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.