BioRx, a national specialty pharmacy and infusion services provider, has been added to the limited distribution network for Zemaira® [alpha-1 proteinase inhibitor (human)]. Zemaira is indicated for chronic augmentation and maintenance therapy in individuals with alpha1-antitrypsin (AAT) deficiency and clinical evidence of emphysema. It was originally approved by the Food and Drug Administration (FDA) in 2003 and is one of only four FDA-approved treatments available today.
"We are very excited to be added to the Zemaira distribution network," said Eric Hill, co-founder of BioRx. "The addition of Zemaira enables us to extend our reach to more patients within the alpha-1 community. We are also excited to expand our partnership with CSL Behring into the alpha-1 marketplace."
AAT deficiency is an inherited condition that raises the risk of developing certain types of lung disease, such as emphysema, especially in smokers. People with AAT deficiency do not have enough of a protein called "alpha-1 antitrypsin" (also called "alpha-1 proteinase inhibitor") in their blood. AAT protein is produced by the liver and helps to protect the lungs from everyday irritants such as smoke, fumes, dust and pollen. Zemaira, a human-derived alpha-1 proteinase inhibitor, acts to inhibit the activity of another protein, neutrophil elastase, which, left unchecked, can degrade the lining of the lungs and lead to pulmonary disease.
"It's a great pleasure that BioRx will be able to serve the alpha-1 community by adding Zemaira to the current portfolio that includes Glassia," Firas Koura, MD, MPH, president of Kentucky Lung Clinic, PSC. "This will also provide a wider variety to choose from based on, first, our patients' needs and, second, their insurance requirements."
It is estimated that 100,000 to 120,000 people in the United States have the severe form of AAT deficiency, though only about 10,000 have been diagnosed. Many alpha-1 patients receive inadequate treatment for years as their condition continues to decline. They may be officially diagnosed with a form of COPD but have alpha-1 deficiency as the underlying cause.
"Medical opinion leaders say that alpha-1 deficiency is not so much of a rare disease, as one that is rarely diagnosed," said Hill. "Since serving our first alpha-1 patient in 2010, we have increasingly promoted targeted screening and detection efforts to help raise awareness of the disease and potential treatment options for diagnosed patients. We will continue to do so now with expanded treatment options and a new partner in this important therapy category."