Cystic fibrosis survivor calls for life-saving drug Trikafta to be funded as Pharmac dangles hope

Drug-buying agency Pharmac has revealed it is willing to go directly to the Government for the extra money needed to fund the expensive but life-saving drug Trikafta.

Cystic fibrosis survivor Ed Lee says the drug is a "miracle" and crucial for his survival. However there's a major problem - the blue and orange pills currently cost over $430,000 a year.

But if they are deemed effective against cystic fibrosis, Pharmac says it could go over its billion-dollar budget and ask for a direct boost to buy them.

Ed Lee - off for a run. A simple pleasure for most of us. But for Ed it's an extraordinary achievement because he was born with cystic fibrosis. And for 38 years, it wrecked his lungs.

"It's like gasping for air," he says.

Cystic fibrosis was filling Ed's lungs with sticky mucus making it hard to breathe. It's a death sentence - what Ed calls a "dark cloud".

"The cloud that says you probably won't live until 40. Many of us don't make it to 18," he says.

"And that's always in the back of your mind for your whole life."

Just over a year ago - hospitalised with a severe lung infection - Ed believed his time was up. He and wife Kalyn were updating their wills. But then came a new drug.

"Trikafta, this is the miracle. The drug that saved my life," Ed says.

Trikafta was not available here so Ed and Kalyn travelled to America to buy it.

"And when I got the box in my hand, I remember that smile and thinking 'I've got it'."

Within hours of taking the first tablet, Trikafta started to work, starting with what's known as 'the purge'.

"All of the mucus you have in your lungs, it all just comes out - 38 years of gunk."

An X-ray before Trikafta shows the lungs clogged. But after Trikafta the lungs are much clearer - Ed can breathe properly again.

"I don't even feel like I have cystic fibrosis anymore," Ed says.

A doctor's report says his recovery is "remarkable". But to his wife, it is much more than that.

"We have Ed back. We have our marriage back. His parents have their son back and their brother back and the world gets Ed back, he's a remarkable human. Yeah, you can't describe it," Kalyn says.

For the first time in their lives Ed and Kalyn are now dreaming of their future.

"Now we can have kids, I'm not going to die at 40," Ed says.

The problem is Trikafta costs more than NZ$430,000 a year. Ed has been able to privately fund this massive cost - he knows he is lucky. But he is now fighting to get it funded by Pharmac, so the 530 other Kiwis with cystic fibrosis can have it too.

And he's taking it to the top with Health Minister Andrew Little.

"Should have been here yesterday. People will die. People will die between now and the time that it is funded," Ed says.

Pharmac's Lisa Williams is revealing that if it is proven to be effective it could go to the Government for extra funding to cover that expense.

"It could, if all of those things turn out to be accurate, we can be fast and we can be swift."

Ed says it's worth it.

"It's a miracle drug. And every person who has cystic fibrosis deserves to be on this medicine."

The simple feeling - of being able to breathe.

A direct bid from Pharmac for extra funding would be highly unusual and it's only been successful twice before. And there's another problem - the drug company Vertex hasn't even applied yet.

But there is some movement. Vertex issued a statement saying: "We are open to discussing creative and innovative ways for people living with cystic fibrosis to access our treatments in New Zealand."

It is cryptic - but patients hope that could mean some sort of compassionate access for those about to die we will have someone facing that prospect tomorrow night.