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pixie · Site Admin Joined: 16 Jun 2007 Posts: 1968

One-year-old James Jenkins has a new liver.

His mother is delighted her son has a new lease of life following liver disease, but she is angry that "ignorance" put him in danger.

Despite raising concerns about his continued jaundice, the mother-of-two says many obvious warning signs of his disease were missed.

Signs, she now knows, should have been blindingly obvious to health professionals and herself.

"Because they said he was OK, I just thought 'fine, he is OK'. That is something I have got to live with," said Maria.

The UK's Children's Liver Disease Foundation (CLDF) has launched a Yellow Alert campaign to raise awareness among both parents and medical staff about the warning signs in newborns.

WARNING SIGNS
Jaundice lasting longer than two weeks
Pale stools - stools should be English mustard yellow or green in bottle-fed babies and daffodil yellow or green in breast-fed babies
Yellow urine - newborns' urine should be colourless

About half of all newborn babies are jaundiced, but only one baby each day will be diagnosed with a liver disease.

The CLDF says something as simple as regularly checking the nappy - looking for colour changes in the urine and faeces - can show which child is at risk.

Catherine Arkley, chief executive of CLDF, said: "The warning signs of liver disease in a new baby are not being recognised and therefore they are not getting their treatment at the optimum time.

"At least 50% of babies will become jaundiced shortly after birth and that is totally normal."

It is particularly common among breast-fed babies.

She explained that the immature liver takes a little time to start working efficiently.

"Most babies do not have liver disease. We are concerned with the babies where the jaundice continues beyond two weeks of age, because normally that baby jaundice should have sorted itself out by then," she said.

"There are signs we can pick up very readily that should alert us.

"The problem is that community health professionals are very often not aware of the signs.

"They often reassure parents that there is nothing wrong and it is not until later on that they find it is abnormal.

"Also the babies look really well and they feed very well and that all fools you."

Maria, 38, said that despite her continued worries about James, she would not have done anything had her husband not become alarmed about their son's continuing yellow appearance, which he noted on his return after being away from home for a few days.

At first Maria was told James had the common breast milk jaundice, but one midwife was concerned at three weeks and asked for a bilirubin test.

The test results showed jaundice, but Maria said she was told by the doctors and community medics that James was doing fine and not to worry.

James had a Kasai portoenterostomy at 12 weeks

"So we just carried on. I even showed one of them his nappy and pointed out the pale poo," Maria recalls.

At 12 weeks, Maria took James back to the GP who sent him to the hospital.

This time he was immediately referred to the Birmingham Children's hospital where he was diagnosed with biliary atresia - a potentially lethal liver disease.

James's surgeon agreed to carry out a special procedure, called a Kasai portoenterostomy. Ideally this should be carried out before the liver becomes damaged and before the baby is eight weeks old.

The surgery appeared to work well, but tests six months later showed the liver damage was continuing and James needed a transplant.

But by now his body was stronger and able to accept the more easily obtained cut-down adult liver.

The Kasai portoenterostomy - where a loop of bowel is used to form a duct to drain the bile from the liver - bought James' vital months to grow.

James, who had his transplant this summer, was born at 39 weeks.

"It is a miracle James is still with us and doing so well now."

Mark Davenport, consultant paediatric liver surgeon at King's College Hospital, London, said an early diagnosis was vital for babies like James.

"The earlier a diagnosis is made, the less damage will have occurred in the liver and the higher the chances of sparing the child from a liver transplant at a young age.

"Time is of the essence when dealing with a condition like biliary atresia. "

http://news.bbc.co.uk/1/hi/health/7027632.stm