09/03/2019
Dr Matt Dun is in a race against time to not only save his daughter's life but the 20 other Australian children who are diagnosed with an incurable form of brain cancer each year.

The University of Newcastle and HMRI biologist spoke with Merryn Porter for Essential Baby describing how his daughter's diagnosis led to his own research, which might one day lead to a cure for children in the future.
The date 17 February 2018 will forever be etched in the minds of the Dun family.
 
It was the day the cancer researcher, along with wife, Phoebe, a General Practitioner, were told their two-year-old daughter Josephine had a deadly tumour growing in her brain stem that would ultimately claim her life.
 
There were no ifs.
 
She was diagnosed with DIPG, a cancer that has no cure and is largely untreatable. Its location makes it inoperable. Chemotherapy is also ineffective while radiotherapy at best slows the tumour's progress in the short-term.
 
DIPG has the worst survival rate of childhood cancer, with most children succumbing to the disease within a year. Less than one per cent will still be alive five years after diagnosis.

"Families are told at diagnosis to go home and make memories," Dr Dun said.

Dr Dun, who has dedicated 10 years of his professional life to finding new treatments for both adult and childhood leukaemias, knew very little about DIPG and was shocked by what he learned in the days after his daughter’s diagnosis.

 
"DIPG seems to have exploded in Australia. There are 20 new cases in Australia each year, which means there are 20 fatalities," he said.

While DIPG is considered rare, Dr Dun said the death rate exceeds more treatable childhood cancers.
 
"We lose 101 children under the age of 15 in Australia to cancer each year, so to lose 20 from DIPG is a lot," he said.
 
Within days of her diagnosis, Josephine underwent a biopsy as part of a clinical trial run by the Kids Cancer Centre at the Sydney Children's Hospital, Randwick, and the Children's Cancer Institute.
 
The Zero Childhood Cancer Program sees cancer cells screened for genetic abnormalities (mutations) and drug tested in laboratories before a team of oncologists, clinical geneticists and scientists determine a personalised treatment program. (Figure below: The journey of a child enroled in the Zero Childhood Cancer clinical trial.)


 

Meanwhile, Josephine underwent 30 straight days of radiotherapy treatment, despite worsening symptoms, followed by another MRI two weeks later, which showed a "huge, horrible tumour", according to her dad. The family began discussing palliative care.
 
Then doctors started Josephine on targeted treatment based on the results of the earlier biopsy. Despite having difficulty breathing and being very unwell prior to the treatment, the little girl improved "almost instantly" according to Dr Dun.

 
"She was more conscious and breathing better," he said. "There is no doubt it saved her life."

While it was a relief, Dr Dun knew the improvement was only a reprieve and started crowd-funding so they would have money available if an international treatment trial became available and also to fund his own research.

"I developed my own research program particularly focused on investigating ways to predict DIPG progression in its early stages, as well as testing new drug therapies with the aim of improving survival," he said.
 
When he is not writing grant applications, you will find Dr Dun in his lab, where he has been able to pinpoint DNA from the tumour in Josephine's blood, which serves as a marker to show tumour growth.
 
While others in the medical profession were sceptical, similar new research from the US mirrored his findings. Sadly, his own analysis of Josephine's blood was one of the first signs of the disease's progression in November 2018.
 
"We had stable disease for a long time. But the treatment never killed the tumour, it just stopped it from growing," said Dr Dun.
 
Now he's using Josephine's earlier biopsy results to see if any existing drug treatments could be effective.

At present he has 13 DIPG tumours growing in the lab and has been hard at work testing 15 to 20 drugs, and drug combinations. He believes one such experimental drug could have a positive benefit but he does not know if it will be in time to save Josephine.

 
Dr Dun's ability to compartmentalise means he has continued his normal workload while welcoming a third child into their family and running in a marathon and a half marathon to raise money for his research, all while spending time with Josephine.
 

The Dun family welcome their newest member. 
 
Although he knows the odds are stacked against them, he has not given up hope of buying a little more time and asserts medical research is the key.
 
Associate Professor David Ziegler is a Group Leader at the Children’s Cancer Institute where his preclinical research focuses on novel therapies for childhood brain tumour. He’s currently running three separate studies into DIPG that are funded by The Kids’ Cancer Project.

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Very little was known about DIPG up until few years ago when research finally shed some light on the disease as Associate Professor Ziegler explained.

"For the first time, we, together with our collaborators overseas, developed the laboratory tools that allowed us to investigate this tumour in greater detail than ever before," he said.
"We now have one of the largest research programs in the world dedicated to coming up with new treatments."
 
Associate Professor Ziegler said researchers are learning more about DIPG by studying the tumours of those children taking part in clinical trials such as Zero Childhood Cancer.
 
"Their tumours are currently getting evaluated by state-of-the-art techniques such as molecular profiling and robotic high throughput drug screening in order to find key targets that would render these aggressive DIPG tumour cells susceptible to specific treatments," he said.
 
As well as establishing a national tumour bank of brain tumours, Associate Professor Ziegler is investigating new treatment strategies, including the use of nanocells loaded with anti-cancer drugs to target DIPG and other tumour cells.
 
"The more drug treatments we can test, the more options we will ultimately be able to provide to patients with DIPG," he said. 
"Fifty years ago, parents of children with leukaemia were told, 'this is an incurable disease, there's nothing we can do'. Now 85 to 90 per cent of those kids are cured.”
 
"We believe we will be able to achieve the same for DIPG patients," said Associate Professor Ziegler.
This story is shared with permission from Merryn Porter who originally wrote the piece for Essential Baby.
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The Kids' Cancer Project is an ACNC Registered Charity 
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