Pioneering liver graft could save boy: Operation for jaundice holds out hope for haemophiliacs

A NINE-YEAR-OLD boy with a rare form of jaundice has had a quarter of his liver replaced in a pioneering operation that could save his life. It is only the second time in the world that surgeons have attempted this procedure, which holds out the hope of a cure for much more common diseases such as haemophilia.

Andrew Foran, from Aldershot, Hampshire, has been jaundiced from birth and has had to spend 15 hours every day on a 'sunbed' so that the ultra-violet light can break down the yellow bile in his skin.

Yesterday, his mother, Angela, said that she had seen her son with pink cheeks for the first time. Andrew and his eight-year-old sister, Sarah, both suffer from a deficiency disease called Crigler-Najjar syndrome, which means that they lack an enzyme, made in the liver, that breaks down bile. In all other respects his liver is normal.

As a result of the enzyme deficiency, bile has been deposited all over Andrew's body including his brain. The condition can lead to brain damage and death.

Last Wednesday, surgeons at King's College Hospital, south London, led by Mr Kai-Chah Tan, performed an orthotopic segmental liver replacement. In the nine-hour operation they cut away about 25 per cent of Andrew's liver and replaced it, in exactly the same place, with a segment of normal donor liver capable of making the missing enzyme.

Animal studies have shown that replacing only 5 per cent of a defective liver will produce enough of the enzyme to have an effect. Without treatment, Andrew had between 400 and 500 units of bile in his blood compared with 20, which is normal. His intensive light therapy brought his count down to 250.

Yesterday, six days after his surgery, the count was 75. His father, who is in the Army, flew home from Cambodia to be with his family. Andrew's mother said yesterday: 'We were at the stage where we really had no choice. Andrew just came home from school, had his tea and went to bed under the lights. I am really glad we went ahead. It is amazing to see his skin turning white and really amazing to see the whites of his eyes look white as well.'

Mr Kai-Chah Tan, who carried out the operation with Mr Nigel Heaton, said that he was very pleased with Andrew's progress. 'The operation is very intricate but we are happy so far. The patient is doing very well. It is the first time in his life that he has been without his light treatment for this length of time.'

He said that the decision to go ahead with the surgery had been made because Andrew had started to show some weakness in his legs, a sign of the beginnings of neurological damage.

'One of the advantages of this operation is that if the graft does not work he will not be worse off. His liver, which is otherwise normal, will go on working.

'This is a genetic disease and we can expect the gene for it to be discovered in five to ten years time so that gene-replacement therapy can be given. The surgery should let him live a normal life until that happens,' Mr Tan said.

But the operation, which cost about pounds 20,000, has far wider implications. It could be used for much more common conditions, such as haemophilia, when a normal liver segment would produce the missing blood- clotting factors, or familial hypercholesterolaemia in which the liver cannot break down the fats in blood.

One American adult has had the same operation as Andrew, being given a segment of liver donated by his father.

This had been partially successful, Mr Tan said. The patient no longer needed light therapy although he is still jaundiced.

(Photograph omitted)