Primary Children's Performs Their First Combined Heart and Liver Transplant

On January 30 to 31, 2012, surgeons at Primary Children’s Medical Center performed the hospital’s first heart/liver transplant on a 13-year-old boy from Tuba City, AZ. Surgeons performed the 19.5 hour procedure transplanting both organs from the same donor with successful results. The patient, Ethan Skacy, was the 15th pediatric patient in the United States to receive this dual transplant since 1997. He is expected to be released from the hospital early next week.

Ethan was diagnosed with familial hypercholesterolemia at age 10. Familial hypercholesterolemia causes the liver to be unable to process cholesterol, which then clogs the arteries of the heart. Ethan’s condition is a genetic disorder and is the result of a two abnormal genes, one being passed from each parent. Many people (1 in 500) carry one of these abnormal genes, which causes high cholesterol but not severe enough to cause a heart attack in childhood. Only 1 in one million people like Ethan receive the gene from both parents. These rare individuals suffer from severe hypercholesterolemia in childhood such that their coronary arteries become blocked and cause heart failure or death early in life if untreated. When Ethan received his transplant, his cholesterol level was over 700—a normal cholesterol level is < 200.

Ethan was first seen by a medical professional for symptoms of familial hypercholesterolemia when he was 5. He had developed growths made of deposits of cholesterol called xanthomas on his elbows. They were removed, but the extent of his condition wasn’t fully discovered until he was 10. Until November of 2011, Ethan was on the transplant list waiting to receive a liver. At that time, his heart had only mild cholesterol-buildup and was still working well; and if his liver could be replaced with a healthy one, his heart would eventually recover on its own. In November, Ethan’s heart condition worsened. His coronary blockage damaged the heart irreparably. It became necessary for both Ethan’s heart and liver to be replaced.

The Skacy family came to Primary Children’s and the University of Utah Medical Center to seek care for Ethan because a life-saving machine that would perform LDL apheresis was not available in their area. LDL apheresis resembles dialysis and eliminates cholesterol from the bloodstream buying time for patients with familial hypercholesterolemia until a liver becomes available.

Ethan’s follow-up care will be lifelong but most intense during the first 6 months after transplant when the risk of rejecting the organs or getting an infection is the greatest. During this 6-month period, Ethan and his family will be required to stay near Primary Children’s. He will be monitored by physicians, laboratory studies, regular ultrasounds of the heart (called echocardiograms), and periodic heart catheterizations in which tiny pieces of the heart muscle are removed to look for rejection using a microscope (heart biopsy).
Ethan has been cared for at Primary Children’s by Dr. Melanie Everitt, cardiologist; Dr. Linda Book, gastroenterologist; Dr. Phillip Burch, cardiac surgeon; Dr. Willem Van der Werf, general surgeon; Dr. Shiro Fujita, general surgeon; Dr. Jack Mulroy, anesthesiologist; and Dr. Chris Miller, anesthesiologist; along with a comprehensive team of intensive care doctors; rehabilitation specialists; nurses; techs; social workers; and child life specialists.