• Lia Stoll

The Alarming Truth About Childhood Dementia (and What You Can Do About It)

Portrait of a girl
The Alarming Truth About Childhood Dementia (And What You Can Do About It)

Maybe it’s happened to you.

You drink your coffee in the morning and watch your beautiful daughter eating breakfast.

You admire her, and laugh at the tower she’s created out of blueberry pancakes.

If you could stop time, you would make this heartwarming moment last forever.

Instead, you notice she seems tired. Her eyelids flicker, and she drops her fork as she falls over.

Sadly, you witnessed your first ever seizure.

A form of epilepsy.

And the possibility that your child could be diagnosed with Childhood Dementia.

But, what’s going on here?

Doesn’t dementia usually strike the elderly?

The simple truth is, no.

And, the worst part?

All forms of dementia are fatal.

Let me guess.

By now, you’re wondering, what is Childhood Dementia? Why is it fatal? And, can I do something about it?

Grab a cup of coffee, and let’s take a closer look.

The childhood illness that exists but nobody knows about

There’s no doubt about it.

Making a diagnosis for a genetic or rare disease is challenging and frightening.

And, childhood dementia is scary.

Like a lurking thief, it robs children of their memory. It strips away their emotional, mental, and physical control. It leaves them lost in a world of their own, a place where parents can no longer reach them.

How does this happen?

Let me explain.

It begins with a group of rare genetic disorders called neuronal ceroid lipofuscinoses (NCLs). They cause severe neurodegeneration in the central nervous system and an increase in ageing pigments in your neurons.

These genetic disorders are found guilty for over 70 types of childhood dementia. The most common are Juvenile Batten disease and Sanfilippo disorder.

According to the Childhood Dementia Initiative, these complex disorders are crippling and progressive. Less than 5 per cent of them have effective treatments.

And, none have a cure.

The result?

Children die before reaching adulthood.

A disturbing thought, isn’t it?

And here’s the part that’s tough to swallow.

Because it’s rare, there is a lack of information and awareness leading to late or improper diagnosis and delayed treatment.

What’s worse?

The disease is more common than you think.

In Australia, a baby is born every 3 days with a genetic condition that leads to childhood dementia, and every year more than 90 children die before they reach the age of 18.

In the UK it occurs in 5–6 births every year.

Yet, in a recent survey by YouGov, only 4% of MPs said they were familiar with childhood dementia as a disease.

On a global scale, 700,000 children and young people suffer from the disorder.

I know you’ll agree with me when I say, this is huge.

So, I guess you’re wondering, what happens to children born with the disorder?

The 4 principal types of childhood dementia

In her article, ‘Childhood Dementia Causes’, Dr. Sanchari Sinha Dutta explains how depending on the age of onset, the neuronal ceroid lipofuscinoses (NCLs) are subdivided into 4 types:

So, stick with me here.

First of all, there’s congenital — it begins before or around birth, and symptoms are seizures and microcephaly, (meaning small head in Greek).

Then, there’s Infantile — this begins at 6 – 24 months, the typical symptoms are deterioration of cognitive and motor abilities, vision loss, and seizures.

Late-infantile — occurs in children aged 2 – 7.5 years, and the symptoms include cognitive and motor abilities, vision loss, and seizures.

Finally, there’s Juvenile— the typical age is 4 – 10 years, and clinical symptoms are cognitive and motor abilities, vision loss, and seizures.

And here’s the really disturbing part.

As childhood dementia advances children find it hard to recognise their family.


So, what happens next?

The long and hard road for medicine and hope

It’s the question you secretly dread.

Is there any hope?

The truth is, despite identification of the disturbed genes, how their deficits lead to disease pathology isn’t entirely understood.

As if that’s not enough, early diagnosis of childhood dementia is problematic.

Why is this so?

For one thing, early symptoms aren’t specific, and they are related to other neurological disorders.

For another, there aren’t any truly effective treatments to stop the progress of dementia.

Want to know the best part?

Let me tell you a little story.

Carla and Dave are excited about having a family and decide to talk to their GP about it.

They want to know how to give their future baby the best start in life.

Their GP recommends eating a balanced diet, regular exercise, and the classic, folic acid.

There’s no discussion about potential genetic risk. None.

