Ruby was just six when she died. Her parents donated her brain tumour to a paediatric cancer research laboratory, hoping her short life might help others.
The children of Everton Park State School are gathering, hundreds of them.
Two by two they march, some of them holding hands, in honour of one of their own — six-year-old Ruby Pringle, who has changed the school, in Brisbane's north, forever.
Today, dressed in the colours of the rainbow, they are celebrating Ruby's short life and cementing her legacy.
Their tiny bodies spell out the words "be kind" on the sports oval.
Kindness has been added to the school's values — alongside responsibility, respect, care, collaboration and achievement — in memory of little Ruby.
The Year One student died on March 1, less than six months after being diagnosed with the cruellest of diseases — a type of brain tumour, known as a diffuse intrinsic pontine glioma, or DIPG, the deadliest childhood cancer.
Treatment protocols for the cancer — which is 100 per cent fatal and affects girls and boys equally — have not changed since before man walked on the moon in 1969.
Life expectancy is just nine to 11 months after diagnosis.
And yet, Ruby continued to attend school until just days before she died. Her parents, Hannah and Andrew Pringle, were keen for her to keep doing the things she loved.
When she was well, her principal Aminta Miller recalls Ruby running around her in circles, shouting her name and telling her what she loved learning about at school.
"She was a beautiful young person," Aminta says.
"When we heard about Ruby, it really made us reflect on what's important to us. We have five values at our school, but we really felt like kindness was missing.
"Ruby was very kind. Her passing has made us all think about what kind of people we'd like to be. It's always important to be kind, because you never know what someone else is going through."
The little girl with sun-kissed wavy long hair was much like others her age — her favourite colour was pink, she loved going on bike rides to the local soccer club with her dad and she had a penchant for giraffes.
Everything changed
However, Ruby's life would never be the same after a morning walk with her family on a Sunshine Coast beach last spring.
It was September 17, just 11 days after her sixth birthday.
The Pringles were visiting Ruby's paternal grandparents, Chris and Ray, at Kawana.
As she walked in the soft sand, Ruby struggled to keep her balance, frequently falling over, unable to walk in a straight line.
"She couldn't walk in the sand. At all," Hannah recalls, the grief etched on her face.
"Every step she took, she fell."
Her dad, Andrew, a landscape gardener, thought Ruby might be dehydrated.
His wife, a children's nurse, later told doctors at the Sunshine Coast University Hospital she wondered whether her daughter was "mucking around".
It was wishful thinking.
When doctors took a box of tissues into a room and invited Hannah inside to discuss Ruby's brain scan, the mum of two daughters, including three-year-old Tilly, knew the news was bad.
"I just screamed," she recalled of being told Ruby had a brain tumour.
Within hours, Ruby and her mum were in an ambulance being transferred from the Sunshine Coast to the Queensland Children's Hospital (QCH) at South Brisbane. Andrew followed later with Tilly in the family car.
At the time, Hannah had worked at the QCH for nine years. She was used to looking after sick children.
However, nothing could have prepared her for that ambulance journey with her daughter, knowing her six-year-old firstborn had a mass inside her head.
She was so distraught when she arrived, she had to be taken into the hospital in a wheelchair.
"The whole time I was having a panic attack," Hannah says.
"As we were getting to the children's hospital, I was getting worse.
"Ruby was wondering what was wrong with me because I couldn't walk or breathe or talk."
On Sunday, September 18, Ruby's parents were told their daughter's tumour was unsafe to operate on and it was DIPG, an aggressive tumour of the brain stem that no child survives.
About 20 Australian children are diagnosed with the cancer every year.
Ruby's diagnosis could not have been more dire.
DIPG tumours form in the pons, a part of the brain stem that links the brain to the spinal cord.
"The pons controls every single bit of your body: your heart rate, your breathing, your movement. Everything. Swallowing, talking," Hannah explained.
The prognosis was bleak.
Go. Make memories, one doctor advised.
The standard therapy is palliative radiation.
It's a protocol that hasn't changed since the early 1960s, when Karen Armstrong, the daughter of astronaut Neil Armstrong, was diagnosed with DIPG as a toddler.
Karen, nicknamed Muffie, died before her third birthday, about seven years before Armstrong made history as the first person to walk on the moon in July 1969.
Monday to Friday for six weeks, Ruby received radiotherapy.
Shut off, alone, inside a hospital room for minutes at a time, she was pinned to a table by a mask over her head.
The prospect is daunting for most adults.
However, for the most part, Ruby faced her treatment with bravery, and even a sense of humour.
A video shows Ruby emerging happily from one radiotherapy session, declaring with excitement: "Peanut butter and jelly time," as health workers removed her mask.
"Even through all this she was the best little girl," Andrew says, proudly.
"She never complained about anything."
While radiation usually extends survival by two or three months in children with DIPG, it failed to work in Ruby's case.
Her parents, however, refused to be paralysed by her diagnosis, doing everything they could to make happy memories with Ruby.
