Community June 30, 2008
A New Light Shines on Transplantation
A few weeks before Easter 2008, Lia Cirelli, a smart, happy 11-year-old from Redding, Connecticut, who rarely got tired, sick or bored, began complaining about stomach aches. One Tuesday afternoon, Laura Cirelli watched her daughter step off the school bus clutching her stomach, pushing against her upper rib cage. The next day Lia's skin turned yellow, almost orange.
After listening to the story on the phone, the Cirellis' pediatrician told them to go directly to Bridgeport Hospital. There the situation intensified, and emergency room doctors told the family they would need to transfer Lia to Yale-New Haven Children's Hospital (YNHCH) by ambulance - or, if possible, by helicopter.
Within hours, Orazio Cirelli, an equipment mechanic, and Laura, a tax assessor's assistant, found themselves listening in shock as pediatric liver expert Pramod Mistry, MD, told them their only child, adopted from China when she was 11 months old, appeared to have a rare condition called Wilson's disease. Lia's body had been retaining copper since birth, and now a buildup of copper was causing her liver to fail. Conclusive tests would take time, and although Lia was awake and articulate, she only had a few days at most to live. The only cure was a liver transplant.
Lia's age and acute illness thrust her toward the top of the donor list, but the initial liver that became available wasn't suitable. So family and friends came, one by one, to New Haven to be tested to see if they qualified as living donors. Incredibly, a 27-year-old social worker and distant relative of the Cirellis' - who was in Connecticut for the Easter weekend - turned out to be a match.
On Easter Sunday, Lia Cirelli became the second patient in Connecticut - and the first child - to receive part of a liver from a living donor. She'll stay home until September to avoid infection, and she'll take immunosuppressant drugs for the rest of her life. Otherwise, her parents are walking with her a mile a day now, and her doctors say she is "perfect."
Advancing in a new direction
If it had to happen at all, the Cirelli family believes they landed in the right place at the right time. Lia's surgeon was 55-yearold Sukru Emre, MD, appointed last July as director of the Yale-New Haven Transplantation Center (YNHTC), and section chief of organ transplantation and immunology in the department of surgery at Yale School of Medicine.
Dr. Emre, a native of Turkey, came to YNHH from Mount Sinai Medical Center in New York, where he turned the transplantation program into one of the best in the nation. He became a rising star in the demanding field of adult and pediatric liver transplant surgeries, performing cutting- edge variations such as domino transplants, in which the imperfect liver removed from one transplant patient might still serve another patient with a shorter anticipated lifespan.
Last year, Yale-New Haven Hospital and Yale School of Medicine joined forces to bolster the Transplantation Center and increase services available in the region. As the leader of this effort, Dr. Emre has the ambitious goal of increasing the current number of liver transplants at YNHH to between 80 and 100 a year. He expects to boost an already thriving kidney transplant program, increasing transplants to 150 a year, and pancreatic transplants, which are far less common, to 20.
"My goal at Yale-New Haven is to replicate what I did at Mount Sinai by creating one of the best transplant programs in the country, with the best possible patient care and excellent outcomes," said Dr. Emre. Data from the United Network for Organ Sharing (UNOS) have
shown that Dr. Emre's survival rates in pediatric liver transplantation have been 99 percent after one year and 93 percent after five years, both of which are well above the national averages.
Excerpt from and article that originally appeared in the summer 2008 issue of the Yale-New Haven Hospital Magazine (pages 2-3)
http://www.ynhh.org/online/magazines/summer08mag.pdf
