Shine a Light

Shuichi Suetani (New Zealand)

Michael (not his real name) is a three-year old boy with Apert’s syndrome.  I was fortunate enough to meet this young boy in 2009.

Apert’s syndrome is a craniofacial/limb abnormality that affects 1 in 200,000 births.  Although it is an autosomal dominant condition, 98% of the cases are sporadic mutation of FGFR2 gene in chromosome 10.

This rare condition is characterised by craniosynostosis (premature fusion of sutures of the skull), midfacial hypoplasia, exorbitism (bulging of eyes) and syndactyly (webbing of fingers and toes). Complications associated with increased cranial pressure are also common.

The treatment is predominantly surgical.  The main aim is to release the fused sutures and correct any abnormalities of the skull.  Surgery should be performed as early as possible for better long-term prognosis.

Prognosis varies and depends largely on how early the surgical treatment was initiated.  Some patients have normal life expectancy, although most are mentally impaired to some degree.

All of the above, I knew before meeting Michael for the first time. These are the things that you can learn in text books.  I even knew what he looked like, from the images on the internet.  I felt like I knew a lot about Michael.  Because he’s a child with a chronic condition, and I knew all about the chronic condition.

What I found out, through home visits and catch up chats with his mother, was how little I knew about Michael himself.  Like his mother told me repeatedly, Apert’s syndrome is only a tiny part of who Michael is.

In this appreciation essay, I have tried to explain my experience through a poem.  I will attempt to explain each stanza of the poem in turn, and hopefully at the end of it, you can have some idea of what this experience has been to me.

Shine A Light
What do you see through your eyes?
Characteristic features in text books
Diffused fingers, decreased cranial size
Another knowledge to go with your looks

Come closer, come right near
Chasing balloons, drawing lines
A busy happy boy, such a dear
Choosing blocks, picking pines

Let me shine a light
(Sure shit happens...)
Let me shine a light
(...but things could be worse)

Auckland, Christchurch, nights away
Surgeons, paediatricians, speech therapist
Fix me, inspire me, make my day
My mother, my father, my older sister

So tell me once again, what do you see?
A tiny part of my life with a funny foreign name
I am who I am, so let me be me.
Were you expecting that we are all the same

Let me shine a light
(He's such a courageous boy...)
Let me shine a light
(...he makes us very proud)

* * * * * *

What do you see through your eyes?
Characteristic features in text books
Diffused fingers, decreased cranial size
Another knowledge to go with your looks

As medical students we are conditioned to pick up diseases.  When you see a patient, what goes through your head is a checklist of characteristic features.  This is a skill that you quickly develop in clinical years.

It’s not only confined to a hospital setting either. You’d be walking down the street and spot somebody with a mask-like face and a funny gait.  He stops and sits down, and you’re looking at his fingers to start pill rolling. And bingo!  Parkinson’s disease.

It becomes increasingly easy for us to make patients into “cases”. Your old neighour Mr Smith becomes a Mr S with COPD.  Your old girlfriend Jenny becomes a Miss L, a 24-year-old woman with a high risk of ectopic pregnancy.

Just because it’s so much easier to talk about people this way. Heck, we’re not even talking about people anymore. Unfortunately, that’s how we are conditioned.

If I was honest, what I saw was diffused fingers and decreased cranial size.  Michael looked a lot like every other boy with Apert’s syndrome.

So the first thing I did when I met him was to tick off the check list. Craniosynostosis, tick.  Midfacial hypoplasia, tick.  Exorbitism, tick. Syndactyly, tick.

Mental picture captured, so that I can spot them if a picture pops out for a multiple choice question.

Come closer, come right near
Chasing balloons, drawing lines
A busy happy boy, such a dear
Choosing blocks, picking pines

I don’t know why, but the first time I met Michael, I decided to go up to him to shake his hand.  I was never going to succeed in that kind of “adult” social interaction.  Predictably, Michael ignored my hand and ran away from me.

His parents laughed and told me not to take it personally.  I’m not sure what I was expecting.  A formal start to this longitudinal assessment?  Maybe.  Whatever I hoped for, all I got was a long-haired blond boy playing on a toy horse.

I just stood back and watch him play at the paediatric outpatients’ playground.  The paediatrician was running late, so I stood there for a good 10 minutes, just watching Michael play.  From time to time, he would look up, just to check that I was still watching him, then he shyly turned away again and start playing with whatever was lying around him.

On my second visit, his mother told me that he is just like every other kid.  This time around, he was a little bit more used to me.  He would come closer to me, but not too close, and would start drawing lines and circles on his homework book.

