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2006 February 27 - Double celebration for family of liver disease girl

The family of a local girl born with a life-threatening liver disease are looking forward to celebrating her first birthday and enjoying a very special Mothers day next month. The picture was much bleaker last March.

After an ultrasound scan when she was 28 weeks pregnant mum Alex and dad Steve from Doddington Road were told that their unborn baby had duodenal atresia.  This is a rare condition where the duodenum (first part of the small intestine) was closed off. This stops food and fluid passing from the stomach into the intestines and often children with the condition are also found to have Down’s syndrome.

Mariella Jack was born on 10th March 2005 at Queen’s Medical Centre, Nottingham, and at just 24 hours old she underwent a corrective operation. It was during this procedure that the surgeon noticed her intestines were malrotated, this was corrected, and that her gall bladder was underdeveloped and he couldn’t see any bile ducts. 

After the operation, blood tests for Down’s syndrome came back negative to the huge relief of the family.  However their jubilation was short lived as doctors explained that they suspected Mariella had biliary atresia, a liver disease in which the bile ducts become progressively blocked leading to irreversible liver damage. 

The family were transferred to the liver unit at St James’s University Hospital in Leeds, one of only three specialist centres in the UK, where the diagnosis was confirmed.

Steve, a countryside service manager, says, “We were told that even if Mariella survived she would have long-term health problems. Mariella is our first baby and we were devastated. We didn’t know that children could get liver disease before she diagnosed, and this made it even harder to cope with.   We have since learnt that two children a day are diagnosed with a liver disease in the UK.” 

Mariella was still only 29 days old when she underwent her second operation, a corrective procedure for biliary atresia called a kasai (named after the Japanese surgeon who pioneered the operation).  It was during this operation that the surgeon identified that Mariella’s spleen was malformed and she had a rarer form of biliary atresia known as ‘biliary atresia splenic malformation syndrome’.  However the kasai was a success and Mariella was finally allowed home for the first time on the 19th April, which the family describe as ‘like being released from prison’. 

Although Mariella is now doing well she will never be out of the woods and will require monitoring and daily medication for the rest of her life.  She may also require a liver transplant sometime in the future if her condition deteriorates.

However the couple are now enjoying spending quality time with Mariella and are also keen to raise awareness of childhood liver disease and the work of Children’s Liver Disease Foundation (CLDF), the charity which offered vital emotional support when Mariella was diagnosed.  Alex has recently become a ‘CLDF Ambassador’, part of a nationwide league of supporters who organise activity and help raise funds and awareness in their local area.

Alex, a speech and language therapist, adds, “CLDF is the only organisation in the UK fighting childhood liver disease.  They have been a lifeline for us over the past year, providing much needed information and emotional support.  We want to help raise awareness and funds for the charity to help them continue their vital work which includes funding research. Like all families of children with liver disease, we hope that this may lead to a cure in the future.”

Mariella will be having a small party at home to celebrate her first birthday on 10th March. The family will also be together for Mothers Day on 26th March and Alex explains this will also be a special day, “I will never take being a mum to Mariella for granted, I cherish every moment.   This experience has also brought me much closer to my own mum who has been a wonderful support, so Mothers day will be extra special this year.”

Catherine Arkley, chief executive of Children’s Liver Disease Foundation, adds, "We are delighted that Mariella is doing so well.   It’s so special for them to be able to celebrate a birthday and Mothers Day together.  Families like the Jack’s are truly devastated with a diagnosis of childhood liver disease.  Fortunately we are here to pick up the pieces as well as work to provide a future for the family.  The Jack’s still do not why or how their child had a liver disease.   The fact is that there are over 100 different liver diseases which can affect any child of any age and quite simply we don’t know why they occur.  We desperately need more funds to continue our support, education and research programmes.”

For further information on the work of the Foundation, please visit www.childliverdisease.org or call 0121 212 3839.

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For further information on this story please contact Rachel Markham, Children’s Liver Disease Foundation, Tel: 0121 212 6012, e-mail: communications@childliverdisease.org

Note to editors:
The Children’s Liver Disease Foundation is a unique national charity that fights childhood liver diseases through funding pioneering research and educating healthcare professionals and the general public. Formed in 1980, it provides professional, emotional support to families affected and is the only organisation of its kind in the UK.

Childhood liver disease – the facts
• More children in the UK are currently diagnosed with a liver disease than childhood leukaemia
• At least two children are diagnosed with a liver disease every day in the UK
• Most childhood liver diseases are life threatening; all mean a lifetime of care
• There are over 100 different liver diseases that can affect babies, children or young people
• For most childhood liver diseases the cause is unknown
• Liver disease has no cure
• CLDF is the only organisation in the UK dedicated to stamping out childhood liver disease