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Alpha-1 Antitrypsin Deficiency (A1ATD) Treatment

The only specific therapy for A1ATD is augmentation therapy. During this therapy,  preparations of A1AT that have been isolated from pooled blood of healthy donors are given by weekly intravenous infusion, thereby elevating the blood levels of A1ATD of the deficient patient 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 A1ATD. However, the lung diseases that it causes can be treated. The initial treatment is similar to that of COPD and emphysema.

The treatment includes:

  • Bronchodilatorsmake 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 & Other Treatments: in advanced disease there are surgical options available such as 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 a 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

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 A1ATD. Smoking can speed up the lung damage associated with A1ATD.

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 A1ATD.