one thing, but I couldn't fathom allowing a surgeon to cut into my son's face.

The volume of his cheeks is asymmetrical, Dr. S continued. I couldn't see the unequal shape of his cheeks so Dr. S tried to point it out to me. Was I insane? Why was I not able to see these things?

In addition, my boy's face wasn't as animated as usual due to low muscle tone – his smile not as broad as normal. I had always thought of his smile as one of his most beautiful attributes.

I couldn't reconcile the beauty I saw in Ben – and the delight he brought me – with his medical picture.

We left the appointment with a referral to Dr. W, the surgeon who did ear work.

I struggled with whether we should pursue the otoplasty. I hated that my son would undergo surgery in large part to make other people feel more comfortable. But by this time, Ben had been diagnosed with a whole collection of physical and learning disabilities related to his syndrome. When I asked Ben, who was unable to speak, if the kids ever teased him about his ears, he hit "yes" on his voice-output device. And when I asked if he thought it was a good idea to have the operation to fix his ears, he again hit "yes." Dr. S said they liked to do facial surgeries before the kids entered kindergarten – to "max out" their appearance and minimize social exclusion and teasing. In addition, there was now a strong functional impetus: Ben needed hearing aids and because his ears flopped forward, the behind-the-ear aids constantly waved back and forth, creating a disturbing ringing in his ears.

I was desperate to even the playing field for my boy and remove even one of the many strikes against him.

Other than telling us that Ben was a good candidate for the ear reconstruction, Dr. W didn't tell us what to expect. I don't know why I didn't ask. My understanding of plastic surgery was limited to episodes of Extreme Makeover. I assumed Ben's ears would be made symmetrical, tucked back at his head and equivalent in height. Like other people's ears, I assumed they would look "the same."

Ben emerged from the operation with his new ears bound in layers of white bandages. Dr. W said the procedure had gone well and we were elated. My only concern was the hot, itchy bandages, which Ben was supposed to wear for a couple of weeks.

Ben was highly sensitive to touch, so to deter him from ripping off the head dressing, I purchased some Teletubby hats that came down over his ears and snapped at the chin.

This lasted for about a week. One day I left Ben watching a video and when I came back a few minutes later bandages were flying through the air. My curiosity got the better of me and I allowed him to unwrap the last couple of rounds.

I was crushed. While his ears sat back closer to his head, they were still asymmetrical and very different in size and appearance.

I squinted and reopened my eyes a couple of times. Was I not seeing properly?

I expected two ears that looked the same – the birthright of most children. What had we done? I wondered. Why had we put him through this?

We went back to the hospital a month later and a nurse took out the stitches. A medical student came in, took one look at Ben and said: "I guess you're here about surgery to correct his ears?" I don't remember what Dr. W said. It was obvious that the student had given us an unsanitized assessment.

In the ensuing months, despite religiously covering them as we were directed, Ben's ears would gradually creep forward until they were again protruding significantly. We went back to see Dr. W. He had been featured in People magazine as the 'Smile doctor' because he was an expert in surgery that enabled kids with Moebius syndrome – a condition that caused weak or paralyzed facial muscles – to make their first smiles. We were surprised that he hadn’t been able to work a similar miracle with Ben's ears.

Dr. W said that he hadn't taken enough cartilage out, and that cartilage had "memory" – it always returned to its original position. He'd been worried about Ben's ability to wear hearing aids if he took more cartilage out. He wasn't sure how to make things better and referred us to Dr. F, a surgeon who specialized in constructing ears in children born without them.

Dr. F was surprisingly cautious. "He'll look different," he said, assessing the impact of additional surgery. "But he won't necessarily look better." How had we moved from a simple, quick-fix ear operation to this sobering statement?

As a parent, I felt duped. Why had the otoplasty repeatedly been explained to us as a simple procedure that would correct Ben's ears? Why was there a steady barrage of media stories recounting success with much more complicated and extensive plastic surgeries?

Dr. F said it was because Ben's ear structures weren't normal to begin with. If you have a normal body structure, he said, it's possible to enhance it – which often means making it bigger or smaller. But it was another kettle of fish when you didn't have the normal structure to work with. In other words, the people who most needed plastic surgery were the ones least likely to benefit from it.

"Ben doesn't seem to be particularly bothered about his ears," Dr. F said.

Was it because Ben had an intellectual disability that he didn't think it mattered? Or was it really that challenging technically? I'll always wonder. A quick Internet search for “lop-ear deformity" produced numerous plastic-surgery sites with impressive before and after pictures.

It grates on me that Ben's ear operation wasn't successful. We were trying to do something relatively minor to help Ben blend in just a little better. We knew he would always stick out with his short stature, developmental disability and inability to speak, but at least his unusual ears wouldn't draw attention away from his face, which in my eyes was beautiful.

