A better
life ... Eight-year-old Rama, who has a metabolic liver disease. She
became Australia's first liver cell transplant recipient at the Royal
Children's Hospital in Melbourne.
If everything goes according to plan, eight-year-old Rama's life could
change forever.
A defective liver that has left her jaundiced and bound to a light
box for treatment eight hours a day for all of her short life may
soon function properly after she became Australia's first liver cell
transplant recipient.
It is hoped the transplant, a relatively simple procedure in which
healthy cells are infused into the liver, will mean Rama, from Melbourne,
will not need a full organ transplant - for which waiting lists are
long- later in life.
Rama, whose family wished to remain anonymous, was born with the genetic
condition Crigler-Najjar syndrome: her liver cannot process the yellow
pigment bilirubin in the blood, which is usually removed from the
body through bile.
"Now, her liver is unable to do this,
and because it's unable to do this the bilirubin builds up in the
blood and can cause brain damage," said Winita Hardikar, head
of hepatology at Melbourne's Royal Children's Hospital, who performed
the procedure with Katie Allen, a pediatrician at the Murdoch Children's
Research Institute. |
| Before the transplant, Rama would spend eight hours
a day in a specially made phototherapy box, which would process the
bilirubin her liver could not. "Unfortunately this is a terrible
thing on her lifestyle because as an eight-year-old she has to wake
up at 4.30 in the morning, sit in this box for three hours, then she
goes to school, then she
|
|
comes home and sits foranother four hours,
"Dr Hardikar said. "So it's a big impingement on lifestyle,
she goes to bed at midnight, and it's really surprising what a bright
button she is."The doctors took cells from a healthy donor liver,
and inserted a catheter into Rama's stomach. Over the curse of an
hour, they slowly injected the liver cells into the main vein that
feeds into Rama's liver.
"What happens is they have this
amazing ability to then find their home in the substance of the liver
and take up, just like Rama's other cells," Dr Allen said.
As with a full organ transplant, there is a chance Rama's body could
reject the transplanted cells, and she is being treated with anti-rejection
medication.
But so far, it appears to have been a success, with Rama able to return
home yesterday. Her condition will be closely monitored, and she still
needs some phototherapy, but much less than she once did.
Had Rama not had the cell transplant, Dr Hardikar said, she would
inevitably have needed a full liver transplant. Transplant waiting
lists are long: doctors have to wait 10 months for a suitable donor
from which to extract the liver cells for Rama's transplant.
The technique means that, in the future, one liver may be able to
provide cell transplants to many more patients, rather than just one
or two as is now the case. It has been used on about five to 10 children
worldwide, including two with the same condition as Rama. Amanda
Dunn. Sidney. 8/12/2004. |
