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The University of Chicago Medicine - Comer Children's Hospital

On the Mend from Biliary Atresia

Many busy young families rely on a to-do list. But for Leah and Matthew Woodcock of Montgomery, Ill., checking off items on their refrigerator dry erase board was a matter of life and death.

Their lengthy list detailed medication reminders as well as their infant daughter Elizabeth's ever-changing tube and bottle feedings. "Looking back, I'm not sure how we did it," Leah said. "But, thanks to prayer and the medical team at Comer Children's Hospital, our daughter is now healthy."

Soon after she was born on May 22, 2013, a test showed abnormally high bilirubin levels in Elizabeth's blood. Bilirubin is excreted out of the liver in bile, a substance that aids in digestion. Elevated bilirubin levels in the bloodstream cause jaundice (yellowing of the skin) and indicate inflammation of the liver or blockage of the bile ducts.

The Woodcock family

Frequent heel sticks on the newborn in subsequent weeks showed the bilirubin numbers trending down. "She was just a little jaundiced," said Leah, "but feeding her and getting her enough nutrition was a struggle, even though I was breastfeeding, pumping and supplementing with formula."

At Elizabeth's two-month check up, the bilirubin level in her blood was 7 (normal range is 0.1 to 1). She weighed a little less than 10 pounds, placing her in the 5th percentile for her age. She was admitted to Comer Children's Hospital under the care of pediatric liver specialist Ruba Azzam, MD, and pediatric surgeon, Andrea Lo, MD.

Azzam performed a biopsy of Elizabeth's liver that was highly suggestive of the suspected diagnosis: biliary atresia, a chronic and progressive disease. Because the bile ducts outside of the liver were blocked, bile could not exit the infant's liver. The biliary atresia was causing liver damage and affecting other vital functions in the body.

Members of Elizabeth’s care team, Pamela Boone, RN, MSN (left); Ruba Azzam, MD, director of pediatric hepatology (center); and pediatric surgeon Andrea Lo, MD.

Azzam referred Elizabeth to Lo for the Kasai procedure -- an operation in which the damaged bile ducts are removed, and a segment of small intestine is sewn directly to the liver to allow for bile drainage.

"Time was of the essence," said Azzam, noting that biliary atresia is the most common indication for liver transplantation in pediatric patients. The Kasai procedure is most effective if performed in the first 45 to 60 days after birth, before the disease has progressed beyond repair. For about 20 percent of infants who undergo the surgery around the age of two months, the procedure establishes a good bile flow out of the liver, and delays the need for liver transplantation. In some, it may completely eliminate the need for transplant.

Lo performed the Kasai surgery on July 29, 2013. Elizabeth quickly recovered and went home from the hospital a week later.

"Her color improved right away," said Leah, "and her bilirubin levels were back to normal by our next visit to Comer Children's Hospital."

But there was more work to be done -- by the medical team and the family -- to help Elizabeth gain weight and improve her health.

"Elizabeth had a few setbacks and still was not feeding well," Azzam explained. "Her growth was slow, and she had signs of progressive liver disease. We tackled these problems with nutritional support." In October, a tube that passes through the nose into the stomach (called a nasogastric or NG tube) was placed so that formula could go directly into Elizabeth's stomach. She was listed for liver transplantation in January 2014.

Working with registered dietician Elizabeth Kwarta, MS, RD, LDN, Matt and Leah followed a strict regimen for Elizabeth's feedings over the next seven months. During the same period, Kwarta helped Leah lose 40 pounds so she could qualify to donate part of her liver, should her daughter need a transplant. Friends, neighbors, and church members supported the family with prayers and financial help.

By spring 2014, Elizabeth weighed 22 pounds, reaching the 67th percentile in her age group. Her liver disease had subsided, and she no longer needed the NG tube.

After discussing her progress at the Comer Children's Hospital pediatric liver transplant multidisciplinary conference in July 2014, Elizabeth's medical and transplant teams changed her status on the transplant list to inactive.

"She may never require a transplant," Azzam said, "but if she does, she is now healthy enough to undergo the operation."

Recently, Leah erased the family's medical to-do list on their refrigerator for the last time.

"It's almost too good to be true," she said.