Pediatric subspecialists, from left to right, Daniel Rhee, Kenneth Ng and Kristin Arcara play key roles in moving the TPIAT program forward at Johns Hopkins Children’s Center.
Pediatric gastroenterologist Kenneth Ng with Riley and his mom, Lindsay Smith, at a follow-up appointment following his TPIAT surgery.
Removal of the pancreas followed by transplantation of the insulin producing islet cells into the liver are shown to avoid lifelong pain and diabetes.
Riley Smith, 15, from Pekin, Illinois, was experiencing, in his own words, episodes of “excruciating abdominal pain” that were occurring with greater frequency. Diagnosed with hereditary pancreatitis with progressively worsening new episodes, he faced a potential lifetime reliance on narcotics to manage the pain. Then, one of his doctors told him about a new pancreas program at Johns Hopkins Children’s Center that is designed just for patients like him. Today, after having his pancreas removed in a complex surgical transplant procedure, Riley is free of the painful episodes and is weaning off insulin injections to a point where he may not need them at all.
Not too long ago, such an outcome would not have been conceivable, says Kenneth Ng, the pediatric gastroenterologist who Riley saw at Johns Hopkins. Two factors, Ng explains, came into play. One is the formation of the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a consortium that expanded understanding of pancreatic disease in children. The other is the collaboration and support of the nationally known Johns Hopkins Pancreatitis Center, which serves adult patients. The result was establishment of the pediatric Total Pancreatectomy with Islet Autotransplantation (TPIAT) program, a treatment initiative for children with chronic pancreatitis or refractory acute recurrent pancreatitis.
“Being part of the consortium has allowed us to not only build upon the foundational knowledge we already have but also cross-talk with other centers to learn and grow our TPIAT program, which translates into even better care,” says Ng. “Also, we’ve been very fortunate in being physically connected to The Johns Hopkins Hospital, home to renowned leaders in pancreatic medicine. We’ve been able to leverage their expertise to get our program up and running.”
Indeed, the adult TPIAT program helped staff the pediatric program with its own expert transplant coordinator, Christi Walsh, who plays the vital role of coordinating the multidisciplinary care required for proper evaluation and treatment, and guiding patients and families through the process. In addition, pediatric surgeon Daniel Rhee worked closely with his surgeon colleagues in the adult pancreas center to learn the nuances of the TPIAT procedure. In this new approach, the source of the pain — the pancreas — is completely removed, and the islet cells that make insulin are isolated and re-implanted into the liver.
“There they can set up shop and over time reestablish their function,” says Ng. “The hope is that it will remove the original environment at risk of these inflammatory events, and still allow the body to do the important job of creating insulin.”
Patients with chronic or recurrent pancreatitis are first seen by Ng to determine if they are candidates for the TPIAT procedure. The patient and family then meet with Rhee to discuss surgical options. Also, Kristin Arcara of pediatric endocrinology counsels the family on the post-surgical course regarding insulin and blood sugar management. If the decision is made to proceed with the procedure, the patient and the family meet with the diverse group of clinicians and professionals who make up the rest of the TPIAT team.
Arcara’s role as an endocrinologist is crucial — meticulous management of patients’ blood sugars is critical for survival of the islet cells and their ability to take over the insulin production that the body needs. Even mildly low or high blood sugars, Arcara explains, create stressful environments for islet cells and make it harder for them to survive.
“The better we can control their blood sugars, the more we can allow those islet cells to rest and take up residence in the liver and recover from the shock of isolation and implantation,” says Arcara. “It’s my job to make sure they preserve their function so patients can go on and not require multiple daily insulin injections or an insulin pump for the rest of their life.”
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Of course, the complex eight- to 10-hour TPIAT surgery, which includes reconstruction of the gastrointestinal tract and re-configuration of the intestine, must come first. “It’s a scary sounding operation for sure,” says Rhee, “but it can be done safely.”
Rhee is the lead surgeon, but multiple teams play a part, including a team to handle the pancreas specimen for extraction of the islet insulin cells. In a four-hour process immediately following resection of the pancreas, these cells must be separated out in a solution to inject back into the patient’s liver. In addition, a pathologist must examine them to ensure they contain no cancerous cells before re-implantation.
The new TPIAT program is complemented by the Johns Hopkins pediatric pancreas center, which offers a range of medical and specialized endoscopic treatments. One such procedure is endoscopic retrograde cholangiopancreatography (ERCP), which is used to diagnose and treat diseases of the biliary system, pancreas and liver. ERCP may help alleviate congestion in the main pipeline of the pancreas, or treat an obstruction in the common bile duct pushing on the pancreas. If missed, consequences may include acute pancreatitis or cholangitis. Ng is one of the few pediatric GI physicians in the country with deep experience using ERCP.
How do young patients respond to TPIAT? The answer is: generally better than adult patients, says Rhee, thanks to young patients’ resilience and healthy residual pancreatic tissue. While lifestyle choices like alcohol exposure and smoking are trigger points in adult disease, genetic predisposition is a dominant factor for children. Most families report significant relief and improvement in their child’s quality of life following the procedure — they typically return to school, social activities and sports. Parents are able to reduce or eliminate the child’s need for narcotic pain relief, but knowing whether the patient will remain on insulin therapy takes longer — sometimes a year or more.
So, how did Riley handle TPIAT?
“Riley is doing great,” says Arcara. “We’ve had one other patient recently, and both are doing better than I ever could have imagined with the use of the most cutting edge diabetes management technologies.”
“We’re very excited about Riley,” says Rhee. “The operation went very smoothly and he’s recovered like a champ. We’ll have to wait a few months out to see how his blood sugars are doing and whether his body can take care of insulin production on its own.”
Rhee adds, “These patients have had an ongoing fear of the pain in their daily lives, saying to themselves, ‘This meal might set off an attack and I’ll have to go to the hospital.’ We take that away and give them peace of mind so they can go back to living their life without fear.”
After eight days in the hospital, Riley was anxious to return home to Charlie, his rescue dog, and school activities like volleyball and theater. During his latest audition, he landed the role of Harry Bailey in It’s a Wonderful Life.
Johns Hopkins Children’s Center is among only a few institutions in the United States offering total pancreatectomy with islet autotransplantation (TPIAT) for patients as young as age 4. The TPIAT team includes specialists in pediatric gastroenterology, endocrinology and surgery, along with anesthesiology, child life, child psychiatry, social work, intensive care, pain medicine and psychological services.