Alpha-1 antitrypsin deficiency in the lungs requires lifelong treatment and monitoring. In most cases care is provided by a specialist for lung diseases known as a pulmonologist. Tip: You can find a directory of doctors who are closely involved in the care and treatment of alpha-1 patients and are therefore very familiar with the clinical picture of alpha-1 antitrypsin deficiency at www.alpha-1-center.de.

Treatment approaches
1. Treatment and improvement of symptoms
Pre-existing lung disease, i.e. alpha-1 pulmonary emphysema, is treated in accordance with the guidelines for chronic obstructive pulmonary disease (COPD). Preventive, pharmacologic and non-pharmacologic measures are available.

Prevention

• Become a non-smoker. The substances in cigarette smoke damage the epithelium in the airways responsible for clearing the bronchi (cilia) and render protective proteins ineffective. Above all, they reduce the effectiveness of (infusion) therapy with alpha-1 antitrypsin. Passive smoking is also harmful. You can find help on this subject here.
• Avoid harmful substances at your workplace (e.g. welding fumes).
• Avoid infections. This includes, for example, getting an annual influenza immunization and immunization against pneumococci (the bacteria most commonly responsible for pneumonia). You should treat infections early and consistently to prevent pneumonia. It is also a good idea to avoid contact with people who have a cold by avoiding public transport and crowds during times of infection.
• Balanced diet
• Genetic counseling: Estimation of your risk of developing disease, early onset of treatment and planning for the future.

Non-pharmacologic measures

• Breathing exercises and physical exercise (see Rehabilitation and Exercise)
• Lung transplant for highly advanced disease stages
• Oxygen therapy for advanced disease (reduced oxygen supply to the body)

Drugs

• Bronchodilators (beta2 agonists or anticholinergics) in the form of sprays (metered-dose aerosols) or powder form inhalation when you have shortness of breath
• Cortisone for inhalation, which acts as an anti-inflammatory substance (for severe disease with frequent acute deterioration  = exacerbations)
• Cortisone in tablet form and/or antibiotics for moderate or severe exacerbations, also in combination with hospitalization

2. Specific therapy to impact further destruction of lung tissue
Depending on the severity of the disorder, it may be sensible and necessary to supply the body with the missing alpha-1 antitrypsin by means of regular infusions. This form of treatment is called augmentation therapy. Concentrated alpha-1 antitrypsin is obtained from the blood of healthy donors. The infusions increase the concentration of the missing substance so that the air sacs in the lungs are protected from further destruction. The decision as to which patients are suitable for this treatment is made by the treating doctor in accordance with regulatory conditions for the drug and national and international treatment guidelines.