When Matthew Russell was born nearly two years ago he was a healthy, vigorous baby. Within a few days he had developed a life-threatening liver disease and when he was three months old had to undergo a major operation which, against the odds, was completely successful and saved his life.

Matthew suffers from biliary atresia. Little is known about the early development of the disease, but the effects become apparent shortly after birth. The biliary tract, which carries bile from the liver to the intestines, becomes inflamed and blocks the flow. Bile consists of the waste products from the breakdown of blood and other metabolic processes in the body and needs to be excreted.

The inflammation spreads back up the bile ducts into the liver itself and, if the flow of bile is not restored through surgery, permanent liver damage will develop. 'Without an operation, these children are likely to be in real trouble by the end of their first year and will probably die shortly after their first birthday. A liver transplant is the only hope for them,' says Professor Alex Mowat, of the paediatric liver unit at King's College Hospital in south London.

He believes that a simple change in postnatal care in this country could revolutionise the care of children suffering from biliary atresia - and many other conditions which develop or become apparent in the first few weeks of life.

After discharge from hospital, babies (and mothers) are not examined by a doctor until the six- week check-up. Professor Mowat would like all babies to be examined thoroughly by a doctor four weeks after birth. As well as picking up babies with liver problems, a four-week check would detect some congenital heart defects that are not noticeable in the first few days after birth, and any dislocation of the hip joints. All these problems would benefit from early diagnosis.

For a child with biliary atresia, the chance of corrective surgery being successful after six weeks - and enabling the liver to function fully again - drop to 30 per cent. Babies who have the operation before they are six weeks old have a much higher chance (80 per cent) of liver function returning.

Professor Mowat says that diagnosis can be difficult. 'The trouble is that

in the first two months of life, apart from mild jaundice, these babies look well. They are growing and gaining weight;

it is difficult to realise that they

are concealing a life-threatening


Matthew's story is typical. 'He suffered from slight neonatal jaundice but that was all, and he was clearly thriving. Between the ages of ten days and two weeks he looked slightly yellow, so we put him in the garden. It was warm and sunny and it was difficult to tell whether or not the jaundice had gone - as a family we have quite dark skin,' his mother, Elizabeth Russell, explains.

'At the six-week check Matthew was still mildly jaundiced. We were told not to worry about it - that he was suffering from breast-milk jaundice. Some days he looked sad and droopy and was a bit limp, but not exceptionally so. At other times he was lively, so we were reassured. His stools were pale, but we were not warned to look out for anything.'

But despite the reassurances, she still felt anxious. 'We had further tests done, but it was not until Matthew was 12 weeks old that we were told he had a serious problem with his liver and we were to be referred as an emergency to King's. The consultant who broke the news to us said that some babies died, some didn't. We were stunned.'

Professor Mowat explains that the jaundice due to biliary atresia is just like ordinary jaundice in that it develops within a couple of days of birth. 'The difference is that the jaundice does not clear. Some midwives and health visitors simply do not realise the significance of this.'

The condition is rare and affects about one baby in 10,000 to 20,000. In the United Kingdom, about 50 are referred every year for surgery. Professor Mowat does not, however, think that that is much of an excuse for not recognising early symptoms: as many as one in 500 of all newborn babies may have a liver-related problem. Moreover, in the nought to four age group more children die from liver disease than from cystic fibrosis and leukaemia.

He says that there are other conditions which can lead to jaundice in the newborn and these all need to be investigated. Some are caused by infection and some by metabolic disorders. Some children might eventually need a liver transplant - as did Laura Davies, who recently travelled to Pittsburgh, in the United States, for a liver and bowel transplant.

Yet mothers like Elizabeth Russell are often told that their babies are suffering from breast-milk jaundice, a benign condition that affects one in 200 breast-fed babies. That usually clears up quickly. After 11 days in infants born at term and 14 days in preterm infants jaundice is very unusual, occurring in only 0.5 per cent of babies admitted to paediatric units.

Professor Mowat says: 'I want colleagues and others to know that jaundice in the newborn can be something to worry about. Doctors and nurses are taught that people with liver disease have dark urine. While this is true of adults and older children, because babies drink so much their urine is so diluted that the colour is weakened.'

According to Professor Mowat, the ominous signs in a jaundiced infant - urine that is not colourless and stools that have no yellow or green pigment - are easily spotted but are rarely volunteered by parents, especially when the child is their first. 'Baby books and health education publications do not mention these important signs,' he points out.

The operation for these babies is called the Kasai procedure, after the Japanese surgeon who pioneered it in the early Fifties. It is undertaken at King's College Hospital, which does 20 to 30 a year, and at Birmingham Children's Hospital. It involves removing all the blocked bile ducts back towards the liver. The liver is then attached directly to the gut so that the bile flows straight into the latter.

The operation is not a guarantee of full recovery. The inflammation does not disappear immediately and, even when the procedure is successful, the bile flow through the liver may take nine months to settle down to the correct speed. The problem is that if the inflammation has managed to take too great a hold on the liver, the tissues become toughened and eventually the liver stops functioning.

Doctors and parents wait anxiously in the days and weeks after the operation. Will the faeces of the baby start to become pigmented, or will they stay pale and colourless?

Elizabeth Russell remembers the time of waiting with pain. 'That was the worst week of all for us. We were in tears for the weekend, waiting at King's while tests were performed and the medical staff decided what to do. The operation had to be put off three times, which was agony.

'Matthew was incredibly lucky. The operation was completely successful, bile started to flow again immediately and his jaundice started to fade. Since then he has been fine. He has to take vitamin supplements and phenobarbitone to stimulate the bile flow, but otherwise he seems a normal child. We have to take him back regularly for check-ups and liver-function tests and he seems to be more susceptible to minor infections. He has been in hospital twice with respiratory problems. We could not hope for more.'

For the foreseeable future, Matthew's prospects are good, certainly until his teens. 'We have been told he will not be able to drink alcohol, he may not live beyond his early thirties or he may need a liver transplant - who knows,' says his mother.

In Japan, the postnatal check takes place at four weeks and all children with jaundice at 14 days have their urine checked. As a result, the success of the surgical correction for biliary atresia has gradually improved. For hundreds of children with biliary atresia yet to be born in this country, a simple change in postnatal practice could save their lives.

Children's Liver Disease Foundation, 40- 42 Stoke Road, Guildford GU1 4HS (0483 300565).

(Photograph omitted)