"I went a bit overboard with the memory-making," Hannah says laughing through her tears.
Disneyland was out of the question, but the family has cherished photos of Ruby and her best friend, Charlie Cushion, on a trip to Cairns with their parents and siblings.
Ruby and her family got to stroke a dolphin.
She fed a giraffe from the balcony of a tree house during a sleepover at a Canberra zoo, and she took a ride in a helicopter.
Hannah even organised a prom night for Ruby at their home.
They had their makeup done together and Ruby walked a red carpet in a white ballet dress, wearing a tiara in her hair.
At Christmas, when Ruby was too weak to walk around the neighbourhood to see the Christmas lights, her dad created their own festive display at home, complete with an artificial snow machine.
Ruby delighted in surprising passers-by with snow showers.
Throughout much of her final five months, Ruby's best friend Charlie was by her side during the good and the bad, giving her hugs throughout.
The pair had been almost inseparable since they were babies.
Their friendship remained strong as Ruby's appearance changed and she could no longer walk by herself, run, jump and play as she once had.
Early in Ruby's illness, a photo shows them both smiling, surrounded by bubbles, glistening in the sunshine.
Videos capture the joy of just being little girls together: playing games, splashing around in a pool and jumping on a trampoline like six-year-olds everywhere.
The "bestie" Ruby nicknamed Cha Cha was frequently by her side during visits to the Queensland Children's Hospital, both enjoying musical therapy sessions together.
Charlie was also there to console her friend after some of her radiotherapy sessions.
Cherished photos of the family's adventures feature inside a cabinet dedicated to Ruby at the Pringles' Everton Park home.
There's a framed painting of a giraffe drawn by Ruby after their trip to Canberra.
It's special because it's Ruby's, but even more so because she did it with her left hand after the DIPG severely weakened the right side of her body.
Inside the cabinet is one tiny half of a best friend necklace, the other given to Charlie in memory of a friendship that could not be broken by cancer.
However, amid the best of humanity, the Pringles also experienced some of the worst at the hardest time of their lives.
Hannah recalls being abused by a man when she parked in a disabled car park to cut the distance she had to push Ruby in Tilly's pram.
"The tumour was affecting her movement … I wasn't able to get a wheelchair," Hannah explains.
"He got angry at me for parking in a disabled car park. I had to say to him how much he upset me because my child is dying, but from the outside, she doesn't look like she's dying.
"Ruby had beautiful long hair down to her bottom. She didn't lose her hair because she wasn't able to have chemo."
Dozens of framed sketches of children who have died with DIPG hang on the walls of a paediatric cancer research laboratory at the University of Newcastle, about 750km south of Brisbane. A picture of Ruby will soon join them.
'Hard to call a cancer rare when you're so close to it'
Laboratory head, Professor Matthew Dun, lost his own daughter, Josephine, to DIPG just before Christmas in 2019. Josie was four years old.
Matt's research originally targeted blood cancers. He expanded his focus when his eldest child was diagnosed with DIPG in February 2018, aged just two.
Despite his cancer research background, and his wife Phoebe's work as a general practitioner, neither had heard of DIPG before Josie's diagnosis.
However, like the Pringles, he rejects the term "rare" when talking about DIPG.
"It's hard to call a cancer rare when you're so close to it," he says.
"DIPG is responsible for more than one-fifth of all childhood deaths from cancer, so DIPG is not rare in terms of its effect on patients and their families.
"I struggle with the term rare, because I speak to families [affected by DIPG] every day of my life. If it was so rare, I wouldn't have these conversations every day."
DIPG took the life of his "best little mate", the daughter who shared his love of Cat Stevens and Beatles music, and who would join him on coffee dates and scooter rides on the weekends, dressed in matching outfits.
"We all miss her terribly," Matt says.
"But she was probably put here for a reason and that's to do all that she could to make sure the patients in the future diagnosed with DIPG are not faced with the same hopeless prognosis of nine to 11 months."
As he dealt with the grief of Josie's prognosis, the devoted dad resolved to give DIPG "one hell of a shake", reaching out to Stanford University neuroscientist Professor Michelle Monje, who studies paediatric brain tumours in the US.
Soon after Josie's diagnosis, Professor Monje sent cell lines derived from 10 donated DIPG tumours to Matt's laboratory to study.
He went to work analysing the tumour cells in a bid to unlock the secrets of the disease that was killing his daughter, hoping to identify new treatments in time to help her and other affected children.
"These patient and family-donated tissue specimens are critical for changing the outcomes for patients diagnosed with cancers like DIPG," Matt says.
"Without them, we would be still performing guesstimation research."
A year into Josie's illness, family and friends set up a charity, RUN DIPG, to fund more research into the cancer. It's raised $3 million for DIPG research in the past four years.
"It's challenging for any kind of cancer research in the Australian climate and has been for some time now," he says.
"We need further investment by the federal government into health and medical research. Our funding rates are historically low and they're much lower than our first world country counterparts."
