Hepatic Encephalopathy

Hepatic Encephalopathy, sometimes referred to as portosystemic encephalopathy or PSE, is a condition that causes temporary worsening of brain function in people with advanced liver disease. When your liver is damaged it can no longer remove toxic substances from your blood. These toxins build up and can travel through your body until they reach your brain, causing mental and physical symptoms of HE.

Hepatic Encephalopathy often starts slowly, and at first you may not be aware you have it. The stages of HE span from mild to severe and symptoms vary depending on how bad your liver disease is. It’s important for you and your family to become familiar with the signs of Hepatic Encephalopathy so you can tell your doctor right away if you think you may have it. Prompt identification and treatment of HE is essential to stopping the progression of this condition. Progression may not stop despite best efforts.

Read on to learn more about HE, its symptoms, stages and treatment. Being a fully-informed participant in your medical care is an important factor in staying as healthy as possible. The American Liver Foundation is here to answer all your questions about HE, one step at a time.

You only have one liver and it’s one of the largest and most important organs in your body. Your liver performs many jobs to keep you healthy including filtering everything that enters your body, such as food, drink and medicine.

After your intestines break down things that you eat or drink into their component parts, your liver is responsible for separating the good stuff from the bad. It sends the good things – such as vitamins and nutrients – into your bloodstream for your body to use and changes the bad or toxic things, making them harmless.

HE is most often seen in people with chronic liver disease. Anything that damages your liver over many years – such as long-term alcohol abuse or chronic hepatitis – can cause it to form scar tissue. As hard scar tissue replaces soft, healthy tissue two things begin to happen:

• The scarred tissue cannot carry out the process of changing toxins into harmless substances like a healthy liver normally would.
• The scarred tissue can block the flow of blood through the liver causing high blood pressure in the veins in and around your liver (called the portal venous system). This condition is known as portal hypertension.

When your liver can’t filter toxins from your blood or when blood flow through your liver is blocked, toxins build up in your bloodstream and can get into your brain.

Ammonia, which is produced by your body when proteins are digested, is one of the toxins that’s normally made harmless by your liver. But when ammonia, or a range of other toxic substances, build up in your body when your liver isn’t working well, it may affect your brain and cause HE.

HE is a serious but treatable condition if caught early and treated promptly. Symptoms often resolve when triggering factors are treated. It’s important to continue treatment for as long as necessary to keep HE from coming back.

Once symptoms become severe, HE can quickly worsen and become a medical emergency resulting in prolonged hospitalization. But with continuous treatment, HE can usually be controlled. So it’s important to tell your doctor about any warnings signs you have as soon as you, or a family member or friend, notice them.

Liver failure occurs if the liver has lost all of its function due to cirrhosis caused by different liver diseases.

What are the common causes of cirrhosis?

• Hepatitis B & C
• Alcohol-related Liver Disease
• Non-alcoholic Fatty Liver Disease (NAFLD) & Non-alcoholic Steatohepatitis (NASH)
• Autoimmune Hepatitis
• Bile duct disease such as Primary Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC)
• Metabolic diseases such as Hemochromatosis, Wilson disease and Alpha-1 antitrypsin deficiency

Liver failure is a life-threatening condition that requires hospitalization. Many people do not have any liver damage symptoms until serious liver problems have already developed slowly and silently.

What are the symptoms and signs of liver failure caused by cirrhosis?
For some people, cirrhosis is diagnosed unintentionally. Cirrhosis often does not have any specific signs and symptoms in the early stage. The non-specific symptoms may be:

• Nausea
• Loss of appetite
• Fatigue
• Diarrhea
• Jaundice (yellowing of the skin and whites of the eyes)

As cirrhosis progresses, symptoms and complications can appear that make it apparent that the liver is not doing well. These could be the symptoms of Hepatic Encephalopathy (HE) and other complications due to cirrhosis. In addition to Hepatic Encephalopathy (HE), following complications are signs of liver damage or cirrhosis:

• Fluid build up and painful swelling of the legs (edema) and abdomen (ascites)
• Bruising and bleeding easily
• Enlarged veins in the lower esophagus (esophageal varices) and stomach (gastropathy)
• Enlarged spleen (splenomegaly)
• Stone-like particles in gallbladder and bile duct (gallstones)
• Liver cancer (hepatocellular carcinoma)

Chronic liver failure indicates that the liver has been failing gradually, possibly for years.

If the liver is failing, a liver transplant may be needed in some cases.

What is a liver transplant?
A liver transplant is the process of replacing a sick liver with a donated, healthy liver. Liver transplants require that the blood type and body size of the donor match the person receiving the transplant. Donated livers come from living and non-living donors. Liver transplant surgery usually takes between four and twelve hours. Most patients stay in the hospital for up to three weeks after surgery. There are currently over 17,000 patients waiting for a liver transplant here in the United States. Historically between 5,000 to 6,000 liver transplants happen annually.
In the U.S., there are more people who need a liver transplant than there are donated livers. The major reason for liver transplants here in the U.S. is hepatitis C.

The process to be eligible for a liver transplant is:

• Person’s doctor refers him or her to be seen at a transplant center;
• At the transplant center, the transplant team evaluates the person’s overall physical and mental health, plan to pay for transplant related medical expenses, and emotional support family and friends will provide;
• Based on the findings, the team decides if the person is eligible for a liver transplant;
• If the person is eligible, the center will add him or her to the transplant waiting list.

The waiting list is prioritized so the sickest people are at the top of the list. The time a person spends on the waiting list depends on:

• Blood type
• Body size
• Stage of liver disease
• Overall health
• Availability of a matching liver

Most patients return to a regular lifestyle six months to a year after a successful liver transplant. In some patients, the liver disease they had before the transplant comes back and they may need treatment or another transplant.

For more information about cirrhosis of the liver and liver transplants, call our helpline at 1-800-465-4837 or consult your healthcare provider.

For more information about Hepatic Encephalopathy stages, consult your healthcare provider.

• Will I get routine labs to monitor my ammonia levels?
• What is the status of my liver?
• Do I have liver damage?
• Will I need a liver transplant?
• (If I need a liver transplant) – Which transplant centers are located in my state?
• What is my approximate MELD score?
• What dosage of lactulose will I be taking? How often should I take it?
• If lactulose alone does not manage my HE, will Xifaxan be added?
• What can I anticipate with HE? Will I have mood changes?
• If I begin to have issues with remembering my medications – will it be possible to have a visiting nurse come to my home?
• What kinds of information can I share with my friends and family about my condition?
• Will I be able to continue to work to my full capacity?
• Will I be able to drive?