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Respreeza (Zemaira) is indicated for maintenance treatment, to slow the progression of emphysema in adults with documented severe Alpha-1 antitrypsin deficiency (AATD) or Alpha-1. It is currently undergoing a health technology assessment by the National Centre for Pharmacoeconomics (NCPE).
Respreeza has been licensed for use in the EU since 2015 and was the subject of a clinical trial in Ireland between 2006 and 2014. Since then, a total of 21 patients here have been offered access to the therapy by its maker, CSL Behring, on a compassionate-use basis. It is estimated that a further 40 patients could benefit from the treatment, which is given in a weekly IV infusion.
Alpha-1 is the most common genetic lung disorder in Ireland and there are currently around 350 people here who have been diagnosed with severe Alpha-1.
The majority of people with Alpha-1 present with emphysema or chronic obstructive pulmonary disease (COPD), explained leading researcher on the condition Prof Gerry McElvaney, Consultant Respiratory Physician and Head of the National Alpha-1 Centre at Beaumont Hospital, Dublin.
Respreeza works by replacing the protein that is lacking in these patients and is currently the only available treatment that targets the underlying condition, rather than the symptoms of severe Alpha-1, he said.
Alpha-1 Foundation CEO Ms Kitty O’Connor said Irish patients using Respreeza have reported that it significantly improves their quality of life by slowing the progression of emphysema, reducing the frequency and severity of chest infections and associated hospital admissions, and thus increasing life expectancy. She told the Medical Independent she is “hopeful of a positive recommendation” by the NCPE.
While the proposed Irish price has not been revealed, Respreeza costs between €60,000- €90,000 per patient per year in other countries.