Dietary support
Children and young people with cystic fibrosis have a high need for extra calories and nutrition. Your child may already take supplements and follow a high calorie diet. Malnutrition and vitamin deficiencies can become worse if your child develops cystic fibrosis-related liver disease. A high calorie diet may not be enough. Extra support will be given by dietitians and the medical team and may include:
MCT (medium chain triglyceride) formulas and diets
MCT is a type of fat. It is more easily absorbed and is a good source of energy. Your child’s dietitian will prescribe special milk formulas and supplements containing MCT. They will also provide a list of suitable foods for older children.
Fat soluble vitamins (vitamins A, D, E and K)
Your child may already take fat soluble vitamins. If they develop liver disease, the medical team may need to change the formulation or dose. Vitamin K is particularly important in cystic fibrosis-related liver disease. It is needed for blood clotting and good bone health.
Nasogastric feeding
Nasogastric feeding may be used if your child needs more calories or is not growing well. This involves passing a very thin, soft tube up the nose, down the back of the throat and into the stomach. A special milk formula will be given overnight using a pump. Parents/carers can be taught how to do this at home.
I was petrified of needing an NG tube for feeding but was really well supported when that day came. It gave her the energy to enjoy things again.
– Parent
Find more information about nutrition
Medicines
Medicines may be used to try to slow down the disease, or to help with symptoms. The choice of medication will depend on the severity of symptoms.
Ursodeoxycholic acid
This is a bile salt that occurs naturally in small amounts in bile. Taking it as a medicine can help make the bile less sticky and allow it to flow more easily from the liver. Some research has shown that the drug improves symptoms and blood test results. However, the long-term benefits are still not clear. So far, research has not shown that it prevents advanced liver disease or the need for liver transplant.
Blood pressure medicines
Drugs may be used to help lower high blood pressure in the portal vein. This will reduce the risk of bleeding from swollen blood vessels in the stomach or food pipe (oesophagus).
CFTR modulators
CFTR modulators are new drugs that are being used in people with cystic fibrosis. They are different to the treatments used in the past because they target the genetic causes of the condition. They may help to ease cystic fibrosis symptoms and slow down its progression.
It is not yet clear if CFTR modulators can help with cystic fibrosis-related liver disease. We do not know if they stop liver disease from happening or slow down its progression. We do know that CFTR modulators cannot reverse scarring caused by advanced liver disease. More research is needed to find out the long-term effects of the drugs.
Invasive procedures
The medical team may use invasive procedures to treat the complications of cystic fibrosis-related liver disease.
Endotherapy
This is used in the treatment of swollen blood vessels in the food pipe (oesophageal varices). There are three different types of endotherapy. The choice of treatment will depend on the results of the endoscopy.
Banding (variceal band ligation)
Banding is done during an endoscopy. The doctor places small rubber bands around the swollen blood vessels to make them close. This stops blood flow and reduces the risk of bleeding. Once a tiny scar has formed the rubber bands fall off. They may appear in your child’s poo (stool) after a few days.
12 years on his liver is still doing what livers do and the portal hypertension has been controlled by the banding programme
– Parent
Glue injection (glue obturation)
This treatment is done during an endoscopy. A medical glue is injected into the swollen blood vessels to seal them off and prevent bleeding.
Sclerotherapy
This treatment is done during an endoscopy. It will be used if your child is too small for a banding procedure or if the swollen blood vessels are in the stomach wall (gastric varices). A medicine (sclerosant) is injected into the swollen blood vessels. This irritates the inside of the blood vessel wall. A scar forms, which blocks the blood flow and reduces the risk of bleeding.
TIPS (Transjugular intrahepatic portosystemic shunt)
This treatment may be considered for older children. It may be used:
- to help manage severe portal hypertension that cannot be controlled with endotherapy
- in young people waiting for a liver transplant
- when a liver transplant is not possible
During the procedure, a small metal tube is placed between the vein supplying blood to the liver (portal vein) and the veins draining blood from the liver back to the heart (hepatic veins).
This new channel or ‘bypass’ reduces pressure in the portal vein and allows blood to flow more freely. It makes swollen blood vessels (oesophageal varices) less likely to bleed. It also reduces the build-up of fluid in the tummy (ascites).
TIPS can be done as a planned treatment or as an emergency treatment if your child has a bleed.
Liver transplant
A liver transplant is used for the treatment of complex portal hypertension or liver failure. Only a small number of children with cystic fibrosis develop advanced liver disease that will lead to a liver transplant.
A liver transplant is an operation to remove a liver that does not work. It is replaced with a healthy liver from another person (donor). A child may receive a whole, split or reduced liver from a deceased donor. It is also possible that they may receive part of a liver from a living donor. A successful liver transplant will help a child survive when the liver is failing. It will also significantly improve a child’s quality of life.
A liver transplant is a major operation. It will only be done after the benefits and risks have been carefully weighed up. The medical team will also assess and monitor your child’s lungs. This is done to make sure that they are healthy enough to cope with major surgery and the recovery process. Following a successful transplant children need lifelong medication and follow up.
Cystic fibrosis-related liver disease will not return after a liver transplant. However, a liver transplant will not cure cystic fibrosis or the effects of the condition in other organs.
Find more information about liver transplantation for children.
Useful resources and support
See the links at the end of the page for more information on our support services.
Cystic Fibrosis Trust
A UK-wide charity providing support for people with cystic fibrosis. They fund research, improve care, provide a network of support, and produce a range of information resources
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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