LIVER CENTER
Eleanor
Diagnosed shortly after birth,
biliary atresia

Eleanor doesn’t look sick. She doesn’t act sick either.

Ballet, art lessons and gymnastics occupy regular time slots on her busy schedule. In a sea of preschoolers, the talkative 4-year-old capped in blonde curls would be the last child pegged as the one with a mysterious and incurable disease.

Yet each minute of Eleanor’s ballet practice and every day she spends in preschool bring her closer to her presumable fate – a liver transplant.

“Eleanor will need a liver transplant before she is in her teens or early twenties because of her condition,” said Saul J. Karpen, M.D., Ph.D., chief of service at Texas Children’s Liver Center and Biliary Atresia Clinic at Texas Children’s Hospital.

That condition is biliary atresia, a progressive liver disease characterized by the liver’s inability to drain itself of bile. In a healthy liver, bile drains into the small intestine through a network of tubes and ducts where it aids in digestion.

With biliary atresia, the ducts and tubes begin to wither and disappear, trapping bile inside the liver. Without the ability to drain bile, the liver saturates beyond its capacity and causes scarring and eventual death of the liver cells.

Simply put, Eleanor’s liver is slowly poisoning itself.

A temporary antidote
Two days after Eleanor was born, an alert pediatrician noticed she was slightly jaundiced; further tests indicated a more serious condition.

“Biliary atresia in its early stages can be mistaken for newborn jaundice because of the yellow skin,” explained Karpen.

However, biliary atresia doesn’t go away – and is fatal if not properly diagnosed within two months of life. On average, biliary atresia occurs in one out of every 8,000 to 15,000 live births and affects mostly females; it is the leading cause of cirrhosis (liver scarring) and the most common reason for liver transplantation in children. The only long-term treatment option for biliary atresia is a liver transplant.

In the United States, approximately 300 to 500 new cases are reported annually yet its cause remains unknown, said Karpen.

A liver biopsy confirmed the devastating news that Eleanor had biliary atresia.

“When we asked where to go to get the best treatment – and we would have gone anywhere in the world – we were told Texas Children’s Hospital," said Eleanor’s mother, Harriet. “Fortunately for us, it was in our backyard.”

At six weeks old, Eleanor underwent the Kasai surgical procedure to attach the small intestine to the liver to drain the excess bile. The Kasai procedure isn’t a cure – just a potential way to keep the disease from progressing. It successfully slows the effects of biliary atresia in about 80 percent of children who undergo the procedure, said Karpen.

Four years after her Kasai procedure, Eleanor lives a normal life. Her biliary atresia still slowly progresses, but her parents and physicians carefully manage it.

The waiting game
In spite of all preventive care, Eleanor’s liver continues to harden and develop scar tissue. When scar tissue develops to a certain point, explained Karpen, the liver can no longer function.

For children like Eleanor, a liver transplant is the closest thing to a cure.

Eleanor will receive her liver transplant when keeping her own liver is more dangerous to her health than surgery to receive a new one, Karpen said.

As a national leader in liver transplants, Texas Children’s Liver Center survival rates rank among the nation’s best at 94 percent. The national average is 85 percent, according to John Goss, M.D., director of pediatric liver transplant service at Texas Children’s Liver Center and associate professor of liver transplant surgery at Baylor College of Medicine.

Next to the skin, the liver is the human body’s largest organ. From its perch just under the diaphragm, on the right side of the abdomen, the liver is the body’s “multi-tasker,” performing more than 500 functions including:

• Cleansing the blood of poisons and waste.

• Synthesizing important proteins contained in blood plasma, including clotting agents and some antibodies.

• Producing globin, one of two components of hemoglobin, the substance that allows red blood cells to carry oxygen.

• Regulating the levels of many chemicals found in the blood.

• Storing reserves of vitamins and nutrients.

• Producing bile, a key fluid in the digestion of fats.

• Regulating water distribution between the blood and tissues.

• Storing and releasing energy in the form of glucose, the key blood sugar that fuels our cells.

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