Alpha-1 antitrypsin deficiency, also known as Alpha-1, is an inherited disorder that causes a significant reduction in the naturally occurring protein alpha-1 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 the 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. One of the available treatments is Prolastin® Alpha1-Proteinase Inhibitor (Human). For 15 years, Prolastin, our proteinase inhibitor product, was the only augmentation therapy available for persons suffering from alpha-1 antitrypsin (ATT) deficiency.
Prolastin,derived from human plasma, is a concentrated form of AAT. Given as prescribed, Prolastin raises the blood and lung levels of AAT. This may help lessen damage to the lungs caused by the enzymatic activity of neutrophil elastase. Because Prolastin therapy augments or replaces AAT, it is known as "augmentation" or "replacement" therapy.
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The most commonly reported side effects with Prolastin,are delayed fever, light-headedness, and dizziness.
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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