Treating and Managing Alpha-1 Antitrypsin Deficiency

How AAT Deficiency Is Treated

AAT deficiency may or may not affect your long-term survival. If you are diagnosed with AAT deficiency, your doctor may or may not suggest treatment based on the results of other testing and the severity of your symptoms. Patients who are diagnosed with AAT deficiency before symptoms occur usually have better outcomes than those who are diagnosed at later stages.

If you have no symptoms, you may be advised to return for regular follow-ups. You should restrict alcohol consumption, get regular exercise and control your weight. You should get a flu shot every year and ask your doctor about getting a pneumonia vaccine.

If your AAT deficiency has caused lung disease, and you are a current smoker, it is essential that you quit smoking and avoid secondhand smoke. Additionally, you should protect yourself from environmental dusts or workplace exposure to toxic substances. Your treatment may include prescribed inhaled medications to control symptoms of COPD, referral to a pulmonary rehabilitation program, oxygen therapy, and antibiotics and inhaled corticosteroids to control symptoms of flare-ups, infections or exacerbations if needed.

If you are not a current smoker and your lung function is rapidly declining, you may be considered eligible for AAT replacement therapy. Replacement therapy will increase blood levels of AAT, but it is not clear that it leads to longer-term improvements in lung function or survival. Replacement therapy is given intravenously. Some patients with advanced lung disease may be referred for lung volume reduction surgery (LVRS) and lung transplantation.

More information

For more information about AAT deficiency is available from the Alpha-1 Foundation. The Alpha-1 Foundation has information about a nationwide network of affiliated support groups for alpha-1 patients and families.

Finding Support

Communicate regularly with your doctors about changes in your breathing and general health. The Lung Association recommends patients and caregivers join our Living with Lung Disease Support Community or attend Better Breathers Club meetings to connect with others facing this disease. You can also call the Lung Association's Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with additional support

Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel.

Page last updated: March 24, 2020

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