'My twin boys are inseparable, but I’m watching one of them deteriorate before my eyes.'

"I want to go home, Mummy," says eight-year-old Jobe. He's distressed and overwhelmed. "Take me home," he pleads.

They're heart-breaking words for his mother, Tenille Koistenin. Because Jobe is at home, it's just that sometimes he doesn't recognise it anymore. 

"Even the safe surroundings he's used to are starting to confuse him," shares Tenille. 

"He's starting to forget the names of the people around him. He feels uncomfortable in new surroundings… he doesn't understand the explanations we give anymore."

These days, Tenille's main goal is to give Jobe the best possible quality of life. It's about making memories, enjoying life's little glimmers when they come along. It's about prolonging the bond between Jobe and his twin brother, Tate, for as long as possible. 

Watch: The Childhood Dementia Initiative. Article continues after the video.

Jobe has Sanfilippo syndrome, one of about 140 diseases that fall under the umbrella of Childhood Dementia. 

Born at just 29 weeks, Jobe and Tate started their lives together in the Neonatal Intensive Care Unit. Although Jobe required some additional medical treatment in his first year, both boys hit their important developmental milestones. 

"Being a twin you get to see them hitting their milestones together in real time, so you do have a comparison," says Tenille. "You tend to keep a close eye on who's doing what and how they're progressing and any differences."

When Jobe was about 18 months old, Tenille noticed his fontanel – the soft spot on a baby's head – hadn't closed yet. When he was two, measurements revealed his head had grown more quickly than expected. 

Jobe was diagnosed with Sanfilippo Syndrome, a type of childhood dementia. Image: supplied.

"That prompted us to get an MRI, and that showed he had some enlarged spaces in his brain, which prompted further tests.

Just after his second birthday, the results came in. Jobe was diagnosed with Sanfilippo Syndrome, a type of childhood dementia. 

Before the diagnosis, Tenille Googled the condition having seen it listed on the pathology forms. 

"Honestly this was the worst outcome that I thought we would be searching for and I didn't think this would be us. 

"Then when we were diagnosed, I knew this was a progressive brain disease, there is no cure, it's fatal, and affects all aspects of the central nervous system."

"An overwhelming grief."

When Tenille gave birth to twins, she'd felt blessed that her two children would have such an incredible bond. Discovering how different Jobe and Tate's paths will eventually become was heartbreaking. 

"They're supposed to go through life side by side… go to school, get their license together."

For the next 12 months, Jobe remained developmentally typical, alongside Tate. But at three, he began to plateau. 

"That's when we started noticing the difference. Tate was starting to count and talk more and Jobe just sat cognitively at the age of three."

Now, Jobe's family lives with constant anticipatory grief, with the knowledge that Jobe will continue to regress. 

"They are about to turn nine, and the scary thing is … only 50 per cent of kids with childhood dementia live until their 10th birthday," says Tenille. 

"Every year you're kind of celebrating what you still have, but then you're grieving the fact you're seeing that terminal figure come closer."

Jobe has a twin brother, Tate, who he adores. Image: supplied.

These days, Tate plays more of a big brother role than a twin, with Jobe's cognitive abilities closer to that of a three year old. 

"They don't enjoy the same things, or play the same games. But the way he interacts with Jobe is with such compassion and patience."

As Jobe gets older, his parents are noticing an increase in his confusion, and a more rapid progression of the disease. 

"He is quite dysregulated. He doesn't like change, he needs routine. He is starting to forget the names of the people around him. 

"It's hard on Tate too because he doesn't know the exact reality of what this looks like for Jobe."

Tenille recently moved her family closer to her mum, to provide their family with some additional support. Tragically, Tenille's mum has now been diagnosed with early onset dementia, at just 69.

"I've got a child with dementia, and I've got a mother with dementia, and it's so heartbreaking that in Australia this is where we're sitting at the moment."

Tenille has stopped working now, and dedicates her time to making memories and being in the moment with her family. 

