By Kathleen Loomes, MD, and Amit A. Shah, MD
B.C. has been a patient in the Fred and Suzanne Biesecker Pediatric Liver Center at Children’s Hospital of Philadelphia (CHOP) since being diagnosed with biliary atresia at 9 weeks of age.
CHOP has been a leader in care for children with biliary atresia since the 1970s, when Morio Kasai, MD, who devised the Kasai operation as a treatment for biliary atresia (BA), came to work at CHOP alongside C. Everett Koop, MD and Louise Schnaufer, MD. Together, they created a specialized surgical program for infants with BA. Now, our Biliary Atresia Clinical Care Program, within the Liver Center, treats one of the largest populations of biliary atresia patients in the United States.
As B.C. had not yet developed complications of liver disease, such as portal hypertension and ascites, she underwent a Kasai procedure soon after her diagnosis was confirmed. After initial improvement in her bilirubin levels, her jaundice worsened and she began to develop complications of end stage liver disease. She experienced decreasing albumin (indicative of decreased production from liver and increased risk of developing ascites), worsening coagulopathy that required subcutaneous vitamin K injections, vitamin D deficiency that required increasing supplementation up to 16,000 units daily and decreasing platelet count, indicative of worsening portal hypertension.
She was referred for liver transplant at 6 months of age, and CHOP’s Liver Transplant Program joined her care team.
Our Liver Transplant Program, led by Elizabeth Rand, MD, works closely with the transplant program at the Hospital of the University of Pennsylvania. Combined, the programs perform an average of 120 transplants a year, with approximately 20 in children. That partnership brings unparalleled experience to CHOP’s patients and makes CHOP one of the larger pediatric liver transplant centers in the country. To date, the program has performed nearly 350 liver transplants, and our graft and patient survival rates consistently exceed national averages.
The next few months were challenging. B.C. needed an NG tube to deliver additional calories, as she suffered from profound malnutrition and fat-soluble vitamin deficiencies. She developed complications of portal hypertension, including variceal bleeding and ascites, which required treatment with diuretics and multiple paracenteses. Her echocardiogram showed signs of cirrhotic cardiomyopathy, which has been shown to be associated with increased waitlist mortality.
B.C. underwent a complete multidisciplinary evaluation and was listed for liver transplantation. Due to her multiple complications of end stage liver disease, she was granted exception points that increased her priority as a transplant candidate.
As her condition grew more critical, B.C. was hospitalized for close monitoring and management of comorbidities. She was admitted to CHOP on March 25, as COVID-19 was sweeping across the United States, hitting East Coast cities like Philadelphia particularly hard.
Like other hospitals in the first few months of the pandemic, CHOP had postponed elective procedures and moved many visits to a virtual platform. However, B.C.’s case was urgent; she needed to be in our inpatient area. The hospital strictly followed all CDC guidelines. All patients were tested upon admission, which continues to be our protocol. CHOP was the first hospital on the East Coast to receive FDA approval for its own lab test. Any suspected COVID-19 patients or caregivers were housed in separate COVID-only units.
B.C.’s father was undergoing the necessary screening to be a living donor and was nearing the completion of the process when a deceased pediatric donor organ became available.
She underwent a successful liver transplant on April 4, three days after turning 10 months old.
Within a week, her mother noticed improving skin tone and appetite. Her cholestasis and coagulopathy had resolved while her liver enzymes were normalizing. As her energy returned, so did her spirit. She was discharged six weeks after receiving her transplant.
Now at 15 months old, B.C. is growing well and beginning to meet developmental milestones that had been delayed due her degree of illness prior to transplant. At each of her now monthly check-ups — carried out in person and under COVID-19 safety protocols — we see something new and exciting that B.C. is doing. She just started drinking from a sippy cup and eating ice cream!
When it comes to caring for children with a rare condition like biliary atresia — with only 250 to 400 news cases a year in the United States — experience counts. At CHOP, the Liver Center, the Biliary Atresia Clinical Care Program and the Liver Transplant Program bring together a multidisciplinary team to support the child and their family from diagnosis through transplant and beyond — even during a pandemic.
As B.C.’s mother says, “It’s safe. Don’t be afraid to go to CHOP. It’s normal to be concerned at the beginning, but everyone knows what they’re doing. CHOP is such a wonderful hospital, I wouldn’t go anyplace else.”
To refer a patient to the Fred and Suzanne Biesecker Pediatric Liver Center, call 1-866-543-5487.