There Be Monsters

Through the 11 months (!) that my twins have been on this earth, I have been exposed to many things and many new concepts.  Along with my "twin mom" hat came a "special needs mom" hat and there have had to be some accompanying adjustments.

It's the second hat, my "special needs" one that has forced me to consider quite a few things that, in my own ignorance, did not even occur to me.  It never even crossed my mind that in this day and age, there would be people that did not even know what Down syndrome was;  however, I have found myself explaining it numerous times over the last year or so.  I don't begrudge this either, as it gave me an opportunity to present it fresh, in a positive light as opposed to dispelling myth.  I myself have had to unlearn and relearn a few things about DS.  When my daughter needed surgery for her inguinal hernia at 6 weeks, there was no doubt in my mind that she would receive exceptional care.  When my son was diagnosed in utero with an Atrioventricular septal defect (AVSD), there was no doubt in my mind that when the time came, he would receive the surgery he required in one of the leading children's hospitals in the world in Toronto.  It was never questioned, as why wouldn't he?  It never crossed my mind that this was never an option for a child with Down syndrome.

Then, a few days ago, I learned of Amelia, a  girl who lives in Philadelphia.  She is a little girl with a very rare genetic disorder known as Wolf-Hirschhorn syndrome (I had to look it up too).  It manifests itself in a variety of medical considerations and some global delays, just like Down Syndrome.  I know about her as she requires a kidney transplant or she will die... and she will die, as she has been refused the operation.  She has been refused a life saving procedure as she, in the words of both the facility and the surgeon, is "mentally retarded".

According to Amelia's mom, Chrissy, even though the family offered to find a donor within itself, the surgeon refused to perform the transplant due to Amelia's "quality of life".  It's not just the doctor either, as this is apparently a matter of hospital policy.  You see, "mental retardation" is a criteria for exclusion from a transplant procedure, like Hepatitis C or HIV.  In fact, it's listed on the form, just under those exact two things.  This was brought to the attention of Amelia's parents, thanks to the stroke of a pink highlighter.

I'm not even sure I can adequately express how shocked I am and to what extent.  You have to understand that I live in Canada, land of universal health care.  Immediately I thought "it must be just that hospital" and began digging.  Then it became "it must be that state" and kept reading.  Finally, it became "this would never happen in Canada".  Well, I was wrong there too.  It has only been in the last while that even surgery to repair defects was an option for kids like Wyatt, never mind transplanting an organ.

In my research, I came across the story of Sandra Jensen, the first woman in the world with developmental delays (specifically Down syndrome) to receive a heart-lung transplant at Stanford University Medical Center in California.  In 1996, 15 years after the first heart-lung transplant at the same facility, long after such a revolutionary surgery had become a matter of routine.  Sandra, an outspoken activist, volunteer and valued member of her community was initially denied the surgery due to her "quality of life".  She, and her family, had to sue the hospital in order for her to have her procedure. Sadly, Ms. Jensen died 16 months afterwards, not from non-compliance, not from rejection, but from a rare form of cancer that can accompany transplants.  Related to this is an international study from 2006 that shows that success rates among those with "MR" or developmental delays are equivalent to those who do not.  Bonus: the study looked at the most common type of transplant, kidneys.

Amelia's parents were told by the staff that it was her "quality of life" that was to be considered and it was put forth that they might not be around in the future and "who would make sure she took her medications then?".  There are two major points here I would like to address.

The first is the phrase "quality of life".  We use this in medicine a lot as a sort of blanket statement to convey a list of complex thoughts surrounding a person's treatment, level of comfort and/or happiness.  Amelia is just shy of her 4th birthday and I'm sure, like other kids with a developmental delay, has a little catching up to do.  At this point, her parents do not know what she will do or achieve as an adult any more than any parent of any pre-school age child.  Is she suffering?  Yes, currently from kidney disease.  Would a transplant alleviate that?  Yes, it would.  She would have a treatment regimen of certain medications (that have manageable side effects).  She will then live, and grow and learn and yes, even be happy.  Am I wrong in assuming that would improve her "quality of life"?