A letter from Federal Health Minister Mark Butler to a family of a child with DIPG in September last year shows that since 2015, just $13.1 million has been allocated to children's brain tumours through the Medical Research Future Fund and the National Health and Medical Research Council.
Only $970,000 was specifically focused on DIPG.
"There is limited federal government funding for DIPG research [here] and, also, around the world," Matt says.
"But, thanks to the courage of many families who travel this horrendous journey with their children — and have the courage to continue the fight after their children have passed away — many foundations have started, both nationally and internationally, that are supporting research in research labs like mine and around the world.
"Without those charities, and without those foundations, we would be still left with radiotherapy as the standard of care going forward."
Promising clinical trials
Soon after Josie's diagnosis, Matt's laboratory was able to identify a drug, Paxalisib, originally dubbed GDC-0084 and owned by an Australian biotechnology company, Kazia Therapeutics, that might be effective against DIPG, based on an analysis of her tumour cells.
"We got permission to test Paxalisib against tumours growing in our lab," he says.
"We identified that DIPG cells were more sensitive to Paxalisib than other forms of adult brain cancers, for which it was in clinical trials being tested against."
Six months after Josie's diagnosis, she became the first child in the world to receive Paxalisib, in combination with another therapy, ONC201, which was in the early stages of being trialled in children with DIPG in the US, thanks to the work of Matt's team.
Although, at that stage, the drug combination had never been tested, Josie was granted compassionate access.
"She tolerated the therapies quite well and it stalled the tumour from growing for an additional four months," Matt says.
Josie lived for 22 months after her diagnosis — about a year longer than what doctors initially predicted. The drug combination gave her and her family a reprieve from some of the symptoms of the disease.
She began to walk again, before finally succumbing to the cancer at home, surrounded by her family, including her brother, George, and sister, Harriet.
Her other brother, Henri, arrived after his big sister's death.
No government funding for drug
Eve Daher was five-and-a-half years old when she was diagnosed with DIPG on March 10, 2021.
She's Tamer and Angie Daher's only child.
Eve had begun to limp sporadically, complained of having a sore wrist and was unable to pick up a piece of paper off the floor. Her left hand stopped working properly.
Scans late into the night identified Eve had DIPG.
"It's like someone rips out your heart 1,000 times," Tamer says, of being told his daughter had incurable brain cancer.
"It's indescribable, the pain."
After approaching medical specialists both in Australia and overseas — and speaking to Matt Dun — the Dahers decided to use money they were saving for a renovation on their Sydney home to give their daughter the same ONC201-Paxalisib drug combination that Josie received.
"We thought: 'If he's given it to his daughter, then we can give it to our daughter'," Tamer says.
Eve has been taking the drugs for almost two years, her parents importing ONC201 from Germany, costing about $3,500 a month.
The Pringles were also giving Ruby ONC201 and have donated about $20,000 worth of leftover medication, six months' supply, to the Dahers for Eve.
Families receive no government funding to pay for the drug.
While Eve has little use of her left hand, and her left foot is "still wonky", her smile has returned to what it used to be before DIPG began affecting the left side of her face.
"There was no symmetry in her face," Tamer recalls.
"After about three or four months on the combination, her smile got better, her moods got better, her overall health just got better. She goes to school almost every day.
"If it wasn't for the limp and the hand, you would not know that she has the worst type of cancer that you can have.
"Eve's the first patient in the world that's been on this combination for any long period, child or adult, so she is the guinea pig."
The Dahers know the future has no guarantees, but they are grateful to Matt and his laboratory for their work into DIPG.
"My daughter has lived quite well for the last two years thanks to him and his team," Tamer says.
"Three or four years ago if Eve was diagnosed with DIPG, there was basically nothing there that you can try, and you're told to go home and watch your child die."
'Not the silver bullet'
Matt Dun warns that not all children with DIPG will respond to the ONC201-Paxalisib drug combination, which has entered clinical trials in Australia and the US.
"It's not the silver bullet," he says.
However, after five years of research — and a bank of 85 DIPG tumours to study — his laboratory has identified potential new combinations of drugs he hopes will be trialled moving forward.
He's waiting on patents before sharing the discoveries with the world.
"Once we get broad consensus for their potential, then we'll establish clinical trials to test efficacy and safety," he says.
"There is still so much work to move these ideas from the research bench and into the clinic."
That will require much more investment.
"Australia is a lucky country," Matt says.
"We're a rich country and we need to invest appropriately to ensure that diseases like DIPG become a chronic illness rather than a uniformly fatal diagnosis."
After Ruby Pringle died last month in Queensland's only paediatric hospice, Hummingbird House, she made a final journey back to hospital.
In one last act of kindness, her brain tumour was removed and donated to Matt Dun's laboratory.
There, it will be studied in the hope it will improve the lives of future children with DIPG.
Credits
- Story: Janelle Miles and Emma Pollard
- Original pictures and video: Chris Gillette, Mark Leonardi and Michael Lloyd
- Digital production: Kelsie Iorio and Heidi Davoren
