‘Miracle’ drug gives hope to CF sufferers

CYSTIC FIBROSIS saloon cars: Raising more awareness of cystic fibrosis is the only way to bring about a change in funding drugs to treat it, says Gisborne woman Paige Cook, who was born with the disease. To help do that and as a surprise for his wife, Ethan Cook and brother Daniel branded their 76G and 77G speedway saloon cars with the Cystic Fibrosis NZ logo and colours. The brothers will have information sheets, a donation box and merchandise at Gisborne Speedway Club events for anyone who wants to help. But the biggest thing you can do is sign the online petition calling for the Government to fund “miracle drug” Trikafta, which currently costs $470,000 a year in New Zealand. The drug would significantly improve and extend 24-year-old Paige’s life beyond her life expectancy of mid-to-late 30s. Picture by Liam Clayton

Like breathing through a straw is how cystic fibrosis is described by Paige Cook — one of only a handful of people in Gisborne and 540 nationwide who have the rare disease.

To help spread awareness of cystic fibrosis, Paige’s husband Ethan Cook and his brother Daniel unveiled special designs on their saloon cars at Gisborne Speedway Club’s meeting last weekend.

Both cars were decked out in cystic fibrosis colours and logos to help spread awareness.

“I was super-surprised. It was so kind and lovely,” said 24-year-old Paige, who was born with the disease.

“It was a gesture to say ‘you’ve got this, we can do it, you’re strong and we’re all in this together’.”

The cruel twist — and why more awareness is key — is that there is a “miracle drug” called Trikafta, which clinical trials have shown significantly improves health outcomes for cystic fibrosis sufferers.

The problem is it is not funded by the Government in New Zealand so comes at an unattainable cost of $470,000 a year. Countries such as the United Kingdom, the United States and Australia fund it for their citizens but not here.

A petition is under way for Parliament to change this.

For Paige, getting Trikafta would mean the difference between her and Ethan having their own family, and her living beyond the estimated life expectancy of mid to late 30s.

“I would be lying if I said the life expectancy didn’t play on my mind a bit.

“If I had children, how long would I be around to raise them? How heartbroken would my family and friends be?

“What I’m more scared of is living a painful and hard life, and that is where Trikafta gives me hope.”

CF is a degenerative disease where sufferers get sicker as they get older.

Paige takes at least 30 pills a day.

Growing up, she never wanted to talk about it.

“I didn’t like the attention or for people to feel sorry for me. I wanted to be normal.

“But then as I got older I realised there would be no changes if no one knew about it.

“That’s when I started to talk about it more and bring more awareness. Now I am an open book and I like it when people ask me questions.”

Her main focus is to get New Zealand funding for Trikafta.

“Cystic fibrosis is an inherited, incurable, life-threatening disorder that damages all our organs, but mainly our lungs and digestive system.”

Paige inherited it from her mum and dad, who did not realise it was in the family and that they were carriers until Paige was born.

Her brother is a carrier but does not have the disease.

“I was just that unlucky one.

“But I like to live my life with as much happiness and positivity as possible.

“I could choose to be bitter or just make the most of my situation.”

Those with CF produce thick and sticky mucus that blocks the tiny tubes of many organs.

“Our lungs develop severe damage over the years from recurrent chest infections.”

Paige suffers breathlessness, wheezing and a chronic cough.

“It’s like breathing though a straw.

Other affected parts of the body are the ears, nose (sinuses), liver, bones, muscles and joints,.

CF sufferers can have serious fertility problems and a high chance of developing CF diabetes.

“My whole life has been doctor visits, going in and out of hospital, medications, breathing therapies (using a nebuliser) and physio.

“I was actually very lucky because my mum is a nurse and she was very on to it.”

■ To sign the petition to make Trikafta funded in NZ, go to