People affected by the most common genetic lung disorder in Ireland have expressed their ‘huge disappointment' at a decision by the Minister for Health not to fund the only therapy which has been shown to slow the progression of the disease.

Alpha-1 (alpha-1 antitrypsin deficiency), also known as genetic emphysema, can cause severe problems with the lungs, liver and skin.

Alpha-1 antitrypsin is a protein, the main function of which is to protect the lungs from infection and irritants such as tobacco smoke. A deficiency of this protein can lead to symptoms such as breathlessness, wheezing and coughing with phlegm.

Almost 350 people in Ireland have been diagnosed with the severe form of Alpha-1, and the Minister for Health's decision specifically affects 17 patients, who have been taking the drug, Respreeza.

Respreeza is administered once a week by intravenous infusion. It works by replacing the protein that is lacking in patients, slowing down damage in their lungs.

The 17 patients had previously been taking the drug as part of a clinical trial, which ended in 2010. They had then continued to receive it on a ‘compassionate use' basis, pending the outcome of the drug manufacturer's reimbursement application to the State.

However on Wednesday, the Minister for Health, Simon Harris, announced that this therapy would not be funded by the State. The manufacturer, CSL Behring, has agreed to provide the drug for an additional two months to allow for further discussions to take place.

According to the Alpha One Foundation, the advocacy body for people affected, it is ‘hugely disappointed' with this decision.

"The foundation is at a loss as to how the Minister cannot recognise the clear benefits of this therapy, as evidenced by the patients who have been receiving it for over a decade. If only the Minister would meet some of the patients who have been receiving the therapy, he would see and hear first-hand the difference it has made to their lives," commented the foundation's CEO, Kitty O'Connor.

Respreeza has already been approved by the European Medicines Agency and is available in eight European countries, as well as in the US.

"The European Medicines Agency and these other countries see the benefits, so why can't the Minister? Patients are devastated at this decision which, if left to stand, is likely to have a hugely detrimental impact on their future health.

"We call on the Minister to adhere to his statutory responsibility to safeguard the health of all of our citizens. We also call on the Minister to meet our patients and to hear what they have to say. These patients have already endured more than their fair share of challenges in life. We ask the Minister to please not give up on them now," Ms O'Connor added.

For more information on the Alpha-One Foundation, click here