Perhaps I should have consulted Ben's sister Lucy before deciding on the ear surgery.

"I just love Ben's big ear," she said spontaneously the other day, while sitting beside him at dinner.

Wow, I thought. Ben had always been a given for Lucy, who was born when he was a toddler. I had never heard her comment on his appearance.

I assumed she was talking about Ben's cupped ear – the one that is the most protruding, the most noticeable, and which most resisted the surgeon's handiwork – rebounding vigorously from attempts to refashion it flat against his head.

"No, I mean his taller ear," she said. His taller ear? How could his less-affected ear be called his "big ear?" Apparently Lucy, now 12, saw something completely different from me when she looked at Ben.

"Flippety flop, flippety flop," Lucy said lightly, acknowledging both the unusual nature of her brother's rabbit-like ears – and her complete acceptance of them.

Read More

I say 'gobbledygook!'

I know nothing about philosophy in the academic sense.

And, after reading this paper published in the Journal of Medical Ethics, I have no interest in knowing anything about it.

In After-birth abortion: Why should the baby live? a philosopher and an ethicist argue that killing newborns – before they have neurological self-awareness – is ethical for children with disabilities, because it's assumed they would be an unbearable burden to their families and society. They also argue that infanticide is ethical for a healthy baby with no disabilities, if that baby might compromise the psychological, social or economic wellbeing of parents, siblings and society.

In a nutshell, Alberto Giubilini and Francesca Minerva argue that if a newborn is disabled, not wanted by his or her mother or too expensive, it's ethical for doctors to kill the baby.

In their article, the academics attribute the right to life only to individuals who can appreciate their existence and have ‘aims’ or dreams for the future, so that depriving the person of that life would be experienced as a loss. If individuals can’t value their life and plan for the future, because their brains are in the newborn state, or because they have a disability, the philosophers categorize them as non-persons who would not be harmed if killed.

“Merely being human is not in itself a reason for ascribing someone a right to life,” is how they so eloquently put it.

The philosopher/ethicist duo say that killing newborns who have severe mental and/or physical disabilities is ethical if the parents would have aborted the child in advance – if only they had the knowledge.

An example given is Treacher Collins syndrome, a rare genetic condition that Giubilini and Minerva describe as causing ‘facial deformity’ and physiological ‘failures,’ including potentially life-threatening respiratory problems.

Hmmmmmm. I thought. I remembered the name Treacher Collins because when my son was a toddler we visited an ear-nose-and-throat specialist at a world-renowned children's hospital who mentioned the syndrome in passing, noting that some of the facial differences were similar to those seen in my son. In fact, when telling us about Treacher Collins, the ENT said that Sylvester Stallone had the condition, and that while people with Treacher Collins had facial differences that required reconstructive surgery, they were intact intellectually and led full lives.

That doesn't seem to line up with the journal article's description of 'physiological failures' and life-threatening respiratory problems.

I did a quick search online and came across this blog by a young woman with Treacher Collins who is a physician in internal medicine, pediatrics and sleep medicine. She is also married and from the looks of the blog, loves her life.

Guibilini and Minerva note that people with Treacher Collins have typical intelligence so they would be aware of how ‘different’ they are and of all of their problems (seeming to imply that this would make death a better option).

They also tell us that 'many parents' would choose to have an abortion if they found out their child had Treacher Collins prenatally (not sure how they produce evidence of this claim, since it is not a standard syndrome tested for).

It seems to me that our philosopher and ethicist were looking for a syndrome that's not part of the regular prenatal testing battery to give an example of a situation in which parents don't have the option of aborting because they don't know of the diagnosis. Unfortunately, they didn't take much time to actually research Treacher Collins or speak to people living with it.

They lament the fact that in 18 registeries in Europe between 2005 and 2009, 'only' 64 per cent of ‘Down-syndrome cases’ (remember, we’re not talking people here), were detected prenatally, and that 1,700 babies with Down syndrome were born to parents who learned of the diagnosis at birth. For some reason, the philosophers don’t make the leap of understanding to consider that perhaps the parents of those 1,700 babies chose not to have prenatal testing because they decided to carry a child to term whether the child had Down syndrome or not.

They end this section with this bizarre statement: “Once these children are born, there is no choice for the parents but to keep the child, which sometimes is exactly what they would not have done if the disease had been diagnosed before birth.”

In this statement I can hear my 13-year-old son whining "It's not fair," and myself responding, "There are lots of things in life that aren't fair. But that's life."

Hello! Who said control over our circumstances is attainable in this life? Coming to grips with our vulnerability is part of the growing-up process which seems to have been lost on these academics. How would these philosophers ‘solve’ the problem of our mortality? Because I am sure there are many people who might not choose to die either.

