Welcome! Our first son, Anthony, was born in Nov 2003 with a rare liver disease called Biliary Atresia. He was transplanted with the left lobe of his Daddy's liver in March 2004. Our second son, Benjamin, was born in June 2008. Check often for updates on our two boys and the other goings on in our household.
Biliary atresia is a progressive inflammatory process that begins very soon after birth. In the most common form, called extrahepatic biliary atresia, the delicate ducts outside the liver are affected first. White blood cells invade the ducts, which become damaged and may whither or completely disappear. Bile is trapped inside the liver and rapidly causes damage and scarring to the liver cells. Further scarring of the liver tissue may result in cirrhosis.
On average, there is one case of biliary atresia out of every 15,000 live births. Females are affected slightly more often than males. In the United States, approximately 300 new cases are diagnosed each year.
The cause of biliary atresia is unknown. Auto-immune mechanisms may be partly responsible for the progressive process that takes place. Recent research suggests that a viral infection in susceptible infants could result in biliary atresia. About 10% of cases have other associated congenital defects in the heart, blood vessels, intestine, or spleen.
Although the cause is uncertain, it is known that biliary atresia affects only newborns; it is not hereditary; it is not contagious; and it is not preventable.
A surgical procedure called the Kasai (hepatoportoenterostomy) can be performed in hopes of saving the liver. With the Kasai, the liver is connected directly to the small intestine, circumventing the gall bladder. The Kasai is usually successful in 1/3 of cases, while the other 2/3 result in the need for a liver transplant. Anthony's team of doctors were unable to diagnose Anthony with biliary atresia, and the Kasai was never performed. We learned after his transplant that the Kasai would never have worked, and we would have traveled the same road to transplant.
1 comment:
Such a cutie.
I swear, Laurie, when I look at these pictures, he reminds me so much of John M. I kind of look at Anthony as a way of seeing what John will look like a little down the road.
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