His older sister, aged nine, had brought home a homework book. Michael wanted his.  So his mother bought a little notebook for him. Now he would bring it out every time his sister practised her spelling.

“He’s showing off to you,” his mother said to me, “aren’t you Michael?”

Michael loves playing in the back garden with his father.  Michael loves running around, jumping around.  Michael loves doing homework with his sister.  He’s just like every other kid.

Let me shine a light
(Sure shit happens...)
Let me shine a light
(...but things could be worse)

That is what his mother said to me. And it pretty much sums up the family’s attitude.

One of the things that she decided from the start is that she was not going to let anything get in the way of Michael’s health. Sometimes it meant having an argument with a specialist and flying to Auckland for treatment.

Michael’s father decided to stay home to look after his son.  It was a mutual decision, but it did mean that the family lost half their income.  But it was better for one of his parents to look after Michael than to have somebody else doing the job.

His mother was over 40 when she fell pregnant with Michael.  She didn’t find out about Michael’s condition till he was born.  Had she known that he was to have a congenital disease, she would have aborted the pregnancy. “I did the amniocentesis for that very reason,” she told me, “but I guess I wasn’t expecting Apert’s syndrome!”

Her initial, honest feeling was “gutted”.  She felt down for a little while, feeling sorry for herself.  It was much more than baby blues and hormone withdrawals.  But her husband was determined to keep his first son. “I do feel a bit guilty, thinking back now.  But at least one of us loved him dearly from the start.”  And soon enough, she had begun to treasure her son.

You can feel sorry for yourself all you like, but there will always be people much worse off than you, and your life ain’t as bad as you think it is.

Auckland, Christchurch, nights away
Surgeons, paediatricians, speech therapist
Fix me, inspire me, make my day
My mother, my father, my older sister

From the start, the family fought as a unit.  Michael’s maternal grandparents are dead and his paternal grandparents are in North Island.  They have no family support in Dunedin.  Wherever Michael’s treatment takes them, they go as a team.  They stay at Ronald McDonald House.  They meet other families dealing with chronic conditions.  They meet nurses who work their hardest to make their stay as pleasant as possible.

When everybody is going through a tough time, everything else becomes very relative.  All of a sudden, you’re not the saddest person in the world.  In the bedroom next door, a young mother is sleeping next to her terminally ill daughter.  The same woman who would come up to you next morning to wish you luck for Michael’s operation.

You realise that people care.  You realise that life isn’t all that bad after all.   If you sit alone in the kitchen, not knowing what to do, a nurse will come up to you with a cup of tea and sit next to you. She just sits there, ready to listen.

One of Michael’s surgeons visits a country in South East Asia every year to give free prosthetic legs to the child victims of landmines. Michael is not the only person being fixed on these long, nervous surgery trips.  Healing doesn’t just happen in the operation theatre.

I can’t help but think that Michael’s family must be an inspiration to somebody else.  I don’t say this to his mother because it sounds so cheesy.  But surely, I keep thinking, they must be an inspiration?

So tell me once again, what do you see?
A tiny part of my life with a funny foreign name
I am who I am, so let me be me.
Were you expecting that we are all the same?

Hopefully, I’ve drawn you a better picture of who Michael is. After talking to his mother and just getting to know Michael, it soon became clear that this congenital disease is only a small part of who he is.

We have a tendency to categorize individuals into broad groups.  I’m not just talking about chronic conditions.  We do this every day, we categorize people by the way they look, the way they talk and the way they interact with you. Judging the book by cover, is how they put it, which is something that my mother told me not to do. Because by doing that, you’re not opening the book, are you?

I guess the bottom line is that, you just have keep reminding yourself that Mr S is not a typical COPD case.  There is never a typical case.  There is never a case.  Mr Smith is your next door neighbour who happens to have COPD.  The same Mr Smith who made you cheese-on-toast after school when you forgot your house key.

A disease should never define a person.  As soon as you let that happen, you’re on a losing streak.  Like Patch Adams said “If you treat a disease, you win or you lose. But if you treat a person, I guarantee you, you'll win, no matter what the outcome.”

Let me shine a light
(He's such a courageous boy...)
Let me shine a light
(...he makes us very proud)

I’d like to close this poem by quoting his mother once again. She admits that having Michael has, at times, been very tough, but it has also brought the family very close.  She tells me that she wouldn’t have it any other way, although if she had a choice, she’s not sure if she would do it all over again.

In a way little Michael has become a symbol of a family’s bond.

And he sure did shine a light to me.  He let me see something that I’d never seen before.  Being touched, is a description people use.

Let me shine a light, the little boy says, so that you can see.

Article first published on the site in 2010.