'We could be dead in 6 months': Chronic lung sufferers on future without life-saving drug

John Hannon from Mallow in Co. Cork, who suffers from the condition, is only the second person in the world to use this drug 10 years ago.

John told Patricia Messinger on C103’s Cork Today show that the announcement was "life-shattering news" for the 17 people who are presently taking the drug in the Republic of Ireland.

Explaining that there were another 20 people to go on the drug in the near future, Mr Hannon outlined the difference between how much the HSE were prepared to pay, and how much the company wanted for the drug.

He said: "There is still a massive void between them, to our knowledge."

He went on to say that it was frustrating for the sufferers who are only interested in maintaining their health with the drug which has made such a difference to them.

He said: "It (the drug) has turned me from being almost an invalid 10 or 11 years ago when I was able to get a free disability parking pass because I wasn't able to walk from my car to the railway station or my car to the office. I was constantly in and out of hospital, I was hardly ever without a chest infection, I used to get bouts of coughing that lasted 20 to 30 minutes.

"Today, I am 68 years of age and I could almost do a day's work, I'm so good at the minute. I hardly ever get any bouts of coughing, if any, and I haven't been in hospital since 2010 for an Alpha 1-related illness."

He confirmed that the other patients on the drug have also had similar improvements to his.

He said: "In fact one or two of them will tell you that they may not be alive today if they hadn't got on it, and I may not be alive today if I hadn't gone on it. When I was 58 years of age, I was told by my consultant that 'you may not see your 60th birthday'.

"That has been turned around 100%."

He said that it is almost certain that he will have to come off the drug on September 30, but he doesn't know what will happen after that.

John said: "My last infusion will be on the 25th of September and nobody knows ... nobody has ever been taken off it after such a long period of time. There is also the risk that up to 2006 our bodies did not have any Alpha 1 protein in them and our bodies got used to handling life without having Alpha 1 in their system to protect them and the lungs from the environmental damage.

"But now having had protein in our bodies for 10 years, our bodies may have become dependent on that protein and they may deteriorate awfully quickly."

The Corkman, who originally helped CSL Behring to prove that their drug works by going on the clinical trial, feels that the company owes the 17 people on the drug something.

"I also beg the HSE to come off their high horse, think of the people who have paid taxes in this country. They are allowing our health service to become a third-world health service and the HSE is not fit for purpose anymore.

"I also believe that the Health Minister has this ability to closet himself and not look outside the box. He is allowing 17 people to go into the unknown.

"Some of us might last for 10 years, some of us could be dead in six months. He is playing God with our lives."

In a statement last week CSL Behring said: “We remain committed to AATD research to improve the quality of life of people living with the condition. Based on this commitment, we are currently in discussion with health authorities in both the US and Europe to finalise a randomised, non-placebo controlled study design which seeks to demonstrate the long-term safety and efficacy of Respreeza.

“CSL Behring is evaluating clinical sites in Ireland which may then be offered the opportunity to recruit severe AATD patients.”