But back to the article.

Giubilini and Minerva note that euthanasia in infants has been proposed by philosophers for children with severe abnormalities who meet two conditions: their lives are not worth living (they don’t define this – although we can assume from earlier comments that they equate an unworthy life with intellectual OR physical impairment that limits a person’s ability to envision or achieve their aims or dreams). The other condition is that they are experiencing unbearable suffering.

They tell us it is reasonable to predict that living with a severe condition is against the best interest of the newborn. They don’t say on what they base this prediction, but it’s interesting to note that while economists analyze quality-of-life years using models that assume low quality of life for people with chronic illness and disability, large-scale studies of self-reported satisfaction in people with disabilities paint a different picture.

Dr. Chris Feudtner, a pediatrician and director of the department of medical ethics at the Children's Hospital of Philadelphia went to great pains to describe this in a talk he gave at the Montreal Children's Hospital last year.

But our philosophers were not listening.

The article is full of contradictions -- on the one hand mentioning studies that find people with severe disabilities are happy, on the other, creating an image of the same people suffering unbearably.

Which one is it?

 They argue that the interests of ‘real people’ – who they define as parents, siblings and society at large – come before those of the newborn, who is a non-person, whether disabled or not. In fact, they suggest the term ‘after-birth abortion’ be used, rather than infanticide, because they compare the newborn to a fetus rather than a child.

“Actual people’s wellbeing could be threatened by the new (even if healthy) child requiring energy, money and care which the family might happen to be in short supply of,” they write.

And: “If a disease has not been detected during the pregnancy, if something went wrong during the delivery, or if economical, social or psychological circumstances change such that taking care of the offspring becomes an unbearable burden on someone, then people should be given the choice of not being forced to do something they can’t afford.” Since they believe the newborn does not yet exist as a person, they perceive the newborn as expendable.

Can't you hear that voice of my 13-year-old: "Why do I have to? It's not fair!"

Guibilini and Minerva even go so far as to say that a healthy newborn could ethically be killed rather than being placed for adoption because the idea of adoption might be more psychologically distressing to the mother.

The killing of disabled newborns would take place within a few days of the birth, they say, which they believe is enough time for the doctors to detect abnormalities. Further, in the case where a child is healthy, the killing can take place outside of any time threshhold as long as neurological assessments determine the child is not self-aware.

Minerva is quoted in the Daily Mail as saying: "This is not a proposal for law. This is pure academic discussion.”

The picture that runs with the piece shows a young woman (20-something?) who was formerly at Oxford and is now at the University of Melbourne.

Perhaps Minerva would like to consider the psychological 'harm' done to the wellbeing of 'real' families like ours who have to read her drivel? We can only hope that in the next few years she gains some life experience, is humbled and comes to her senses.

The editors of the Journal of Medical Ethics defended publishing the article in the name of intellectual freedom. I'm more interested in intellectual rigour.

Read More

A fate worse than death?

I attended a fascinating conference Wednesday at the Montreal Children's Hospital on ethics related to the care of children with disabilities.

One of the themes was cultural devaluing of children with disabilities – and how it can play out in decision-making about care, including whether to withdraw care from premature newborns with significant disability.

A neonatologist in the audience made the following comment.

“There is a feeling among my colleagues – an unspoken and probably unconscious bias – between physical and mental disability. Sometimes neonatologists think if you're not perfect mentally, you're better off dead. But when it comes to physical disability, they will go a long way with interventions.”

Neonatalogists care for newborns with complex, life-threatening problems related to prematurity, illness or congenital disability.

I think the stigma that intellectual disability is worse than physical disability is widespread, even within the disability community itself.

We can't know if what the doctor said about the pervasiveness of this attitude in neonatology is true. Perhaps she is ascribing her own bias to others. But it would certainly be shocking if specialists trained in the care of disabled newborns held such views.

According to the American Academy of Pediatrics, neonatologists go to medical school for four years, then train as pediatrics residents for three years, then train for three more years in newborn intensive care. That’s a decade of learning.

Is it reasonable to expect that in this 10-year span the attitude that intellectual disability is worse than death would be challenged?

Neonatologists, as part of their training, should be required to have extensive interaction with families of children with intellectual and other disabilities, so they have a firsthand understanding of what life with these conditions is like. I hope we hear from a neonatologist about what kind of exposure to families of children with disabilities is included in their training.

At the Montreal conference we also heard about the Disability Paradox – that people with serious disabilities rate their lives as good or excellent while able-bodied people, particularly medical professionals, rate quality of life in people with disabilities as poor.

