In the 1840’s, a French physician looked at a diseased liver and coined the word cirrhosis from the Greek kirrhos for tawny. A common myth is that cirrhosis is caused solely by alcoholism. Actually, there are many causes. Examples are hepatitis B, C, and D, fatty liver disease, excess iron, certain drugs and supplements, and other liver diseases.

One in five people with chronic hepatitis C virus infection (HCV) will develop cirrhosis. It starts when the liver tries to eliminate HCV, by producing more liver enzymes. But for some, the liver can’t keep up with the pace and it builds scar tissue. This scar tissue is called fibrosis. Most with HCV have fibrosis and will not progress past this stage. If the damage becomes severe, the liver hardens into cirrhosis. If it happens, the process usually takes twenty or more years.

One way to describe cirrhosis is to picture a drip coffee maker. If you lived in a place where the water is hard, mineral deposits will build up in the holes the water passes through. In time, deposits accumulate so that water can’t pass through. Your coffee maker would have cirrhosis.

“Cirrhosis has been linked to as little as 2-3 daily glasses of alcohol for healthy women and 3-4 for men, according to the National Institute of Diabetes, Digestive, and Kidney Disease.”

There is a problem with this analogy. The mineral deposits can be cleaned out with various solutions, such as white vinegar. Although the liver acts as a filter, fibrosis and cirrhosis aren’t simply clogged up vessels. The liver is damaged because the constant assault from inflammation actually changes the architecture of the tissue. For this reason, products that promise to cleanse the liver are misleading because it is not an organ that can be “flushed out” with herbs or supplements.

Signs & Symptoms

The liver is referred to as a non-complaining organ. Although twinges are common, most patients don’t run to the clinic, writhing with liver pain. Typically patients find out they have HCV via a routine lab test, such as when applying for insurance or attempting to donate blood. Or, labs may be ordered to address vague complaints such as fatigue.

This silent feature is good news for those who abhor pain and bad news for those who can’t stand uncertainty. If the disease is silent, how does someone know if they have cirrhosis? There may be symptoms such as fatigue, appetite loss or nausea. However, these are symptoms of a host of other conditions, including HCV. So again, how does one know if they have cirrhosis? Your health provider can tell you.

The liver is normally soft and smooth. On exam, if your liver feels hard and coarse, this is a sign. The spleen may be enlarged. You may have red, spidery blotches, usually on upper body and face, called spider nevi.

The signs and symptoms of cirrhosis may be caused by other conditions, some of which are very minor. For instance, a red spider vein on the face may be just that. Don’t assume, don’t panic, don’t self-diagnose.

Lab tests are critical. Some typical lab abnormalities include:

• Decreased platelets – the sticky part of the blood that helps clotting

• Increased blood clotting times

• Low cholesterol

Lab abnormalities that occur later in cirrhosis may include low albumin and high bilirubin,both produced by the liver.

Ultrasound and other imaging studies may be ordered but are not reliable diagnostic tools. A liver biopsy is the surest way to confirm cirrhosis. For most, the risk of liver biopsy-related complications is low. This risk increases if there is cirrhosis, so make sure the physician performing the biopsy is experienced. If the procedure is done in a hospital during July, ask if a new resident will be doing the biopsy. If the answer is yes, ask yourself if you are comfortable with this since this is the start of rotation for new residents.

If lab tests point to a bleeding risk, a safer option is to have a transjugular biopsy. This procedure is done under sedation. A long, thin tube is guided to the liver through an incision made in the neck’s jugular vein. Liver tissue is obtained via a biopsy needle threaded through the tube. If there is accidental bleeding, the blood flows into the liver’s own vein rather than leaking into the abdomen.

Two Stages of Cirrhosis

Sometimes the symptoms of cirrhosis are not subtle. A friend found out she had cirrhosis after vomiting blood. Her liver was severely damaged. She probably had cirrhosis for awhile. I’ll explain the bleeding in a moment.

Cirrhosis is categorized as compensated or decompensated. Compensated cirrhosis means that the liver is performing relatively well in spite of the damage. Decompensated cirrhosis means that the liver is not functioning properly. Decompensated cirrhosis is also known as end-stage liver disease (ESLD). About one in five of those with HCV-induced cirrhosis will progress to a decompensated stage.

