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How is Alpha-1 liver disease treated?

At this time, there are no specific treatments for Alpha-1 liver disease. In its most severe form, the only treatment is liver transplantation. Also, there is no treatment to prevent the onset of the liver disease. The focus of care is on managing health problems as they come up and keeping patients as healthy as possible. All patients with Alpha-1 should be immunized against hepatitis A and B. They should also have regular physical exams, liver function tests and abdominal ultrasound exams. People 50 and older who have decompensated (worsening) cirrhosis due to Alpha-1 are at increased risk for hepatoma (“liver cell cancer”). As a result, they should get periodic CT imaging of the liver. Staying away from tobacco smoke and alcohol while eating a nutritious, well-balanced diet is also important.

Unlike lung disease caused by Alpha-1, there is no role for “augmentation therapy” — periodic injections or doses of the missing or deficient AAT protein. This therapy does NOT help the liver.

Liver transplantation is surgery to remove a sick liver and replace it with a healthy one. A transplant is needed when a patient’s diseased liver gets worse over time until it is working so poorly that the patient may die. Most often, more than one doctor will decide if a person needs a liver transplant and if it is safe for them to have one. It can take a long time to get a healthy liver. The liver usually comes from someone who has just died. However, sometimes part of the liver from a living person is used. Due to the lack of donated organs, there is no guarantee that a donated liver will be available. For this reason, the decision to put someone on a transplant waiting list may be made long before a person truly needs one.

The Liver and Alpha-1 Antitrypsin Deficiency – Alpha-1 Foundation