Pediatric Living-Related Liver Transplantation
"The ability to perform living-related liver transplants makes us less dependent on the short supply of donor organs," Dr. Selby notes. "There is a limit to the number of cadaver livers (livers from brain-dead donors) available--a number that falls far short of demand. Living-related liver transplantation offers new opportunity, all three of our patients have done incredibly well."
Interestingly, in two of the living-related liver transplantation operations performed at CHLA, grandmothers donated a section of their livers to their grandchildren. Both grandmothers were in their mid-40s, and both children were approximately six months old. In each case, the donor surgery, which takes about four hours, was performed at USC University Hospital. The liver was then driven to CHLA, where Dr. Selby transplanted the liver section in a separate four-hour surgery.
"The recipient and donor livers must have the same blood type," Dr. Selby points out. "We also screen the donors for hepatitis and make sure the liver has not been damaged. We estimate the size of the section to resect--either a full lobe or just the left lateral segment--based on volumetric measurement and CT scans."
The recovery time for the donor is five days and for the recipient about two weeks. "The ability to perform living-related transplants differentiates us from other liver transplant programs in the area," notes Dr. Selby. "It's an exciting advance for young patients who face end-stage liver disease."
In addition to addressing the issue of the limited donor organ pool for livers, living-related liver transplantation also gives surgeons the option of transplanting a liver that is in excellent condition--reducing the complications that can occur from cadaveric livers. "Although the liver still functions in a patient who is declared brain dead," notes Dr. Selby, "The length of time it remains in the body can compromise the quality of the organ. With living-related transplants, we use organs that generally are in excellent shape."
Critical Hours Post Transplant
Patients are placed on a national transplant waiting list run by UNOS (United Network for Organ Sharing). Patients are then selected based upon time on the list, medical acuity, blood type and body size. Once the organ becomes available, the patient is taken to the operating room. During a procedure lasting from size to eight hours, the diseased liver is removed and the donor liver is implanted.
After either living-related and cadaveric liver transplantation, the patient continues to receive immunosuppressive drugs first administered in the operating room. The initial 48 hours following the transplantation are critical--even if the liver looks healthy immediately following the surgery. Within 24 to 48 hours, the transplant team will know if the organ is functioning properly. After discharge, patients return regularly for monitoring of organ rejection as the greatest chance of rejection is during the three months following the transplant. Major risks during this period are related to opportunistic infections. However, in general, liver transplant recipients can lead a full and improved life.
Dynamic Medical Component
Dr. Thomas, head of Gastroenterology and Nutrition at UCMG and CHLA, runs the medical aspect of liver transplantation. In his Division, he cares for children with acute liver failure or chronic liver disease whose only option for survival is liver transplantation. "We handle the medical care and management of these children up until they actually have the liver transplant," Dr. Thomas notes. "And we share the care with Dr. Selby's group after they are transplanted as well."
Dr. Thomas explains that the most comon condition that requires liver transplantation is biliary atresia, in which the external biliary system is absent. An operation can be performed in young infants to replace the absent external biliary system with a segment of intestine. The operation is called a Kasai portoenterostomy. "In one-third of the patients, the operation helps correct the problem," Dr. Thomas says. "In another third of patients, this corrective surgery is not completely successful and they may need a transplant when they reach school age. And the remaining third of these patients need a liver transplant immediately."
Another liver disorder that can lead to end-stage liver disease is fulminant liver failure, which can be caused by poisoning, for example from mushrooms or the result of a reaction to medication, or from viral hepatitis. Other groups of disorders involve problems of metabolism, such as Alpha-1 antitripsin deficiency and glycogen storage disease. Some children also suffer from autoimmune liver disease, which is similar in mechanism to systemic lupus or rheumatoid arthritis.
Connection to Small Bowel Program
Kathryn D. Anderson, M.D., Chief of Surgery at UCMG and CHLA, notes that with the addition of the liver transplant program, CHLA is now a full-service transplant center. UCMG's specialists perform heart, lung and kidney (both living-related and cadaver) and bone marrow transplantation. In addition, there is an active group researching small bowel transplantation--a challenging procedure for patients with short gut syndrome and severe intestinal dysfunction.
"In short gut syndrome, the intestine is lost and patients can't eat or digest their food," notes Dr. Anderson. "Consequently, they require a lifetime dependency on total parenteral nutrition (TPN)."
Dr. Thomas adds that over the years, this dependence on TPN can cause progressive damage to the liver, and eventually the patient will experience end stage liver disease. This damage may be caused by three factors: septic events, which can occur from blood infections; the fact that there is not normal food intake to stimulate bile flow, resulting in a liver filled with sludge; and the constitution of the TPN ingredients themselves.
"When we begin to perform small bowel transplantation, which I predict will occur in the next few years, we must also have the capacity to perform liver transplants on these patients," Dr. Anderson says. "The Liver Transplant Program fulfills this need."
What makes the CHLA Liver Transplant Program particularly special is its emphasis on families and follow-up care, notes Barbara Gross, R.N., M.S.N., the administrator who oversees both the liver and cardiothoracic transplant programs at CHLA. "Children have unique needs, and at UCMG and CHLA, we are able to focus on that," Gross notes. "We are particularly sensitive to family issues, and keep family members involved and educated throughout the process."
Gross notes that UCMG and CHLA have extensive experience in helping patients return to a normal lifestyle following treatment. "We work with family members and the child's school to help them reintegrate back into their previous lifestyle," Gross notes. "It's our strength as pediatric specialists and a tertiary facility."
Expansion of Program
Dr. Selby says he is extremely pleased with the progress in the CHLA Liver Transplant Program and looks forward to expanding it every year. "The Hospital is well-organized and the administration is dedicated to the success of all transplant efforts," Dr. Selby notes. "This support is essential as we move forward with the Liver Transplant Program as well as our small bowel transplant endeavors. We have developed some great working relationships here, and feel confident that many more children will benefit from these coordinated efforts."
For referral and consultation call Dr. Rick Selby at (323) 442-5908 or Dr. Daniel Thomas at (323) 669-5454.