'We'll watch our child waste away': The forgotten children living with a death sentence

Each year in Australia, as many children will die from dementia as die from cancer. So why is it that childhood dementia remains relatively unknown? And what measures are in place to support these overlooked children and their grieving families?

Sarah Matthews and her young daughter Charlotte, who has childhood dementia. Source: Supplied

Key Points

• Childhood dementia is a "devastating and hopeless" diagnosis because there is no cure, says the Childhood Dementia Initiative (CDI).
• Sarah Matthews knows she will have to watch her three-year-old daughter Charlotte "waste away".
• The CDI is calling on the government for funding to enhance research into treatments and better support families.

Sarah Matthews, 39, knows she will outlive her daughter Charlotte. And before that, she'll be forced to watch her child lose the ability to walk, talk and swallow.

The now-three-year-old was diagnosed with an extremely rare genetic condition called Cockayne Syndrome when she was 16 months old. The condition causes her brain to degenerate.

Charlotte has childhood dementia.

Sarah and her partner Brett first became concerned when their daughter wasn't growing at the same rate as other babies of her age, and had difficulties swallowing milk.

The diagnosis of the generative condition, which spared their five-year-old son Finn, and the subsequent diagnosis of dementia have been devastating for the young family.

"It's been hugely emotionally taxing. There's a lot of grief in all of this that we experience daily," Sarah told SBS News.

But even worse for Sarah and Brett is facing what inevitably will come.

"Charlotte essentially is going to have a much shorter and harder life than most children. She probably has around 10 to 20 years. We are most certainly up against something that we can't ultimately change all that much."

Three-year-old daughter Charlotte Matthews will likely only live for 10 to 20 years. Source: Supplied

"It's a feeling of anticipatory grief, that something is coming and something is coming soon.

"It's really hard to manage that on a day-to-day basis, knowing that we are essentially going to have to watch our child waste away."

Calls for help

Megan Maack, CEO of Childhood Dementia Initiative (CDI), describes a diagnosis of childhood dementia as "devastating and hopeless".

"It's hopeless for the families, hopeless for the doctors, hopeless for everybody who's supporting these affected families," she said.

This heartbreaking reality for many families is highlighted in a report by the CDI presented at Parliament House on Monday, which has the aim of saving lives and improving the quality of life for children living with dementia.

The State of Childhood Dementia in Australia 2024 report calls for urgent action to improve research into treatments that can improve and extend lives, as well as better support for families.

The report was informed by government-funded research in 2022 into the needs and barriers to healthcare and support for children with dementia and their families in Australia.

"Australia is the first country to recognise and respond to this problem in a coordinated way," Maack said.

"We have the potential to lead the world in progress for children with dementia."

On Monday Health Minister Mark Butler said the government would deliver $750,000 in funding to the CDI to support children and families affected by childhood dementia.

"We are making it easier for families to get a diagnosis of this rare condition by delivering continued healthcare professional education for more timely and appropriate care," Butler told SBS News.

"This funding will also develop the care and support solutions that will enhance the quality of life for the children impacted by this neurocognitive decline condition."

What is childhood dementia and how common is it?

Childhood dementia results from progressive brain damage that can be caused by more than 100 genetic conditions.

Around one in every 2,900 babies are born with a condition that causes childhood dementia, which means a baby is born with a childhood dementia disorder every few days in Australia.

"Childhood dementia is more common than you would expect and it is shocking that most people haven't heard of it," Maack said.

A graphic card showing childhood dementia in numbers. Source: SBS News

The reason for this, she says, is that each condition causing childhood dementia has historically been considered in isolation as a rare disease.

"But there is a huge commonality in how the symptoms across all of the conditions present, and the needs of both the child and their entire family are consistent.

"Now we want to articulate these common needs so we can address them."

A death sentence

Maack explains that a child with dementia typically develops normally initially before showing symptoms, often around the age of two or three.

"The early signs are typically developmental delays, which don't ring alarm bells. However, as the disease progresses, the children suffer quite significant symptoms due to progressive brain damage."

Similar to adults with dementia, these symptoms can include memory loss; confusion; difficulty concentrating, understanding, learning, and communicating; personality changes; severely disturbed sleep; behavioural issues like hyperactivity; and emotional issues such as anxiety and fear.

Childhood dementia results from progressive brain damage caused by a range of more than 100 genetic conditions. Source: Getty / Catherine Falls Commercial

As the disease advances, children lose abilities such as speaking, walking and even hearing or sight, often experiencing debilitating seizures. This relentless decline, coupled with chronic pain and distress, makes childhood dementia uniquely cruel.

"In my practice, I see firsthand how childhood dementia is different from other chronic childhood diseases," explained Professor Michelle Farrar, a child neurologist and clinical academic at UNSW Sydney.

"Childhood dementia is an ever-changing condition, with new and more severe symptoms constantly emerging on a scale much greater than any other condition.

"The connection with the healthcare system and support must be exceptionally strong and responsive in ways that aren't required for other conditions."

Childhood dementia is an ever-changing condition, with new and more severe symptoms constantly emerging.

Professor Michelle Farrar, a child neurologist and clinical academic at UNSW Sydney.

There is no treatment or cure for childhood dementia, and the prognosis is dire: 50 per cent of children will die before the age of 10, and 70 per cent will not reach their 18th birthday.

All will die prematurely.

Neuroscientist Professor Peter Schofield AO says this isn't good enough.

"Survival rates for children with cancer have risen to 84 per cent and there is concerted action to achieve 100 per cent," he said.

"Childhood dementia, in comparison, sits at zero per cent survival and children are suffering and dying. There's a strong moral imperative here for immediate action."

Lack of funding and support

Because it can take a few years for children with dementia to decline, they are often "out of mind and out of sight", Maack says, leading to a lack of necessary support for terminally ill children and their families.

Many families experience prolonged grief disorder after losing a child and high levels of pre-loss grief while caring for a child with dementia.

As childhood dementia progresses, children lose abilities like speaking, walking, and even hearing or sight. Source: Getty / chameleonseye

"These families live with these diseases for many years, and they aren't part of the hospital system. They're fighting for everything they need and feel lost in the community. We aim to establish standards and consistency in how all these conditions are managed," she said.

Research into treatment faces significant obstacles due to a lack of funding, infrastructure and clinical trial capacity. Fewer than 2 per cent of children with dementia in Australia can join a potentially life-saving clinical trial.

Maack would like to see ongoing investment to enhance research into treatments and to create a national centre of expertise that provides consistent standards of care for families regardless of location, and ensures children with dementia have the opportunity to access therapeutics through clinical trials.

"We know treatments are in the development pipeline, and we want to provide access to these treatments as quickly as possible," she said.

"There has been no improvement in survivorship over time for children with dementia, unlike other chronic conditions where we've seen increased survivorship and improved quality of life. We need the same progress for children with dementia."

The need to hope

Sarah and Brett are hopeful for any medical interventions that may improve Charlotte's dire prognosis.

"At this point in time, you're essentially told your child has this condition, it will worsen, and there's nothing that you can do about it in the meantime," Sarah said.

"As parents, it's incredibly difficult to accept that you can't improve your child's life."

They're pinning their hopes on increased funding into treatments that may improve their daughter's quality of life and perhaps extend it a little bit.

"Right now, we're simply hoping that in the coming years, something will come out of the woodwork."