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