Families and medical experts are calling on the Federal Government to commit more funding to find treatments for a rare and aggressive childhood cancer that kills about 20 Australian kids every year.
Diffuse intrinsic pontine glioma (DIPG) is a tumour that develops in a part of the brain that controls important body functions such as heartbeat, breathing, sleeping, balance and bladder control.
Some of the symptoms include difficulties with balance and swallowing, headaches and odd eye movements.
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Adelaide boy Samuel Scully, 7, was diagnosed with the cancer in January this year after his mother noticed his speech was slurred.
“He’d lost his two bottom teeth at the same time, so I just kind of put it down to that,” Scully’s mother, Alison Harrison, said.
“He also had a fall at school, so I thought maybe he had a concussion.”
Scully was rushed to the emergency department and within a few hours, brain scans showed he had a tumour the size of a peach that had no cure.
“I think every single person on that floor of the Women’s and Children’s Hospital heard me screaming … I received a death sentence that day too,” Harrison said.
Scully was given six weeks of radiotherapy and then put on a clinical trial known as PNOC-022 for children with DIPG.
Typically, children with DIPG live about 11 months after their diagnosis, but Scully’s tumour has shrunk 75 per cent while on the clinical trial.
Harrison said, while the medication will prolong his life, at any given moment the cancer cells could stop responding to the drugs and Scully’s health will quickly decline.
She said she tries to keep a brave face around her children, but she dreads the day the family will be given news the treatment is no longer working.
“When Sammy sees my tears, he asks what’s wrong. And I just say, ‘mummy’s having a sad day or it’s happy tears’,” Harrison said.
“But I’m just broken as a person. In my moments of downtime when the kids are all asleep, I just cry.
“I lay next to Sam every night when he’s asleep and just listen to him breathing and think about later,” she said.
Harrison said there was a desperate need for more funding to find treatments and cures for this aggressive type of cancer.
“It’s all these beautiful, beautiful children that have such a bright future,” she said.
Clinical trials
University of Newcastle biochemist Professor Matt Dun lost his daughter Josie to DIPG in 2019 when she was four years old.
Having been through the torment of his own child having cancer, he’s dedicated the last few years of his work to finding treatments, even setting up a charity to fund the research because he said government funding was insufficient.
“(It’s) tragically underfunded. It’s funded by mums and dads,” Dun said.
Dun is part of the PNOC-022 clinical trial which began in 2021 and is now open in 31 hospitals around the world — including all eight Australian and New Zealand major paediatric oncology hospitals.
The trial is testing the effectiveness of drugs ONC201 and Paxalisib.
“We use two different drugs to try to combat the way these tumour cells specifically produce energy and grow,” Dun said.
So far, seven children from around the world who are on the trial have survived longer than two years, and one child in Sydney has survived five years, Dun said.
He said from the trial’s preliminary data, there has been a nine to eight month survival extension for patients who have also completed radiotherapy.
The team working on the PNOC-022 trial hope by mid-2024 they will be able to report on the trial’s results and the drug’s side effects, which Dun said could include nausea, rashes and mouth ulcers.
If the results demonstrate success, they will apply to the United States Food and Drug Administration to approve the drug, and then the Therapeutic Goods Administration in Australia.
Another trial known as Levi’s catch is also underway at Sydney Children’s Hospital in Randwick which will use CAR-T cell therapy to genetically modify a patient’s own immune cells in a laboratory to teach them to target and attack the tumour.
While the PNOC-022 treatment is not believed to be a cure, Dun said the clinical trials could help eventually find one.
“Hopefully, that will unlock another piece of the puzzle that will help us target whatever the tumour is using as a resistance mechanism and (in future) hopefully (we can) include additional therapies or change therapies part way through treatment.”
Federal Department of Health representatives met with Dun and other experts on Friday, December 15 to discuss research opportunities into DIPG and report back to the minister.
Health Minister Mark Butler said the Commonwealth Government was investing $700,000 to develop a new treatment that targets DIPG.
“We’re at a very early stage right around the world in understanding the condition,” Butler said.
“I’ve instructed the Department of Health and Aged Care to work with DIPG Australia to find new research opportunities — research offers the best hope for driving meaningful change for these children and families.”