And we heard about research that shows that the way a health problem is framed influences the decision a family makes about treatment choices. How clinicians order information, the words they choose and even the tone of their voice can have a significant impact on how parents of children make medical decisions.

If a neonatologist were to believe that intellectual disability is a fate worse than death, how could that bias not seep through when conveying this diagnosis to families? Wouldn't that belief run contrary to communicating that a child with intellectual disability has value?

When a life hangs in the balance, I believe parents should receive the most complete information about intellectual disability, including the perspectives of families whose children live with it.

Read More

Listen to the children, ethicist says

We underestimate the ability of even young children to participate in health-care decisions and need to recognize them as part of the decision-making team with their own preferences, said Franco Carnevale, a Montreal nurse, psychologist and clinical ethicist speaking at the 2011 Canadian Association of Pediatric Health Centres Annual Conference yesterday in Ottawa.

“The child has not been on the radar as a recognized decision-making agent,” Carnevale said. “I’d like to see a paradigm shift from adults making decisions about children as moral objects to seeing children as moral subjects with their own insights and perspectives on what’s right and wrong, what’s just and unjust, and what’s good and bad.”

While he doesn’t view children as independent decision makers he says physicians and parents have an obligation to provide them with information adapted to their understanding in an attempt to solicit their “willfull cooperation” in a treatment plan.

“We have a tendency to underestimate children’s capacities to participate in decisions,” Carnevale said. “Those decision-making abilities can develop better in children if we engage them earlier on, and children appreciate having a say about their treatment plan.”

Carnevale said professionals tend to overestimate or underestimate the burden of a particular treatment for youth. For example, he spoke about a 4 1/2 year old girl on long-term ventilation who viewed her pap machine in the same way as an adult might view eyeglasses. “She called it ‘her pap’ and she likes it and is happy it made her feel better.”

On the other hand, he spoke of a 15-year-old teen who opposed having a defibrillation device implanted in his chest because the resulting bump would harm his body image.

By tailoring information to different levels of understanding and listening to the child’s wishes, we communicate an “underlying respect for the child’s dignity,” Carnevale said.

This is important whether or not the child has a developmental disability or can verbalize their wishes, he said.

Carnevale was speaking during a town hall that was moderated by Dr. Brian Goldman, host of the CBC show White Coat, Black Art.

Dr. Margaret Lawson, a pediatric endocrinologist at the Children’s Hospital of Eastern Ontario, echoed the importance of children and parents to healthcare decision-making and said “families are the most underutilized resource."

She spoke of a shared decision-making model in which two-way communication occurs between two experts: the doctor and the family. “You need expert knowledge about the condition... and you need to know how the family feels about risk and what their preferences and values are.

Lawson said a decision tool developed at CHEO “helps take families through the process of making an informed decision that is consistent with the family’s values.”

This includes identifying treatment options, benefits and harms, and the family’s values, including which benefits and harms matter the most to them.

Dr. Lawson emphasized that patient engagement is not just ‘parent’ engagement and the child’s voice is essential.

Read More

Should babies with Trisomy 18 be treated?

Many of you remember our interview with Barb Farlow, who lost her baby daughter Annie (above), who had Trisomy 13, after she was rushed to hospital in respiratory distress. Barb later read Annie's medical records and learned a "not for intubation" order had been written without consent.

This month Barb co-authors a fascinating article in Pediatrics -- the official journal of the American Academy of Pediatrics -- that address the ethics of offering heart surgery to the parents of a baby with Trisomy 18. Most children born with the condition die in the first year of life.

In An Infant with Trisomy 18 and a Ventricular Septal Defect, Barb and two North American neonatologists respond to questions about whether heart surgery should be offered and, after a surgery results in complications and the child can't be extubated, whether life support should be withdrawn over the objections of his parents.

The article concludes with these comments from Editor Dr. John Lantos:

Cases of trisomy 13 or 18 highlight an area of deep disagreement. Most parents would not want an infant with these conditions. Many of them are grateful for the prenatal diagnosis that allows them to terminate an affected pregnancy. Others choose a different course and either forego prenatal diagnosis, or, as in this case, use the information to make decisions about obstectric and neonatal care. Doctors are similarly deeply divided; some feels that aggressive treatment is futile and should not be offered, and others defer to parents. Drs. Okah and Janvier reflect this professional disagreement. Ms. Farlow speaks for the parents who come down on the side of treatment. These cases raise the most fundamental questions about the value of life, the meaning of personhood, and the limits of parental and professional authority. Deference to parents is generally the right course unless the infant is clearly suffering from ongoing treatment that is unlikely to be of benefit. The doctors in this case did the right thing: they worked to find common ground. As often happens, the infant surprised everybody.

Read the article and tell us what you think. Louise

Read More