Listing gives hope to some with disease

HOBART’S Jack Dyson and about 40 other Tasmanians who live with cystic fibrosis will be able to access a new medication now it has been added to the Pharmaceutical Benefits Scheme.

Mr Dyson, 21, said the cost of the lift-extending medication had been “ridiculously” prohibitive in the past but he hoped to have a prescription next month.

“It will not fix my condition overnight but hopefully it will add 10 to 15 years to my life expectancy which is currently 37 years of age. The drug focuses on the cause of cystic fibrosis not the symptoms,” he said.

Liberal Senator Jonathon Duniam said he had been lobbying Canberra about adding Orkambi to the PBS for more than a year.

“The rollout of this medication will be significant in helping patients extend their lives,” Senator Duniam said.

“Patients would otherwise need to pay up to $250,000 a year for Orkambi but they will now only pay a maximum of $39.50 per script, with concessional patients paying just $6.40.”

Orkambi will be available on the PBS for all patients over the age of six who have the most common form of cystic fibrosis — two copies of the f508del mutation in the CFTR gene. Around Tasmania, 110 people live with cystic fibrosis but not all have the form which can be treated with the new drug.

Cystic Fibrosis Tasmania Executive Officer Paula Wriedt said the listing would benefit about 40 Tasmanians.

Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. A defective gene causes a thick, sticky build-up of mucus in the lungs, pancreas and other organs.

Orkambi has been shown to slow the rate of decline in lung function and reduce the number of infections.