Alpha₁-antitrypsin deficiency, also known as Alpha-1, is an inherited disorder that causes a significant reduction in the naturally occurring protein alpha₁-antitrypsin (AAT). Reduced levels of AAT result in the body being less able to protect itself from elastase, an enzyme in the white blood cells that normally helps to fight bacteria and that can cause damage to the air sacs in the lungs if not neutralized by AAT. AAT deficiency may predispose an individual to several serious illnesses, most commonly emphysema beginning in the third or fourth decade of life. Less frequently, AAT deficiency can result in liver disease, or a skin condition called panniculitis.

It is believed that AAT deficiency affects as many as 200,000 people in North America and Europe. AAT deficiency is most common among Caucasians of Northern European descent. It is estimated that as many as 95% of people with Alpha-1 have not been identified.

There are many components to treating AAT. There is a specific pharmacologic treatment that helps restore, through replacement therapy, the natural balance of enzymes in the lungs, protecting them from the damage caused by neutrophil elastase. For 15 years, our proteinase inhibitor product, Prolastin^®, Alpha₁-Proteinase Inhibitor (Human), was the only augmentation therapy available for persons suffering from alpha₁-antitrypsin deficiency.

View the Prolastin product monograph.

Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor if you have any questions about your health, your symptoms, or your therapy.

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