Emily is eight years old and already losing her short-term memory and her ability to speak. She has juvenile Batten disease or CLN3. Some describe it as 'childhood dementia'. It's an incurable and degenerative neurological disorder that eventually leaves children bedridden and most children with the disease won’t ever experience adulthood.
“When she says to me, 'Mum, when I grow up I'm going to be a teacher or a queen! I'm not going to tell her otherwise,” says Julia. “I think she needs to have hope, she needs to have self-esteem, she needs to know that she can do things.”
Everyday life for us as a family is day by day.
Richard Webster is the head of neurology at The Children’s Hospital at Westmead and said many people associate dementia with the elderly.
“Most people aren't aware that there are dementias that come on in childhood,” Dr Webster said.
“So most people are aware of diseases like Alzheimer’s disease and other diseases where people lose their cognitive skills, their ability to do day to day things and have problems with their behaviour and we see the same problems in Batten disease,” he says.
The Mickler family are still coming to terms with their daughter's diagnosis and say they want to cherish every moment with her.
“I think you find that there are always good moments. You have your bad moments but there are always good moments and we need to yeah just focus on making as many good moments as we can,” says Julia.
The Cartwright family, based in far-north NSW, have had several years to come to terms with the diagnosis of Battens disease. Their two sons – Dane, 22 and Matt, 19 – are now young adults, but daily tasks like walking are no longer possible and they find it difficult to speak.
Their father, Glen Cartwright, hasn’t told the boys about the disease. He wants to share their story to help other families in their situation.
“We've never explained the exact situation and their diagnosis to the boys… I just couldn't do that to them,” says Glen.
“Everyday life for us as a family is day by day. You can't get too far ahead and take it as it comes,” he says.
While there is no cure for Batten disease, researchers say they now have a better understanding of the different forms of the disease.
Both the Mickler and Cartwright families know it may be too late for their children, but they’re hopeful a medical breakthrough will come for other families in their situation.
