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Cystic Fibrosis and Liver Disease

Some children and young people with cystic fibrosis may have had prolonged jaundice as an infant. This usually disappears in a few months after birth. However, a number of children and young people with cystic fibrosis develop liver problems later in life regardless of whether they had prolonged jaundice in infancy.

Liver disease is very variable and progresses at different rates. Each child or young person therefore needs individual assessment and follow up.

Which children or young persons with cystic fibrosis get liver disease?

It is not possible to predict who will be affected. Boys seem to be affected more than girls.

The development of liver disease may be more likely in children who have had "meconium ileus" as a baby.

What happens to the liver in cystic fibrosis?

Damage to the liver starts in the small bile ducts. The bile produced by the liver cells into these ducts becomes stickier than normal and blockage of the ducts follows (this very occasionally causes jaundice). The surrounding liver tissue then becomes damaged and scarred, which may be referred to as biliary fibrosis. This is not unlike the underlying process occurring in the lungs. In time the fibrosis progresses and eventually spreads throughout the liver. This causes the liver to become hard and the blood flow through it more difficult.

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