What Is Hepatic Encephalopathy?
You've had liver disease for a while, but now you notice something different about the way you act and feel. Maybe you forget things or get confused when someone's talking to you. Your friends may tell you that your speech sounds slurred or that you say things that aren't appropriate.
And it isn't only changes in behavior. You might feel sluggish or find that you can't move your hands well anymore. Or your breath doesn't smell the way it should.
What's going on? Is there a link to the liver problems you've been dealing with for years?
There could be. You may have hepatic encephalopathy (HE), a disorder caused by a buildup of toxins in the brain that can happen with advanced liver disease. It affects a lot of things, like your behavior, mood, speech, sleep, or the way you move.
Sometimes the symptoms are so mild that it's hard for anyone to notice. But whether you have obvious signs or just a few subtle changes, it's important to see your doctor. The right treatment can help keep your condition under control, but if you don't take care of yourself, your condition will continue to worsen.
Hepatic Encephalopathy Causes
Your liver is "a workhorse," says Don Rockey, MD, a gastroenterologist and hepatologist at the Medical University of South Carolina. Its big job is to constantly clean up toxins that get into your blood from your muscles and organs, including your gut, says Rickey, who's also a volunteer with the nonprofit American Liver Foundation.
Hepatic encephalopathy almost always starts with a damaged liver. When the damage is severe, key cells in your liver die off or don't work right anymore, Rockey says. That means toxins can build up in your bloodstream and travel to your brain. That can cause the mental and physical symptoms of HE.
Liver diseases
Many different liver diseases and conditions can lead to hepatic encephalopathy. They include:
Acute liver failure. This is a sudden, severe, liver injury, which can be caused by viruses, drugs, or by other illnesses. The most common cause in the United States is an overdose of acetaminophen, the key ingredient in Tylenol.
Chronic hepatitis C or, less commonly, hepatitis B. These viral infections can linger in your body, causing inflammation and damaging your liver over many years. This can lead to permanent, severe scarring of the liver, called cirrhosis, which greatly increases the risk of hepatic encephalopathy.
Metabolic dysfunction-associated steatohepatitis, or MASH. This is severe inflammation of your liver caused by excess fat cells. If you don't get a diagnosis and treat it in time, It can lead to cirrhosis and HE. The condition was previously known as NASH, or non-alcohol related steatohepatitis. It's a more advanced form of metabolic dysfunction-associated steatotic liver disease (MASLD), previously known as fatty liver disease. These disorders are more common in people with obesity and type 2 diabetes.
Autoimmune liver disease. These include disorders such as autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. They all can cause cirrhosis and HE.
Alcohol misuse disorder. Alcohol use over many years is another common cause of cirrhosis and hepatic encephalopathy.
Presence of TIPS
Some people with liver disease develop high pressure in veins that drain blood from the bowels to the liver. That can lead to internal bleeding and fluid buildup in the belly area. To solve these problems, your doctor may insert a tube, called a transjugular intrahepatic portosystemic shunt, or TIPS, to let blood bypass your damaged liver. A possible risk is that the toxins that remain in this unfiltered blood get to your brain and cause hepatic encephalopathy.
Your body sometimes creates a natural shunt past damaged liver tissues, causing the same buildup of toxins and risk of HE.
Urea cycle disorders
These rare conditions, which cause a buildup of ammonia in the bloodstream, can cause HE in people with healthy livers, Rockey says. The problem is that the liver can't keep up with the heavy load of toxins. About 1 in 250,000 babies born in the United States have one of these disorders.
Triggers and risk factors
Having any sort of liver damage, whether it's sudden or long-standing, increases your risk of HE. Up to 80% of people with cirrhosis have at least mild symptoms of hepatic encephalopathy.
Once you have liver damage, HE is more likely to develop or get worse if you have:
GI bleeding. Bleeding anywhere in your intestines, stomach, or esophagus (gastrointestinal or GI bleeding) can lead to high ammonia levels in your bloodstream. A damaged liver can't remove the excess ammonia, so it can get to your brain, causing HE symptoms.
Infections. Infections, including bacterial infections, also can increase ammonia levels in the blood and put extra strain on the liver, increasing the risk of HE.
Kidney problems. When your kidneys don't work well, that can lead to more waste products in your blood and a higher risk of HE.
Other triggers and risk factors can include:
- Constipation
- Dehydration, from not drinking enough fluid, diarrhea, vomiting, or other causes
- Medications like sleeping pills, pain relievers, or water pills
- Binging on alcohol
- Electrolyte problems such as low sodium or potassium
- Surgery
Hepatic Encephalopathy Types
Your doctor may classify your HE as one of three types, depending on the main cause. They are:
Type A. This is caused by sudden, or acute, liver failure. Common causes include viral infections and drug overdoses. The sudden breakdown in liver functioning can lead to brain swelling. It's a medical emergency.
