Liver

Cystic fybrosis related liver disease is common (abnormal biochemical tests and ultrasound scans) but it rarely causes symptoms. Once cirrhosis is present but not causing symptoms treatment may be given to prevent further cirrhotic change, this includes:

• Ursodeoxycholic acid – this drug increases bile flow but can cause diarrhoea as side effect
• Vitamin K

Advanced liver cirrhosis may cause the following complications:

• Jaundice – a yellow discoloration of the skin and eyes that develops because the liver cannot excrete waste products
• Varices – swollen veins, most often found at the bottom of the oesophagus that bleed easily and may cause acute haematemesis (vomiting blood) or malaena (altered blood in the stool).
• Ascites – abdominal swelling due to leakage of fluid in to the abdominal cavity always a sign of advanced liver disease
• Encephalopathy – confusion and neurological signs always a sign of advanced liver disease

The effects of advanced liver disease can be managed for a short time but the only hope of return to normal function is liver transplant. It is unlikely that advanced liver disease with ascites and encephalopathy will be managed at home unless the child cannot or will not have a liver transplant or is terminally ill.