Hepatic Encephalopathy: What Caregivers Need to Know

What is hepatic encephalopathy?

A healthy liver cleans the blood. It turns waste products, such as ammonia made by bacteria in the gut, into harmless substances. When cirrhosis has badly damaged the liver, those toxins build up and reach the brain. The result is called hepatic encephalopathy, or HE. It changes how a person thinks, acts, sleeps and moves.

HE can come on slowly over days or suddenly within hours. The good news is that it often gets better with prompt treatment. It is also one of the main warning signs that cirrhosis has become decompensated, so it deserves attention every time.

As a caregiver, you matter here more than almost anyone. You see your loved one every day, so you are often the first to notice that something is off, long before a doctor would. This guide is part of our caregiver hub.

Early signs caregivers notice first

Early HE can be subtle. Your loved one may not notice it in themselves. The 2014 AASLD/EASL guideline notes that relatives are often the ones who report the first personality changes. Early signs described in the guideline and by NIDDK include:

• Sleep changes. Very sleepy during the day, and sometimes awake, restless or mixed-up at night. This is why many families first notice confusion at night.
• Personality or mood changes. Irritable, flat or uninterested, unusually cheerful, anxious, or "just not himself."
• Forgetfulness and poor focus. Losing the thread of a conversation, repeating questions, trouble with simple math like making change.
• Clumsier hands. Trouble with handwriting, buttons and other small, coordinated movements.
• Slurred or slow speech and slow, sluggish movements.
• "Flapping" hands (asterixis). When the person holds their arms straight out with wrists bent back, the hands jerk or flap down and up, like a bird's wings.

A simple habit helps: ask your loved one to write their name and the date at the same time each day, and keep the pages. A sudden change in handwriting is easy to show the liver team.

The stages of hepatic encephalopathy (West Haven grades)

Doctors grade HE from minimal to grade 4 using the West Haven criteria, described in the 2014 AASLD/EASL practice guideline. Here is what each grade can look like at home.

Doctors group minimal HE and grade 1 together as covert HE, and grades 2 to 4 as overt HE. Grades can change quickly, sometimes within the same day.

What sets off an episode

The AASLD/EASL guideline says a trigger can be found in nearly every episode of HE, and that finding and treating it is the cornerstone of treatment. Common triggers named in the guideline are:

• Constipation. Fewer bowel movements give gut bacteria more time to make ammonia.
• Infection, such as a urinary infection, pneumonia or an infection in belly fluid (ascites).
• Bleeding in the stomach or gut. Black, tarry stools can be a sign.
• Dehydration, including from vomiting, diarrhea, too much lactulose or too much water pill (diuretic).
• Low sodium or potassium in the blood, and kidney problems.

The guideline also stresses taking HE medicines exactly as prescribed. And it lists sedating medicines (such as benzodiazepine anxiety pills and narcotic pain medicines) and alcohol among the things that can cause confusion of their own, so the team will want to know about them.

Sometimes no trigger is found. That is not your fault, and it is still worth reporting every episode.

Treatment you can help with

Lactulose

Lactulose is a liquid sugar medicine. It draws ammonia out of the blood into the bowel, where it leaves the body in the stool. For liver disease it is usually taken several times a day. The 2014 AASLD/EASL guideline says the dose is adjusted to produce 2 to 3 soft bowel movements a day.

• Keep a simple daily log of doses and bowel movements. It is the single most useful thing you can bring to the team.
• Fewer than 2 soft stools a day? Let the team know. Constipation can bring confusion back.
• Many watery stools a day? Let the team know. The guideline warns that too much lactulose can cause dehydration and can itself bring on HE.
• Do not change the dose on your own unless the team has given you written instructions for doing so.
• Diarrhea is a known side effect, and the guideline notes that too much lactulose can cause severe skin irritation around the bottom. Ask the team about a barrier cream.

It is normal for people to dislike lactulose and want to skip it, especially before an outing. Gently remind your loved one that missed doses are a common trigger, and ask the team how to plan around trips.

Rifaximin

Rifaximin is an antibiotic pill that lowers the number of toxin-making bacteria in the gut. The guideline recommends it as an add-on to lactulose to help prevent HE from coming back after a repeat episode. It is used together with lactulose, not instead of it.

Food

Do not cut protein on your own. The guideline advises against routine protein restriction, because people with cirrhosis need protein to keep their muscles. It recommends small meals spread across the day and a late-evening snack. Ask the team for a dietitian.

Keeping your loved one safe at home

Driving

Even hidden (minimal) HE can slow reaction time, and the AASLD/EASL guideline notes it can impair driving. Keep your loved one from driving until the liver team says it is safe. This can be a hard conversation; it may help to let the doctor deliver the news.

Medicines

• Take over the pill box if your loved one is confused. A weekly organizer and a checklist help.
• Avoid sleeping pills, sedatives and tranquilizers unless the liver team approves them.
• Check with the team before any over-the-counter product, including pain relievers, sleep aids and supplements.
• Make sure every doctor and pharmacist knows about the liver disease and the HE.

Falls and other risks

• Use night lights, clear walkways and keep a phone within reach.
• Help with stairs and showers during and after an episode.
• Consider taking over the stove, money and important paperwork while thinking is clouded.
• Keep the house alcohol-free.

"But the ammonia test was normal"

Many families are told about ammonia and expect a blood test to settle the question. It usually cannot. The 2014 AASLD/EASL guideline says a high ammonia level alone does not diagnose HE, does not show how severe it is, and does not predict how things will go. Doctors diagnose HE mainly from symptoms and an exam, after ruling out other causes of confusion such as infection, low blood sugar, a stroke or a head injury from a fall.

The same guideline says a normal ammonia level should make the team re-check the diagnosis. So a normal number does not end the conversation. It means everyone, including you, should keep looking closely at what is happening. Your description of the changes at home is real evidence. Share it.

HE is a reason to ask about a transplant

The AASLD and the American Society of Transplantation advise that transplant evaluation should be considered once a person with cirrhosis has a major complication such as hepatic encephalopathy, ascites or variceal bleeding, or once their MELD score reaches 15. NIDDK notes that a transplant is the only treatment for liver failure, also called end-stage liver disease.

If no one has raised a transplant referral yet, you can. Ask the team, "Does this episode mean we should be referred for transplant evaluation?" Learn who qualifies for a liver transplant and how the liver transplant waiting list works. Transplant centers will also want to know who will be the support person; see what transplant centers expect of caregivers.

When HE keeps coming back despite treatment and transplant is not an option, comfort becomes the focus. Our guide to end-stage liver disease at the end of life covers that path with care.

Looking after yourself

Living with someone who has HE is hard. The person you love may say hurtful things, wander at night or not recognize what is happening. That is the illness, not them. Sleep loss and constant watchfulness wear caregivers down. Ask family or friends to cover a night, and read our guide to caregiver burnout.