Doctor encourages locals to continue giving generously to CF research

"I don't want to see children dying of CF, I don't want to see young adults dying of CF," the Royal Children's Hospital doctor said.

"I want them to be able to live a long, unrestricted life.

"Because the long-term survival at this stage is in the low-40s."

It's a dream shared by countless CF patients across Australia including Echuca’s Shari Butcher, founder of local CF fundraiser Breathe Easy.

She was Dr Phil's patient from birth until she was almost 19, just one in a multitude of patients he has cared for throughout the years.

"He would see me for my three-monthly outpatient check-ups until I transitioned to adult care at The Alfred Hospital," Shari said.

"When I was diagnosed with CF at one month old, Phil said to Mum and Dad, 'don't wrap Shari in cotton wool, but don't forget she has CF' and that's how I lived and grew up."

It is a challenging, often heartbreaking, chat Dr Phil has had with more families than he can remember.

He has been in the respiratory department at the Royal Children's for nearly 30 years and is currently director of respiratory medicine at the hospital.

In 1999, he worked alongside families to establish the Royal Children's Cystic Fibrosis Research Trust with the aim to raise funds for the hospital's CF research.

Twenty years later, the trust has raised more than $3 million from charity events across the state — including Breathe Easy right here in Echuca-Moama.

"Shari's work over the past five years has been wonderful," Dr Phil said.

"Money raised from events like these has funded research that is already making a difference.

"In 1998, the average age of a patient at death was about 18 and now it's well into the 40s.

"Similarly, in 1998 about 30 per cent of people in Australia with CF were adults and now it's 55 per cent. So they're certainly living a lot longer."

In the past 10 years, researchers have started developing specific drugs to correct the underlying biochemical defect which is the major disease-modifying effect.

"We haven't seen the long-term benefits yet because they're such recent drugs but that certainly is the direction of a lot of therapy," Dr Phil said.

"Medicine is becoming more personalised so we identify which drugs work effectively for a particular patient.

"There are suggestions they are having an effect in the short term, reducing exacerbation and the number of times people need to go to hospital for treatment."

But when it comes to a "cure", Dr Phil said things were a little more complicated.

"These drugs will only be commenced when the patient is actually born with the condition so they won't be cured from there," he said.

"We could eventually move on to therapies that are administered in utero to prevent the child actually developing the issues of CF.

"But the main drive we have at the moment is to identify patients early through newborn screening and initiate treatment very early on.

"We believe children born in the past few years will have survival into the 50s and we want to see that push out even further."

In the meantime, Dr Phil encourages communities like Echuca-Moama to continue giving generously to CF charities.

"There's an ongoing battle to actually identify the appropriate therapy for everybody and that takes money and research," he said.

"We've done a fabulous job throughout the past 25 years but I think there's a lot more we can achieve.

"And it's really the support of people in the community that allows that to occur."

The fifth and final Breathe Easy Cocktail Party will be held at Radcliffes on Saturday, September 7 from 7pm to 11.45pm.

Tickets cost $115 per person and include entry, entertainment, drinks and a finger food package.

For more information and to book a ticket, visit stickytickets.com.au/87873/breathe—easy—cocktail—party—2019.aspx