Although there is currently no cure for alpha-1 antitrypsin deficiency, and at present no specific treatment, early diagnosis and education for the patient on having a healthy lifestyle and the need for prompt medical attention at the first signs of lung infection are vitally important. Treatment is mainly supportive aimed at reducing symptoms and attempting to slow the progress of disease.
Alpha1 Lung
In patients with an Alpha1 lung condition supportive treatment is given as for other patients with chronic lung disease which include Inhalers, Antibiotics, Healthy eating, Lifestyle, Rehabilitation, Supplementary oxygen, Surgery options.
Preventing or slowing the progression of lung disease is the major goal of AAT deficiency management. It is most import to avoid anything which stimulates inflammation of the alveoli (the small air-sacs in the lungs). The major irritants are smoking, asthma and respiratory infections.
Most patients are identified only after they develop lung disease, and the goals of treating AAT deficiency emphysema are similar to those for treating other forms of emphysema.
In the United States, Canada, and several European countries, lung-affected AATD patients may receive intravenous infusions of alpha1 antitrypsin, derived from donated human plasma which is known as augmentation therapy.
Although this therapy is not readily available in the UK recent trials using Prolastin (Produced by Talecris now Grifols) have been completed in Birmingham the results of which have proved Prolastin to be effective in slowing the progression of emphysema. However, augmentation therapy has been used successfully in the UK for the treatment of Panniculitis.
Alpha1 Liver
In patients with an Alpha1 liver condition there is no specific treatment and in its most severe form the only treatment is surgery. There is also no treatment to prevent the onset of the liver disease, augmentation therapy is not appropriate for liver-affected patients The focus of care is on managing health problems as they arise and keeping the patients as healthy as possible.