Type B. This is the type caused by a natural or surgically implanted (TIPS) shunt that reroutes blood past your damaged liver. Doctors may call either kind a portosystemic shunt.
Type C. This is the type caused by chronic liver failure and cirrhosis. It is the most common. The underlying liver diseases often include hepatitis C, chronic alcohol use disorder, and diseases linked to fat in the liver, such as MASLD and MASH.
Hepatic Encephalopathy Symptoms
There's a lot of variety in the way HE affects people. Not everyone has the same symptoms. For some folks, symptoms may be very slight or come and go.
In some cases, the effects of hepatic encephalopathy start slowly and then get worse bit by bit. But sometimes, they hit you hard all at once.
There are some mental signs to watch out for. For instance, you may:
- Get confused
- Forget things
- Feel nervous or excited
- Notice a sudden change in your personality or behavior
- Speak or act inappropriately to others
- Not feel interested in things
- Get cranky
Of course, when it comes to changes in behavior, sometimes you're the last one to realize that something is off. So if you've got a long-term liver disease, ask your family and friends to be on the lookout for shifts in your personality. Tell them to be honest with you if they see your mood has changed or you're just not acting like your old self.
You may also notice some changes in your sleep patterns. You could feel sleepy during the day or stay up late at night.
Some physical changes can start creeping in, too. You may notice that:
- Your breath smells sweet or musty.
- It's hard to move or use your hands.
- When you hold out your arms or hands, they shake or flap.
- Your speech sounds slurred.
- You feel slow or sluggish when you move your body.
Hepatic Encephalopathy Diagnosis
It may be hard to notice hepatic encephalopathy at first. Your family or friends may see changes in your behavior or movement before you do.
If you have a liver disease or had one in the past, your doctor may ask you if anyone has told you about changes in your personality. They'll examine you for signs of the physical symptoms that go along with the brain disorder.
They may also give you a blood test to check for high levels of ammonia. That's a sign that your liver is not clearing it from your bloodstream the way it should. Too much of that toxin can build up in your brain and lead to HE symptoms.
You might also get:
- Ultrasound testing to look at blood flow through your liver
- A CT or MRI scan to look at your brain
- An electroencephalogram (EEG) to look at electrical activity in your brain
Questions for Your Doctor
If your doctor diagnoses you with hepatic encephalopathy, some things to ask them are:
- What stage of the brain disorder do I have?
- What treatments do you recommend?
- Will my symptoms get better or go away?
- Will I have to change my diet?
- Will I still be able to work, take care of myself, and drive?
Hepatic Encephalopathy Treatment
You've got a number of choices. A lot depends on your specific situation. For instance, your doctor will take into account things like:
- What triggers your episodes
- How severe your case is
- The types of symptoms you get
- How serious your long-term liver disease is
- How old you are and your overall health
If your case is mild, your doctor may or may not recommend drug treatment. In more advanced cases, they will likely prescribe at least one of these drugs to reduce the toxins in your body:
Lactulose or other laxatives. Lactulose is a type of sugar that can make you have more bowel movements. This helps get rid of some toxins from your body, like ammonia, which can trigger HE. Your doctor or nurse will explain how to figure out the right amount. Usually, they'll want you to adjust your dose until you are having two to three bowel movements a day. An alternative, used in some hospitalized patients, is polyethylene glycol (PEG), the solution you use to clean out your bowels before a colonoscopy.
Rifaximin and other a ntibiotics.Rifaximin (Rifagut, Xifaxan) may help by curbing bacteria that create toxins when they digest your food. Another antibiotic, neomycin (Neo-Fradin), is an alternative.
Other possible treatments include:
Hemodialysis. In some cases of acute liver failure, doctors might use a dialysis machine to remove toxins from your blood.
Shunt closure or reduction. You might need a procedure to redirect blood flow if a shunt is causing your HE.
Liver transplant. Your doctor might recommend getting on a waiting list for a liver if your cirrhosis leads to HE or other serious complications.
Your doctor may also ask you to take some other steps that can improve your symptoms:
Change your diet. Nutritional support can help you get better. In the past, doctors recommended a low-protein diet for people with HE, Rockey says, but that's no longer the case. If eating meat and other animal protein makes your symptoms worse, you might need to get more protein from plant foods or take amino acid supplements. You might also be advised to eat small meals through the day to limit strains on your liver.
Skip alcohol. Even a little bit can be risky for you because it damages your liver.
Treat infections. They can affect the way your liver works. You may need antibiotics to get rid of them.
Treat urinary blockages. If you can't pee normally, it can trigger your HE. Treatment for the infection or disease that's causing it can help.
Stop some medications. Certain medicines, such as acetaminophen, nonsteroidals medicines, sedatives, or narcotics, may cause problems with your liver.
