Cirrhosis of the liver
We understand that you may have a lot of questions when your child is diagnosed with cirrhosis.
- What exactly is it?
- What are the causes of cirrhosis?
- What are potential complications in my child’s case?
- What are the treatments?
- What are possible side effects from treatment?
- How will it affect my child long term?
We’ve tried to provide some answers to those questions here, and when you meet with our experts, they can explain your child’s condition and treatment options fully.
What is the liver, and what does it do?
The liver is the body’s second largest organ, located in the right side of the abdominal cavity below the diaphragm and above the right kidney and intestines. The liver helps the body in hundreds of ways:
- All of the blood coming from the stomach and intestines passes through the liver through a large vein called the portal vein. The liver turns nutrients from the food we eat and chemicals from the medicines we take into forms that the rest of our bodies can use.
- The liver helps clean the bloodstream of harmful substances and poisons.
- The liver makes bile, which contains chemicals to help us digest the food we eat.
- The liver helps control blood sugar and cholesterol levels
- The liver makes the proteins that allow blood to clot normally.
What is cirrhosis?
While the liver has an amazing capacity to heal itself if damaged by illness or injury, in the case of long term damage the liver may become scarred. The most severe form of scarring is called cirrhosis.
What happens to the liver in cirrhosis?
The normal healthy liver has a smooth, shiny surface. Over time, as the liver tries to recover from long-term illness or injury, hard scar tissue can replace the liver’s healthy tissue. When this happens, blood cannot flow through the liver as easily and the liver cannot work as well. Once it has started, if the damage continues, the scar tissue in the liver will gradually replace the liver’s healthy tissue, and the organ can actually start to shrink and take on a shriveled appearance. When the scarring (also called fibrosis) gets to the point of causing nodules instead of a soft, smooth liver, it is called cirrhosis.
What causes cirrhosis?
Because cirrhosis is always linked to some kind of long-term disease or injury to the liver, it can be caused by a large number of disorders. In adults, chronic alcohol ingestion, viral hepatitis, and fatty liver are the most common causes. In children, biliary tract disorders and genetic conditions top the list of causes:
- Biliary atresia, a condition that occurs in the first month or two of life, a blockage of the ducts that bring bile from the liver to the gallbladder and intestines.
- Autoimmune hepatitis, in which the body’s immune system turns against the liver
- Cystic fibrosis, an inherited chronic disease in which the body produces unusually thick, sticky secretions.
- Alpha-1 antitrypsin deficiency, a genetic disease that prevents the liver from building a protein it normally releases into the bloodstream.
- Chronic viral hepatitis caused by the viruses hepatitis B or C. These viruses cause the liver to swell, which can encourage scar tissue to form.
- Primary sclerosing cholangitis, an acquired condition which causes inflammation and narrowing of the bile ducts inside and/or outside of the liver
- Wilson disease, an uncommon inherited disorder in which the body cannot rid itself of excess copper found in many foods; the copper then builds up in the liver.
- Non-alcoholic fatty liver disease (NAFLD), a buildup of fat in the liver most commonly caused by obesity. Many children with NAFLD have a more serious condition called non-alcoholic steatohepatitis (NASH), which can lead to severe liver scarring.
- Some congenital (inherited) heart defects
- Other less common disorders
While in adults overconsumption of alcohol is the most common cause of cirrhosis, it is important to stress that no amount of alcohol consumption by a parent, even during pregnancy, can cause a child to develop cirrhosis.
Signs and symptoms
What are the symptoms of cirrhosis?
Cirrhosis itself generally produces no symptoms early on, though your child may experience symptoms related to the underlying medical condition that caused the liver damage. As it worsens, cirrhosis may cause:
- Loss of appetite
- Nausea or vomiting
- Weight loss or difficulty gaining weight
- Abdominal pain or swelling
- The appearance of spider-like blood vessels on the skin
With time, cirrhosis may also lead to additional serious problems, including:
- A yellowing of the skin or the whites of the eyes (called jaundice)
- Bruising or bleeding easily, or nosebleeds
- Swelling of the legs or abdomen from build up of fluid. In the legs this fluid buildup is called edema; in the abdomen it is called ascites.
- Confusion or difficulty thinking (encephalopathy), caused by a buildup in the bloodstream of waste products from food.
- Failure of the kidneys to work properly
Because in cirrhosis blood cannot flow as easily through the liver, the pressure in the vein entering your child’s liver, called the portal vein, may increase, a condition called portal hypertension. This condition can cause its own symptoms and complications, and has its own treatments. The increased blood pressure in the portal vein can also affect how blood circulates in the lungs, causing conditions called hepatopulmonary syndrome and portopulmonary hypertension.
How is cirrhosis diagnosed?
Doctors usually make a diagnosis of cirrhosis based on a combination of symptoms, medical history, physical exam, and blood tests. In some cases, our doctors may order a liver biopsy to confirm the diagnosis, which will help them see the extent of the scarring in the liver.
For details, see Tests.
How do you treat cirrhosis?
While in most cases there is no way to cure cirrhosis, our doctors will work with you and your child to control it and keep it from getting worse. They will also help understand and treat the underlying illness or damage that is causing the liver to scar.
Cirrhosis can also raise your child’s risk of developing liver cancer. And in advanced cases, cirrhosis can cause the liver to start to fail altogether. If that should happen, we may refer your child for a liver transplant.
For details, see Treatment and Care.
How can cirrhosis affect my child in the long term?
- Because a damaged liver cannot break down medicines as quickly as if it were healthy, medicines – including over-the-counter medicines and vitamin or herbal supplements – may work more strongly than before, or may not be made into the active forms by the sick liver. For this reason, if your child has cirrhosis, you should always talk to his or her doctor before starting any new medications or supplements, even vitamins.
- Cirrhosis may make it harder for your child to gain or maintain his weight and healthy nutritional status.
- Cirrhosis may be an additional risk factor if your child has other medical problems or needs surgery.
- If your child has portal hypertension from cirrhosis, she may have more long-term effects.
Cirrhosis is a chronic condition, and for this reason your child will likely have to seek care for it for the rest of his or her life. The Center for Childhood Liver Disease can help you and your child plan for the eventual transition from pediatric to adult care.
Questions to ask your doctor
You and your family play an essential role in your child’s care for cirrhosis. It’s important that you share your observations and ideas with your child’s treating physician, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.
You’ve probably thought of many questions to ask about your child’s cirrhosis. It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your child’s appointment. That way, you’ll have all of your questions in front of you when you meet with your child’s treating clinician and can make notes to take home with you.
Some questions to ask your doctor might include:
- How did you arrive at this diagnosis?
- Are there any other conditions my child might have instead?
- Does my child need further testing?
- What is causing my child's cirrhosis?
- What is the long-term outlook for my child?
- What medications will you prescribe, and what are the possible side effects?
- How should I talk to my child about this condition and her long-term health?
- Do I need to make any changes to my child’s home and school routines?
- Can you point me to educational and support service resources for children with cirrhosis in my area?
- What other resources can you point me to for more information?