¦ At least one baby a day is born with liver disease every day in the UK.

¦ Liver disease in babies is indiscriminate – there is no specific group at risk.  Everyone needs to "think liver".

¦ There are more than 100 liver diseases that can affect babies. For most, the cause is unknown.

¦ Most liver diseases are life-threatening and mean a lifetime of care.

¦ Biliary atresia is a lethal liver disease which is unique to childhood and affects approximately 50 babies in England every year. If left untreated it is fatal with a mean age of death of two years. It is vital that biliary atresia is identified and surgical treatment given at an experienced centre before the baby is eight weeks of age.

¦ Prolonged jaundice

Jaundice is the name given to the baby’s yellow appearance of the skin and the whites of the eyes and is very common in newborn babies. It reaches its peak at about four days and then gradually disappears in most babies by the time they are two weeks old.

Prolonged jaundice can be a sign of liver disease and is defined as jaundice persisting beyond two weeks of age in a term baby and three weeks in a pre-term baby.

¦ Persistent yellow urine staining the nappy can be a sign of liver disease – urine in a healthy baby should be colourless.

¦ Persistent pale coloured stools may indicate liver disease – healthy coloured stools can be described as English mustard yellow in bottle fed babies and daffodil yellow in breast fed babies.

¦ A significant number of babies with prolonged jaundice will have breast-milk jaundice. This is harmless and disappears without the need for treatment. Breastfeeding can be continued safely.

¦ Parents are frequently reassured that prolonged jaundice is breast-milk jaundice. In some cases the cause will be liver disease which is potentially life threatening. The only way to distinguish between breast-milk jaundice and liver disease jaundice is by doing a special blood test – a split bilirubin blood test.

¦ Newly born babies with persistently pale stools and / or yellow urine should be referred to a paediatrician immediately for investigation.

¦ All suspected babies should be referred promptly to a doctor.

¦ A simple split bilirubin blood test costing £1.50 should be conducted to rule out liver disease in a baby with prolonged jaundice and/or showing other signs. This can be carried out anywhere in the UK.

¦ If the conjugated bilirubin level is greater that 20% of the total bilirubin (identified by the split bilirubin test) the baby should be referred immediately for investigation by a paediatrician.

¦ Further investigations will be needed to identify the liver disease.

¦ Treatments vary according to the liver disease. Some are managed by diet, some medical intervention and surgery is required in others.

¦ Approximately 50 babies will be diagnosed with biliary atresia every year in England.   These babies will need an operation called Kasai portoenterostomy (named after the Japanese surgeon, Professor Morio Kasai who developed the procedure in 1959). Kasai portoenterostomy needs to be carried out before the liver becomes damaged and, ideally, needs to be performed before the baby is eight weeks old.

¦ If surgery fails in babies with biliary atresia, the baby’s only other option is a liver transplant costing around £80,000 and means a desperate wait for a donor organ.

¦ There are three supra-regional centres in England designated to carry out Kasai portoenterostomy: Birmingham Children’s Hospital, King’s College Hospital, London and St James’s University Hospital, Leeds.

¦ These units are specialist children’s liver centres caring for children from all over the UK and provide surgical (including liver transplant) and medical management.

¦ Healthcare professionals can contact the three specialist paediatric liver centres directly for advice.

¦ Identifying babies with liver disease can be difficult. For community healthcare professionals it is only one of many diseases for which they need to be alert.

¦ Parents are unaware of the signs and think yellow urine is normal.

¦ For those babies with liver disease the consequence of a late diagnosis is death or a liver transplant at a very young age. The human cost is incalculable.

¦ CLDF is the only organisation in the UK dedicated to stamping out childhood liver disease.

¦ CLDF is launching the Yellow Alert campaign, a campaign targeted at parents, parents to be and healthcare professionals and aims to highlight the signs of liver disease in the newborn baby and the steps to be taken in those babies identified with the signs.

¦ CLDF intends that Yellow Alert will save lives and reduce the need for liver transplant.

¦ CLDF intends that Yellow Alert will empower parents to be alert to the signs as well as healthcare professionals.

 (12/10/07)