Ben's story

This is a story that D'Arcy wrote about Ben (above, in a much earlier photo) and read at his Life Plan day. It's a beautiful piece because he totally pegs Ben when he calls him a serial giggler. When I first read it, I was going to 'edit' it, as D'Arcy's take on some events is very different from mine. But this is D'Arcy's story as much as Ben's, so I didn't touch it. Louise

Ben's story
By D'Arcy McGovern

Ben is a giggler. A serial giggler. No one knows when a fit of the giggles will catch him; at the dinner table; in the pool, gazing out the car window; late at night curled up in bed.

"What's so funny?!" I always ask. But he never tells me. Of all the riddles that surround Ben, questions marks and mysteries that blanket him like a fog, I'd love to know the answer to that.

Because Ben, viewed from the perspective of most of us, might have a lot to be unhappy about.

Ben has Langer-Giedion Syndrome, a rare genetic disorder. That doesn't mean much to most people, but the result is pretty easy to see. Ben is small, about the size of a six year old, though he just turned 17. He cannot speak. Instead he uses some signs, gestures, sounds. "Um-ah" is Dad. "Ma!" is no. He has weak muscles and bones that are covered with bumps that can sometimes grow so large as to be painful. Two of those bumps, on his hip and knee, had to be removed last year because they were making it hard for him to walk. Ben still limps, but he's back walking again and uses his walker and wheelchair less and less.

Movement has been a struggle for Ben for a long time. At age two, Ben was still crawling long after most kids are up and running. He went to many physiotherapists. We built tiny wooden boxes and forced his legs and feet into the proper positions to stand, climb and walk. There were hundreds of hours of these sessions when he cried -- no, screamed -- from beginning to end. But at long last, he stood and walked.

He went to specialists in Detroit, in New York City, in Syracuse who tried to understand why he couldn't talk. They poked and prodded, made him wait, asked endless questions, tried pills, potions, x-rays and surgery. They advised more therapy and when one therapy proved useless, the next was tried, and the next, and the next with Ben coaxed, begged, and pushed into endless rounds of repetition, tears and frustration. There were weeks when doctors and therapists appointments came every day.

And Ben got sick. Every winter we had season's tickets to the doctor's office. Ear infections. Because of the odd internal structure of Ben's ears, he was very much more susceptible to infections, with the fluid pooling up behind his eardrums, causing pain and reducing his hearing. He had eight sets of tubes surgically inserted in his ears to help drain the puss, each working for a short while before blocking up and being pushed out by the building tidal wave of goo.

Ben stopped growing. He was given growth hormones -- daily painful needles for months -- to no effect. He grew thin. A high-fat diet pumped cream and butter into his system. And then a G-tube -- a tube cut into his stomach, with liquid nutrients forced into him to try to get his little body to grow. The tube in his stomach had to be changed frequently, the old one yanked from his belly while he was held down screaming, a new one jabbed in and inflated.

Tears. Tears all 'round.

At nursery school, Ben mostly played alone. The nannies, the moms, the other kids were unfailingly polite. But Ben couldn't communicate, and the other kids, jabbering away a mile a minute but met with stoney silence, slipped away where Ben couldn't follow.

Kindergarten at one school ended abruptly after Ben was found bound to a chair -- punishment for some minor discretion.

And then Ben was touched with a fairy sprinkling of luck. He found an alternative grade school -- Alpha -- filled with eager, compassionate kids and teachers, who brought Ben into their family. And Ben experienced the miracle of friendship -- real friends to play with, to go to birthday parties, to swap toys, to share their lives.

Ben started to read, comic books and picture books, gazing at them for hours, going back to the same ones over and over. And computer games, a source of endless mirth and often the spark for an explosion of giggles. He had electronic friends: Reader Rabbit, Putt Putt, Freddy Fish, Pajama Sam and printed ones, Geronimo Stilton and Captain Underpants. The real world Ben found so hard to relate to came a little closer through the virtual world of books and games.

I have stood amazed as Ben has made his way through the world, stunned that so much pain, so little return for so much effort, has left him not bitter and disconnected, but happy, open and even at times serene. I have said more than once that Ben is like a balm for my soul. On my worst days, I can come home and melt into his little arms, swallowed up by calm. Ben bears no grudges, makes no judgments, accepts all faults. And he is always ready for a good laugh.

So, I'd like to know why Ben giggles. But not because I need an answer. I just want to join in the fun.

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He's on a roll

Ben caught more than 6 fish today!

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Too disabled? Not disabled enough?

It bothers me when criteria for services for kids with disabilities is so restrictive.

