Biliary Obstruction: Amanda's Story
Patient Story Highlights
- After months of worrying and knowing something serious was happening inside Amanda's body, her parents were lead to Michael Wilsey, M.D. at John Hopkins All Children's Hospital.
- During the process of removing Amanda's gallbladder, it was discovered that she would need an advanced endoscopic procedure done which required a specifically trained gastroenterologist.
- Wilsey and his team are working to build a therapeutic endoscopic program to address the complicated and advanced needs of children like Amanda.
Amanda’s odyssey started with what seemed like a dirty pair of feet.
“You just took a shower, so why are your feet dirty again?” her mother, Elyse, would ask. “I had no idea what was really going on.”
No one did.
Months went by. Remedy after remedy did not help. Amanda’s worried parents knew something serious was happening, but to that point, no one, including Amanda’s pediatrician, had been able to figure it out.
“She was so itchy. She had rashes and she never, ever felt good,” Elyse explains. “We tried every lotion, soap and medicine we could find. She had endoscopies, colonoscopies, she was treated for reflux. We even treated her for scabies but that wasn’t it.”
They were at their wits end and would continue to be until the odyssey led to Michael Wilsey, M.D., vice chair of the gastroenterology division and vice chief of the medical staff at Johns Hopkins All Children's Hospital.
A Key Referral
Elyse is convinced it all traces back to a badly broken leg that Amanda sustained during a bike-riding lesson near her Fort Myers home when she was 14. Her leg required several surgeries, plenty of antibiotics and lots of screws to put her back together again. The leg healed, but something still wasn’t right. Before the surgery, she could eat anything. After, not much agreed with her. There was vomiting, diarrhea, rashes and those strange dirty feet. A regular school schedule was becoming nearly impossible. Amanda’s studies suffered and she had to give up dance classes.
Those dirty feet eventually solved the mystery: They were actually jaundiced.
Gallbladder surgery was scheduled at a Fort Myers hospital. The gallbladder was easily removed, but she continued with jaundice and abdominal pain after the surgery. Her adult gastroenterologist found an unexpected mass obstructing the bile duct, which he could not remove during an endoscopic procedure. He immediately halted the testing and quickly referred her to Johns Hopkins All Children’s Hospital. Elyse didn’t wait for insurance approval- she put Amanda in the car and set out for St. Petersburg where Wilsey would be waiting.
Because certain gallbladder issues in adults are not as common in children, there aren’t many pediatric gastroenterologists trained in advanced therapeutic endoscopy techniques. A reasonably common therapeutic endoscopy procedure for adults—which involves going through the throat to access the pancreas and biliary tree to drain bile, endoscopic retrograde cholangiopancreatography (ERCP)—is what Amanda required. Fewer than 20 pediatric gastroenterologists in the United States are specially trained to do this procedure on children.
Wilsey, who is a “national clinician of distinction” in pediatric advanced therapeutic endoscopy, has been performing the ERCP procedure in children for more than 15 years. He is one of two pediatric gastroenterologists in Florida—and one of fewer than 20 nationwide—with this expertise. He often gets referrals from around the southeastern United States and speaks internationally on the topic to raise awareness of the need for pediatric training.
Wilsey met Amanda and her mother and went to work.
“Obstruction of the bile duct causes yellowing of the skin and itching, which Amanda clearly experienced,” Wilsey explains about her mysterious dirty feet and unexplained rashes. “Left untreated, biliary obstruction can progress to infection, sepsis and even death. With Amanda's case, we needed to evaluate her liver and biliary tree to determine the cause of the biliary obstruction, which turned out to be an impacted gallstone. We were able to remove it during her procedure.
“ERCP allows us to combine fluoroscopy and endoscopy to diagnose and treat diseases of the pancreas, gallbladder, biliary system and liver, even in young children,” he says. “Adult gastroenterologists are trained to treat adults, but children are not little adults. Performing ERCP in children requires special training, and Johns Hopkins All Children’s is dedicated to meeting the unique needs of children.”
Wilsey explains that meeting the unique circumstances of children such as Amanda is why his team is building a therapeutic endoscopic program designed to address their complicated and advanced therapeutic endoscopic needs. “We are focused on endoscopic outcomes research, and we are currently studying the risk factors and predictors of gallstone formation in children,” he adds. Wilsey is a founding member of a prospective, multicenter international endoscopic database initiative (PEDI) designed to study the indications and technical outcomes of ERCP in pediatric patients at 14 centers worldwide.
Amanda, now 17, finally feels good again and is ready to do a little studying of her own. She began college classes this winter. Other than Amanda, no one is more relieved than Elyse to have it all behind them.
“We are so ready to move forward," Elyse says. "We loved Dr. Wilsey. He provided us with all of the information we needed in easy-to-understand language. Johns Hopkins All Children’s is really family-based. We appreciated the private rooms and how special they make their patients feel.”
This story first appeared in Leading Care magazine.
Pediatric Gastroenterology at Johns Hopkins All Children's Hospital
The pediatric gastroenterology, nutrition & hepatology division at Johns Hopkins All Children's Hospital in St. Petersburg, Florida, takes care of infants, children and adolescents with a wide range of chronic and acute disorders of the gastrointestinal tract.