The real issue here is the underlying text:  "Amelia is delayed and therefore broken".  If she has the surgery and takes the anti-rejection meds, she could theoretically have a seizure, which theoretically could deprive her brain of oxygen and cause her brain damage.  It's ok to impose this on a "normal" child, but "her brain is already damaged" (the surgeon's words) so theoretically she could end up very very damaged, or dead.  Theoretically.

What isn't theoretical is that dead is what she definitely will be without the transplant.  She will go into kidney failure and be made as comfortable as possible, given the circumstances.  The comfort measures, by the way, will be given out of respect to her "quality of life".  I can't be the only one that sees the irony here.

Along with this is the assumption made by the staff at CHOP that a grown Amelia would be unable to care for herself, specifically, she would be unable to remember to take her medication.  This is a blatant example of the type of discrimination that delayed persons face every single day.  We don't know that any more than any other kid.  We can't see into the future.  My BFF is a Diabetic Educator and she has many delayed patients, most of them with Down syndrome.  She lights up like a Christmas tree when these patients visit.  Despite their varying level of functioning and unlike the rest of the population, these patients are compliant with their treatment regimen, take it seriously and do as much as they can to better themselves. Most of her patients cheat on their diets, don't take their insulin or diabetic meds properly and always think they know better than her because, after all, she is just a nurse.  Her special needs patients make her day and it always makes me smile to hear it.

My mental health patients vary in their degree of functioning due to nature of their illnesses.  Are they not worthy of life saving transplants?  I have similar stories to my BFF as some of my "lower functioning" patients are more compliant and hold jobs and contribute to their community.  I wish I could say the same thing about some of the "higher functioning" ones.

Every special need parent, when they get that new hat, considers the future for their child.  I have touched before on some of the financial considerations that have to be addressed and how easy they are to achieve.  The idea that Amelia's parents have not considered this is laughable, the idea that a social worker would suggest this as a reason that this girl would be denied a new kidney is insulting to both this family, to all special needs families and to social workers in general.

I hope the ethics committee reconsiders their decision and I hope this family finds another surgeon.  I also hope that the medical community looks a little more closely at bio-ethics in relation to the special needs community.  I dare say, they will find a lot of work to do.  There still be monsters in them thar' waters.

Dave Hingsburger, a special needs blogger, has put forth in a recent post the suggestion that January 23, 2012 be the first International Day of Mourning and Memory of the Lives of People with Disabilities. The date is no accident;  it is the day in 1996 that Sandra Jenson received her transplant.  In his words:

"The day would be one of remembrance of those whose lives were not celebrated or remembered, the lives of those who were slaughtered by care providers or brutalized to death by bullies. It would also be a day to remember the entire disability community - the elders who came before and who made the world different and better. It would be a day where a moment was taken to pause and reflect and remember."

I like that.  

So many people with developmental delays are still hidden away, world wide, to this day.   Despite all our efforts, there is still so much work to be done.  So many lives to celebrate.  So much predjudice to dispel.

I hope that we are not too late and I hope little Amelia gets her kidney.  She is a little girl with a visual intellectual disability and because of that, does not qualify for an organ transplant.  Amelia does not live in some far flung third world country, but in the US.  Amelia deserves to live and to grow.  She deserves the ability to explore her highest potential.  As do all children with developmental delays.

There be monsters, all right.  And they are us.

I will be observing January 23rd.  For Sandra, for Amelia, for the hundreds of thousands of kids like Wyatt who unlike him, did not get the chance to feel the sun on their face or fall asleep in their mothers arms.  For those locked away and hidden from view, for those left to starve.  For those denied medical care that is readily given to others.  For those denied their basic humanity.  For those parents and advocates that have had to sit helplessly by and watch it happen.  For those humiliated and shamed by a thoughtless public who continues to dismiss developmentally delayed individuals and their struggles to achive equality.  It will be for them.

I hope for Amelia's sake, that we are honouring more than her memory.