Screen for Biliary Atresia to Save Lives

Screen for Biliary Atresia to Save Lives

Nemours Children’s Health experts are using a simple screening tool to catch biliary atresia early – here’s how you can too.

Biliary atresia is a rare and serious liver condition in newborns. It happens when the bile ducts inside and outside of the liver are blocked from inflammation and scarring, causing bile to build up and damage the liver.

This condition is treatable with swift diagnosis and appropriate care. But it’s often missed early on – which can cause a child to develop cirrhosis, portal hypertension, and liver failure.

Abnormal stool color is an early, and lifesaving, warning sign for biliary atresia. That’s why experts at Nemours Children’s are training health care providers and parents on using a simple stool screening tool to catch biliary atresia early.

What are the symptoms of biliary atresia?

Biliary atresia only affects infants. The exact cause is unknown, but it’s thought that inflammatory, viral, genetic or toxic factors may play a role.

Symptoms of biliary atresia show up within the first two to six weeks after birth. They can include:

  • Pale stools, including yellow, chalk white or tan in color
  • Dark urine
  • Jaundice
  • Failure to thrive
  • Enlarged spleen
  • Abdominal swelling

About 10–20% of infants with biliary atresia will also have severe congenital abnormalities that can involve the spleen, intestines and/or the heart and vascular system.

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Providing Screening Tools to Doctors and Patients

The pale stools seen in biliary atresia indicate there is a problem with bile reaching the intestines. Bile backs up and damages the liver, leading to scarring and other digestive problems.

To help identify pale stools, Nemours is distributing stool color screening cards and training nurses to share this vital information with new parents.  The cards show normal infant stool colors compared to stools that lack bile and are therefore “acholic.” If there is a problem, parents are encouraged to call the doctor right away or see an experienced pediatric hepatologist for evaluation.

Nemours began the biliary atresia stool screening program with local partner hospitals. They’re expanding the program to more hospitals so that infants can get the lifesaving care they need.

How is biliary atresia treated?

A surgical procedure, called a Kasai procedure, can significantly slow disease progression to cirrhosis and liver failure and allow children to live healthier lives. The earlier surgery is performed, the better the outcome. It’s best to get surgery within the first 30 to 90 days of life.

Surgery involves replacing damaged bile ducts with the loop of an infant’s small intestine to allow bile to properly drain from the liver. It takes weeks to months to measure surgery success.

Even with successful surgery, biliary atresia complications often continue. About 75% of kids will need a liver transplant by the time they become adults.


Liver Transplantation Options for Kids

Today, children who need liver transplants have more options than ever before, including living donor liver transplants and deceased donor liver transplants.

  • Living donor liver transplants are when organs, or partial livers in this case, are donated by a living person. They can come from a parent, a blood relative, or even an unrelated donor. With living donor transplants, doctors only need to transplant a small segment, or lobe, of an adult liver. The liver develops within a few months and grows with the child.
  • Deceased donor liver transplants are another option for kids. A liver donated from an adult can be split into two portions. Split liver transplants help more kids get liver transplants, more quickly.

About Our Transplant Center

Our solid organ transplant team based at Nemours Children’s Hospital in Wilmington, Delaware, partners with specialists across the country to provide excellent care for their patients with liver disease. We’re national leaders and innovators in pediatric liver transplants.

In fact, we’re among the first to perform living donor liver transplants in children. More than one third of our patients receive a living donor liver. We also have vast experience in deceased donor split liver transplantation, shortening the time to transplant for many kids.

When you refer patients to Nemours, you can trust they’ll get expertise and personalized care from the start. We begin evaluation quickly and help families through the transplant process. Our program offers:

Shorter liver transplant wait times. Our average wait time is 12 weeks, with a third of our patients receiving transplants within 30 days of being listed. Our pre-transplant mortality rate is zero percent.

 Care from some of the best in the nation. Our pediatric gastroenterology and GI surgery teams are recognized among the best in the country by U.S. News & World Report. Our renowned transplant surgeon is instrumental in building international transplant programs and sharing expertise as a professor, researcher and medical editor.

Highly advanced pediatric multidisciplinary care. We use an Integrated Practice Model that organizes a team of specialists who collaborate before, during and well after transplant. These include hepatologists, gastroenterologists, infectious disease specialists, anesthesiologists, pediatricians and many others.

Collaborations with recognized donor programs. Nemours teams work closely with adult transplant surgeons at Thomas Jefferson University to evaluate living organ donors safely and effectively.

Support team that sets us apart. Our advance practice providers and support team are highly skilled. Our transplant nurses have decades of experience guiding and nurturing families. We also have skilled dietitians, psychologists, social workers, Child Life specialists and many others who know the unique needs of transplant patients and families.

Meet Our Team

Refer a Patient: 302.651.4889

Learn More: Nemours Liver Transplant Program

Read More at Nemours KidsHealth: Biliary Atresia

 

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