This rare, incurable brain cancer takes 20 Aussie kids every year

Exclusive: “Go home and make as many memories as you can.”

That’s what the parents of children diagnosed with the rare and incurable brain cancer Diffuse Intrinsic Pontine Glioma (DIPG) are told.

“There’s no fight. There’s no hope. There’s nothing you can do,” Brisbane mum Chloe McDonnell told nine.com.au.

McDonnell’s son Archie was diagnosed with DIPG 18 months ago, after a family member noticed one of his eyes behaving oddly.

He has already outlived his prognosis.

Most children with this tumour die within nine to 12 months of diagnosis.

After the standard 30 rounds of radiation following diagnosis, Archie completed another 12 rounds earlier this year and was part of a clinical drug trial.

But the family have now been told they have no further treatment options.

“You’ve got an 11-year-old and you’re trying to explain to them that they’re going to die, and there’s nothing you can do,” McDonnell said.

“That’s it. There’s no options. There’s absolutely nothing.”

McDonnell said her clever and adventurous boy had a cheeky, sarcastic wit and was obsessed with planes.

He had dreams of becoming a pilot and had been involved with the Australian Air League youth group.

The weekend before he was diagnosed, the fit and active 11-year-old had played his first full game as catcher for the Narangba Demons Baseball Club.

Now having lost most of his mobility, he is most happy playing games on his computer, or spending precious time with his mum, dad David, brother Baxter and his best mate Jake, who McDonnell said has been an incredible friend.

Archie recently told his mum he wants to meet Neil the Seal.

“You just live every day one day at a time, if anybody asks me, I just say we’re plodding along, we just enjoy waking up every morning and enjoying the day,” McDonnell said.

“(But) he’s just not the same kid.

“He can’t walk, although if you told him he was going on a flight simulator, he’d get up some stairs, sure, if there’s anything to do with planes.

“But yeah, he’s lost the use of his left arm... his speech has started to go... as time goes on, Archie will be unable to do anything.”

‘The worst conversation you can have’

DIPG tumours grow in a part of the brainstem called the pons that is responsible for crucial bodily functions like breathing and heart rate.

They cannot be surgically removed, because the cancer cells spread and mix with healthy tissue, and they do not respond to chemotherapy.

Radiation therapy only works to slow the tumours’ growth.

And we don’t know what causes them.

There’s no known environmental cause and only about seven per cent of children diagnosed have been identified to have a genetic predisposition.

Until about 10 years ago, it was considered too dangerous to even attempt a biopsy - meaning until recently, DIPG hadn’t been studied in labs.

“It’s the worst conversation you can have with a parent,” leading childhood cancer researcher Professor David Ziegler told nine.com.au.

Ziegler is the group leader of the Brain Tumours Group at the Children’s Cancer Institute, Chair of Clinical Trials for the Zero Childhood Cancer Program and head of the neuro-oncology program at Sydney Children’s Hospital.

“Most cancers have at least, even if it’s a small per cent, a tiny chance of cure or at least some treatment that that may be effective, but this is really one of the worst, one of the most aggressive.

“It’s one of the only cancers for which there’s no treatment, no chance of cure even from day one.”

In 2011, Ziegler led the team that established the first tumour bank for DIPG in Australia, with tumours donated by parents of children after they had died.

These first specimens allowed the research that has now identified five drugs that are effective at killing DIPG cells and led to the first clinical trial. Another two trials are in development.

According to Ziegler, it has been parents driving the bulk of the funding and awareness.

“Part of the challenge is it’s a rare tumour, it’s not something that most people have heard of, meaning it’s not as well funded as other cancers that we hear a lot about, breast cancer, bowel cancer, et cetera.

“So pharma companies... there’s not a lot of incentive for them to invest in this area.

“In large part it’s been parents who’ve been doing a lot of the fundraising to try and raise the funds to do research, and also generate awareness leading to support from the government as well.

“We are making progress, but we still have a long way to go.”

Reasons for hope

Ziegler said research now showed biopsies of DIPG tumours are safe and can help match a child’s tumour type with the most effective treatment.

“Sometimes these tumours are actually not what people think they are, so they might be a slightly different tumour type and also we find that many of these have genetic changes for which you can actually match them to a drug treatment,” Ziegler said.

“For the first time, we are extending the survival of these children by doing that.”

He said there was also research underway into CAR T-cell therapy, which takes the child’s own white blood cells and genetically engineers them to fight the tumour.

“We’re still not curing all these children, but we’re actually starting to make some ground, starting to shift the goalposts where we can actually have treatments that are starting to do something.”

Ziegler said 50 years ago, childhood leukaemia was also an incurable disease. It now has a 90 per cent cure rate.

“In leukaemia, it’s not just one drug that’s the cure, but multiple drugs used in combination therapy.

“That’s what gives me a lot of hope.

“I feel like we’re seeing the initial responses that they saw when they started using the right treatments for leukaemia 40 years ago, where they started to see some kids going into remission.

“We’re starting to find better treatments that are actually starting to see kids actually responding and having a longer lifespan.”

And like DIPG, it was parents who fought for the funding and investment into leukaemia research.

“It was parents who said ‘we need to make a difference’, so they started doing fundraising and garage sales and gala balls and sausage sizzles and all sorts of things, and that was what raised the money that allowed them to start doing lab research and clinical trials.

“The more investment we have, the more different drugs we can test, the more trials we can open, the faster we’ll be able to learn and and make the same advances.

“I think we really are at a turning point where hopefully we’ll be having less of those terrible conversations and having more positive ones and more hope in the future.”

While Ziegler’s Brain Tumours Group pushes forward with research, family and friends have rallied around Archie to do the only thing they can do, make memories.

There is a GoFundMe fundraiser to help Archie live his dreams in the time he has left.