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.
When it comes to childhood cancer, none is more terrifying than DIPG. For cancer researcher Dr Matt Dun, his daughter's diagnosis has led to his own research, which might one day lead to a cure.
February 17, 2018, is a day which will be forever etched in the mind of Dr Matt Dun.
The leading cancer researcher with the Hunter Medical Research Institute, and his wife, Phoebe, a GP, were told their then two-year-old daughter Josephine had a deadly tumour growing in her brain stem that would ultimately claim her life.
There were no "ifs". The type of brain cancer Josephine has, DIPG, or diffuse intrinsic pontine glioma, has no cure and is largely untreatable, partly due to its location which 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 says.
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 Josephine'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 says.
While DIPG is considered rare, Dr Dun says 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 says.
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.
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.
"She was not doing very well. In fact, we began discussing palliative care," says Dr Dun, before doctors started Josephine on targeted treatment based on the results of the earlier biopsy.
Josephine, who was very unwell and was having difficulty breathing prior to the treatment, improved "almost instantly", says Dr Dun.
"She was more conscious and breathing better," he says. "There is no doubt it saved her life."
But despite their 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 says.
His family and friends have helped raise almost $230,000 to date through donations, fundraisers and a running team, which includes Dr Dun.
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.
"We had stable disease for a long time. But the treatment never killed the tumour, it just stopped it from growing," says Dr Dun.
Dr Dun is also using Josephine's earlier biopsy results to see if any existing drug treatments could be effective.
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.
His ability to "compartmentalise" means he has continued his normal workload, welcomed a third child into their family, ran a marathon and a half marathon to raise money for his research and spent time with Josephine.
While he knows the odds are stacked against them, he has not given up hope of "buying us a little more time" and says "medical research is the key".
Associate Professor David Ziegler, who is leading the Zero Childhood Cancer program, says very little was known about DIPG until the past few years, when research has finally shed some light on the disease.
"For the first time, we, together with our collaborators overseas, have developed the laboratory tools that allow us to investigate this tumour in greater detail than ever before," he says.
"We now have one of the largest research programs in the world dedicated to coming up with new treatments."
Associate Professor Ziegler says researchers are learning more about DIPG by studying the tumours of those children taking part in the program.
"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 says.
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 says.
"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."
Donations towards Josephine's treatment can be made here.
Those wishing to fund Dr Dun's research can find details here.
Donations to Associate Professor David Ziegler's research can be made here.