Cystic fibrosis and liver disease




Some children and young people with cystic fibrosis may have had prolonged jaundice as a baby. This usually disappears 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 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.

 

How is a diagnosis made?

Most children and young people with cystic fibrosis have regular check-ups which include:

 

Physical examination

Signs of liver disease may be found during a routine examination. These include:

  • Enlargement of the liver (hepatomegaly)
  • Enlargement of the spleen (splenomegaly) or both spleen and liver (hepatosplenomegaly)
  • This is rare and usually only seen when liver disease is very advanced
  • Visible veins on the abdominal wall which suggests poor blood flow through the liver (portal hypertension)

 

Liver function tests

Blood tests can be useful in monitoring the liver function but they may remain normal despite progression of liver disease. Find out more about liver tests.

 

Abdominal ultrasound

This shows the size and texture of the liver, the blood flow through it and any obvious abnormalities of the bile ducts including swelling and the presence of stones or blockages. It also enables accurate measurement of the size of the spleen and gall bladder.

If a liver problem is suspected following the above tests then occasionally a liver biopsy may be recommended. This may help to show the extent of the damage to the liver.

 

What are the main effects of liver disease in children with cystic fibrosis?

  • Portal hypertension  (increased blood vessel pressure caused by scarring of the liver)
  • Enlarged spleen
  • Disturbance of the normal working of the liver. This may include poor growth, reduced absorption of dietary fat soluble vitamins A, D, E and K, ascites , and an increased tendency to bruise or bleed

 

What treatments may be considered?

 

Medication

  • Supplements of fat soluble vitamins may be required and in most cases are essential
  • Download our leaflet to find out more about the medication which may be prescribed

 

Nutrition

Calorie and protein requirements are high and supplementary feeding is often required. Find out more about nutrition and liver disease.

 

Diagnosis and treatment of varices

  • Endoscopy – looking directly at the oesophagus and stomach with a flexible tube
  • Sclerotherapy – the injection treatment of oesophageal varices
  • Banding – reduces bleeding from varices by placing a tight band around them to make then shrivel up
  • TIPS – a radiological procedure which reduces blood flow through the varices by directing in through a channel created in the liver
  • Medication may be prescribed to reduce gastric irritation. Download our leaflet to find out more

 

Liver transplantation

Transplantation can be an effective treatment for children with cystic fibrosis who have severe liver disease. A full assessment of nutrition, lungs and liver disease and associated complications is needed to decide whether the liver alone needs to be replaced, or the liver and the lungs.

Find out more about liver transplantation.

 

Download CLDF’s leaflet on cystic fibrosis and liver disease

 

This website provides general information but does not replace medical advice. It is important to contact your/your child’s medical team if you have any worries or concerns