When Sharon McBride’s baby was diagnosed with biliary atresia, a form of liver disease, she was stunned to discover that there is very little awareness of the disease, which is in fact more common than childhood leukaemia. Every day in the UK at least two children are diagnosed with liver disease, a condition which is always lifelong and often life threatening.
What is childhood liver disease?
Other than the brain, the liver is the most complex organ in the body, processing food to make and store energy and filtering unwanted substances from the body.
There are over 100 different liver diseases that can affect babies, children or young people and for most of them the cause is unknown. Sharon’s daughter, Hannah, was diagnosed with one particular form of liver disease called biliary atresia which is unique to babies, affecting about 1 in 16,000 live births. The bile ducts are either missing or damaged which is usually
as a result of an inflammation in the bile ducts at birth. “Hannah was apparently a completely healthy newborn,” Sharon says. “We first realised there was a problem when her jaundice continued after she was two weeks old.
Blood tests showed Hannah had a high ‘conjugated bilirubin’ which meant that the jaundice was caused by an obstruction of bile flowing from the liver. A scan showed a cyst on the bile duct and during surgery to remove the cyst the specialist discovered Hannah had biliary atresia”.
How many children are affected?
According to the Children’s Liver Disease Foundation (CLDF) two children are diagnosed with liver disease every day and the charity are currently providing practical and emotional support to over 5000 families across the UK. Around fifty of the children born with liver disease each year are diagnosed with biliary atresia.
Sharon says, “Biliary atresia is very difficult to diagnose and most babies have to go through lots of tests to make the diagnosis as certain as possible. It cannot usually be confirmed until surgery.”
What causes it?
Sometimes the cause of liver disease can be hereditary or genetic. Conditions which are hereditary include progressive familial intrahepatic cholestasis (PFIC) and Tyrosinaemia type I, both of which appear in the first few weeks of life and need treatment and possibly a transplant.
However, for most childhood liver disease the cause is unknown. When the liver cells are damaged then inflammation known as hepatitis occurs, disrupting some or all of the liver functions. The causes of liver damage include:
- An obstruction to the flow of bile out of the liver.
- An infection acquired before the baby was born, during delivery or caught at an early age after birth.
- Metabolic liver disease. If one of the chemical processes occurring in the liver is faulty, liver damage may occur. The cause is usually genetic.
- Certain drugs or poisons will damage the liver
- Poor blood supply
Like many liver diseases, the cause of biliary atresia is unknown although it is not thought to be hereditary.
What are the signs and symptoms?
Like so many people, Sharon admits to knowing nothing about liver disease before Hannah’s diagnosis.
“We were completely ignorant as to what the liver actually did or how it could go wrong,” she says.
However, when Hannah was still jaundiced – yellow – when she was four weeks old she had a blood test at the local hospital.
Sharon says, “Hannah had pale yellow stools and bright yellow urine - classic symptoms of obstructive jaundice.”
Other symptoms include:
- Jaundice – yellow skin and the whites of the eyes
- Nausea and vomiting
- Abdominal swelling caused by an enlarged liver
- Ascites – swelling of the abdomen caused by fluid
- Poor weight gain
Can it be detected in pregnancy?
According to the CLDF there are some liver diseases of infancy which can be detected by pre-natal diagnosis but for the vast majority of liver diseases they cannot be seen on an antenatal ultrasound scan.
If a child is diagnosed with a genetic liver disease then in many cases mothers can be offered pre-natal diagnosis in subsequent pregnancies. Some conditions only occur after birth and diseases like biliary atresia seem to occur randomly.
There is a 1 in 14,000 birth chance and you can’t predict which baby it will affect.
Is there a cure?
Rachel Markham, communications officer at the CLDF says, “Today there is no cure for liver disease, just medication to cope with the symptoms of liver disease.
Children’s Liver Disease Foundation is the only organisation in the UK dedicated to stamping out childhood liver disease. We fund vital research into childhood liver disease which will hopefully one day find a cure”.
Liver disease can be treated with medicines, special diets and surgical procedures. Sometimes a liver transplant is needed.
Hannah underwent surgery called a Kasai procedure to restore the flow of bile from her liver. In 33 per cent of cases the procedure is unsuccessful and a transplant is needed in the first year of life but Hannah has recovered well and has not needed to be hospitalised since.
Sharon says her husband Andy took the enormity of Hannah’s condition on board much quicker than she did, and her older daughter Caitlin, whilst too young to fully understand the implications of the disease, knows more about doctors and hospitals than most children her age.
About the CLDF
The Children’s Liver Disease Foundation (CLDF) the only organization in the UK dedicated to stamping out childhood liver disease. As well as funding vital research into childhood liver disease and offering support to children and their families, they also fund vital education programmes.
Rachel says, “We run study days for healthcare professionals including health visitors and midwives to help promote early diagnosis and referral - which is so important in cases such biliary atresia where quick treatment is vital.” The charity, headed by Catherine Arkley, a leading authority on paediatric liver disease, has also developed over 50 different information
leaflets for parents.
Sharon found the information and support from the CLDF invaluable and would recommend parents wanting to know more should get in touch with them.
“They have lots of information and great family support,” she says but adds, “Childhood liver disease is not a priority for government funding and research in this area has to be funded by the Children’s Liver Disease Foundation.” Although Hannah is doing very well, she will have liver disease for the rest of her life.
“She is just like any other nearly 18 month old, and full of life,” says Sharon. “The prognosis for biliary atresia is liver transplant, and Hannah will almost certainly need a transplant one day, but no-one can predict when, until it becomes imminent.
Not many people have heard of childhood liver disease or how devastating it is.
It is important that new parents know how to spot when newborn jaundice is symptomatic of something else that needs treatment.”
Rachel Markham says, “Many families fundraise for us – they find that having a positive goal helps them through some of the difficult times. But we desperately need more funds to continue our support, education and research programmes.
We have a wide range of challenges that people can take part in or they can make a donation. Our website (www.childliverdisease.org) has lots of information on how people can help.”
Although Sharon admits to feeling numb for a while, she then suffered depression and became obsessed with her daughter’s illness. She says to parents going through the same thing, “Take it a day at a time. Learn about your child’s diagnosis and treatment and keep your own records.
It has helped me to make contact with others in the same position. Use the diagnosis for good and raise awareness of childhood liver disease and organ donation.”
- Contact the CLDF at www.childliverdisease.org or call them at 0121 212 3839.