Saved by a stranger
It did not come entirely as a surprise when Ms Katherine Chong's third child, Lim Si Jia, was diagnosed with glycogen storage disease eight years ago, when she was eight.Si Jia's brother, who is two years older, also developed the disorder when he was around the same age. Ms Chong's elder daughter, 21, is the only one of her children who does not have the disease."Every parent would be very sad," said Ms Chong, 52, who works in the insurance sector. "But there is a reason for everything, and we had to accept the reality."The genetic condition is estimated to affect one in every 100,000 people globally.Si Jia, like her brother, would eventually have to get a liver transplant to stay alive. Her brother received a new liver from a brain-dead patient last year.
Random act of kindness
For Si Jia, now 16, the donor was Mr Lim Kok Seng, 54, who simply wanted to give part of his liver to someone on the national waiting list who needed it the most.It is the first time that a non-directed liver transplant–where the donor does not come forward with arecipient in mind–has taken place in Singapore.People with glycogen storage disease cannot produce an enzyme that is needed to break down the body's stores of a compound called glycogen.When this happens in the liver, glycogen accumulates and often results in the organ swelling.As glucose–or blood sugar–is derived from glycogen, people with the disorder often also sufferfromlow blood sugar levels.So, Si Jia had to regularly drink a solution of uncooked starch–such as cornstarch mixed with water–before bedtime every night.
Running out of time
While she could attend school like other children, there was still an urgent need for a transplant.At a height of only 1.49m, Si Jia is shorter than the average adult woman here, who is about 1.6m tall."If we didn't do a transplant in time, she would remain at her present height throughout her puberty," said Professor Quak Seng Hock, who heads the paediatric gastroenterology, hepatology and nutrition division at the National University Hospital.A tumour–benign, but which could be potentially cancerous–was also discovered in Si Jia's liver.Last year, there were a total of 38 liver transplants, 20 of which used livers from dead donors. There were 54 people on the waiting list for a liver transplant last year."I'm really grateful to Mr Lim, and I really admire his courage and determination," said Si Jia, who requires lifelong medication to prevent her body from rejecting her new liver.
Professor Krishnakumar Madhavan, co-director of the National University Centre for Organ Transplantation, said the majority of living organ donations worldwide are directed–that is, the donorhas a specific recipient in mind.Said Prof Madhavan: "This is the first time in our experience with somebody who steps up and says, 'I want to donate; it doesn't matter to whom.'"Mr Lim, who simply wanted to be able to help someone, decided not to wait until after his death to donate his liver because he was not sure whether it would still be in good working order by then."When you are above 60, you know, complications do come in all forms," he said. "Even if I made the pledge, my liver might not be good (enough) to help any more."He also wanted to make the donation before he turns 55, as doctors generally recommend that people who want to donate their organs do so before this age.Typically, potential donors go through a lengthy counselling process over months to make sure they are aware of the risks and still want to go ahead.They are given the option to back out at any time before the surgery. Both sides do not know who the other person is until after the operation–and only if both agree to disclosing their identities–so astoavoid feelings of obligation.Mr Lim met Si Jia, who now has 60 per cent of his liver, for the first time about a month ago. The rest of his liver will regenerate within three months, doctors said.Said Mr Lim: "I had only one request, which was that (the liver) be given to a younger (recipient), so that they have much more life ahead of them."A version of this story first appeared in The Straits Times.(Photos: Mark CheongST and 123RF.com)