Hirschsprung's disease

Omegaven saves livers

Many children who have complex intestinal surgery can’t eat normally for a long time. These children are usually placed on an intravenous method of feeding called pareneteral nutrition (PN).

• PN provides the necessary nutrition for children until their digestive systems adapt and they can eat on their own.

PN has revolutionized treatment for diseases such as Hirschsprung’s and short bowel syndrome. But its prolonged use often damages the liver, potentially leading to liver failure and the need for a transplant . And unfortunately, infants are at the greatest risk due to the small size of their livers.

Back in 2001, Children’s surgeon Mark Puder, MD, surgical resident Jenna Garza, MD, and pharmacist Kathy Gura, PharmD, decided to conduct studies in mice to see why PN was causing liver disease.

• They found evidence that the fat used in standard PN solutions, called Intralipid, was contributing to liver disease by causing fat to accumulate in the liver.

They then tested Omegaven, an IV fat mixture made from fish oil. Fish oil contains omega-3 fatty acids, which have been shown to prevent fat accumulation and have anti-inflammatory properties.

• As they had hoped, PN using Omegaven as the fat prevented liver injury in the mice.

Surgeon Rusty Jennings, MD had heard of Puder's research and wanted to try Omegaven in one of his patient. Since Omegaven wasn't approved for use in the United States, Puder had to receive special permission from the FDA to use Omegaven rather than Intralipid in his PN solution.

• Within eight weeks, the baby's liver function improved so much that he was removed from the liver transplant list.

Puder later treated a second child, a premature baby whose bowel had ruptured; he too had complete resolution of liver disease. Now, more than 100 children at Children’s have received Omegaven.

Puder and colleagues are now conducting a formal clinical trial aimed at preventing liver disease in PN recipients.

• Their work has caused a worldwide shift in treatment.