But after six months on the Australian trial of a new drug, his health had improved remarkably. "He's like a new boy," she said. "He has so much more energy and stamina, and he's so happy. "His confidence and self-esteem have shot up, he eats normally, and he's so cheerful and positive all the time rather than being down and tired. He's a completely different boy." Today, 10 months on from that epic hockey game, Connor, now 15, has continued to thrive and hasn't had to be rushed to hospital once, whereas he'd normally spend between nine and 15 weeks there a year. But last week came the most crushing blow of all: the government ruled that the new drug, Orkambi, should not be made available on the Pharmaceutical Benefits Scheme (PBS), pushing its cost up to an estimated $5000 a week for Connor, or $260,000 a year.

"That kind of money is unattainable for people, no one in the world can afford that," said Mrs Philpott, 45, a teacher. "It's horrifying not to be able to give a child a better chance at life because of a decision like this." For her and husband Shane, also 45, a mine machine-driver, the decision is doubly tragic: their seven-year-old daughter Macyn also has CF, although, at her young age, her body hasn't yet started to deteriorate at such a fast pace as her big brother's. Their youngest child, Hamish, five, was born clear. For the other 1000 or so Australian CF sufferers the drug could help, that decision has been shocking, CF NSW CEO Michele Adair said. "On the trial happening here, many say their quality of life has improved significantly so to take that away from them now is absolutely devastating," she said. "We believe the government should make the drug available while it continues to negotiate a fairer price with the drug manufacturer Vertex."

The drug was rejected for the PBS on the basis of cost and evidence of a "substantial" benefit. A spokesman for the Department of Health said it was decided not to recommend PBS listing "based on an unacceptably high and uncertain incremental cost-effectiveness ratio at the requested price by the sponsor, and uncertainty around the impact on long-term improvements in lung function and survival … The net cost to government was more than $100 million in each of the first five years of listing." But, with the average age of death due to CF in Australia standing at 27 years, and even small increases in lung function – like the average three per cent the trials found – leading to huge improvements in health and slowing the rate of health decline, the sufferers and their families say the cost of not making the drug available is incalculable. "They're not looking at the big picture, at the lifetime cost on the health system of all the treatment they need, at all the hospital beds we're taking up, at the cost of not having healthy, happy members of the community who can all contribute," Mrs Philpott said. "Connor's increase has been six per cent and, for us, for the past 16 months, our sadness had been replaced by hope. To have that hope taken away and know we've going to have to watch him deteriorate again … that's so horrifying.

"My child isn't a statistic. He's my heart and my living breath, my world. For them to say my child isn't worth the chance of a longer life, it breaks my heart. Every child deserves a chance." BITTER PILL Clinical trials show Orkambi has cut relapses of Cystic Fibrosis by 39 per cent, meaning a 61 per cent fall in the number of hospitalisations. It has also led to a 56 per cent decrease in the need for intravenous antibiotics among sufferers. It's now available under subsidy in the US, Germany, France and Austria – but has been rejected in Britain for reasons similar to those listed in Australia.

In Australia, sufferers will now have to pay an estimated $260,000 a year for the drug, as against $200-$1500 on the PBS, according to financial circumstances.