Adam's Story - Children's Vision Day

It's two years this week since we found out Tricky had a skull deformity that would require surgery. As awful as it is to go through something like that, the people that I've met because of it, more often than not, turn out to be truly amazing.

I was lucky enough to be introduced to Jennie by the staff at Princess Margaret Hospital. They pair up parents of newly diagnosed kids with those who have gone through it before, and her son, Adam, had walked (well, crawled - they were babies after all) the same path. Instantly we were on the same team, bonded by this intense, shared experience. And as time passed we moved from being fellow cranio mamas to actual friends.

Now you’re probably wondering why I’m blabbing on about Craniosynostosis when the title of this post has Children’s Vision Day in it. Well Adam, who is now three years old, is going through another rough patch and Jennie graciously let me interview her in the hopes that together we can raise awareness. I've never actually interviewed someone before, so I went all serious, pretended I was Jana Wendt and just wendt for it:

Can you tell us the name of Adam’s condition?
Adam has a condition known as bilateral retinoblastoma. 

What exactly is retinoblastoma and how common is it?
Retinoblastoma is a cancer of the retina, the inside lining of your eyeball. Some children only have the tumours in one eye, Adam was unlucky and has it in both eyes (which is where the ‘bilateral’ bit comes in).

I’ve never actually heard of retinoblastoma before, how common is it?
It is quite rare, it accounts for only 2% of all childhood cancers, and I have been told there are only about 3 cases a year in WA.

What signs had you noticed that lead you to think something might be wrong and seek medical advice? 
I had noticed a strange reflection in one of Adam's eyes (leukocoria). His pupil looked white in certain lights and at certain angles. It didn't seem to be causing him any problems, he wasn't bumping into things or complaining of not being able to see. It's strange the way you can come up with reasons for things, I was thinking that it was just something that I hadn't noticed, that maybe it was because he was now a bit taller and I was seeing his eye at a different angle. After maybe a couple of weeks I started to think that really there was something not quite right, that it really shouldn't look like that, but I didn't think anything was seriously wrong.

Adam's left eye showing leukocoria

I made an appointment for both of my kids to have an eye test at the optometrist. My daughter went first and her eyes were good. Then it was Adam's turn. The optometrist asked if I had any concerns and I told her about the white reflection. She first tested his vision with both eyes and it was good, then she covered his "bad" eye and again his vision was good, then she covered his "good" eye and my heart nearly broke.

He started moving his head around, trying to see from his good eye and it was so obvious that he was blind in the bad eye. How could I not have realised? My heart broke for my little boy and I felt like absolutely the worst mother in the world, how could I not have known that he was blind in one eye?

She then looked in his eye and told me that she thought he had a cataract. This would require surgery to remove the lens of his eye, which could then be replaced with an artificial lens and that he would get his vision back. She gave me a referral to a paediatric ophthalmologist. I went home and rang my husband (who was away travelling for work) and burst into tears, telling him that Adam was blind and would require surgery on his eye. After I had calmed down a little bit, done some Googling, I told a few friends, and I remember saying "at least it's not cancer". As traumatic as him having a cataract would have been, I wish that is all it was!

I rang to make an appointment to see the ophthalmologist and then next available appointment was in about 2 months time. I really didn't think I could wait that long and I started getting quite upset on the phone, so the receptionist started looking for another appointment. Luckily she found a cancellation for 2 days time, so I grabbed it!

By this time my husband was home, and so he came to the appointment too. First the ophthalmologist repeated the vision test that the optometrist had done. Then he put dilating drops in Adam's eyes and after they’d taken effect he looked in to his eye.

I will never forget that moment. He said "I'm afraid it's not good news. It's a tumour". 

Can you describe how you felt when he said that? It must have been such a shock.
I'm almost sure the world stopped turning for that briefest of moments. Yes, shock is probably the only way to describe it. I didn't quite get it, I had to say to the ophthalmologist "you mean it's cancer?" and he said yes.

The fear I felt was enormous and I started thinking what will happen to him? How will he cope? Will I lose my little boy?

I imagine with a cancer diagnosis, everything happens very fast. What treatment has Adam had so far?
The day after the diagnosis Adam was sent to PMH to have a CT scan and then an MRI and a really thorough examination of both eyes under general anaesthetic. These tests showed that there were tumours in both eyes, but that they appeared (thankfully) to be confined to the eyes only - there is a risk with this type of cancer that it can "escape" via the optic nerve to the brain. The tumours in his left eye were too large to be treated and the only option was for them to remove his eye. Thankfully the tumours in the right eye were considered small enough to be able to treat with laser surgery and chemotherapy.

The following week Adam was admitted and his eye was removed (enucleation) in one operation and a few days later another operation to have some laser surgery and to have chemotherapy port put in.

So far he has had two rounds of chemotherapy and we are expecting that he will be having chemo for at least another 6 months. On top of this, every 6 weeks or so he has to have another general anaesthetic and either more laser surgery, an MRI or just an examination.

That is some intense treatment for such a little fella! How has Adam coped?
He’s amazing! People talk about the resilience of children, and it is so true! It has made me so much more aware of the emotion and “baggage” that we attach to illness. Adam has none of the associations that we as adults attach to cancer, he knows that his eye was sick and that he had to have an operation and he knows all about his chemotherapy port - his "button", but he doesn't have the dread and fear that we as adults have.

Adam showing off his temporary prosthesis

I can’t imagine how hard it would be for you, how are you dealing with the stress?
With wine and chocolate!

But really just trying to live by all those clichés that you hear - one day at a time, make the most of every moment, be happy now because you don't know what's around the corner.

If you could tell parents one thing, what would it be?
Again, all the usual clichés - treasure each moment with your children and family because you never know when it will be your last. Get your childrens eyes tested. Trust your instincts as a parent. If you think something is not right, follow it up.

Thank you so much for sharing your and Adam's story, Jennie, and best of luck to Adam for his operation today to have his new prosthesis fitted!

If you would like to donate to childhood cancer research at PMH there are a number of ways you can do this:

1. Support the Telethon Adventurers – their most recent adventure to raise money involved climbing Grand Paradiso in Italy with a giant poster of all the kids currently being cared for in the cancer ward of PMH (Adam’s picture is bottom right).
   
   
   
   
2. Support the Perth Bloggers and Tweeters Big Walk team. We’re all walking for different reasons but with the same goal – to raise $500 for PMH. If you’re in Perth, join our team (the password is Glowglow) and if you don’t want stroll around the river with us you can just donate.
   
   
3. Buy Lexie’s Look & Cook Book - all proceeds raised from the sale of this book go towards purchasing non-invasive eye testing equipment for PMH to make the process of diagnosis and treatment less painful and scary.