Ethan Skacy grew up in Tuba City, AZ with his mom, dad, brothers and sisters. When he was 5-years-old, he was seen by the family physician because he had developed some growths on his elbows. They were removed. When Ethan was 10, it was discovered that he had familial hypercholesterolemia – or in other words, he was a little boy with high cholesterol. The growths on his elbows when he was 5 were actually excess calcium that his body couldn’t process.
Familial hypercholesterolemia causes the liver to be unable to process cholesterol, which then clogs the arteries of the heart. Ethan’s condition was 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 in adulthood, 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. The coronary arteries of these rare individuals become blocked and cause heart failure or death early in life if untreated.
For three years doctors watch Ethan’s heart and liver closely and tried to manage his high cholesterol. The Skacy family came to Primary Children’s from Arizona because of a life-saving machine that would perform LDL apheresis. LDL apheresis is like dialysis and eliminated cholesterol from Ethan’s bloodstream which helped his liver do its job, and helped his heart stay as healthy as possible. Ethan was on the transplant list to receive a new liver because if it was functioning, his heart would eventually recover on its own. Hearts are like that. In November of 2011, his heart condition worsened. Coronary blockage damaged Ethan’s heart irreparably, and it became necessary for both Ethan’s heart and liver to be replaced.
A couple of days after Ethan’s 13th birthday his family received the call that a donor had been identified, and Ethan would receive his much needed transplants. On January 30 to 31, 2012, surgeons at Primary Children’s performed the hospital’s first combined heart/liver transplant on Ethan. The surgery took 19.5 hours, and transplanted both organs from the same donor. Ethan was only the 15th pediatric patient in the United States to receive this dual transplant since 1997.
Ethan is doing well, but his follow-up care will be lifelong. It will be most intense during the first 6-months after his transplants 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; pharmacists; rehabilitation specialists; nurses; techs; social workers; and child life specialists.
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