Gift Will Grow Program for Pancreatic Disease

August 12, 2015
Contact: Dr. Sue Rhee or Dr. Emily Perito (415) 476-5892

The UCSF Benioff Children's Hospital San Francisco Pediatric Pancreas Program is on its way to becoming a national referral center for pancreatic disease, thanks to a generous gift from Marc and Lynne Benioff that targets this rare but increasingly recognized children's health problem.

Very few medical centers have expertise in pediatric pancreatic disease, and UCSF's recently established program has exceptional depth in this field. It includes not only expert clinical services but also participation in an international research consortium and an NIH-funded fellowship training program in pediatric gastroenterology. This programmatic strength makes UCSF a magnet for both patients and physicians in training.

"Pancreatitis was once thought of as a predominantly adult disease, but we have seen a dramatic increase in pediatric referrals over the last few years as physicians realize that it affects children, too," said pediatric gastroenterologist Dr. Sue Rhee, who serves as medical co-director of the program with colleague Dr. Emily Perito. Because the disease is relatively rare, it is important that children be evaluated and treated by a multidisciplinary, experienced team, Rhee said.

Acute and Chronic Disease

Acute pancreatitis, which is characterized by severe abdominal pain, occurs when the pancreas becomes inflamed. Chronic pancreatitis can develop when repeated bouts of inflammation cause irreversible scarring of the organ. In some cases the pancreas loses its ability to produce digestive enzymes and insulin, which can lead to malnutrition or diabetes. The causes of pancreatitis are not well understood, but anatomic abnormalities and genetic disorders may predispose a child to developing the disorder. Blood tests and imaging studies can diagnose the disease and a variety of therapies can be used to treat it.

Diagnostic and Treatment Options

Young patients are referred to the program through the Division of Pediatric Gastroenterology, Hepatology and Nutrition. Children receive a comprehensive evaluation that includes consultations with pediatric gastroenterologists, hepatobiliary specialists, endocrinologists, pediatric pain specialists, surgeons, and dieticians. Genetic testing may also be recommended to detect any of the mutations known to be associated with a hereditary predisposition to chronic pancreatitis.

A definitive diagnosis generally requires imaging the pancreas with endoscopic ultrasound; magnetic resonance cholangiopancreatography (MRCP); or endoscopic retrograde cholangiopancreatography (ERCP). Gastroenterologist Dr. James Ostroff, known for his extensive experience with ERCP in adults, has successfully adapted this procedure for use in children. As in adults, ERCP can be used for a variety of interventional procedures when the pancreas has been scarred by chronic disease. These include dilation of the pancreatic duct, removal of stones and/or stent placement. Medical and nutritional interventions are also used to ease digestive problems.

Surgery may be appropriate for some patients with chronic disease. This can include partial pancreatectomy or a procedure that connects a portion of the small intestine to help drain the pancreas (Puestow procedure). Selected patients with refractory disease may also be candidates for total pancreatectomy and islet autotransplantation, a procedure offered at only a few institutions, including UCSF. Islet cells are removed in a complex process from the pancreas and then infused back into the patient, where they typically engraft in the liver. This autotransplant prevents the development of insulin-dependent diabetes in children who undergo the procedure.

Pain management is a key element in caring for patients with pancreatitis. The UCSF Benioff Children’s Hospital San Francisco Integrative Pediatric Pain and Palliative Care Program (IP3) draws on the expertise of hospitalists, pediatric anesthesiologists and nurse practitioners to effectively manage children's pain both in and out of the hospital. The team uses pharmacologic and and nonpharmacologic approaches, such as biofeedback and acupuncture.

Fellowship Training and Research

The Pediatric Pancreas Program offers the next generation of physicians an opportunity to develop expertise in the diagnosis and treatment of pancreatitis through UCSF's NIH-funded fellowship training program in pediatric gastroenterology. Created under the leadership of Division Chief Dr. Melvin Heyman in 1973, it is the oldest training program of its kind in the country.

Research is also an important programmatic focus. Dr. Heyman serves as a principal investigator in the International Study Group of Pediatric Pancreatitis, a multinational, multi-center NIH-funded project established in 2013 to characterize pancreatic disease in children. Approximately 350 children have been enrolled in INSPPIRE, including 40 through UCSF Benioff Children’s Hospital San Francisco. In addition, UCSF fellows conduct research through grants awarded by the Cystic Fibrosis Foundation, with the goal of better understanding why patients with this genetic disease are at increased risk for pancreatitis.

For more information about the UCSF Benioff Children's Hospital San Francisco Pediatric Pancreas Program, contact Dr. Sue Rhee or Dr. Emily Perito at (415) 476-5892.

About UCSF Benioff Children's Hospital San Francisco
UCSF Benioff Children's Hospital San Francisco creates an environment where children and their families find compassionate care at the forefront of scientific discovery, with more than 150 experts in 50 medical specialties serving patients throughout Northern California and beyond. The hospital admits about 5,000 children each year, including 2,000 babies born in the hospital. For more information, visit www.ucsfbenioffchildrens.org.

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