Cystic fibrosis: Tauranga's 'Cystic Sister' says her sister could still be alive if Trikafta was available

Nikki Reynolds-Wilson pictured in 2019 after her sister Kristie Purton died from cystic fibrosis. Photo / Andrew Warner

Kristie Purton died from cystic fibrosis in 2019. Her sister Nikki Reynolds-Wilson says Kristie could still be alive if a $330,000-a-year drug was available. Kristie and Nikki are known as Tauranga's 'Cystic Sisters' and were both born with cystic fibrosis - an inherited disorder that clogs the lungs and pancreas with thick, sticky mucus. Nikki's message to the Government about publicly funding Trikafta is clear: "It saves lives". Journalist Megan Wilson tells her story.

Kristie Purton could still be alive if Trikafta was available.

That's the view of her sister Nikki Reynolds-Wilson, who is backing the call to publicly fund Trikafta - a drug that treats the underlying cause of cystic fibrosis.

The Tauranga sisters were born with cystic fibrosis and had both undergone lung transplants. They became well-known in Tauranga as the 'Cystic Sisters' and spent years raising awareness of the disorder, twice completing 65 days of good deeds in the community.

But Kristie died in March 2019 after her body rejected the lungs. She was 35.

Nikki, 31, says Trikafta would not have helped Kristie with her reaction to the transplant.

"But had [Trikafta] been available, she could have avoided having the transplant and still be here."

Her message to the Government is to stress how important Trikafta is.

"It saves lives."

Kristie Purton pictured in 2018. Photo / Andrew Warner

The Bay of Plenty Times has spoken with several families who are living with cystic fibrosis and who are urging the Government to fund Trikafta.

Rotorua mother Trish Daniels faces losing her second child OJ Daniels, 19, to cystic fibrosis if the drug is not funded and Tauranga father Glenn Ford worries his 11-year-old son Charlie Ford's life will be "cut short" without it.

Whakatāne brothers Ashley and Troy Watson also both have cystic fibrosis. Ashley, 28, has access to Trikafta free through a compassionate managed access programme funded by the drug's manufacturer and supplier. But Troy, 20, is "too well" to be eligible and without Government funding, there is "no chance" for someone like him.

Cystic fibrosis is a genetically inherited condition that produces thick and sticky mucus and mainly affects a person's lungs and digestive system.

It affects about 540 people in New Zealand. Specialists believe the average life expectancy of a person with cystic fibrosis is mid-to-late 30s.

A petition calling for public funding of Trikafta was presented to Parliament last month, saying clinical trials have shown "significant improvement" in health outcomes for people with the condition.

Nikki Reynolds-Wilson said having a lung transplant was not a cure for cystic fibrosis. Photo / Andrew Warner

Speaking to the Bay of Plenty Times, Nikki says funding Trikafta would prevent people with cystic fibrosis from getting a transplant which cost "a lot more" than the drug.

She says lung transplants are publicly funded and cost a minimum of $1 million.

Trikafta costs $330,000 per year.

"The drug is a lot cheaper than that so it makes no sense - why not fund a drug to prevent having a lung transplant?

"Transplant isn't a fix - it just gives you a bit longer.

"It still means we have a short life expectancy, we still have to take medication. It's kind of like swapping cystic fibrosis for something else."

Nikki says a lung transplant gives people with cystic fibrosis a longer life expectancy of between five and eight more years. But it is different for everyone.

"Kristie only got one year."

Nikki Reynolds-Wilson pictured in 2019 at Kristie Purton's funeral. Photo / George Novak

In August, it will have been six years since Nikki had her lung transplant.

"In the back of your mind, you feel like each year you arrive... one year closer. But that eight-year mark - is that kind of it? You just don't know."

Nikki says the lung transplant has allowed her to live "like normal". She goes to the gym regularly, works part-time and is studying to be a primary school teacher.

With her partner and her 10-year-old daughter Skylar, Nikki moved to Christchurch a month ago with the goal of buying a house. She plans to eventually move back home in a few years.

When asked how she dealt with facing a shorter life expectancy, she says it is "definitely a struggle" - particularly since Kristie died.

"It's just really hard not having that person that gets everything."

Nikki says younger patients and children need Trikafta so they possibly would never need a transplant.

She references the Watson brothers and says if both had access to Trikafta, "theoretically they should have a much longer, healthier life and probably avoid having a transplant".

Whakatāne brothers Troy and Ashley Watson both have cystic fibrosis. Photo / Andrew Warner

Pharmac operations director Lisa Williams says Pharmac would like to fund Trikafta but it operates on a "fixed budget" that the Government sets.

"We can't avoid the fact that Trikafta is a very expensive medicine.

"There will always be more medicines we want to fund than we can afford. This means we have to make difficult choices about what medicines are funded."

The price, along with costs or savings to other parts of the health sector, is a considered factor during the assessment of funding applications, she says.

Pharmac acknowledges there is an "unmet health need" for another effective treatment for patients with cystic fibrosis and it has placed Trikafta on its options for investment list.

"While Pharmac can negotiate some of the best prices for medicines in the world, when faced with a medicine which has such a long patent life and no competition, such negotiations can be challenging."

Williams says Vertex has a monopoly on the drug because it is the only one that can make and sell the medicine.

"This means there is no competition, thus limiting our influence over the price Vertex decides to offer to sell Trikafta in New Zealand."

Pharmac continues to talk to Vertex about the cost, but cannot provide a timeframe for if or when a decision will be made to fund Trikafta.

The Ministry of Health was contacted for comment.

Kristie Purton and her "day of magic"

A Day of Magic Charitable Trust was founded after its chief executive Melanie Quin gifted Kristie "a day of magic" before she died.

The charity gives terminally ill New Zealanders a day to celebrate their life with friends and family.

Melanie said she knew of Kristie from her college days and called Kristie when she heard of her terminal illness.

"I just rung her and just said, look is there anything you'd wished you'd done? And she said actually... I've always wanted to fly in a helicopter."

A Day of Magic Charitable Trust chief executive Melanie Quin. Photo / Andrew Warner

Melanie organised the day for Kristie, her friends and family which was "a complete surprise". It included a morning out in Pilot Bay on a launch, a helicopter ride and a private performance by Tiki Taane.

She did it as a "personal gift" to Kristie and also donated professional photography and videography to them.

"After that came off beautifully... I just couldn't believe that there wasn't really anything out there like it.

"And then the charity kind of was born from that day."

Nikki said it was a "really nice day" but saw "how hard" the day was for Kristie.

"You know why you're doing it and she was so sick, and it was really hard."

Since then, Melanie has helped five families have their "day of magic" through her charity.