Prem Menon, M.D. Vimla Menon, M.D.
Asthma, Allergy and Immunology Center
In the lungs, white blood cells produce an enzyme that destroys invading germs and digests damaged or aging cells. The alpha-1 protein neutralizes this enzyme to ensure that it does not overdo its job and only attacks aging or damaged cells. At least 100,000 people in the US are deficient in the alpha-1 protein. This deficiency causes the enzyme to be overactive in the lungs destroying healthy lung tissue. This is known as Alpha-1 Antitrypsin Deficiency.
Alpha-1 Antitrypsin Deficiency is often confused with asthma or chronic obstructive pulmonary disease (COPD), causing Alpha-1 Antitrypsin Deficiency to be undiagnosed an estimated 90% of the time.
Symptoms of Alpha-1 Antitrypsin Deficiency Include:
Severe, persistent asthma, early-onset emphysema or COPD, and damage to the lower lungs are consistent with Alpha-1 Antitrypsin Deficiency. A blood test should be performed to confirm diagnosis.
While there is no cure, there are several treatments available. A patient with Alpha-1 Antitrypsin Deficiency may receive similar treatment as patients with asthma or COPD. Bronchodilator inhalers help to open the airways and inhaled corticosteroids help reduce inflammation.
The deficient alpha-1 protein may also be replaced through weekly intravenous infusions known as augmentation therapy. Augmentation therapy can help slow the progression of lung tissue destruction.
It is recommended that all patients with COPD or asthma with airway obstruction that is not completely reversible through standard treatment should be tested for Alpha-1 Antitrypsin Deficiency. It is important for patients who have the Alpha-1 Antitrypsin Deficiency phenotype or the heterozygote phenotype to stop smoking immediately, as smoking can significantly accelerate lung tissue destruction.
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