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Pediatric Total Pancreatectomy with Islet Auto Transplantation Process

Our Pediatric Pancreas Center is recognized as a center of excellence by the National Pancreas Foundation for the care and treatment of pancreatitis. We are also a contributing member of INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE project), an NIH-funded research project comprised of 26 pediatric centers around the world focused on children with pancreatitis.

Goals of TPIAT

The main goal of pediatric total pancreatectomy with islet auto transplantation (TPIAT) is to reduce pain and improve overall quality of life for patients with acute recurrent or chronic pancreatitis by removing the pancreas, the source of their pain. Additionally, by isolating and re-implanting the insulin-producing islet cells which reside in the pancreas, we aim to preserve patients’ ability to make insulin to regulate their blood sugar (glucose) levels and avoid complete insulin deficiency, which would result in very difficult to manage diabetes mellitus.

Pretransplant Evaluation

Patients are first evaluated in the Pediatric Pancreas Center by a pediatric gastroenterologist where the patient’s medical history and previous records are thoroughly reviewed. Additional testing including blood, stool, imaging, and/or endoscopy may be required based on the initial consultation. If TPIAT is deemed a reasonable surgical option, the patient would also be evaluated by pediatric surgery, pediatric endocrinology, social work, and Child Life, all arranged by our program coordinator. Patients will receive education regarding the surgical procedure and about postoperative management, including the administration of insulin.

Patients may also be referred to the Kennedy Krieger Institute’s Pain Rehabilitation Program. Cases are all reviewed together by our multidisciplinary team and management approach is individualized in order to best meet patients’ needs. Upon approval for TPIAT, families will work with our program coordinator to arrange for all necessary preprocedure evaluation and scheduling. This will likely include glucose tolerance testing, pre-operative lab tests, and medications including pancreatic enzymes.

Surgery

The TPIAT procedure involves surgical removal of the entire pancreas followed by reconstruction of the gastrointestinal tract. Once the pancreas is removed, our islet cell isolation team immediately processes the pancreatic tissue to isolate the insulin-producing islet cells. Our operating room is specially designed to allow this process to occur within the same room. This minimizes the time the organ is without blood flow, which is important to maximize islet cell yield. The pancreas and islet cells are examined by our pathologists to ensure no abnormalities. The islet cells are then carefully infused into the liver, where they will eventually begin to resume function and produce insulin, with the goal of allowing patients to be partially or completely independent of needing to take insulin over time.

Post-Transplant Process

After surgery, the patient is transferred to the Pediatric Intensive Care Unit (PICU) for postoperative care. Our expert team of pediatric intensivists, surgeons, endocrinologists and pain specialists will closely monitor the patient’s recovery. Postoperative pain will be carefully managed. Additionally, the patient will require intensive blood glucose monitoring and IV insulin infusion to ensure blood glucose levels remain within a very narrow range in order to allow for the freshly transplanted islet cells to rest and maximize their chance of survival.

During the hospital stay and in the days following discharge, the patient and family will receive further training on administration of insulin and use of cutting-edge diabetes management technologies. After hospital discharge, patients will have very close contact with the pediatric diabetes team in order to optimize blood glucose control at home. Our team approach, which shares similarities with our adult TPIAT program, is optimized for our pediatric patients offering individualized care that promotes timely recovery. Patients are usually discharged home after 7 to 14 days.

All patients will remain on insulin therapy for at least 3 months after surgery. Long-term endocrine follow-up will consist of evaluation of islet function with glucose tolerance testing at regular intervals and management of insulin therapy. Patients can expect longitudinal outpatient follow up with members of the pediatric TPIAT team until the age of 22, at which point their care is transitioned to our adult TPIAT program.

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