Hadspen’s Luke Emery, 11, lives with cystic fibrosis, and his family had hoped the drug, Orkambi, worth $250,000, would be put on the Pharmaceutical Benefits Scheme.

“Every time it went up for considering, there were high hopes, especially the first time I guess,” his mother, Donna, said.

Orkambi showed positive results for cystic fibrosis patients in a medical trial, but the Pharmaceutical Benefits Advisory Committee refused to recommend it to the PBS on Friday.

According to media reports, the committee was continuing to negotiate with drug company Vertex over the price.

The drug has been found to reduce the need for hospital visits, some daily medications and physiotherapy sessions.

Luke is currently on 13 tablets a day, just to keep him functioning normally.

“When you add into that getting sick, which is much more common with a condition like he has, he then has to add in more medications, more treatments, more physio, and then hospital visits on top of that,” Mrs Emery said.

“Our main aim is to keep him well and keep him from having to have all that extra medication and that’s what this particular medication can do. It can keep him well at that same maintenance level for longer so that he doesn’t have to face hospital, so that he doesn’t have to take extra medication on top of what he already has.”

Because of his cystic fibrosis, Luke has low immunity and is at greater risk of infection, so his parents have to constantly be aware of people’s health around him – at school, out in the public, and at home.

“Anytime family is unwell, we have to ask them to stay away, especially in winter time in Tasmania, like the flu epidemic at the moment is a real worry. It’s a real worry every year.”

And the more times Luke contracts an infection, the more irreparable damage is done to his lungs.

“We can get a cold and get over that cold. Luke, on the other hand, can get a cold and he’s more susceptible to bacteria getting into his lungs and that bacteria can sit in his lungs and grow there and cause ongoing infections that keep coming back all the time.

“He has a certain lung function percentage and once that drops down, it is very hard, if not impossible, to get up again.”

Luke has been in and out of hospitals his whole life. When he does get sick, it’s a difficult road to recovery.

“It’s similar to someone who’s suffering any other illness that causes immunity deficiency - like cancer and things like that.

“It can cause him irreparable damage to his lungs if he is exposed to something - a virus that causes him to become unwell. It can take years off his life.”

Cystic fibrosis has an average life expectancy of about 37.

Mrs Emery said the current fight over getting Orkambi on the PBS was just about money.

“But it makes sense to keep young people well. It makes sense to get medication that can halt the progression of a disease further.

“It may not increase any lung function as such, but it can halt the exacerbation of infections and the need for hospitalisation which is costing money anyway.

“For someone who’s 18 or 19, they could be on 30 per cent lung function - they need it now.

“The more time we waste arguing about money, the more lives are lost.”