McGowan Institute for Regenerative Medicine
faculty member Julie Fuchs, MD (pictured), is an assistant professor of surgery at the University of Pittsburgh and practices in the Division of Pediatric Surgery at Children’s Hospital of Pittsburgh of UPMC. An area of interest within Dr. Fuchs’ research efforts is in exploring novel treatments for short bowel syndrome and liver failure. Recently, a Boston Globe staff writer contacted her for input regarding a therapy option currently available for young patients living with this ailment.
Short bowel syndrome (SBS, also short gut syndrome or simply short gut) is a malabsorption disorder caused by the surgical removal of more than two thirds of the small intestine, or rarely due to the complete dysfunction of a large segment of bowel. Most of these cases are acquired, although some children are born with a congenital short bowel. Babies born with SBS lack the ability to digest food or absorb nutrients.
In newborn infants, the 4-year survival rate on total parenteral nutrition (TPN) is approximately 70%. TPN is often referred to as IV nutrition (feeding directly into a venous catheter). Some studies suggest that much of the mortality is due to a complication of the plant-based TPN, especially chronic liver disease. Much hope is vested, however, in Omegaven, a type of fish oil-based TPN feed, in which recent case reports suggest the risk of liver disease is much lower. Omegaven is rich in omega-3 fatty acids.
At Children's, Omegaven has been used in about two dozen patients and is "very promising," said Dr. Fuchs. "We've had very good results, but we're planning to study it long term in more detail."
Omegaven - which typically costs $50 to $100 a day - is not approved in the United States but is made available to individual patients with permission of the FDA. Today, that means parents must make the choice to give their very sick baby an experimental treatment. Omegaven’s German manufacturer, Fresenius Kabi, supports clinical investigations needed for Omegaven to gain federal approval for use on children facing liver failure because of IV nutrition.
In 1981, Children's Hospital opened one of the first short bowel clinics in the nation and is recognized internationally for the successful surgical and non-surgical management of children suffering from this disorder. To this end, the nation's first multidisciplinary, comprehensive Intestinal Care Center has been established and is available to care for patients diagnosed with complicated intestinal disorders.
Illustration: McGowan Institute for Regenerative Medicine.
The Boston Globe (01/09/09)
Children’s Hospital of Pittsburgh of UPMC Intestinal Care Center
Short Bowel Syndrome, Wikipedia
Bio: Dr. Julie Fuchs