And, like most enthusiastic, first-time parents, Carla and Dave don’t have nightmares about genetic disorders crushing their lives.

Carla is now pregnant and the regular trimester tests begin, making her feel safe.

And baby Dana? She sails through her tests with no problem.

So, Carla and Dave go home, clueless about being tested for a sprinkling of genetic conditions diagnosable.

And, that’s only half the story.

Now, baby Dana is born and Carla and Dave are enchanted. Life is perfect.

At 6 weeks Dana’s regular health check announces she is a healthy baby.

What Carla and Dave don’t understand is that newborn screening covers only half a per cent of diagnosable genetic conditions.

What Carla and Dave don’t know is, to be included in the newborn screening panel a disorder must have an approved treatment.

Well, I can give you my thoughts but…they won’t be pretty.

As Dana grows, Carla begins to notice symptoms; like the size and shape of her head, her enlarged spleen, chronic ear, and nose infections.

Regular screen tests are carried out but show nothing abnormal.

The GP finds it odd but thinks nothing more of it.

At 22 months, Carla begins to report lagging cognitive skills and troubling behaviours in Dana. Her instincts now screaming — something is wrong.

Yet, health professionals continue to dismiss her, telling her every child is unique and developmental stages vary.

The trouble is, isolate these symptoms and they don’t raise suspicion, but add them up and warning bells start ringing.

By this time, Dana is labelled as a neurotic mother creating problems where there are none.

Far fetched? Not at all.

Just short of her 4th birthday, and after extensive and expensive tests, baby Dana is diagnosed with Sanfilippo syndrome.

For the first time it dawns on you; in an empathetic world, future parents would be offered genetic screening tests.


Now, since childhood dementia is irreversible, delayed diagnosis is dangerous, leading to premature death.

And the worst part?

Dana is missing out on the life-changing and life-saving opportunities that genomics can offer.

Because, even though there’s no cure for childhood dementia yet, there is hope in gene therapy.

The emotional wrecking ball demolishing your life and how to contain it

Just imagine stepping into your doctor’s office, and she begins to explain what your child is experiencing.

Childhood Dementia.

Two words you will never forget.

Like carrying stones on your shoulders the emotional strain is heavy.

You feel a crushing sense of sadness. You’re left with anxiety, guilt, and unrest.

That’s when you know you have to take care of yourself.

So, let’s try to focus on lightening the load, shall we?

Dr Alessandro Simonati; a child neurologist and head of the clinical service of neurodegenerative disorders of childhood at the University of Verona, says. ‘The quality of complex care these children require is 24-hours-a-day.’

What does this mean?

It means you can’t face this alone.

It means you don’t need to face this alone.

It means you can reach out for help.

One way to do this is by creating check-ins.

Ask family and friends to visit on a consistent and regular basis.

Let them ask you how you are feeling.

Let them take you for a walk.

Let them show you, you are not alone.

Brain research shows when releasing emotions our logical brain, functions better.

One of my favourites: Find a Listening Partner, from Hand in Hand Parenting.

It’s the priceless skill of having an adult listening partner as a way to release feelings getting in the way of being present and meeting your needs.

This means you will be more prepared to manage stress and find solutions.

To top it off, if you’re like most people, your marriage can begin to feel like a rubber band; stretch it too far and you’ll find yourself in emotional chaos and break down.

So, what can you do?

You’ve probably heard the advice a million times; try to take time to nurture your relationship.

This can be anything from sneaking in a quick cup of coffee together, to holding each other’s hands as you watch your kids play.

And, it doesn’t stop there.

For example, one family fighting Battens disease created a website, Olie’s Army saying, “This is our fight to spread the word and ensure Ollie and Amelia’s voices are heard in a hope to save children’s lives in the future.”

Last but not least, there are life-changing organizations like Childhood Dementia Initiatiative and campaigns like Dementia Strikes Children Too — you can reach out.

Ok. I know what you’re thinking.

It sounds simple. But, it isn’t.

I know.

That’s why it’s important.

It’s important to remember there is hope, and — help.

The answer to your unfathomable challenge

There’s no denying it.

One moment your heart feels like exploding from the short-lived joy of a giggle or a hug, and the next you’re so sad, you find it hard to live a day longer.

But just remember.

There is hope, and — help.

So take the next step, and— reach out.

Life continues in a way.

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