She says, "Mum, when I grow up, I'm going to be a teacher, mum, queen,"
And I'm not going to tell her otherwise.
She needs to have hope. She needs to have self esteem.
We've never explained the exact situation and their diagnosis to the boys.
And I couldn't do it cause I just couldn't do that to them.
So they've always been lead to believe that one day the doctors will make it all better.
The concept of childhood dementia is foreign to a lot of people.
We as neurologists do see it, but it's always very sad when we see it.
Emily is, oh she's full of life. She likes music and we used to refer to her as a friend magnet.
We can see that she's slowing down, but she's still full of life
I see Emily as she is now, as an 8-year-old who just reminds me of a 4-year-old
We’re the Mickler family and our daughter has juvenile Batten Disease.
Normally we think of dementia as being a problem for sort of older age people.
Battens Disease is a dementia that comes on during childhood.
Most of the problems start with difficulties in vision and then later on problems with thinking appear.
Epilepsy is quite common, so children have seizures.
I'm Richard Webster, I'm a Neurologist and I treat children with Battens Disease.
Battens Disease is a progressive problem. The incidents in Australia is about 1 in 100,000.
So if you look at all the live births, one child is at risk of that. It's an incurable problem.
At the moment, it does feel like a race against time. So since I got the diagnosis I've never been as busy as I am now.
Trying to get all the supports in place. Trying to get memory books made.
Was that me?
That was you. You kicked that one in.
I love my sister. I can't describe how much I love her, but a lot.
They usually lose their eyesight and when they're a teenager, they get bedridden.
And then after that, some last until their 20s, but usually in their 20s they die.
She'll say something like, "Mum, when I grow up, I'm going to be a teacher, mum, queen."
Often Alex will be around and we'll look at each other and then we'll say,
"Yeah Em, you're gonna be a teacher, mum, queen."
There are lots of things we could imagine and fear for the future, but we don't know whether
they're going to happen and focusing on them, I think, would make it even more likely that they happen.
I think it's very distressing for parents.
I think for the child sometimes it's not as distressing although it may be
but I think there's real sadness watching children who are able to do things before being not able to do things.
Where is he? Dano.
How are you big fella?
It plays on our minds I guess constantly but that's something you just get used to.
I don’t beat myself up about it every day going, "Oh, this is what's going to happen."
It's just we do do it day by day.
I think that's harder now cause they're getting older, so you know it's getting closer and you think of I, I don't
know if Glen thinks of that, but I think of you know, "They're going to die." I think of the funeral.
I think of things like that.
We're the Cartwright family. Our two sons have Juvenile Battens Disease.
I remember distinctly, I was driving in a truck and I can still see exactly where I was now driving and Karen was
talking to me and her words were going into my ears, but it was just in a void and you can't
You can't comprehend it.
You can't process that.
Despite all their difficulties and what have you, right down to the core of it, they're like your typical 19 and 22
year old boys in that they wanna be builders. They wanna climb mountains and do all that sort of stuff.
Drive a car.
Drive a car. Play sports.
Have girlfriends.
Yeah, do all that sort of gear.
Dane's, he's the more...he's into factual things like he likes watching documentaries. He loves his reptiles and
football, of course, they love their NRL footy.
And then Matt's more the fun, he likes all the--
He's the clown.
Yeah, he's the clown. Matt the rat.
The dementia seems to have set in now, which is challenging as well again because their behaviour is very,
one minute they can be happy then next minute they can just change in to being aggressive. They've got this
aggression in them now. Anger, frustration
and sadness
Yeah and sadness.
Their long term memory is vivid. Extremely good.
Things that we've clearly forgotten about they etched well into their minds, but current day, if I was to tell Dane
now something within five minutes it would probably he, he would not recall that.
Yeah, they don't really know day night, they don't know anything like that now.
Everyday life for us as a family, present day, is day by day now. Kaz has taught me to live like that.
You can't get too far ahead.
And take it as it comes. And when they're good, take the good days and when they're not so good,
just deal with those, eh?
Yeah, we're a good team.
There’s Matt with his big fish that he caught… In typical Matt style.
The darkest moment for me, would be just knowing that my boys just battle a lot, you know they're, a lot of the
time they're not happy cause they're just physically and mentally can't do what they, they'd love to do.
In terms of a cure I think medicine is still a way off. The reasons for that is that a lot of these treatments need to
be given into the brain and it's quite difficult to deliver treatments into the brain.
I think it is probable that gene therapy will help, but at the moment people are just trying to refine the way to
use gene therapy to treat a problem like this.
There's still hope and it just won't happen, I don't believe it's gonna happen the hope of curing the disease in our
boy’s time. But I hope that will they will definitely cure this disease one day especially for other families.
The doctor says it sounds promising
So while we need to be realistic that it might not happen in time for Emily
there's a seed of hope that maybe gene therapy will work.
Cherished moments are when they score their first goals and tries at footy
they're exceptional days.
when they're just having a good laugh, a good belly laugh.
It's just nice to see them when they smile, I love it.
Because you know they're in a good place and they're happy.
Emily loves listening to The Corrs, the band.
They often have Irish folk style violin parts.
Emily sits there, she plays the violin while she listens to it.
I think you find there are always good moments.
You have your bad moments.
But there are always good moments, and we need to focus on
making as many good moments as we can.