Personal Stories

Transplantation for Short Bowel Babies

Despite assertions by Kyle's previous physicians that he would not survive without both intestinal and liver transplants, the team at the Pediatric Intestinal Rehabilitation and Transplant program was able to complete an isolated intestinal transplant while saving his liver. "Today he is doing very well," reports Steven J. Lobritto, MD, Interim Chief of Pediatric Gastroenterology.

This scenario is emblematic of the way the program defies the odds on a regular basis, performing heroics in a field not always known for great optimism. Under the direction of Dominique M. Jan, MD, Professor of Clinical Surgery, the program offers a unique method of independent management of children with intestinal failure and liver disease. Its strength lies in a comprehensive team approach: a multidisciplinary group including a pediatric surgeon, a transplant surgeon, gastroenterologists, hepatologists, nutritionists, and nurse practitioners, works together to determine the best course of treatment for each child. According to Dr. Jan, "This team approach results in far superior care than is available at centers that rely on a surgical or medical approach alone."

Kyle Cramer (center) received an intestinal transplant at age 14 months in October 2005 and no longer needs TPN (total parenteral nutrition). Kyle is flanked by surgeon Dominique Jan, MD (far left), Kara Ventura, NP, and his parents, Cara and Ben Cramer.

Most children with Short Bowel Syndrome (little or no functional intestine) have to be nourished by total parenteral nutrition (TPN, or nutrition through an intravenous line), which can contribute to the development of liver failure. The center has special expertise in minimizing this risk, says Dr. Lobritto. "Proper management of the nutritional balance in TPN is one of the most important keys in preventing liver damage. With proper balance of nutrients, it is often possible to avoid both liver and intestinal transplantation, and to even encourage oral feeding."

"We make every effort to use the intestine that a patient has left, and give the patient a chance to adapt," explains Robert A.Cowles, MD, Assistant Professor of Surgery. When nutritional rehabilitation is not sufficient, however, the program provides the full spectrum of surgical options. Some children may be candidates for the Serial Transverse Enteroplasty procedure (STEP), an operation to lengthen and reshape a segment of intestine into a longer, thinner intestine. Dr. Jan, who has performed many intestinal transplants worldwide, emphasizes that "if patients with short bowel syndrome receive proper treatment early on, many complications – and potentially the need for intestinal or liver transplantation – can be avoided."

In children with biliary atresia (a congenital defect of the bile duct that prevents the liver from excreting bile), emergency surgery called porto-enteros-tomy, or the Kasai procedure, is essential. "If this is done early enough, the injured bile duct can be reconnected with the small bowel before the liver is destroyed," says Dr. Jan. Although a number of U.S. institutions perform the Kasai procedure for biliary atresia, their survival rates fall short of the 98% survival rate achieved by R. Peter Altman, MD, Surgeon-in-Chief at Morgan Stanley Children's Hospital of NewYork-Presbyterian, and his colleagues. "Our surgeons perform reconstructions of the biliary system with incredible expertise," says Dr. Jan. Dr. Altman, who is world-renowned among the best pediatric surgeons for this procedure, is currently training other surgeons at Columbia University Medical Center in the procedure.

When a child requires a liver transplant, a family member will frequently volunteer to donate a portion of their liver, according to Dr. Lobritto. Using tissue from living donors has several advantages, including the ability to choose a healthy donor and to plan the transplant in advance, so that the child's medical team can ensure he or she is fully optimized for the operation.

Surgeons at this center regularly perform operations that many centers won't consider, in children of every age. "Our knowledge of the liver gives us the ability to do things that other centers simply don't do," states Dr. Lobritto. In this past year, Dr. Jan performed a successful living-related liver transplant in a premature, failure-to-thrive newborn who weighed only five pounds, and in a five-day old baby with metabolic liver disease.

"We are not doing transplants to give our patients an extra six months," says Dr. Lobritto. "That would be a failed transplant.We do them to help them grow normally and have normal lives. That is what we look forward to."