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Lung disease is the most common problem associated with a deficiency of alpha1-antitrypsin (AAT). AAT is produced by the liver and shields the body from damage caused by neutrophil elastase. Neutrophil elastase is an enzyme produced by white blood cells.
Under normal conditions, neutrophil elastase helps fight bacteria that cause infection. However, if not neutralized by AAT, neutrophil elastase can destroy healthy lung tissue.
Alveoli are tiny air sacs in the lungs, which are responsible for taking in oxygen and releasing carbon dioxide. When adequate levels of AAT are not present, the enzymatic activity of neutrophil elastase is not neutralized and the fine elastic tissue supporting the alveoli is destroyed. Over time, enough alveoli are destroyed to cause the lungs to lose much of their elasticity, resulting in emphysema. Therefore, people with a deficiency of AAT are at high risk for developing emphysema.
Emphysema is a condition in which there is an over inflation of structures in the lungs known as alveoli or air sacs. This over inflation results from a breakdown of the walls of the alveoli (as shown in the top right picture), which causes a decrease in respiratory function and often breathlessness.
Important Safety Information
Prolastin®, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 PI (alpha1-antitrypsin deficiency) with clinically demonstrable panacinar emphysema. Weekly Prolastin® therapy has demonstrated a low occurrence of side effects. In clinical studies with Prolastin®, reactions were observed in 1.16% of infusions, the most common events being fever (0.77%), light-headedness (0.19%), and dizziness (0.19%). As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated. Individuals with selective IgA deficiencies who have known antibody against IgA (anti-IgA antibody) should not receive Prolastin®, since these patients may experience severe reactions, including anaphylaxis, to IgA which may be present.
You are encouraged to report negative side effects of prescription drugs
to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Please click here for Prolastin® full Prescribing Information.
Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor or healthcare provider if you have any questions about your health or any of your medications.
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