When Alex Mickler’s younger sister Emily was diagnosed with Batten disease at the age of seven, she didn’t quite understand what it was.
When she was diagnosed in 2017, Emily, who Alex and her family describe as a “friend magnet”, had already gone blind and had been searching for the cause for three years.
“Mum and Dad, they sat down and told us, me and Ben [brother]they explained what it was and what was going to happen to it, and I think I knew then what was going to happen,” Alex recalled.
“But I didn’t fully understand what it would be like for us as a family and I think that meant I wasn’t really ready.”
Alex, in the background in a navy shirt, recalls the moment his parents explained his sister Emily’s condition. (ABC News: Kathleen Calderwood)
Batten disease is an extremely rare, fatal and incurable neurological condition in which children who were previously healthy and reaching developmental milestones lose their motor skills, speech, sight and ability to eat.
Children with Batten disease also experience seizures and infantile dementia.
“One of the conversations I have most often with parents and families is that trying to explain what is happening to the affected child to siblings and peers and other children is very difficult,” said neuroscientist Ineka Whiteman.
“It’s hard for these children to understand that this was a typical, seemingly healthy brother or sister that they had and grew up with for a period of time during their childhood, to suddenly realize that this child is losing skills they once had.had.
“They’re seeing severe behavioral changes, cognitive changes, and trying to explain that to kids — it’s a big challenge for families.”
Emily was seven years old when she was diagnosed with Batten disease. (ABC News: Kathleen Calderwood)
A creative way to help
Dr. Whiteman has written a book called Max and Abby about two brothers, one with Batten disease.
As part of the process, he interviewed siblings and families of children with the condition, including Alex Mickler.
In the book, Max loses his ability to sing, read books and ride a bike.
Dr. Whiteman describes what’s going on in his brain using the analogy of garbage trucks that don’t take the garbage away.
Dr. Ineka Whiteman says the children struggle to understand why their sibling is losing his skills. (Supplied)
It also explores the frustration experienced by his sister Abby, when Max misbehaves and is not disciplined or getting full attention from his parents.
It’s something Alex can relate to.
“Max keeps them up at night and they always have to take care of him. I relate to that because Emily usually needs 24-hour care, there’s always someone there to help her,” she said.
“And sometimes that means mum and dad don’t have as much time for me and Ben, which sometimes feels a bit lonely.”
Dr Whiteman hopes the book will help siblings feel less alone in their experiences.
“Even though this affected sibling who has Batten disease may be taking a lot of time and care from mom and dad, spending a lot of time in the hospital and giving a lot of care, these siblings are still loved, they are still very much part of this family unit.”
Have difficult conversations
For Alex’s mother, Julia, the book would have helped not only her children, but also their friends, teachers and even some adults, she said.
“A book is less confronting, even a lot of adults we talked to about the diagnosis…they didn’t know how to deal with it and we had a range of different reactions.
“I think a book like this opens up a debate, makes it less confrontational, you can talk about another person, instead of this particular child.
“A great thing about books is that you can explore your own feelings through a character and it’s easier to step back from those feelings and talk about them.”
One of the siblings in the book Max and Abby has Batten disease. (ABC News: Kathleen Calderwood)
The book has been published by HammondCare, which provides specialist support for children with dementia and their families, and is available free of charge.
Although it was written with Batten disease in mind, Dr. Whiteman hopes it will help families with children with other forms of childhood dementia.
Alex said she hopes the book will help others facing a difficult diagnosis to know there are still fun times ahead.
“It puts a lot of emphasis on the fact that life is not always easy, but also on the fact that there are good times,” he said.
“It has brought the family together, because we all have to look after Emily and work together as a team.”