Dr. J. Philip Boudreaux, Professor of Surgery at LSU's Health Sciences Center and Director of Transplantation at Children's Hospital, explains some of the possible causes for a young child to need an organ transplant.
"Very young children, under the age of 5, typically need a transplant because of a congenital birth defect. Other times, it's not an anatomical defect but a metabolic one. For instance, there are what we call inborn errors of metabolism. In the liver, these are certain chemical defects or processes that can cause kidney failure or even brain damage. Since the liver is like a chemical factory with over 5,000 metabolic functions, the chemical defects can be corrected by giving the child a new factory with all the processes in place."
Alagille Syndrome is generally considered an anatomical defect in which there is a shortage of bile ducts within the liver. The syndrome can present symptoms involving the liver in infancy, or, in Chloe's case, as early as birth. When she was born, her skin was jaundiced, or yellow, indicative of liver problems. It was later revealed that her father, Kenny Smith, suffered from the syndrome and that he had undergone a successful liver transplant operation at the age of 18. After they performed a liver biopsy on her at 3 months, doctors confirmed that Chloe had Alagille Syndrome and her liver was already in distress.
Immediately, her name was placed on the national waiting list for organ transplants, which is maintained by UNOS (United Network for Organ Sharing). UNOS operates under contract from the U.S. Department of Health and Human Services and coordinates the Organ Procurement and Transplantation Network, whose members include transplantation centers, local organ procurement agencies, volunteer organizations, laboratories and the general public. Anne Paschke, spokesperson for UNOS, explains that the waiting list isn't exactly a list.
"When people hear the word 'list,' they typically think of a static list, but that's not the case," she says. "The waiting list is really a pool of information in our computer system that is used when a donor is identified. When information is entered about a potential donor into this database, a ranked list of potential recipients for each organ will be generated. This database is used to match only organs of diseased donors and possible recipients."
Organ transplants in very young children like Chloe are rare. In 2003 with 25,786 transplant recipients overall, there were only 759 organ transplant recipients under the age of 5 reported nationally. However, even with these low national figures, the demand outweighs the supply. Tragically in 2003, 115 children died waiting for organ transplants. In Louisiana, the total number of transplants for children under 5 was only 13. There were no reported deaths in Louisiana in 2003 for those children waiting for a transplant.
Another option is a living donation. This occurs when someone, usually a relative or a friend, steps forward and volunteers one, or part of one, of their organs. Since many organs -- liver, lung, intestines and the pancreas -- can grow in a recipient after the transplant operation, there is no need, except in the case of the kidneys, for a living donor to give an entire organ. In the case of the liver, the organ has regenerative properties that allow the organ to grow back in the donor after the transplant. This ability allows surgeons to fit adult organs into small children. And surprisingly, an adult-size kidney can fit in a baby as small as 5 kilograms.
There are other advantages for having a living donation. Generally, the donors are healthier, younger and have undergone a battery of tests to determine their physical and mental capabilities for making the donation. Additionally, there is less preservation time, which refers to the amount of time that passes between removing the organ and transplanting it into the new host. With living donors, doctors have the luxury of planning a transplant, so the donor and recipient are geographically close to each other, often in the same hospital.
This is not the case with a deceased donor. Thousands of miles might separate the donor from the recipient. Plus, the donor's parents must come to realize that their child is dead, but that, with this terrible tragedy, they can save a life.
"Pediatric donors are extremely rare, which is a good thing in the grand scheme since most kids survive major traumas and other types of injuries," Boudreaux says. "However, every now and then medical science won't be successful and you'll have a lethally brain-injured child. The donors are in the hospital, usually in the intensive care unit, and 90 percent of the time, machines are keeping the heart beating and the lungs breathing. The heart is pumping oxygenated blood to the organs, so the organs are still alive but the brain is not."
Sadly, continues Boudreaux, the machines do provide false hope. "There is a difficulty for people when they're seeing the EKG monitor blinking away and the ventilator moving the lungs up and down (to understand) that the patient is dead when in fact they are."
Once parents agree to donate their child's organs, it then becomes a scramble to enter the data into the UNOS database, generate lists of possible recipients (a deceased donor generally donates more than one organ), find the actual recipients, and then make arrangements for the organs to be removed, delivered and transplanted into their new owners. Incredibly, this whole process usually takes place less than 24 hours after the organs become available.
As Chloe Smith became sicker, her mother, Reyhana Couvillion, began to wonder if she could be her child's donor. During a phone interview from her home in Merryville, Couvillion recalls the meeting she had with Chloe's doctors: "I asked if there was any way I could test for it. They said it was very unusual for the mother to test. I said, 'Well, I want to see if I can.' After the test, they felt I could do it. They said I was the best match she could have."
Doctors decided to delay the operation on Chloe until she reached a body weight of 22 pounds. Unfortunately, even with the aid of fat-enriched baby formula and other additives for more than four months, Chloe did not grow in weight or length. And while the average age for babies to begin walking is 12 months and the average age for crawling is 8 months, after a year, Chloe hadn't attempted either. She was very weak, with little of the usual frenetic baby energy. Her bilirubin count, a reddish-yellow chemical pigment occurring in the blood and bile causing jaundice, soared. Chloe and her mother found themselves traveling more and more to New Orleans. There, the doctors would run test after test confirming what Couvillion already knew: Chloe's condition was worsening.
Doctors decided to proceed with the operation. On the morning of Oct. 27, 2003, a surgical team at Mercy Memorial Hospital led by Boudreaux and assisted by Dr. Thiagarajan Ramcharan removed the left lateral segment of Reyhana Couvillion's liver, about a third of the organ, packed it in an ice chest, and delivered it to Children's Hospital where another group of doctors and Chloe were waiting. For the next eight hours, Boudreaux and his team, including Dr. Ivonne Daly, worked to ensure a successful transplant. The results were immediate and gratifying.
"They said that as soon as she woke up, her eyes were wide as can be and went from yellow to white in seconds," Couvillion says. "Within a week of the surgery, she learned to crawl and then walk. Now, she's nonstop and full of energy. I guess she's trying to catch up on that year she missed."
The prognosis for Chloe is mostly positive. She will continue to take anti-rejection drugs for the rest of her life, and her immune system is severely challenged due to the residual effects of liver disease. Yet the piece of her mother's liver now inside her continues to grow and should last a lifetime. Boudreaux is looking closely at the next few months.
"In the world of transplantation, we talk about success in terms of one year of patient survival and beyond. If they make it through the first year, the likelihood of them making it through many years is quite good."
Boudreaux believes that the majority of organ transplants for children under the age of 5 comes from live donors. This is an encouraging sign considering the advantages of the living donor and that, according to the 2003 OPTN (Organ Procurement and Transplantation Network)/SRTR (Scientific Registry of Transplant Recipients) Report, pediatric transplant candidates generally face a higher mortality rate than their adult counterparts.
Still, no matter how the organ is received, the donation provides life for the recipient. Boudreaux, who is a tireless advocate for organ donation and yearly makes any number of presentations to interested groups, likes to show his audience a slide of a sunset/sunrise on the horizon. Boudreaux explains the ambiguous photo.
"I ask the audience which is it: a sunrise or a sunset. Half raise their hand for the sunrise and half for the sunset. I'll tell them that it's both, because the sunset on one individual's life is the sunrise on eight others through organ donation."