"We try to do as many of those small glimmer moments as we can as a family. We can always work more later, we can always work longer once this moment has passed."

What is childhood dementia?

Essentially, childhood dementia is a progressive brain disease. There are about 145 conditions that cause dementia in childhood, all of which are genetic in origin. 

"Children are born seemingly healthy and most kids will follow a normal development trajectory for a while," says Childhood Dementia CEO, Megan Maack.

"But their development will slow and plateau, and then decline and regress."

Presentation of symptoms varies, with some presenting as babies, while others don't present until their teenagers. The most common age for a child to be diagnosed is between two and three.

"Symptoms are very similar to those experienced by adults with dementia," Megan says. 

"Cognitive decline, confusion, memory loss, hallucination, behavioural changes, sleep disturbance. It might look different in a child's body, but it's the same process of decline."

Unfortunately, all children who have one of these conditions currently face a very limited life – 50 per cent will die before the age of ten. 

"It's a dreadful prognosis and unfortunately what we're seeing is that during those short lives, the quality of life is not great."

Jobe's mum, Tenille, is now focused on making memories with her family. Image: supplied.

Devastatingly, one in five families will have more than one child with childhood dementia, but diagnosis can be slow and traumatic. 

"Families wouldn't immediately think about dementia as a potential diagnosis and neither do health professionals unfortunately," Megan says. 

"A lot of the initial symptoms don't ring alarm bells. For example, a delay in development can be quite common in little children. Most times it's not dementia - people might assume it's autism or ADHD. Their parenting might even be questioned."

Megan is trying to bring the issue of childhood dementia into the light, after decades of neglect that has left families desperate and helpless. 

"We haven't seen an improvement in survivorship ever," says Megan.

"When we look at Cystic Fibrosis or childhood cancers, we've seen over the last couple of decades a beautiful improvement in survivorship and treatment, not only to prolong children's life but to manage the condition. 

"During their short lives, children are not getting adequate health care. There aren't any standards, there isn't sufficient expertise within the health symptoms. Where you live, who your doctor is, will dictate your treatment.  The type of care that the child gets depends on the family's ability to advocate which is really inequitable."

As a result, families are completely decimated when they receive one of these diagnoses.

"The emotional and financial strain is crushing on these families. Most parents change their work status. One in three give up their careers entirely to care for children. Marital breakdown is really significant among this community as well. 

"The level of anxiety and stress that exists in these families is huge, and they are reporting there isn't psychological support, no specialist services available to help them navigate through. 

"This is a cohort that has the highest level of unmet needs in the paediatric system today, and has been invisible across health, across policy and society in general."

Children with childhood dementia suffer terribly – their lives are cut short, they suffer physical and emotional pain, and they lose their ability to engage, play, talk and dance. 

"Until you've watched a child go through an episode it's really difficult to explain the level of true suffering these children have. It's something we can't continue to look away from, we can't continue to ignore, we have to give these children a better quality of life."

The future for Jobe, and kids like him.

Jobe has recently started at a special school, but the support Tenille receives is minimal. 

"The medical professionals don't understand the diversity of the issues that we're dealing with. It is quite complex, and because it involves every aspect of their day to day wellbeing, there's so many different elements of what that care looks like to keep him happy and safe."

Tenille says a coordinated approach is required in terms of medical awareness, research and strategies around what care plans look like. 

"The lack of research progress doesn't give very much hope. But we need that hope and we need that action in research. I want to see those numbers of survival increase. Even if it's not in Jobe's lifetime, I want it to be in the next generation of children's lifetime."

In the meantime, Tenille is focused on making memories, whatever they look like. 

"We don't sweat the small stuff anymore. We want to see the day through Jobe's eyes and the innocence that he portrays," says Tenille. 

"Seeing the love he has for his brother, seeing him laugh, running around and just taking in each moment. 

"He is teaching us so much. Everyone that meets him learns something from Jobe, and it changes their life in some way."

Feature image: supplied.