“Childhood” and “dementia” are two words we wish we didn’t must use together. But sadly, around 1,400 Australian children and young people live with currently untreatable childhood dementia.
Broadly speaking, childhood dementia is attributable to any one among greater than 100 rare genetic disorders. Although the causes differ from dementia acquired later in life, the progressive nature of the illness is identical.
Half of infants and kids diagnosed with childhood dementia is not going to reach their tenth birthday, and most will die before turning 18.
Yet this devastating condition has lacked awareness, and importantly, the research attention needed to work towards treatments and a cure.
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More concerning the causes
Most kinds of childhood dementia are caused by mutations (or mistakes) in our DNA. These mistakes result in a spread of rare genetic disorders, which in turn cause childhood dementia.
Two-thirds of childhood dementia disorders are attributable to “inborn errors of metabolism”. This means the metabolic pathways involved within the breakdown of carbohydrates, lipids, fatty acids and proteins within the body fail.
As a result, nerve pathways fail to operate, neurons (nerve cells that send messages across the body) die, and progressive cognitive decline occurs.
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What happens to children with childhood dementia?
Most children initially appear unaffected. But after a period of apparently normal development, children with childhood dementia progressively lose all previously acquired skills and skills, resembling talking, walking, learning, remembering and reasoning.
Childhood dementia also results in significant changes in behaviour, resembling aggression and hyperactivity. Severe sleep disturbance is common and vision and hearing may also be affected. Many children have seizures.
The age when symptoms start can vary, depending partly on the actual genetic disorder causing the dementia, but the typical is around two years old. The symptoms are attributable to significant, progressive brain damage.
Are there any treatments available?
Childhood dementia treatments currently under evaluation or approved are for a really limited variety of disorders, and are only available in some parts of the world. These include gene substitute, gene-modified cell therapy and protein or enzyme substitute therapy. Enzyme substitute therapy is on the market in Australia for one type of childhood dementia. These therapies try to “fix” the issues causing the disease, and have shown promising results.
Other experimental therapies include ones that goal faulty protein production or reduce inflammation within the brain.
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Research attention is lacking
Death rates for Australian children with cancer nearly halved between 1997 and 2017 because of research that has enabled the event of multiple treatments. But over recent a long time, nothing has modified for kids with dementia.
In 2017–2023, research for childhood cancer received over 4 times more funding per patient in comparison with funding for childhood dementia. This is despite childhood dementia causing a similar variety of deaths every year as childhood cancer.
The success for childhood cancer victims in recent a long time demonstrates how adequately funding medical research can result in improvements in patient outcomes.
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Another bottleneck for childhood dementia patients in Australia is the dearth of access to clinical trials. An evaluation published in March this yr showed that in December 2023, only two clinical trials were recruiting patients with childhood dementia in Australia.
Worldwide nevertheless, 54 trials were recruiting, meaning Australian patients and their families are left watching patients in other parts of the world receive potentially lifesaving treatments, with no recourse themselves.
That said, we’ve seen a slowing within the establishment of clinical trials for childhood dementia internationally lately.
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In addition, we all know from consultation with families that current care and support systems aren’t meeting the needs of youngsters with dementia and their families.
New research
Recently, we were awarded latest funding for our research on childhood dementia. This will help us proceed and expand studies that seek to develop lifesaving treatments.
More broadly, we’d like to see increased funding in Australia and world wide for research to develop and translate treatments for the broad spectrum of childhood dementia conditions.
Dr Kristina Elvidge, head of research on the Childhood Dementia Initiativeand Megan Maack, director and CEO, contributed to this text.
