Alpha-1 Liver Disease Treatment

Reviewed by Karina A. Serban, MD, Robert A. Sandhaus, MD, PhD, FCCP

What is the treatment for Alpha-1 liver disease?

The cirrhosis associated with adult Alpha-1 liver disease should be treated the same as cirrhosis of any cause. This includes treatments to monitor portal hypertension (elevated blood pressure in the circulation of the liver); monitoring and treatment of esophageal varices; adjustment of medication doses to account for changes in metabolism of drugs in an injured liver; and other measures. If the liver disease continues to worsen in spite of these measures, liver transplantation is the only potential cure.

Severe infant liver failure in Alpha-1 is always treated with liver transplantation, which cures the disease by replacing the failing liver with a normal donor liver that has normal Alpha-1 genes. A successful liver transplant leads to normal blood and lung levels of normal alpha-1 antitrypsin protein. Liver transplant, in general, is a very successful procedure, but the availability of donor livers is outstripped by the demand. Sadly, some people who need a transplant may not be able to get one in time.

Children and adults diagnosed with one or two abnormal Z genes should be educated to avoid alcohol and medications or herbal products with liver toxicity. They should be immunized against hepatitis A and B, and against hepatitis C, when that immunization becomes available.

Family members of people with one or two abnormal Z genes should receive genetic counseling, as an early diagnosis of Alpha-1 liver disease in family members will have a significant effect on their prognosis.
People with cirrhosis may receive treatment with:

  • vitamin supplements to help with clotting disorder and blood cell count

  • diuretics to help with the fluid retention

  • antibiotics to prevent infections of the ascites fluid or absorption of bacterial products from the gut

  • propranolol (a medication)

  • bands/clips/sclerosis (treatments for varices in the esophagus using a scope) or TIPS (transhepatic intraportal shunt, a surgical procedure) to help decrease the blood flow from the liver to the esophageal varices.

 

How does Alpha-1 liver disease affect my life expectancy?

If you have one Z Alpha-1 gene and no liver disease, you have a normal life expectancy.

A quarter of people with two Z Alpha-1 genes will develop liver disease at some point in their lives. If the Alpha-1 liver disease progresses rapidly towards cirrhosis of the liver with complications, and you are not eligible for liver transplantations, then life expectancy can be greatly decreased.

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