Treatment for AAT Deficiency
For people who have begun to show symptoms of AAT related emphysema, replacement (augmentation) therapy, may be a treatment option that can help protect the lungs against the destructive enzyme, neutrophil elastase.
Replacement therapy consists of giving a concentrated form of AAT that is derived from human plasma. It raises the AAT level in the bloodstream. Once you start replacement therapy, however, you must undergo treatment for life. This is because if you stop, your lungs will return to their previous level of dysfunction and the neutrophil elastase will again start to destroy your lung tissue.
Not only does replacement therapy help slow the loss of lung function in people with AAT related emphysema, but it may also help to reduce the frequency of lung infections. In a study published in Chest, replacement therapy was shown to have a strong relationship with a significant reduction in the frequency and severity of lung infections associated with AAT related emphysema. The study concluded that while emphysema remains irreversible, less frequent, severe lung infections can help slow the progression of AAT related emphysema and lead to a higher quality of life.
For more information about AAT deficiency or replacement therapy, talk with your healthcare provider or visit the American Lung Association or the Alpha-1 Foundation.
Sources:
American Lung Association. Alpha-1 Related Emphysema http://www.lungusa.org/site/pp.asp?c=dvLUK9O0E&b=35014. November, 2006.
American Thoracic Society. American Thoracic Society/European Respiratory Society Statement: Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency".
ATS: December 2002. ERS: February, 2003.
Alpha-1 Foundation. http://www.alphaone.org/
Lieberman, J. "Augmentation Therapy Reduces Frequency of Lung Infections in Antitrypsin Deficiency: A New Hypothesis With Supporting Data". Chest 2000; 118; 1480-1485.
Stoller, J., M.D., FCCP. "Clinical Features and Natural History of Severe Alpha-1 Antitrypsin Deficiency". Chest 1997; 111: 123S-128S.
