The Lester family - 6-year-old Hazel, mum Jane and dad Gareth - pictured at their Stokes Valley home. Jane has cystic fibrosis and will soon start taking the life-extending drug Trikafta, after Government-funding was approved. Photo / Hagen Hopkins
On her 13th birthday, Jane Lester got a tattoo.
She and her mum walked into a Christchurch parlour and, one after the other, had pigments injected into their skin.
“She made me go first”, Lester says of the novel mother-daughter day out.
“The tattooist thought it was hilarious.”
But getting inked at 13 isn’t the punchline of this story. The punchline is that Lester’s telling it 27 years later.
“Mum was always very open to me experiencing as much as I could, to get in as much as possible while I was able to.
“Little did either of us know that I’d still be kicking at 40″, she says, noting her milestone birthday this month.
The married mum-of-one has cystic fibrosis, an incurable genetic condition where thick, sticky mucus builds up in the lungs, digestive system and other organs, causing a range of symptoms and shrinking the average life expectancy to the mid to late 30s.
The mutation Lester has - delta F508 - is the most common among the 530 Kiwis with cystic fibrosis.
This week Pharmac confirmed that eligible patients aged 6 and over will, from April, 1 be able to get publicly-funded doses of Trikafta, a medicine developed by US pharmaceutical Vertex that treats the cause of cystic fibrosis for around 90 per cent of those with the condition.
“We’re thrilled to announce that Trikafta will be funded and available from next month,” said Pharmac Director of Operations Lisa Williams.
“This decision is a significant milestone. To have a medicine with this level of investment secured for our community is a big deal.”
When first approved for use in the US in 2019, the drug was described by one American doctor as “not a cure, but the closest thing we have”.
Last August, Pharmac acknowledged Trikafta could, on average, give people with cystic fibrosis benefits equivalent to 27 more years at full health.
Among them, Lester.
Given the damage already done to her body by 40 years of living with cystic fibrosis, she isn’t expecting another 27 years.
But the extra years are likely to be still be more than she’d ever dared hope for, and the Stokes Valley mum’s voice falters as she talks about what that means.
She and husband Gareth had always known at some point he’d be a single dad to their daughter Hazel, 6.
Trikafta means that now might be “a lot further in the future”.
“I might get to see my daughter grow up.”
Words are a feeble envoy when tasked with conveying what it means for a little family at the bottom of the world to find out they get more years together.
But this also isn’t a Hallmark movie.
The story didn’t end in the years after a teenage Lester got a butterfly tattooed near her heart - her tribute to a late friend from the cystic fibrosis community - and it doesn’t end now, with the elation of more years to enjoy life, and those with whom she shares it.
Time might be a gift, but it doesn’t come free.
A longer life than expected means those starting on Trikafta next month now face decisions once unthinkable for many in their community - like tertiary study, chasing a career, buying a home, starting a family and saving for retirement.
We’ve already seen the elation, and anguish, in our living rooms.
Last month, Married at First Sight Australia 2023′s bride-to-be Lyndall Grace broke down while talking about what Trikafta meant for her. The drug has been available to eligible Australians since early last year.
“[Before Trikafta] how could I ask someone to be with me forever, when my forever is a lot shorter than theirs?”, said Grace, who was diagnosed with cystic fibrosis aged 3 weeks.
“Now it’s honestly a miracle, I’m going to find the love of my life … I’m going to be able to have kids.”
The Perth accountant’s story - the good bits, and the more challenging - is no different for those with the condition on this side of the Tasman, Cystic Fibrosis New Zealand chief executive Lisa Burns says.
“When the [public funding] announcement came out there was a huge amount of emotion, and now that’s started to settle it’s about, ‘What this will mean? How will I get my medicine? How will my body react?’
“You don’t have to have cystic fibrosis - we all know what it’s like to step into an unknown situation. It’s terrifying at times.”
Some with the condition hadn’t previously thought it was important to focus on education, or were grappling with career decisions they never thought would matter.
Like Grace, some now realise parenthood might be possible.
Even boring life decisions, like whether to join KiwiSaver, had to be re-considered, Burns says.
“Before, you didn’t plan to grow old.”
But Trikafta is also not a cure, she says.
“Yes, it’s absolutely going to make life better and it’s going to stop the decline and the damage, but it’s not going to reverse damage already done.
“These people will still have cystic fibrosis. They’re still going to have emotional, financial and physical challenges to navigate, it’s just they’ve got more years in front of them than they had before.”
Lester’s already in KiwiSaver, albeit for different reasons than most.
“It was purely because I can’t get life insurance, and I wanted something to be able to financially support my family when I go.”
Happily, that support should now be coming her own way - which means giving her fund a bit more attention.
“I might actually have to start contributing properly, because it’s going to help me in the long run if I do get to retirement age.
“I don’t want to be a financial burden on my husband at that point.”
Cystic fibrosis takes much more than it gives, but the condition did prompt the couple to make one financial decision that’ll make them the envy of homeowners everywhere being smashed by rising interest rates.
They aggressively paid down their mortgage and are now just two or three years from being debt free.
The plan was to be financially secure when her health worsened, allowing her to work part-time, or even retire early, to spend more time with Hazel, Lester says.
“Trikafta means I don’t get to retire as soon or go part-time as soon.
“But”, she says laughing, “that’s fine”.
Everyone’s experience with cystic fibrosis is different and Lester puts her “oldie” status down to being fortunate she didn’t struggle to maintain her weight, as many with the condition did.
That, she believes, kept her overall health more stable.
She’s one of just two survivors among 16 peers with cystic fibrosis growing up together in Palmerston North, Lester says.
