Two Rancho Penasquitos residents, who did not know each other, recently benefited from the first “domino” liver transplant procedure in San Diego County.
On May 28, 2004, UCSD Medical Center surgeons Ajai Khanna, M.D., and Marquis Hart, M.D., Director of Abdominal Transplant, transplanted a cadaver liver into Brock Wiles, a 25 year-old patient with a metabolic disorder who was not able to use his functional liver due to a genetic disorder. Wiles’ liver was consequently transplanted into My Huynh, a married father of three suffering from liver cancer.
“Both patients would likely have died without the transplants,” said Khanna, M.D., Director of Pediatric Abdominal Transplantation and Transplantation Research, “It was the first time we attempted a procedure of this type in San Diego County. And it was the first time anywhere, as far as we know, that a patient with this type of metabolic disorder and a liver cancer patient underwent this unique procedure. We felt confident the patients would do well, and in fact their recoveries have exceeded our expectations.”
Wiles suffered from Maple Syrup Urine Disease (MSUD), a rare metabolic disorder that occurs in one in 225,000 births.
|"We felt confident the patients would do well, and in fact their recoveries have exceeded our expectations.”
“The disorder affects the way the body metabolizes certain components of protein. MSUD patients are not able to digest protein and must follow an extremely strict diet,” said Bruce Barshop, M.D. of the UCSD Division of Biochemical Genetics in Pediatrics.
The condition is life-threatening and surfaces during the first week of life; many, if not most patients lose their lives in childhood or suffer permanent neurological damage. MSUD derives its name from the maple syrup smell of the urine.
“With a new liver Mr. Wiles is now cured of the disease,” said Barshop. “He can now get on with his life.”
Wiles mother, Celeste, said she was looking forward to a more normal life for Brock and the entire family. She said it has been a difficult struggle to manage the unusual disease. Brock said he’s looking forward to eating hamburgers.
Khanna said surgeons transplanted Wiles’ liver into the patient with liver cancer for several reasons.
“First and most importantly, due to the long transplant waiting list Mr. Huynh would not have survived long enough to receive a cadaver liver; the rapidly growing cancer would have made him untransplantable,” Khanna said. “Because Mr. Huynh did not suffer from Maple Syrup Urine Disease and his body was not lacking the enzyme, we felt the lack of the enzyme in the liver would not hinder his body’s ability to use Mr. Wiles functional liver the way Wiles’ body was unable to utilize it. It was a win-win situation for everyone.”
Elliott Alpert, M.D., UCSD Liver Center Hepatologist agreed saying “the domino liver transplant was a very unusual procedure because rarely do we get a liver like Brock’s with just a genetic defect that we can overcome. It gave us an opportunity to give Brock’s liver to Mr. Huynh and help save his life.”
Although Brock had to follow a restricted diet consisting mostly of vegetables, doctors anticipate Huynh will not have to follow similar limitations.
The two patients finally met at UCSD Medical Center – Hillcrest on June 9th, the morning of their discharge and learned they lived in the same San Diego neighborhood, Rancho Penasquitos.
The UCSD Center for Transplantation stands alone in the region as a nationally recognized multi-organ transplant program. No other health care network offers such a broad spectrum of transplant programs, including kidney, combination kidney-pancreas, pancreas, liver, heart, lung, combination heart-lung, and bone marrow/stem cell. By providing this array of programs in a single setting, the UCSD Center for Transplantation offers its patients the unique advantage of a medical team that has shared expertise across the spectrum of transplant options. With over three decades of experience, the UCSD Center for Transplantation has given thousands of patients of all ages a new chance at life.
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