Twenty-one years ago, Orla Keane was diagnosed with a rare genetic disorder called Alpha-1.
Orla first discovered she was a carrier of the genetic disorder when her symptoms were triggered by a cleaning agent.
She experienced a coughing fit, and asthma-like symptoms, which led to the diagnosis of a disease that only affects 60 people in Ireland today.
However, she was placed on the trial drug and became virtually symptom-free - "It seriously works, I just cannot understand how they can justify their decision."
Orla is understandably upset. Last week, the HSE announced that they would no longer be funding the drug that totally transformed her life and that of 20 other patients.
Orla, who has been part of the medicinal Alpha One trial for over ten years, has expressed her concern about the decision.
"I'm concerned about what my life will be like when I come off the medicine. I have been symptom-free for years - I'm genuinely afraid."
"We are being held to ransom at this stage, once we're taken off the drug it is a matter of time - the HSE would want to get our hospital beds ready."
Orla, whose young sister also carries the genes that trigger Alpha One, said that the medicine would not be costly, as it works.
"If we're taken off the drug, patients will wind up back in hospital, costing the state more money than the price of the drug. Since starting the drug, I have not been hospitalised once - but we're entering the unknown now."
The Alpha One Foundation is now calling on the Minister for Health to reconsider the decision not to fund Respreeza therapy for people with Alpha-1.
Currently, there are 21 people (including Orla) being treated with the drug on "compassionate-use" basis, with CSL Behring, the company providing the drug, urging the Government not to stop therapy.
Extreme regret has been expressed at the decision by the HSE to cease the reimbursement of the life-changing therapy, Respreeza.
The Foundation is calling on the Minister for Health to reconsider the decision. They said the health of 60 people now hangs in the balance.
Alpha-1 is a genetic condition that can cause severe lung disease. Respreeza is the only treatment https://t.co/7BBZowtteD pic.twitter.com/aH8MNT3ZtC
— Alpha One Foundation (@Alpha1_Ireland) April 26, 2017
Respreeza is the only therapy approved to treat the emphysema caused by severe Alpha-1 and is already available and reimbursed in twelve European countries.
Professor Gerry McElvaney, the Consultant Respiratory Physician involved in the clinical trial of Respreeza in Ireland, and Chair of the Alpha One Foundation, said more needs to be done:
"The drug approvals process is not working. The drug, Respreeza, has been shown to be effective in slowing progression of emphysema. The only question is one of cost. It is absolutely clear that those adjudicating on this matter are not in a position to comment or to make a decision on the efficacy of the drug. Respreeza definitely slows the progress of emphysema.
All of the 21 Irish patients have experienced an improvement in the quality of their lives. No Irish patient died during the study. If the European Medicines Agency and 12 countries in Europe agree that the drug works and is effective, why is the HSE saying it isn’t? The longer patients are not receiving this drug the more lung damage they will suffer. This lung damage cannot be reversed".
Orla and all others suffering from Alpha-1 are now entering into the unknown when it comes to their health. She says it's a scary prospect:
"Who knows what will happen, I have three weeks left of medicine, and then I'm on my own."