Her comparatively better health, and a determination to live life on her own terms despite the condition, led her to push ahead with major life decisions others hadn’t, or couldn’t.
Among those decisions, electing to have a baby, although she stopped at one - Hazel was conceived via IVF - because she didn’t want to “put that pressure on my body [again]”.
Her and her husband’s decision to have a child came after much discussion, including with counsellors as part of the IVF process, Lester says.
They decided that with a good dad and the “support and embrace” of the extended family their child would, like others who lost a parent, “get through” its mother’s likely premature death.
“Maybe that’s selfish, but I just think, ‘Everybody’s going to die, and we never know when that’s going to happen’.
“You can’t live your life waiting for that to happen, you have to live your life as if it’s not going to.”
And although longer life is a joy, there are still aspects of her new reality that are unnerving, Lester says.
“After 40 years of that high-salt diet, the idea that this might cause my body to go normal, and I’m going to have to start actually monitoring what I eat and changing all my habits in terms of what I eat is quite scary.
“[Now] you’re looking at things, as my husband likes to call them, “the normies” have to think about - health issues like high cholesterol and heart-related issues and being overweight, and those kind of old-people issues that were never going to be in my future, because you don’t think there’s going to be a future.”
If she sounds blunt it’s because, for her generation born with cystic fibrosis, it was, Lester says.
“It was made very clear to us that we shouldn’t expect anything, we shouldn’t expect a full life. We wouldn’t be adults.
“My biggest joy [with Trikafta] is that kids with CF aren’t going to grow up with those limited expectations.”
Facing her mortality so early in life had an impact, there were “certainly some moments in my teen years”, Lester says.
And she jokes of having a midlife crisis in her early 20s, embracing the party lifestyle and “not looking after myself”.
But that was also when she moved to Wellington and began working, first in administration and later moving up through various related roles to her current position as a project co-ordinator in IT for Datacom.
Now, entering her fifth decade, and thanks to Trikafta, her sights are shifting to project management roles within the organisation.
“I’ve just pottered along doing things that speak to my skills and my strengths, and just happened upon roles. But perhaps I would’ve done that differently if I’d known I was going to get older, and get old enough to see Trikafta.
“Now I have to think about what I want to do with my life, because now I might have a career and a progression.”
Lester’s mostly surefooted about the new opportunities Trikafta promises, but apprehension would certainly be understandable, clinical psychologist Dougal Sutherland says.
“Obviously, the overwhelming feeling is probably going to be something like hope and excitement, and thinking about new things you’ve never thought of before.
“And it also might be quite overwhelming, because you might feel like you have a whole lot of decisions to make all of a sudden.”
Add to that, misplaced guilt over appearing “not thankful”, fearing being a burden on family by living longer, and trying to break the habit of thinking something doesn’t matter because “that’s 30 years away and I won’t be around”.
“There’d potentially be quite a mindset adjustment to getting used to thinking about a future that you’ve never thought about before.”
The development of the drug certainly puts its beneficiaries in a situation to which few could relate.
“We often hear about the cases of ‘Oh, it’s cancer and you’ve only got six months to live’, whereas this is something where they say, ‘We’re going to extend your life’.
“That would take a bit of getting used to … it’s not something many of us will ever experience.”
Trikafta’s emergence may seem sudden to many, but the drug’s been in development for at least a decade, University of Auckland dean of medical sciences John Fraser says.
What was new was the combination of medicines elexacaftor, ivacaftor and tezacaftor to create the triple combination therapy marketed under the brand name Trikafta.
“It’s the result of good science, it really just reflects the fact we’re getting to terms with a lot of the mechanisms of a lot of these diseases, and coming up with drugs that really do behave like blockbusters.
“But there have been a number of new drugs that have been developed that work exceptionally well.”
HIV antiretroviral therapies that saved millions worldwide similarly came from good science, and were probably developed faster than Trikafta due to the greater number of patients, Fraser says.
“In the US there was a lot of pressure put on the national institutes of health to come up with solutions for the Aids epidemic that was killing a lot of people in the US.
“And let’s not forget the mRNA vaccine for Covid, which was developed in nine months.”
Maybe, but news of Trikafta - approved for use in the US by their Food and Drug Administration in October 2019 - was first reported in New Zealand the following August, followed 11 months later by drug-maker Vertex’s application to Pharmac to have the medicine in New Zealand.
Perhaps it just feels faster when you’re on the frontline fighting to get a life-prolonging medicine into the hands of those who need it.
“I’ve not had a long life in the world of medicines”, says Burns, the Cystic Fibrosis NZ chief executive.
“But it does seem like a pretty incredible journey. And the process happened very quickly in New Zealand for our standards.”
“May you live all the days of your life”, wrote Jonathan Swift nearly 300 years ago in Polite Conversation, one among the Anglo-Irish satirist’s many works, most famously Gulliver’s Travels.
Living isn’t a choice as long as air fills our lungs, but when a medical condition reminds you of its precarity with every breath, it doesn’t really matter whether words to live by appear as stinging satire from the 18th century, or wellness woo-woo from the 21st.
Her 40th birthday, like all the days of her life, will be lived fully, Lester says.
“I don’t really think about the future. I also don’t think about the past.
“I don’t have any regrets because there’s just no time to think about those things and wishing, you know, ‘should’ve, would’ve, could’ve’.”
It’s always been “hope for the best and go with the flow”, with likely extra years thanks to Trikafta “a bonus”, she says.
“I’ve just always lived with that awareness that there’s impermanence about life, so I’ve taken opportunities where I can. We’re just going to keep going as if my life will continue - this just gives us all that little bit more security that it will continue.
“And whatever future we face is whatever future we face.”
The 19-year-old disappeared without her wallet or mobile phone.