Treat constipation. You may need to eat less meat and more vegetables and other foods with plenty of fiber. This can help you have more regular bowel movements so you can flush toxins from your body.
Hepatic Encephalopathy Complications
If you have hepatic encephalopathy, you can get other problems such as:
- Heart and breathing problems when acute liver failure causes brain swelling
- Seizures
- Lasting problems with thinking and memory
- A higher risk of death over the next year or two, if you have multiple HE episodes
Taking Care of Yourself
The best thing you can do to keep your HE under control is stick with the treatment plan that your doctor suggests.
Since you already have a lot of medical stuff to do because of your long-term liver disease, it might seem like your new treatment is a big nuisance. But it's really important. Don't skip any doses of medicines, and make sure you follow your doctor's diet advice. Ask your friends and family to help you stay on track.
Also, make sure you stay safe. Your health care team may suggest ways to make your home safer, if you are agitated or unsteady. And you may have to stop driving.
If you stick to the treatment plan, your symptoms can improve and sometimes go away.
What to Expect
HE can be divided into stages according to how severe your symptoms are. The stage will help you and your doctors pick the right treatments and lifestyle changes to ease symptoms and protect your liver and brain. In later stages or sudden severe cases, emergency treatment can be life-saving.
HE is graded based on signs and symptoms:
Grade 1: Mild. You may have slurred speech, trouble sleeping, or find it hard to concentrate.
Grade 2: Moderate. You may feel like you don't have much energy. Some personality changes and confusion may become more obvious, like acting odd or forgetting things.
Grade 3: Severe. You may be extremely confused and not be able to speak coherently. You could also get extremely sleepy or pass out, though you'll wake up when someone tries to rouse you.
Grade 4: Coma. In this phase, you may pass out and not respond to pain or someone trying to wake you.
These grades may sound scary, but remember, HE can be treated if you act promptly. Work with your doctor closely and follow their instructions carefully.
Getting Support
Even though treatment can often keep your HE from getting worse, there may be times you'll need to get some care at home to help you when your symptoms are acting up. A caregiver can help you with some daily tasks. For instance, they can:
- Shop for food
- Help you dress and wash
- Watch to see if your symptoms get worse
You also need to reach out to family and friends to get emotional support. They know you best and can give you the backing you need while you manage your symptoms.
It helps to talk to people who know about liver disease and HE firsthand. They'll understand just what you're going through. The American Liver Foundation has information about how to find support groups in your area. It also has a lot of useful advice on a website that's all about the diagnosis and treatment of hepatic encephalopathy.
Hepatic Encephalopathy Prevention
If you have a healthy liver, preventing HE is mostly a matter of keeping it that way. That means:
- Limiting alcohol
- Preventing hepatitis A, B, and C with vaccines, safe sex, and washing your hands
- Avoiding medications, herbs, and supplements that can damage your liver
- Exercising and eating well
If your liver is already damaged, such healthy habits are even more important, Rockey says. You should make sure you are up to date with hepatitis vaccinations. And you should completely avoid alcohol and medications like sedatives and narcotics, he says.
Of course, you also should follow all your doctors' advice about controlling your liver condition, whether it's taking medications to treat hepatitis C or managing your weight, blood sugar, and cholesterol to limit damage from MSALD and MASH.
Researchers also are looking at whether some medications might prevent a first episode of HE in people with cirrhosis, Rockey says.
If you've already had HE at least once, you may need to take medications such as lactulose or rifaximin for a long time to keep it from returning.
Takeaways
Hepatic encephalopathy is a potentially serious complication of liver damage. It can happen from sudden liver damage, like the kind caused by taking too much acetaminophen. But more commonly, it happens when you have chronic liver disease. If that's you, it's important to watch for signs of it – like confusion, memory problems, and sleep issues – and talk to your doctor. Treatment can limit and sometimes reverse the damage.
Hepatic Encephalopathy FAQs
What is the most serious sign of hepatic encephalopathy?
Any sign of HE should be taken seriously, because in the most severe cases, HE can cause coma and even lead to death. If you see signs like severe drowsiness or confusion, get help right away.
Is hepatic encephalopathy reversible?
Yes. Some people recover completely. That can happen after successful treatment for sudden liver failure, for example. A liver transplant also can lead to a complete recovery, though it can take years. In other cases, you may have some lasting symptoms or your HE may come back.
Can you have hepatic encephalopathy without cirrhosis?
Yes. While HE most often results from chronic liver damage and cirrhosis, it also can be caused by sudden liver damage, which doesn't involve cirrhosis.
When can you stop lactulose in hepatic encephalopathy?
Once you've had an episode of HE, your doctor may ask you to keep taking lactulose for a long time, especially if you still have cirrhosis and other risk factors for HE. You should keep taking it unless your doctor tells you it's OK to stop.