Ernie Bindel (46), who has a rare form of the genetic condition called G551D, has been taking the trial drug Kalydeco for three years, which he said had improved his health and kept him out of hospital.
‘Between 21 and 42, I would have been in hospital every year, if not twice a year,’ he said.
‘I haven’t been in hospital since I have taken the drug ” you are sick less, you are healthier.’
However, the US-manufactured drug costs between $250,000 and $300,000 a year, so Mr Bindel hopes the Federal Government will approve it and subsidise it through the Pharmaceutical Benefits Scheme this month.
Diagnosed with cystic fibrosis when he was 14, Mr Bindel said he first went into hospital for it when he was 21 and for half his life activities like walking up a couple of flights of stairs left him trying to catch his breath.
‘The important thing with cystic fibrosis is that you are active and you exercise because you clear your lungs,’ he said.
With life expectancy for people with cystic fibrosis normally the late 30s or 40s, Mr Bindel said he now felt he could start planning his future.
‘My wife and I are looking at starting a family ” that is something that when you don’t know how long you have, you don’t look forward to as much,’ he said.
‘What it takes to be a father, you can’t do that if you are sick all the time ” I can make that commitment now and look after someone else.’
During the trial, staff at the Lung Institute of WA regularly tested him for effects of the drug.