Cirrhosis in Children: Symptoms and Treatment

What Is Cirrhosis?

Cirrhosis (pronounced sur-o-sis) is a stage of liver disease that occurs when cells in the liver are damaged and scar tissue forms. This scar tissue causes blood flow to be blocked and waste products to build up in the body. In cirrhosis, normal areas of liver are surrounded by scarred areas that do not function properly.

Cirrhosis in pediatric patients causes normal areas of the liver to be surrounded by scarred areas that do not function properly.

People often think of cirrhosis as a disease caused by long-term alcohol abuse. While this is sometimes a factor in adults, cirrhosis in children often stems from a wide variety of liver disorders, including (but certainly not limited to):

• Hepatitis B and Hepatitis C
• Autoimmune hepatitis
• Inherited diseases:
  • Glycogen storage disease
  • Tyrosinemia
  • Wilson disease
  • Alpha1-antitrypsin deficiency
  • Cystic fibrosis
• Bile duct diseases:
  • Biliary artresia
  • Sclerosing cholangitis
  • Congenital hepatic fibrosis
  • Choledochal cysts
• Drugs and toxins:
  • Isoniazid
  • Methotrexate
  • Excess vitamin A
• Fatty liver disease

Cirrhosis Symptoms in Children

Liver cirrhosis symptoms itself often causes no symptoms early in the disease process. Symptoms start when there is portal hypertension and/or the liver begins to fail, as scar tissue replaces healthy cells. Symptom severity may depend on the extent of liver damage.

A person in the early stages of cirrhosis may feel fatigued and weak. Sometimes he or she will experience abdominal swelling that feels tender or painful. Family may notice the person has a poor appetite or is losing weight.

As the disease progresses, bile flow is blocked or stopped, and jaundice (yellow skin or eyes) appears. The same bile pigment, bilirubin, which is responsible for the yellow skin tones of jaundice can turn urine dark. Bleeding and bruising can occur more easily and take longer to heal. Other later symptoms, some due to complications, include:

• Reddened palms
• Loss of body hair
• Enlarged liver
• Enlarged spleen
• Appearance of thin, purplish-red, spidery looking blood vessels on the skin, especially around the navel
• Water retention and swelling in the legs and abdomen
• Vomiting blood
• Itching
• Abdominal infections
• Forgetfulness or confusion
• Tremors
• Inability to fully process drugs
• Enlarged, twisted, thin-walled blood vessels called varices in the esophagus and/or stomach that can rupture and lead to life-threatening bleeding
• Liver cancer

Cirrhosis Diagnosis

If your child's doctor suspects that your child has cirrhosis, he or she will perform tests to confirm or rule out the diagnosis. Tests may include (but are not limited to):

• Blood Tests – to assess how well the liver is working and determine a cause
• CT Scan, Ultrasound, MRI or Liver/Spleen Scan to identify changes in the liver
• Liver Biopsy – analyzing a sample of liver tissue removed via a thin needle inserted into the liver

Cirrhosis Treatment

In general, cirrhosis cannot be cured or reversed, doctors treat it with the following goals:

• Controlling the cause of the liver damage
• Preventing additional damage
• Treating symptoms and complications
• Treating underlying medical conditions

Your child's doctor may prescribe drugs to treat the underlying cause of the liver disease. Other medications may be used to control symptoms or fight infections. Some medications are prescribed to get rid of excess fluid in the body or reduce the risk of a blood vessel breaking. Others help your child's body cut down on its absorption of harmful waste products or toxins.

If the complications of cirrhosis can no longer be controlled, or if the liver is in danger of no longer functioning, a liver transplant is often the best option.

Many of the liver disorders that cause cirrhosis in children are not preventable, but there are precautions you can take. Make sure your child receives all recommended immunizations including influenza and hepatitis vaccines at the times your pediatrician recommends. If your child needs to take medications that may damage the liver, follow your doctor's recommendations about blood tests.

Balanced nutritional intake is important for people who already have cirrhosis of the liver can prevent or slow further liver damage by following their doctor's instructions regarding diet. Your child may need extra calories to grow properly and to maintain adequate overall strength. If the cirrhosis is more advanced and compromises the liver's ability to process protein properly, the doctor may recommend limiting protein. The doctor may also recommend limiting salt in your child's diet, because salt tends to make the body retain water. They may also advise avoiding raw seafood. Make sure your child takes any vitamin supplements prescribed. Due to increased risk of infections, doctors recommend vaccines against flu, pneumonia, and hepatitis for people with cirrhosis.

One of the dangerous complications that can arise in an individual with cirrhosis is variceal hemorrhage. This occurs when an enlarged blood vessel in the esophagus and/or stomach breaks open and causes bleeding. Typically if this occurs one may vomit blood (which could be bright red or black like coffee grounds). Alternatively, blood might be noted in the stools – it could be bright red or black and tarry. This occurrence is a medical emergency. Immediate medical attention should be sought, either by calling for an ambulance or by going to the nearest medical facility.

Learn about other Liver Disease States.