How can cystic fibrosis affect the liver?

How can cystic fibrosis affect the liver?

The liver is a large organ found at the top of the tummy (abdomen) on the right side. It is made up of cells (hepatocytes), blood vessels and bile ducts.

The liver has many important jobs in the body including:

• cleaning (filtering) the blood
• making blood plasma proteins, clotting factors, cholesterol, vitamin D, hormones, and bile

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Location of the liver

People with cystic fibrosis have a gene which means they can’t move salt and water between their cells properly. This causes fluids in the body to become thicker. The fluids can block the body’s tubes and passageways.

In some people, cystic fibrosis affects bile. This is a fluid made by the liver. The bile becomes thicker than normal and blocks the bile ducts. Blocked bile ducts can cause damage and scarring to the tissues around them. This is called biliary fibrosis. It is like the damage which can happen in the lungs of people with cystic fibrosis.

My son had no symptoms of liver disease… no one had mentioned livers to us before, so it was quite a shock.

-Parent

In some people, the damage spreads throughout the liver as the cells start to die. The healthy cells are replaced with scar tissue (fibrosis). This makes it hard for blood to flow through the liver. It may eventually lead to severe scarring of the liver, known as cirrhosis.

If cystic fibrosis affects the liver, it is called cystic fibrosis-related liver disease (CFLD).

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

Around 40 out of 100 people with cystic fibrosis have changes involving their liver. These changes are often mild. Most people show no signs or symptoms and do not develop serious liver problems.

Around 5-10 out of 100 children with cystic fibrosis develop liver problems that have an impact on their health.

Cystic fibrosis-related liver disease usually develops in the first 10 years of life. We do not know who will be affected. We also do not know why some people with cystic fibrosis get liver disease and some do not.

Some research says that cystic fibrosis-related liver disease may be more common in:

• boys
• children who had a condition called meconium ileus as a baby. This is where a baby’s first poo (stool) gets stuck in the small intestine.
• children and young people who have the SERPINA1 gene. This is a gene for another condition, called alpha-1 antitripsin deficiency, which can also affect the lungs and liver.

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Next: Diagnosis and monitoring

Special thanks

Information reviewed: July 2024

Next review date: July 2027

We would like to thank staff at each of the specialist paediatric liver centres: Birmingham Children’s Hospital, King’s College Hospital and Leeds Children’s Hospital. We would also like to thank the families who helped us produce this information.

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