Friday, February 26, 1999 Published at 05:45 GMT
Liver disease babies dying 'needlessly'
The Kasai operation is very intricate
Children with a rare liver disorder may be dying unnecessarily because they are not being treated at specialist centres, according to a BBC programme.
If they fail, the only other resort is a liver transplant that can be risky for very young children.
Without any treatment, children usually die before reaching their second birthday.
Bile duct disorder
Around 50 babies in England and Wales are born with the liver condition each year. It means the tiny bile duct to their liver is blocked and poisons build up, causing jaundice.
The current treatment for the condition is an operation called Kasai-poroenterostomy.
The operation involves taking a loop of intestine and forming it into a pipe to drain the bile from the liver.
A spokeswoman from the Children's Liver Disease Foundation (CLDF), which has been monitoring problems with Kasai, said: "It is a very intricate operation."
Children are often very ill or malnourished and require a lot of care. Even after the operation, they need to be fed a special diet to build them up.
They may also need drugs and will be monitored to ensure they are free of jaundice.
Recent follow-up research by the foundation shows that hospitals that only carried out one operation a year had a 17% success rate, compared with a 64% success rate for those performing five or more operations.
If the operations are unsuccessful, the children will need a liver transplant. But some are too young or too frail to have an operation.
In some cases, the transplants fail and another operation is needed. The foundation says some children have as many as three transplant operations.
Victoria Iddon was too sick to have a transplant because her local hospital failed to refer her for one early enough.
She died before her first birthday. Her hair had turned yellow because of the jaundice.
In 1985, the government opted to designate specialist centres for treating children with biliary atresia.
The CLDF considers three fall into the category. They are Birmingham Children's Hospital, King's College Hospital in London and St James' Hospital in Leeds.
The move was designed to encourage doctors to refer children on for treatment.
But in 1995, the government reversed this decision.
Campaigners believe that the creation of local trust status in hospitals has also encouraged NHS managers to do more operations locally.
The CLDF wants a reversal of this position. And it says this would save money. A Kasai operation costs around £7,000 plus follow-up care and drugs.
A liver transplant costs around £40,000 together with drug costs.
However, chief executive Catherine Arkley says the Department of Health "will not act unless it receives recommendations from the relevant professional bodies".
Mark Stringer, of the British Association of Paediatric Surgeons, says he also backs the case against local hospital operations.
A Department of Health expert group is meeting to discuss the issue in March and could consider redesignating up to six specialist centres around the country.
A spokeswoman said the 1995 reversal was based on advice from the Royal College of Surgeons, which now appeared to have changed.
However, the government says it cannot stop surgeons from carrying out the procedure if they want to and their hospital backs them.
Nottingham's Queen's Medical Centre has already decided to refer to specialist centres after figures showed 19 of the 29 babies it had operated on for biliary atresia had died.
BBC Two's Trust Me, I'm a Doctor reports on biliary atresia at 8pm GMT on 26 February.