“Childhood” and “dementia” are two words we’d moderately not use together. But unfortunately in the realm 1,400 Australian children and young people they live with currently incurable childhood dementia.

Generally, childhood dementia is attributable to any of those greater than 100 rare genetic diseases. Although the causes differ from dementia acquired later in life, the progressive nature of the disease is the identical.

Half infants and youngsters diagnosed with childhood dementia won’t live past their tenth birthday, and most will die before the age of 18.

However, this devastating disease has lacked the attention and, importantly, the research attention needed to develop treatments and cures.

More in regards to the reasons

Most forms of childhood dementia are so-called caused By mutations (or errors) in ours GOUT. These errors result in quite a lot of rare genetic diseases that in turn cause childhood dementia.

Two thirds dementia in children is attributable to “inborn errors of metabolism“. This signifies that the metabolic pathways involved in breaking down carbohydrates, lipids, fatty acids and proteins within the body usually are not working.

As a result, neural pathways stop working, neurons (nerve cells that send messages to the body) die, and cognitive function progressively declines.

Childhood dementia is related to rare genetic diseases.
Maxim Ibragimov/Shutterstock

What happens to children with childhood dementia?

Most babies initially appear unaffected. But after a period of apparently normal development, children suffer from childhood dementia progressively lose all previously acquired skills and skills, comparable to talking, walking, learning, remembering and reasoning.

Childhood dementia also results in significant behavioral changes comparable to aggression and hyperactivity. Serious sleep disturbances are common, and vision and hearing disturbances may additionally occur. Many children have seizures.

The age at which symptoms begin can vary, partly depending on the precise genetic disorder causing dementia, but the typical is around two years. The symptoms are attributable to significant, progressive brain damage.

Are there any treatments available?

Currently treating childhood dementia under evaluation or approved are for a really limited variety of conditions and are only available in certain parts of the world. These include: gene exchange, genetically modified cell therapy and protein or enzyme substitute therapy. Enzyme substitute therapy is available in Australia one type of childhood dementia. These therapies aim to “fix” the issues that cause the disease and have shown promising results.

Other experimental therapies include people who objective defective protein production or reduce inflammation within the brain.

There is a scarcity of research attention

Death rates of Australian children with cancer almost by half between 1997 and 2017 because of research that has enabled the event of many therapies. But in recent a long time nothing has modified for kids with dementia.

Between 2017 and 2023, pediatric cancer research received greater than 4 times the funding per patient in comparison with research funding childhood dementia. This is despite childhood dementia causing: similar variety of deaths every year as a childhood cancer.

Success for childhood cancer patients in recent a long time shows how adequate funding of medical research can result in improved patient outcomes.

Dementia is not only a disease of older people.
Miljan Zivkovic/Shutterstock

Another bottleneck for pediatric dementia patients in Australia is the shortage of access to clinical trials. Some evaluation published in March this yr found that as of December 2023, only two clinical trials in Australia were recruiting patients with childhood dementia.

However, 54 trials have been recruiting globally, meaning Australian patients and their families must watch as patients in other parts of the world receive potentially life-saving treatments without the choice of self-treatment.

That said, we have now seen a slowdown in creation Clinical tests lately worldwide within the treatment of childhood dementia.

Moreover, we all know from consultations with families current care and support systems they don’t meet needs children with dementia and their families.

New research

We were recently awarded new financing Down our research for childhood dementia. This will help us proceed and expand research to develop life-saving therapies.

More broadly, we’d like increased funding in Australia and world wide for research to develop and translate treatments for the big selection of conditions related to childhood dementia.

This article was originally published on : theconversation.com

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