Siblings diagnosed with childhood dementia not expected to live past their teens

There is no time like the present for South Australian mum Renee Staska.

All three of her children have dementia and aren't expected to live past their teens.

Like many, she never knew the cruel disease affected kids, but has now joined the fight as scientists work as fast as they can to help.

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"I don't have time on my side, so I can't just sit back and hope that something's going to change," Staska told A Current Affair.

Eight-year-old Hudson, six-year-old Holly and four-year-old Austin have Niemann-Pick disease type C.

It's one of more than 70 genetic conditions that trigger childhood dementia.

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For Hudson, symptoms have already started.

He struggles to read and write at school, while Holly's challenges are different.

"She breaks her own heart because she can't keep up sometimes and she can't, you know, she tries so hard to understand why it's not working for her," Staska said.

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When little Austin was eight months old, he had an enlarged liver and spleen, prompting doctors to investigate and discover the rare genetic condition.

"I was given it on a piece of paper. They sort of said, look, this is what it is," Staska said.

"It's terminal. There's no treatment, there's no cure."

The terminal condition is triggered when both parents have a mistake in their genes.

Staska's two eldest children had a 25 per cent chance of inheriting it.

"I decided to get them blood tested, so I could stop worrying all the time and they both came back positive as well," Staska said.

"I cried and I cried and very soon we were introduced to palliative care, which I wasn't even aware was a thing for children."

The children develop appropriately to a certain age before they start to regress by losing their memory, body functions and then the ability to walk, talk and eat.

"Most children with Niemann-Pick type C don't live to see their 20th birthday," Staska said.

Scientists like Dr May Aung-Htut hold all the power.

At Perth's Murdoch University, Aung-Htut and her team are developing a drug to reduce the level of fat production in children with dementia.

"The problem with these children is they have genetic defects that they cannot get rid of these fat molecules," Aung-Htut said.

"They start to build up and this build-up leads to a toxic environment which causes cell death in the brain."

In a landmark investment, the federal government granted $2.7 million for childhood dementia research - a collaboration between three major Australian universities.

"The time it takes from the discovery to the clinic varies a lot, so it can take as long as 20 years or it can take as short as 10 months," Aung-Htut said.

It's estimated about 2300 children are currently living with dementia and 75 per cent of them will die before they reach their 18th birthday.

Meg Maack from the Childhood Dementia Initiative said the death rate is comparable to that of kids with cancer.

"The health systems are not meeting those needs. Families have to fight for every scrap of support and care that their child receives and that's just unacceptable," Maack said.

Staska said her children's bravery helps her get up and fight another day.

"I have to be strong for them, so that they can be strong," Staska said.