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Sunday, May 29, 2016
Inherited Disorders and Birth Defects
Alpha 1 and Life Expectancy in Children
My grandson was diagnosed with alpha 1 at 6 weeks old. He is now 6 yrs old and had an esophagile bleed 2 weeks ago. He looks like the poster child of a starving child. We know he will probably need a liver transplant. What is the life expectancy of a child at this stage of alpha 1?
Although lung problems such as emphysema is a more common problem in people with alpha-1 antitrypsin deficiency (about 75% of people with alpha-1 have lung problems), only about 15% of patients have liver disease. In children, alpha-1 can show up as liver disease such as hepatitis that causes jaundice in newborns or children, or liver scarring (cirrhosis). The liver disease affecting children may be variable – some mild while others are more severe. But one of the most common reasons for a child to have a liver transplant is because of alpha-1 antitrypsin deficiency.
There are no good numbers to say what the life expectancy is in children with alpha-1 because it can be so variable. It really is based on what the general health of the child is and how often they have complications. The best people to help answer this question will be the specialists treating your grandson.
The Childhood Liver Disease Research and Education Network (website below) is a very good website that has additional information about alpha-1 antitrypsin deficiency. The Alpha1 Association is a national support group with information as well.
Anne Matthews, RN, PhD
Associate Professor of Genetics
School of Medicine
Case Western Reserve University