Symptoms and complications of cystic fibrosis-related liver disease

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

You may not notice the early signs of cystic fibrosis-related liver disease. This is because your child may show no symptoms. In most children, any symptoms are mild, and no treatment is needed. If symptoms do occur, the severity can vary a lot from person to person.

Symptoms may include:

Enlarged liver (hepatomegaly)

This is a larger than normal swelling of the liver. It may cause tummy (abdominal) discomfort or lead to your child “feeling full”.

Enlarged spleen (splenomegaly)

This is a larger than normal swelling of the spleen. It may happen as a result of a complication called portal hypertension. It can cause tummy (abdominal) discomfort or lead to your child “feeling full”.

Overactive spleen (hypersplenism)

A possible side effect of an enlarged spleen is an overactive spleen (hypersplenism). As the spleen gets bigger, it traps and destroys platelets from the blood. This makes them unavailable for their role in blood clotting. This may lead to increased bruising and nosebleeds.

Malnutrition and faltering growth

Malnutrition is a common symptom of cystic fibrosis. Children and young people with the condition often have trouble gaining weight. This can have an impact on their growth and development. They have a high need for extra calories and nutrition. Your child may already take supplements to help with this.

Malnutrition and vitamin deficiencies can become worse if your child develops cystic fibrosis-related liver disease. A high calorie diet may not be enough, and your child may need more support. This may involve special formulas, diets, supplements and tube feeding.

Jaundice

This happens when bilirubin trapped in the liver passes back into the bloodstream. Bilirubin is a natural waste product made when old red blood cells break down. If there is too much, it causes yellowing of the whites of the eyes (sclera) and skin. Jaundice is a late symptom of cystic fibrosis-related liver disease.

Gallstones (cholelithiasis)

This is the name give to small stones that form in the gallbladder or bile ducts. If a stone blocks a bile duct this can cause severe pain and /or jaundice. Gallstones also increase the risk of infection in the bile ducts (cholangitis). In most cases gallstones are found on routine ultrasound scans but cause no problems.

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What are the possible complications of cystic fibrosis-related liver disease?

In a small number of children and young people, damage to the liver becomes worse over time. This causes the liver to become stiff and scarred (fibrosis). It can lead to severe scarring, known as cirrhosis. The effects of severe scarring (cirrhosis) of the liver are:

Portal hypertension

When damage happens inside the liver, it stiffens. This makes it more difficult for blood to flow through it. This causes high pressure in the portal vein. This is the blood vessel that carries blood from many organs in the tummy (abdomen) to the liver. This pressure is known as portal hypertension.

Portal hypertension can make the spleen get bigger. It can also cause swollen blood vessels in the food pipe (oesophageal varices). These blood vessels have thin walls and may bleed. This may cause your child to vomit blood or pass black, tarry poo (stools). Both symptoms need urgent medical attention.

The management of portal hypertension may be complicated by a condition called pancreatic insufficiency. This is common in children and young people with cystic fibrosis.

Find out more information about portal hypertension in children.

Fluid in the tummy (ascites)

Ascites is the term for a build-up of fluid in the tummy (abdomen) It is caused by:

• increased pressure in some of the blood vessels
• the reduced ability of the liver to make an important protein called albumin

The main symptom of ascites is swelling of the tummy (abdomen).

Find more about ascites in children.

Fluid in other parts of the body (oedema)

Oedema is the term for a build-up of fluid in the body. This fluid makes the tissues swell. Oedema is most likely to occur around the legs, ankles and feet.

Liver failure

Liver failure happens when large parts of the liver become damaged and scarred. The liver can no longer do its job and is said to be failing. This is a late stage of liver disease. It happens rarely in children and young people with cystic fibrosis-related liver disease.

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Next: treatment and support

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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