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There are many ways you can help children and their families get the care they need.
There are two main reasons why biliary atresia is a problem:
1. It makes it harder for bile to get to your child’s small intestines. Without enough bile in the small intestines, two problems happen:
2. It causes liver damage. This may happen in two ways:
Children with biliary atresia have special nutritional needs. Our registered pediatric dieticians can make recommendations including:
If your baby can’t take in the amount of calories needed to maintain a normal growth rate, we may recommend liquid feedings. These are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus and into her stomach.
We know that biliary atresia is caused by inflammation and scarring of the bile ducts, but what causes the inflammation and scarring is still unknown.
Biliary atresia does not seem to be linked to anything the mother did during pregnancy, such as any medications she might have taken.
There are two types of biliary atresia:
1. embryonal biliary atresia
Infants with biliary atresia may be born with jaundice, but usually appear healthy at birth. Most often, you or your child’s pediatrician will notice signs within the first two weeks to two months of your child’s life. These may include:
Signs that may appear later include:
These are later signs, not usually in the first months of life.
While there’s no cure for the condition, there are ways for your child to have a longer and healthier life. The first step is surgery, called the Kasai portoenterostomy, in which the blocked bile duct is bypassed using a piece of your child’s intestine. Afterwards, special formulas and vitamin preparations, as well as other medications, can help your child grow and help to preserve her liver function.
Nonetheless, many children who have Kasai portoenterostomies early in life will need a liver transplant at some point.
Current national statistics confirm that about 85 percent of children who have liver transplants survive the first year, and most children who survive the first year go on to live into adulthood.
Q: My baby was born jaundiced. Should I be concerned?
A: Not necessarily – many babies have jaundice (yellow discoloration of the skin and whites of the eyes) in the first week or two of life. This is a different kind of jaundice from biliary atresia, and does not mean there is a liver problem.
But if the jaundice goes away and then returns, or lasts longer than 2 weeks, that's often a sign that something is wrong, and you should talk to your child’s pediatrician.
Q: What’s the long-term outlook if my child has biliary atresia?
A: Unfortunately, there are no medications that can unblock your child’s bile ducts or encourage new ducts to grow. Early surgery (Kasai portoenterostomy) and good medical care can help your child to live longer and have a better quality of life. If your child’s liver starts to fail, a liver transplant can be life saving.
Up to 66 percent of children with biliary atresia, even those who undergo the Kasai portoenterostomy, will eventually need to have a liver transplant.
Q: Can biliary atresia be detected before birth?
A: No, there is no prenatal test that can detect biliary atresia.
Q: If my child has biliary atresia, does that mean that I’m more likely to have another child with the condition?
A: No, researchers believe that biliary atresia isn’t genetic, and the condition doesn’t recur in families with any frequency. Even in identical twins, one infant may have biliary atresia and the other may not.
Q: What’s the difference between bile and bilirubin?
A: Bile is a golden yellow fluid made by your child’s liver. It’s stored in the gallbladder and passed through the common bile duct into the intestine, where it helps digest fat.
Bilirubin is a yellowish pigment that is found in bile. It is a chemical formed by breakdown of red blood cells.
Q: What causes jaundice?
A: Jaundice occurs when there is too much bilirubin in your child’s blood (hyperbilirubinemia). Because bilirubin is yellow, it causes a yellowing of the baby's skin and tissues.
Not all yellowing of the skin is jaundice, however. Some babies eat a lot of orange vegetables, such as carrots, and their skin may appear yellow or orange. In that case, the whites of the child’s eyes remain white, and blood tests show that the child’s bilirubin levels are normal.
Q: Why would there be too much bilirubin in my child’s blood?
A: The problem starts as your child’s old red blood cells are broken down. As this occurs, hemoglobin (the protein molecule in red blood cells) is changed into bilirubin and, in a healthy system, is removed by the liver. If the liver can’t remove the bilirubin, it gets backed up in the blood.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”