Biliary Atresia

WHAT YOU SHOULD KNOW:

• Biliary (bil-e-AR-e) atresia (ah-TRE-zhah) is a serious liver and bile duct disease that is seen in infants. This occurs when the bile ducts inside and outside the liver are blocked or missing. Bile flow from the liver and gallbladder going to the intestines (bowel) is affected. The bile gets trapped and stored up in the liver, causing damage to the liver cells. Over time, this scarring may prevent blood flow through the liver and make it stop working permanently. The exact cause of biliary atresia is not known. Infections, toxins (harmful substances), or problems with the immune system or how the bile ducts were formed may cause biliary atresia.
  
  
• Common signs and symptoms may appear shortly after birth or from 2 to 8 weeks after birth. Your baby may appear jaundiced (yellow skin or eyes). He may also have abdominal (belly) pain, an enlarged abdomen, very dark urine, or clay-colored stools (BMs). Abdominal ultrasound, x-rays, cholangiogram, liver or gallbladder scan, or liver biopsy may be used to diagnose biliary atresia. Treatment may include surgery or medicines to ease his symptoms. The earlier biliary atresia is found and treated, the better the chances that your baby may survive. With treatment, such as medicine and surgery, your baby's symptoms may be relieved and his quality of life may be improved.

CARE AGREEMENT:

You have the right to help plan your baby's care. Learn about your baby's health condition and how it may be treated. Discuss treatment options with your baby's caregivers to decide what care you want for your baby.

RISKS:

• Treatment for biliary atresia may cause unpleasant effects. Some medicines may cause nausea (upset stomach), vomiting (throwing up), drowsiness, high blood pressure, or kidney problems. Your baby could get an infection or bleed too much with surgery. Babies with biliary atresia are at higher risk for poor growth and nutrition. If left untreated, the blocked bile and toxic waste products may cause permanent liver or brain damage. This makes very few children survive beyond the age of two years.
  
  
• Sometimes, even if the ducts are restored by surgery, problems may still occur. These problems include infections, easy bleeding, increased pressure in the veins, or ascites (collection of fluid in the abdomen). The earlier biliary atresia is found and treated, the better the chances that your baby may survive. Ask your child's caregiver if you are worried or have questions about his disease, care, or treatment.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that your child may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your child's medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done to your child. Make sure all of your questions are answered.

Blood tests:

Your child may need blood tests to give caregivers information about how his body is working. The blood may be taken from your child's arm, hand, finger, foot, heel, or IV.

Diet:

A caregiver, called a dietitian, may talk to you about your baby's feeding and nutrition. You may also discuss with your baby's caregivers if it is OK to breastfeed your baby. High-calorie liquid feedings may be given through a tube called a nasogastric (NG) tube if your baby cannot drink milk. As he improves, he may be given soft foods that are rich in fiber. Some examples are applesauce, cooked cereal, yogurt, and other baby foods. Ask your baby's caregiver for more information about his feeding and nutrition.

Emotional support:

Stay with your child for comfort and support as often as possible while he is in the hospital. Ask another family member or someone close to the family to stay with your child when you cannot be there. Bring items from home that will comfort your child, such as a favorite blanket or toy.

IV:

An IV is a small tube placed in your child's vein. Caregivers use the IV to give your child medicine or liquids.

Medicines:

Your baby may need one or more of the following:

• Antibiotics: This medicine is given to help prevent or treat an infection caused by bacteria.
  
  
• Anti-itching medicine: Caregivers may give your baby this medicine to help keep his skin from itching. This may be given in an IV, as a shot, by mouth, or as a skin lotion. Sometimes this medicine can make him sleepy.
  
  
• Biliary agents: These medicines work by decreasing the fats released by the liver into the bile.
  
  
• Diuretics: These medicines help get rid of extra fluid in your baby's abdomen, lungs, or brain. He may urinate more often when taking diuretics.
  
  
• Steroids: Steroid medicine may be given to decrease inflammation (swelling). There are many different reasons to take steroids. This medicine may have side effects. Ask your child's caregivers about steroids.
  
  
• Vitamin supplements and special formulas: Vitamins A, D, E, K, and B complex may be given to make sure your baby is getting enough nutrition. Special formulas, that have easy to digest fats, may also be given to your baby.

Nasogastric tube:

This is also called an NG tube. An NG tube is put into your baby's nose and down to his stomach. This keeps air and fluid out of the stomach during surgery. Your baby may need an NG tube if his abdomen gets too full or if he throws up a lot after surgery. Food or medicine may also be given through an NG tube.

Neurologic signs:

These are also called neuro signs, neuro checks, or neuro status. During a neuro check, caregivers see how your child's pupils react to light. They may check his memory and how easily he wakes up. His hand grasp and balance may also be tested. How your child responds to the neuro checks can tell caregivers if his illness or injury has affected his brain.

Tests:

Your baby may need one or more of the following tests:

• Abdominal ultrasound: An abdominal ultrasound is a test that looks inside of your baby's abdomen. Sound waves are used to show pictures of his liver, gallbladder, and bile ducts on a TV-like screen.
  
  
• Abdominal x-rays: Abdominal x-rays are pictures of the organs inside your baby's abdomen. Caregivers use these pictures to look for problems, such as an enlarged liver or spleen or blocked intestines.
  
  
• Cholangiogram: This test checks to see if the bile ducts are open or blocked. A dye is injected into your baby's gallbladder and goes through the bile ducts. X-rays are taken to learn if the dye flows normally into the small intestines and the liver.
  
  
• Liver and gallbladder scan: This test may also be called a HIDA scan. This is a test to look at the liver and gallbladder. Your baby will be given a small amount of radioactive dye in his IV. Pictures are then taken by a special scanner that can see the dye in his body. Caregivers look at the pictures to see if his liver and gallbladder are working normally.
  
  
• Scintigraphy: This is a test to look at the flow or movement of blood through the liver and bile ducts. Your baby is given a small, safe amount of dye in an IV. Pictures are then taken of his abdominal organs and blood vessels.

Treatment options:

Treatment for biliary atresia aims to restore a normal bile flow, to relieve symptoms, and prevent serious problems. Caregivers may do a surgery to make an open bile duct so bile can drain from the liver. Success is more likely if this surgery is done before the baby is two months old. If this surgery fails, a liver transplant may be needed. This is done by removing your baby's whole liver, or a part of your baby's liver, and replacing it with a healthy, donated liver.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

Learn more about Biliary Atresia (Inpatient Care)