A new drug to treat cystic fibrosis has been put on the pharmaceutical benefits scheme -  Rachael Griebenow with daughters Zoe 1yo and Skyla 9yo of Jensen.
A new drug to treat cystic fibrosis has been put on the pharmaceutical benefits scheme - Rachael Griebenow with daughters Zoe 1yo and Skyla 9yo of Jensen.

“I’m not going to die any more mum”

SKYLA Griebenow is only nine years old but already she knows she won't live as long as most people.

The Bohlevale State School Year 3 student has cystic fibrosis and her average life expectancy was 37 years.

But that has changed thanks to a drug that could add an extra 20 years to her life becoming available.

The drug, Orkambi, will be added to the Pharmaceutical Benefits Scheme from October 1.

The drug previously cost $250,000 a year, but from the start of next month it will cost about $40 per script.

"I cried for probably three-and-half hours when I heard and then I called my best friend," Skyla said.

Cystic fibrosis mostly affects the lungs and digestive system and there is no known cure.

Sufferers are on medication their entire lives and spend long periods being treated in hospital.

Skyla's mum Rachael said cystic fibrosis had a big impact on her daughter mentally and physically.

"She goes through lots of stuff that kids her age don't usually go through," she said.

"We've had to have conversations about her life expectancy and the effects of cystic fibrosis and that obviously plays on her mind a lot.

"So when she found Orkambi had been approved there was a lot of relief, not just for me and her dad, but also for her.

"When she found out, she actually said, 'I'm not going to die any more mum' … I guess kids her age don't have to think about dying."

Rachael said having Orkambi on the PBS was "massive" for her family which also included husband Rodney and one-year-old daughter Zoe.

"It's the first drug of its kind that actually treats the underlying cause of cystic fibrosis rather than the symptoms it causes," she said.

Last year Skyla spent 17 weeks in hospital battling the disease and has been in hospital about five weeks this year.

"She's fed for 12 hours overnight through a feeding tube into her stomach, so eventually we will now be able to get rid of that tube," Rachael said.

"She had to have a port put into her chest because of all the veins have collapsed in her arms because she's had so many IVs."

At the moment Skyla takes about 40 tablets each day and taking Orkambi is expected to cut that in half.

Rachael said the medication would help her daughter live a more normal, healthier life.

"Potentially it will give her an extra 20 years, that's on top of her life expectancy which currently sits at 37," she said.

"She will spend less time in hospital and more time in school, more time doing normal stuff, planning a holiday at the moment is impossible because of the amount of time she spends in hospital."

The drug will help about 1200 people with a specific strain of cystic fibrosis.

Health Minister Greg Hunt said the medicine would change people's lives

"So many people have worked so hard to get this medicine, which will now literally be in the hands of parents and kids from this week and change their lives," he said.

Cystic Fibrosis Queensland chief executive officer Petrina Fraccaro said the drug helped stop the disease from worsening.

"The key is to stop the deterioration," she said.

"This will maintain people's health and wellness, so when the cure comes (Skyla) is in the best possible condition to be really receptive to that cure."

Ms Fraccaro said before Orkambi was placed on the PBS it was simply unaffordable to families.