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Cirrhosis of the liver

  • No parent expects a doctor to say that his or her child has cirrhosis. However, hundreds of children in the United States are diagnosed with cirrhosis – scarring of the liver caused by underlying illness or damage – every year.

    Over the next few pages we will introduce you to the basics of cirrhosis, its causes, signs and symptoms, and how the physicians in the Center for Childhood Liver Disease at Boston Children's Hospital care for children diagnosed with cirrhosis.

    The liver is a large organ (the second largest in the body) responsible for processing nutrients from our food and medications in ways that allow the rest of the body to make use of them. It also acts as a big filter for the bloodstream, removing poisons and toxins as well as byproducts from our cells and tissues. The liver helps to control your blood sugar and your cholesterol, and it produces chemicals that help your blood to clot.

    The liver’s ability to repair and regrow itself following damage or illness has amazed us for centuries, even back to the days of ancient Greece (hence the myth of Prometheus). Because of its regenerative ability, a person is able to have a large portion of his liver removed, or donate a portion of his liver for a liver transplant and the removed portion will grow back with no long-term problems.

    But this ability is not unlimited. If the damage becomes chronic, particularly due to underlying illnesses such as disorders of the biliary tract, genetic conditions, or viral infections, the liver’s repair mechanisms fail and instead the organ begins to scar, compromising its processing and filtering capacities. If the underlying damage continues, the scarring progresses, and eventually much of the liver’s healthy tissue will be replaced with scar tissue. This abnormal regeneration with scarring (called fibrosis) is the hallmark of cirrhosis.

    Because it's hard to reverse this scarring once it has started, the main goal of treatment is to keep it from advancing further, generally by addressing whatever underlying medical condition has caused the liver to scar in the first place. If uncontrolled, cirrhosis can have serious complications such as portal hypertension and hepatopulmonary syndrome. It can also increase a child’s risk of liver cancer.

    While cirrhosis in adults is often caused by overconsumption of alcohol, in children disorders of the biliary tract (a tube that carries bile from the liver to the gallbladder and intestines) and inherited or genetic conditions are the most common reasons the liver become injured and at risk to form scar. It is important to stress that no amount of alcohol consumption by a parent, even during pregnancy, can cause a child to develop cirrhosis.


    How Boston Children’s Hospital approaches cirrhosis

    The physicians, nurses, and staff in the Center for Childhood Liver Disease have a deep understanding of the complexities and complications of cirrhosis. Home to one of the few dedicated teams of specialists who are board-certified in Pediatric Hepatology and Transplant, the Center will work with you to provide the compassionate and family-centered care that will help bring the condition under control.

    We give families the resources, information, and support they need in order to manage cirrhosis as well as possible. And because cirrhosis is a chronic condition, we can help you plan for your child’s eventual transition to adult care. 

    Cirrhosis of the liver: Reviewed by Mauren Jonas, MD
    © Boston Children’s Hospital, 2011

  • 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.

    Causes

    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
    • Weakness
    • 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.

    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.

    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? 
  • The first step in treating your child is forming an accurate and complete diagnosis. Your child’s doctor will usually make a diagnosis of cirrhosis based on a combination of symptoms, medical history, physical exam, and blood tests.

    In most cases, our doctors will order a liver biopsy to confirm the diagnosis, and help them see the extent of the scarring in your child’s liver.

    Your doctor may gauge the severity of your child’s cirrhosis using a system called the CTP or Child-Turcotte-Pugh (“Child”) score. This score combines a set of three laboratory-based measures (bilirubin, albumin, and prothrombin time) with two bedside ones (the presence and severity of ascites, and the level of encephalopathy). While this scoring system was designed for adults and has its limitations, it draws a good picture of both the status of your child’s liver and the overall changes in the body caused by cirrhosis.

    Additional monitoring

    Regardless of the underlying cause, once they make a diagnosis of cirrhosis our doctors will keep track of your child’s condition by several means, including periodic:

    • Blood tests, to check how well your child’s liver is working as well as his or her fluid balance.
    • Ultrasounds, which use high frequency sound to help your child’s doctor to see his or her liver. Ultrasounds are painless, non-invasive, and use no radiation.
    • Physical exams.

    In addition, our doctors will want to check your child’s blood oxygen levels every year to see whether he or she is developing hepatopulmonary syndrome, in which increased blood pressure in the liver’s portal vein causes changes in how blood circulates through the lungs. We will also monitor your child’s kidneys for signs of complications.

    Our doctors may also want to occasionally conduct an endoscopic exam to see whether new, weakened blood vessels called varices are growing in your child’s esophagus in response to the irregular blood flow in the liver. To do this, they will either use either an endoscope – a small, flexible tube – to examine the inside of your child’s esophagus, or ask him or her to swallow a small capsule containing a camera, which will pass through your child’s digestive tract.

    Because cirrhosis can raise your child’s risk of developing liver cancer, our doctors will want to conduct regular cancer surveillance.

  • At Boston Children’s Hospital, we take a multidisciplinary approach to the care of children with cirrhosis. Our Center for Childhood Liver Disease is one of the few centers with a dedicated team of specialists who are board-certified in pediatric hepatology and transplant.

    Cirrhosis has no cure; once the liver starts to scar, in most cases the scarring will not go away. Therefore, our focus is to keep your child’s cirrhosis from becoming worse, largely by treating the underlying illness or damage that caused the scarring to start in the first place.

    While the specific course of treatment will therefore be chosen largely based on your child’s underlying medical condition, there are some things that we do for all children with cirrhosis:

    • Encourage healthy eating. Good nutrition is key for all people with cirrhosis, young and old. It is especially important for children with cirrhosis or any other liver disease to eat well so as to maintain growth. For this reason, our doctors may prescribe caloric supplements or special formulas.
    • Monitor for complications. Cirrhosis can lead to several complications affecting many organs and systems in the body. Our doctors will keep a close eye on your child to catch any complications early.
    • Treat complications if they arise. For instance, our doctors may prescribe diuretics to reduce any swelling in your child’s legs or abdomen. If he or she develops varices and they start to bleed, our doctors will treat them, either endoscopically or surgically. They may also ask to conduct additional tests if your child starts to show signs of portal hypertension or hepatopulmonary syndrome, and treat him or her accordingly.

    Because a damaged liver cannot break down medicines as quickly as if it were healthy, medicines – including over-the-counter medicines or vitamin or herbal supplements – will work more strongly than before. 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.

    If your child’s cirrhosis cannot be treated and his or her liver starts to fail, a liver transplant may become necessary.

    Because cirrhosis is a chronic disease, 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.

  • Right now, the only way doctors can be sure of how much fibrosis (scar tissue) is in your child’s liver is from a liver biopsy. Although many liver biopsies are performed at Boston Children’s Hospital each year, this is considered an invasive procedure. That means it is associated with some risk, often requires sedation or anesthesia, may cause some discomfort, and is somewhat expensive.

    Physicians in the Center for Childhood Liver Disease at Boston Children’s are conducting research into other ways of finding out if your child has cirrhosis or how much scar tissue is present in your child’s liver. They are using a combination of special research blood tests and a technology called transient elastography to assess the severity of liver scarring. 

    The elastography is done with a machine called a FibroScan (Echosens, Paris, France), which uses ultrasound technology to measure the stiffness of the liver. This is a quick, easy, and painless exam. If the research shows that these tests work, your child and others will need fewer liver biopsies in the future!

  • Boston Children's Hospital
    Center for Childhood Liver Disease
    300 Longwood Avenue, Fegan 5
    Boston, MA 02115

    Phone: 617-355-5837
    Fax: 617-730-0716

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