New drug gives hope to 14-year-old boy with cystic fibrosis

Thursday, 11 February 2021

Bruno Cettina’s life has changed significantly since he gained access to a “wonder drug” that helps people with cystic fibrosis.

A new drug has transformed the life of a 14-year-old boy who lives with cystic fibrosis.

Remuera’s Bruno Cettina is playing basketball and going mountain biking for the first time after medicine funded by Pharmac increased his lung capacity by 91 per cent in 11 months.

Kelly Oliver has cystic fibrosis and is doing a fundraiser to raise money so kids with the illness can get the same treatment.

The drug is yet to be approved by Medsafe, but was funded through an 'exceptional circumstances process” for people with high needs.

Cystic fibrosis is an inherited life-threatening disorder which damages the lungs and digestive system.

Bruno is one of 35 New Zealanders with a specific mutation of the disease treatable with a drug called ivacaftor.

Pharmac approved funding for ivacaftor in March last year. Bruno’s mother Ana said it has “completely changed” his life.

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Bruno’s mother Ana says her son can now withstand long periods of exercise after the drug increased his lung capacity by 91 per cent.

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Bruno is passionate about sports like cricket, and since accessing the medication he has started playing basketball and mountain biking.

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Bruno would be in hospital every four months due to his lung problems, but since accessing the Pharmac-funded drug he is yet to be admitted.

“It’s amazing, we’ve lived the last 15 years making the most of our time because it’s been very real that we may lose a child in our lifetime,” she said.

Ana says the drug has allowed Bruno to put on weight, so the 14-year-old now looks his age.

“But this really is a miracle drug, it’s known universally as a wonder drug for people with cystic fibrosis.”

Ana said Bruno’s health has dramatically improved, and he looks like “a whole different person”.

“He’s able to eat food and actually put on the weight that he’s eating,” she said.

“Now he looks like a normal teenager as opposed to looking ten years old, and really skinny. He’s put on over ten kilograms.”

Bruno plays soccer and cricket for his school, King’s College, and has joined a local basketball club since gaining access to the medication.

“When he was born his dad and I decided if his life was going to be short he had to be really active, because that was a way to move the mucus around the body,” Ana said.

“So he’s always been active, but he can now withstand long periods of exercise. He’s been on mountain bike rides in the past year which he probably never would have done before”

Bruno has been admitted to Starship Hospital on average three times a year for most of his life, but since starting ivacaftor he is yet to go.

“Long term it should lead to a greater life expectancy. So as opposed to being in his forties, we’re looking at a normal life span,” Ana said.

Ivacftor will be added to the pharmaceutical schedule on March 1 once approved by Medsafe.

Medsafe approval is an important process in ensuring the safety of a medicine before it is made available for New Zealanders.

Pharmac decided to enable funding for ivacaftor through an exceptional circumstances process, while waiting for approval from Medsafe for both formulations to be granted.

Deputy medical director Dr Pete Murray said a number of patients with high health needs were key to this decision.

“Pharmac prefers to fund medicines that have been assessed and approved by Medsafe, however, we need to balance this with making medicines available in a timely manner to meet a high health need,” he said.

“We know the huge impact medicines like ivacaftor can have on an individual’s health and wellbeing, and we are thrilled to hear stories like Bruno’s.”

Ivacaftor costs $383,225 per person per year, although Pharmac was able to negotiate a discount, the amoutn of which is confidential.