A Napier man suffering from a rare and incurable disease has had to close his business and sacrifice everyday normalities so he can travel overseas every fortnight for life-saving treatment.
But Bruce Wells considers himself lucky.
The 48-year-old is one of seven Kiwis suffering from the incurable Pompe disease that, when left untreated, causes muscle deterioration, respiratory failure and premature death.
The drug Myozyme is available in New Zealand at between $500,000 and $2 million per patient per year, but is not funded by government drug-buying agency Pharmac.
In June, Mr Wells travelled to Kansas City University Medical Centre to begin a six-month drug trial with American pharmaceutical company BioMarin.
"I have put on 10kg and I just feel fantastic," he said. In August, a drug trial site re-opened in Adelaide and now Mr Wells travels there once a fortnight to receive 4.5-hour long treatments of the intravenously administered drug, BMN 701.
On January 2, Mr Wells signed a five-year treatment plan for the enzyme replacement therapy.
He said there were risks involved, but as the drug was not available in New Zealand, he had no choice.
"I feel very lucky that we are part of the trial but there's consequences attached to that - it's not just a trip around the corner. For some people it means they can't work, I am lucky that I'm not in that position but there are other people that are."
Mr Wells works for Napier City Council at the National Aquarium of New Zealand in Napier.
His business, Art Deco Pets, will close on Thursday.
"The council have been brilliant, but I had to close down the pet shop because my wife couldn't run that by herself successfully and I can't spend the time there that I used to do."
Pharmac Medical Director Peter Moodie said extensive research indicated the benefit was not strong enough to justify funding for Pompe disease.
"Treatments need to be assessed on their merits and supported by good-quality evidence demonstrating benefit, in order for funding to be justified," he said.