Addressing the unthinkable – The cruelty of childhood dementia needs cross-sector collaboration

Expanding scope for wider results

An unthinkable condition but one that’s important to hear about and face. The childhood dementia initiative has found that around 2000 kids are living with a form of dementia at any given moment in Australia. That’s 2000 families that are trying to manage parenting and loving their children while navigating a degenerative condition with no cure. Dementia Action Week is an opportunity for all leaders to rally around the scope of, and collaborative action towards, all those dementia impacts, diagnosed and family alike regardless of age. 

145 genetic disorders contribute to childhood dementia

In 2023 a collection of experts across Australia conducted a collaborative study to try and provide answers to the families desperate for answers and understanding. The team of clinicians, medical researchers and other experts were able to, for the first time, define the 145 genetic disorders that directly contribute to childhood dementia.

Dr Nicholas Smith, Head of Paediatric Neurodegenerative Diseases Research Group at the University of Adelaide noted, “This study provides a clearer picture of who is affected by these devastating and under-recognised conditions.”

“The 145 disorders we have classified as childhood dementia are complex and varied. Tragically, what they share in common is the heartbreaking, progressive neurocognitive decline and a severely shortened life expectancy.”

What experts clarified is that through the presence of any of the identified genetic disorders, the condition of childhood dementia was a result of progressive brain damage. They found that, similar to adults with the condition, children showed similar symptoms such as confusion, memory loss, personality changes, significantly disturbed sleep and struggling to concentrate, understand, communicate and learn.

The study highlighted that the dementia symptoms would usually start exhibiting when a patient was around two and a half years old but that diagnosis was typically delayed, with the average age of formal diagnosis being four years old. Megan Maack, the CEO and founder of the Childhood Dementia Initiative shares that many families call the length of time it takes to navigate getting a formal answer as, “a diagnostic odyssey”.

Maack highlights the consecutive bombshells that leave many families reeling, “we went from having what we thought were healthy children, to a child with a minor developmental delay, to this horrific diagnosis of childhood dementia.”

Depth of support needed

Dr Kristina Elvidge, Head of Research at the Childhood Dementia Initiative says, “Childhood dementia is a cruel condition that robs children of the skills that they have only just learned – to speak, play and to recognise their loved ones. This can happen over months, years, or decades until eventually the brain loses the ability to keep the body alive.”

Maack shared with ABC Radio, a particular cruelty of the condition, where her children, “born seemingly healthy, hitting all of their early milestones, it was only around the age of two that we started to notice some developmental delays.”

Elvidge says, “currently treatment options are severely limited, hard to access and research to develop effective treatments and cures is poorly funded. Improvements to care and support for affected families are also desperately needed and we hope this new research will lead to better outcomes for these children.”

Families should not face the stunning, and oftentimes powerfully overwhelming situation of a childhood dementia diagnosis, as Mack describes, “we were facing the slowing of development, the plateau, but ultimately the regression.”

Maack shares openly, “the time from a developmentally delayed child and death is accompanied by some dreadful symptoms for families to manage.”

“Families like mine manage fifteen to twenty medical specialties…as symptoms progress they become increasingly difficult to manage”.

Maack explains, as children lose the ability to communicate frustration and anxiety intensifies, there are, “psychological symptoms, behavioural issues, sleep is interrupted, they stop being able to eat”.

“Every aspect of daily care and daily life requires assistance”.

“It’s unrelenting”.

An urgent collective response

Experts involved in the study have been vocal about the need for cross-sector collaboration to help research, manage and support the families and their ability to provide the best quality of life to their children. Dr Smith implores leaders in government, and his peers across specialties and sectors, to be a part of shaping systemic support to lighten the load for families managing the horror of this degenerative condition.

“The collective burden of childhood dementia is significant, and this new research will raise awareness of the scale of these conditions with researchers, clinicians, government and policymakers both nationally and internationally.”

With the average life expectancy for children diagnosed to be only nine years old, statistics show the need for collective intervention and support early, with 70% of children dying before eighteen.

Leveraging skills from across healthcare and aged care, there is significant opportunity for improvements to occur in the treatment and support of childhood dementia. The nature of the disease means families are seeing the decline of their children after joyous milestones are reached like walking and talking, and managing that care, in heartbreaking proximity.

Maack describes it as, “watching a child disappear before your eyes.”

Cross-sector solutions

Dr Smith shares that while resources have rightfully gone into researching, treatment and care improvement of adult dementia, there is the further need to leverage the learnings from this area into the support and care of children with the condition. He notes, “Whilst there is significant resourcing for adult-onset dementia, similar services do not exist for childhood onset disease, despite their need.”

In the development of clinical models of care, of support at home, of research scope and results, there is opportunity for considerable collaboration. Building up research and programs, to systemically support and provide coping mechanisms for the families, and children with childhood dementia, is desperately warranted.

Maack notes that while progress has been made, “what we have now is this understanding of dementia as a lifespan disease that also impacts children”, she calls for further reform, “but we don’t have a paediatric health system that’s set up in a way that accomodates their needs.” For effective reform to be brought about, it is worthwhile for leaders in aged care, with decades of dementia experience in adults, to be a part of the critical need for change in the treatment of the condition in children, to support peers in the paediatric medical space.

Families, just as much as the children diagnosed, need to be supported in their unthinkable journey. Maack shares that as much as the administrative and medical requirements of care need support, the grief is paramount too.

“I’m grieving the little girl my daughter once was, she is gone… there aren’t specialised psychological supports.”

“Most of the families we deal with have never been referred to psychological help…there are huge gaps in services available to our families”.

During Dementia Action Week, and with Childhood Dementia Day just around the corner on September 20, there has never been a better time for leaders across health and aged care to assess what can be pivoted towards, what can be leveraged to help support the families and children that need the lift of brilliant and heart-felt minds. As a collective there is the opportunity to improve, to care and to ensure that no family ever has to try and cope alone.