At the age of 12, Kerrie Taylor was diagnosed with a rare form of cystic fibrosis.
She was not expected to have a long life.
"There were lots of times that growing up you always hear that you might make it to 18, you might make it to 21," she said.
"There were times when you were really sick and you're thinking, 'Am I going to have those special occasions like everyone else is going to?', but I always say this, 'There's always someone else worse than you are'.
Impact on everyday life
Fifty-two-year-old Ms Taylor lives with her husband, Stu, at North Beach on SA's Yorke Peninsula.
She was one of more than 3,500 people in Australia who lived with the disease, which could make everyday life incredibly difficult for some.
"Our lungs will get infections quite a lot," she said.
"We always have mucous on our lungs and it's sticky and it clogs our lungs up and then it makes our lungs deteriorate.
Ms Taylor had trouble doing everyday tasks such as going for a walk, making the bed, and sleeping.
"You'd be lying down you'd be scared to move and that is because you felt like if you moved your lungs were going to erupt like a volcano and everything was just going to come up like all the mucous," she said.
Although Ms Taylor has experienced difficulties arising from the condition, she stayed positive and said she had "never let cystic control me".
Time for a transplant
In 2019, Ms Taylor began preparing for a double-lung transplant that was to take place in either Melbourne or Sydney.
She had attended pilates sessions to ensure her legs and lower body were strong enough for the post-surgery recovery journey.
This was when her specialist approached her about trying a drug called Trikafta for free on compassionate grounds under the Managed Access Program.
"We thought if I can have the chance of going onto medication that would give me the opportunities to see where I could go and I always had the transplant as a back-up," she said.
In December, Ms Taylor started taking Vertex's Trikafta: two tablets in the morning and one at night.
She had to be monitored in hospital for two weeks because a potential side-effect could have been a sensation of drowning while the body expelled her excess mucous.
Fortunately, she was lucky.
"I wasn't wheezy, I wasn't coughing, my chest feels so light — my volcano has gone away in my lungs."
Ms Taylor's lung function has since gone from 27 to 36 per cent and her quality of life has vastly improved.
Pilates instructors at Advanced Physio Solutions, Nick and Kara Rendell, were surprised by how quickly Ms Taylor's condition had improved.
"[We're doing] a lot more repetitions, we've increased the strength quite a bit as well and just probably the variety of exercises — she's doing a lot more," Mr Rendell said.
Ms Rendell has known Ms Taylor since their junior softball days as children.
Making the drug affordable
Ms Taylor and others are now calling for Trikafta to be listed on the Pharmaceutical Benefits Scheme (PBS).
The drug was approved by the Therapeutic Goods Administration last week after its safety and efficacy were tested, which Ms Taylor said was a step in the right direction.
However, for it to be on the PBS, the Pharmaceutical Benefits Advisory Committee (PBAC) must weigh up the cost-effectiveness of the drug and assess patient affordability.
Cystic Fibrosis Australia CEO Nettie Burke said if the drug were listed on the PBS it would drop substantially in price, which could be up to $400,000 per year for patients.
"When it's on the PBS, depending on your income status, you'll pay between $6 and $40," Ms Burke said.
"On April 23, [after the PBAC makes its recommendations] we find out if 2,200 people who suffer from cystic fibrosis will be eligible for Trikafta."
What the research says
Ms Burke said the clinical trials and compassionate care program research had shown promise.
"In the clinical trials, a few people had mild side effects but none of these were seen to be significant," she said.
"They ran overall for about 24 weeks and that provided the data to show the drug worked incredibly well.
However, the trials and programs had only measured the short-term effects.
"At the moment, we are seeing a number of people coming off the transplant list because they are getting compassionate access to Trikafta," Ms Burke said.
"Maybe down the track they may need a transplant, but we don't know because we don't have that long-term evidence."
The results of the PBAC's decision will be announced on April 23.
