The only specific therapy for alpha-1 antitrypsin deficiency (AATD) is augmentation therapy. During this therapy, preparations of alpha-1 antitrypsin protein that have been isolated from pooled blood of healthy donors are given by weekly intravenous infusion. This elevates the blood levels of AATD to levels that are protective for the lung.
Well-designed studies have shown that augmentation therapy helps to preserve lung function and decreases the number and severity of lung infections.
There is no cure for alpha-1 antitrypsin deficiency. However, the lung diseases that it causes can be treated. The initial treatment is similar to that of emphysema, a type of COPD.
The treatment includes:
- Bronchodilators: makes breathing easier by relaxing the muscles around your airways. Bronchodilators can be short-acting or long-acting. Short-acting bronchodilators last about 4-6 hours and are used as needed. Long-acting bronchodilators last 12 hours or more and are used every day.
- Inhaled corticosteroid medications: can reduce inflammation of the airways, making it easier to breathe. They may have side effects, including bruising, oral infections and hoarseness.
- Antibiotics: should be given early for bouts of bronchitis and COPD exacerbation.
- Flu and pneumococcal vaccines: yearly flu vaccination and pneumococcal vaccination every 3-5 years is also recommended.
- Surgery and other treatments: in advanced disease there are surgical options available such as lung volume reduction surgery and bullectomy. There are also experimental procedures such as valve and coil placement into segmental bronchi, in an attempt to shrink the emphysematous areas and improve the function of the less affected areas of the lung.
Eventually, patients with very advanced emphysema may require lung transplant surgery. Lung transplantation involves removing a damaged lung and replacing it with a healthy lung from a deceased donor. Lung transplants can significantly improve lung function and quality of life, but it involves many risks, including infection and rejection.
Lifestyle Changes
Your doctor may recommend pulmonary rehabilitation. This involves a variety of methods designed to improve your well-being. It may include an exercise program, training in managing your condition, nutritional counseling, and psychological counseling. Pulmonary rehabilitation involves a team of specialists who work together to help you manage your condition and function at your best.
In addition, it is very important not to smoke if you have AAT deficiency. Smoking can speed up the lung damage associated with alpha-1 antitrypsin deficiency. You should also avoid things that irritate your lungs, such as pollution, dust, chemicals and ozone.
Finally, you should minimize your alcohol consumption to protect your liver, which is also affected by AATD.
Ready for an Appointment?
To learn more about treatment options for alpha-1 antitrypsin deficiency, schedule an appointment or call 800-TEMPLE-MED (800-836-7536) today.
