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Liver disease and cystic fibrosis

Cystic fibrosis (CF) can cause the blockage of small ducts in the liver, leading to CF-related liver disease. Although this only happens in about eight per cent of people with CF, and can sometimes be managed by drugs, it is a serious health risk and can require a liver transplant.

Here we answer some of the most common questions about how cystic fibrosis (CF) affects the liver, including:

How common is cystic fibrosis-related liver disease?

Around 40% of people with CF will have some liver abnormalities, although only around 5–10% of the CF population will experience problems with their health as a result.

In recent years, an increase in prevalence of CF-related liver disease (CFLD) has been noted, although this is most likely because people with CF are now being screened for liver disease from a young age.

How does cystic fibrosis affect the liver?

Liver disease in people with CF can be related to problems with the drainage of bile out of the liver via the bile ducts and gallbladder, or to scarring in the liver, which is known as fibrosis and cirrhosis.

Bile ducts and gallbladder

Cystic fibrosis causes problems with the normal secretion and function of bile due to cells being unable to transfer chloride effectively. This means the bile can become sticky, causing irritation and inflammation in the ducts and potentially obstructing bile ducts by the formation of gallstones.

Fibrosis and cirrhosis

Scarring, or fibrosis, can develop in the livers of people with cystic fibrosis. In severe cases, this can lead to cirrhosis, which is a more severe form of scarring that is irreversible and can affect the normal functioning of the liver.

Liver tissue becomes hard when it is scarred, and this causes increased pressure in one of the blood vessels flowing into the liver (portal vein) leading to the development of enlarged blood vessels (varices) surrounding the digestive system, as well as enlargement of the spleen. This is known as portal hypertension.

Scarring can also be caused by fatty changes in the liver, also known as steatosis. It is not known why this happens, but fat can irritate the liver and cause scarring.

What are the symptoms of cystic fibrosis-related liver disease?

The majority of patients with cystic fibrosis-related liver disease will experience no or mild symptoms and signs related to their liver disease.

As children are screened from a young age, a diagnosis of CF-related liver disease can be based on some changes seen on a liver ultrasound scan or mildly abnormal liver blood test results in the absence of any signs or symptoms.

For patients with more advanced liver disease, the following signs and symptoms can be noted:

  • Hepatomegaly (enlarged liver)
  • Splenomegaly (enlarged spleen)
  • Jaundice (yellowing of the skin and the whites of the eyes)
  • Bleeding (bruising, nosebleeds and visible veins on the abdominal wall)
  • Poor growth and muscle wasting
  • Ascites (build up of fluid in the abdomen)

Find out more about the signs and symptoms of CF-related liver disease by downloading our factsheet.

How is cystic fibrosis-related liver disease diagnosed?

Tests carried out in a cystic fibrosis clinic can look at the structure and appearance of the liver, or the function of the liver. These include:

  • Ultrasound scan
  • MRI scan
  • Blood tests
  • Liver biopsy

Find out more about the tests used to diagnose CF-related liver disease by downloading our factsheet.

Jaundice

Jaundice is where the whites of the eyes and the skin develop a yellow tinge, which may be due to liver problems. People with CF can develop liver problems as a result of their condition, but also as a result of a reaction to drugs and even due to some infections.

If you suspect you have developed jaundice it is important to contact your doctor; don’t just put the problem down to your cystic fibrosis.

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