Take a straw and try to breathe through it.
That’s how Tayla Purves felt before she was placed on Trikafta.
Ms Purves has cystic fibrosis, a genetic disease that primarily affects the lungs and digestive system. She was 182 centimetres tall, weighed only 60 kilograms, and had accepted that she would die young.
There is no known cure for cystic fibrosis.
From today, Trikafta is available on the Pharmaceutical Benefits Scheme (PBS) for those aged two to five. It has been available for those aged 12 and older since April 2022 and six to 11-year-olds since May last year.
Ms Purves says she felt the effects of the medication almost instantly after she started a clinical trial in 2019.
“I could feel the air hit the bottom lobe in my lungs,” Ms Purves says.
“I could feel how cold it was, which yeah was pretty cool.”
During the trial, her lung function went from 40 to 80 per cent and as her health improved, she was finally able to gain weight.
Devastated and grieving
Unfortunately, that progress was undone when complications with Ms Purves’s liver forced her to abandon the trial.
“I was pretty miserable off it, just because you had that normality, you had a taste of what it felt like … and then you’ve gone backwards,” she says.
The “CF cough” came back and her mental health suffered. Ms Purves’s only hope was to wait and hope that Trikafta would be added to the PBS.
Each time the medication was denied by the Pharmaceutical Benefits Advisory Committee she was heartbroken.
“You’re angry, but you’re devastated as well,” Ms Purves says.
“You’re grieving whenever they wouldn’t put it through.”
Shortly after the drug was added to the PBS her application to go back on the treatment was approved.
“It’s a really weird feeling because you’ve prepared yourself to die, and then all of a sudden … I had doctors and nurses telling me I was going to outlive them all,” Ms Purves says.
With her health improved from the medication, Ms Purves hopes to continue her studies in Communications and Creative Writing.
Life built around the disease
First came the purge.
Adam Holmes coughed up mucus for two days as his lungs started to regulate after being placed on Trikafta to treat his cystic fibrosis.
On the third day, he could finally breathe with his lung function stabilising at 75 per cent.
While Mr Holmes was grateful for his improved health, he was shocked by how much his disease had limited him.
“My life was kind of turned upside down because I could just breathe normally,” Mr Holmes says.
“It was actually very traumatic thinking, ‘Oh, this is how I should be living my life.'”
Like many patients, Mr Holmes was diagnosed as an infant and the intensity of his treatment plan increased as he got older.
During school camp, he would be taken out of his activities twice a day to do physiotherapy.
By the age of 12, he required yearly trips to the hospital.
“Because I was sick my whole life, I kind of built my life around cystic fibrosis,” Mr Holmes says.
After suddenly being told he had many years to live, Mr Holmes felt lost.
“Being healthier was just a bit like, ‘Oh, what do I do now?'” he says.
“I guess, what’s, like, my purpose?”
To combat his mental health struggles, Mr Holmes decided to lean into his music and focus on connecting with other members of the CF community.
Last year Mr Holmes and his band Egoism held a fundraiser called Cough It Up to raise funds for cystic fibrosis research.
“It was surreal to see the support from everyone,” he says.
Part of your identity missing
From a young age, Emily Naismith watched her peers die from cystic fibrosis.
She grew up well aware of the life expectancy of those who, like her, had the genetic condition.
“I didn’t really worry about that,” Ms Naismith says.
“It was more just like, that’s how it is.”
For this reason, Ms Naismith always had short-term thinking.
“When my husband and I bought an apartment, I thought about it in the context [of], ‘Oh well, if I have to stop working we need to still be able to afford the repayments,'” she says.
Now at the age of 36 and taking Trikafta, her perspective has shifted.
When Ms Naismith meets new people, she no longer has the signature cough to tell them she is unwell.
“I recently did karaoke with my sister and I didn’t even think about, being able to do karaoke well,” she says.
“And she’s like, ‘I noticed how you can actually just sing and you don’t get puffed out anymore.'”
But discovering a new self isn’t as simple as some may think when a disease has become part of your identity.
“It’s a complex thing to grapple with because it’s like I want to feel good,” she says.
“But there’s a part of me that you feel like you are missing.”
Ms Naismith has been stable on Trikafta for more than two years and is writing a book about her experience.
Survivor’s guilt
While Trikafta has been life-changing for many, there are some mutations of cystic fibrosis that are not eligible for the medication.
For this reason, some who struggle with their mental health feel ungrateful now they are physically healthier, Mr Holmes says.
“It’s the survivor’s guilt, and I have it,” he says.
“People are still dying, and there’s no cure, but I’m healthy. But mentally it’s been really challenging.”
Mr Holmes hopes more people in the cystic fibrosis community have more conversations regarding mental health and life beyond the disease.
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