Recently we were referred to the local agency providing service for youth with intellectual disabilities. We wanted a consultation with a behaviour management person to see if we could reduce Ben's tendency to pick at scabs when anxious.

I was asked to send a copy of Ben's most recent psychological report. I was surprised when the intake person called back to say that they wanted to look at previous reports because Ben's scores might be too high to be eligible for their service.

The intake person said the agency only served youth with moderate to severe intellectual disabilities that place them below the 1st percentile. However, among the disability community, this organization is known as serving youth with intellectual disabilities period. When I asked my colleagues if they knew this provider only served a segment of this population, they did not.

I didn't have previous reports handy, so I had to contact the psychologist and sign a release consent to get copies. If I didn't work at Holland Bloorview, I would have had to make a trip to pick them up. Tomorrow my husband will take them into work to fax them.

Parents don't have time to play these games. My son requires a service and was referred to this agency by his developmental pediatrician.

It's amazing how many programs are closed to youth because they are either "too disabled" or not "disabled enough."

I guess we will wait while the agency's psychologist pores over these lengthy reports to see if he or she can find a way to exclude my son.

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The boy is back

This just in from Camp Kennebec on day 2:

Ben slept fantastic last night. The storm was brutal but we got through it. Ben and I just got back from fishing. He caught 4 fish and we saw a huge turtle. Was amazing. 

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Disability and quality of life

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.

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When 24-7 caregiving destroys a marriage

The following piece was written in response to our recent posts on the mental-health impacts of parenting kids with disabilities. This story was submitted by a blogger who is known to many of you for her wisdom, compassion and humour. And now -- courage. She wonders if there are others out there like her.

When 24-7 caregiving destroys a marriage
How one woman's world gradually shrank away

I never imagined it could happen to me.

I am educated, self-reliant, and was nurtured in a secure, loving home. I ended up, however, in a psychologically-abusive relationship. Psychological abuse leaves the victim blaming herself and questioning her own perceptions over the behaviour of the abusive partner.

It started when my child had a catastrophic illness that left her with severe disabilities.

Over an 11-year period, I slowly but surely abandoned everything -- family, job, friends -- to care for my child. My spouse could not, or would not, ever do it on his own.

First he insisted that help was required from staff or myself. Then the rule became that there must be two people at home at all times -- staff and myself, or spouse and staff. And when there was no staff, it had to be me and him.

He never went back to work and I wasn't allowed to.

I let go of everything that stressed him out. And stressed he was, with Post Traumatic Stress Disorder. But it was never enough. I could not open a window or it would cause allergies. I could not make a morning coffee until he was watching our child a foot away from the bed.

I could not go out unless I had permission and there was 'enough coverage.' As a result, I haven't been alone in the house for at least nine years. But I believed it was for 'the cause' -- our child -- and that my spouse was an exceptional man for sacrificing so much for her.

It was our child he used to hold me emotionally hostage.

He believed it was only our unwavering hyper-vigilance and constant focus on 'improvement' which kept her alive and well. No staff was good enough, no doctor, no therapist, no school. As my world got smaller, so did my child's. We lived in a room, watching constantly for 'trouble.'

It was through blogging that I came to understand what was happening to me. I became acquainted with a whole world of disability, and caregivers of the severely disabled. I learned how they lived and became acutely aware that something was very wrong in my world.

I got counselling, often in secret. Counselling helps the abused step back cognitively and process the situation, so that she can understand the nature of the relationship and the abuse. It also provides tools to help the victim develop better boundaries so that she can extricate herself from the relationship.

It took me over a year to ask for a divorce. Interestingly, it was my spouse's insistence that we get couple's counselling that reinforced my decision. I became aware of my issues, was able to see where I was off-track in my perceptions, and learned what to do to move ahead. My spouse was shocked that the therapist didn't get me to 'see the error' of my ways.

Now, as I finally plan to leave, child in tow, my partner says I am selfish and am causing our daughter to suffer more, deteriorate and even die.

I will move ahead regardless.

I am on my way out. There is a long road ahead. I will have to deal with my spouse for as long as I have 'our' child. But I have the tools and the growing self-confidence to speak up and move forward in life. In the end, it will benefit not only me, but my beautiful disabled child, who, along with me, will gain a new life.

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Kumon man

Ben has never been able to write because of weakness in his hands.

I started him on Kumon reading but figured I would always have to fill the letter blanks in. It's hard for him, but he's doing it now. And he'll even do a whole book by himself -- quite miraculous considering the degree of his attention problems.

And he's able to identify most words when asked "show me ___________"

I know writing is supposed to aid reading so I'm very happy about this.

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