Let’s go back to the coffee maker analogy. Imagine that the holes through which the water passes are starting to get clogged from mineral deposits. This happened gradually. You probably didn’t notice that it took longer to brew. Maybe you thought the coffee maker was getting old. But it worked and every morning you still had coffee, so there was no reason to suspect anything. Then one day, the holes are completely clogged to the point that everything gushes back out the top of the machine. You have a mess of steam, water, and coffee grounds.

It is similar with cirrhosis. Blood flow eventually backs up into the liver’s portal vein. The circulatory system tries to push blood through the damaged organ. The blood pressure in the portal system increases. This is known as portal hypertension.

At this point, everything in the area is affected. The spleen gets bigger because blood backs up into it. Splenomegaly is the term for an enlarged spleen. Platelets get trapped in the spleen, reducing the body’s ability to clot. Also because of the increased pressure, varicose veins called varices form in the esophagus and stomach. These can burst, as in the case of my friend who vomited blood. The body’s decreased clotting ability combined with bleeding varices is potentially life-threatening.

“Compensated cirrhosis means that the liver is performing relatively well in spite of the damage. Decompensated cirrhosis means that the liver is not functioning properly.”

The liver has over 500 functions, so portal hypertension affects nearly every bodily system. Here are some of the complications of advanced cirrhosis:
• susceptibility to bodily bruising and bleeding
• severe itching
• osteoporosis – bone loss
• increased infections
• cachexia – loss of muscle mass
• increased or decreased blood sugar
• breast enlargement in men along with atrophy of testes
• menstrual irregularities
• kidney abnormalities
• ascites – fluid retention, particularly abdominal and ankle swelling
• jaundice – yellowing of eyes and skin
• cola-colored urine and/or clay-colored stools
• varices – esophageal and gastrointestinal bleeding
• hepatic encephalopathy – mental changes caused by accumulation of toxins in the brain, such as poor memory and concentration or impaired sleep

As stated earlier, the liver will compensate for the decreased function of 80% of those with HCV-induced cirrhosis. This means that the majority of those with cirrhosis are able to function despite cirrhosis. Most of these haven’t progressed enough to warrant a referral for liver transplant evaluation. Many will live long and active lives. These patients need regular medical visits to monitor their health, particularly because those with HCV and cirrhosis have an increased risk of liver cancer. Early diagnosis is a critical factor in liver cancer survival rate.

Although this subject is frightening, take comfort in this: 1) The majority of us with HCV will not progress to cirrhosis. 2) Of those that do progress, the majority can still live full, productive lives. Next month’s column will go into more detail, concluding with how to help ourselves live with cirrhosis or avoid it altogether.

Part Two

http://www.hcvadvocate.org/news/newsLetter/2009/advocate0209.html#3

Cirrhosis was the subject of last month’s column. Two points were emphasized. One, the majority of those with chronic hepatitis C virus infection (HCV) will not progress to advanced liver disease. However, roughly one in five of those with HCV have cirrhosis. This leads to the second point. A full, productive life is possible even with this level of liver damage. Now we’ll go into more detail, concluding with how to help ourselves live with cirrhosis or avoid it altogether.

The best way to prevent cirrhosis is to eliminate or minimize excess inflammation. Living with HCV means there is already a potential source of inflammation. This is the result of the immune system’s attempt to eradicate HCV. Over time, inflammation may lead to tissue damage, or fibrosis. If the damage becomes severe, the liver hardens into cirrhosis. Fibrosis is common with HCV but does not usually advance to cirrhosis. If it does, progression to cirrhosis usually takes twenty or more years.

Treatment is a potential remedy for HCV-induced damage. The liver has remarkable recuperation power, so removing the source of damage allows the liver to recover. Even if HCV isn’t permanently eradicated, there is often some benefit. For example, I underwent treatment in 2003. My liver biopsy showed dramatic improvement although I had not had a sustained response. Even cirrhotic patients have shown improvement from treatment.

There are other potential causes of liver inflammation. Alcohol is a common one. The amount of alcohol that can cause tissue damage is surprisingly low. The maximum safe alcohol allowance for healthy adult women without liver disease is one drink daily. For healthy adult men without liver disease, that maximum is 2 drinks daily. These recommendations drop to zero for those with HCV, substance abuse, other medical conditions, pregnancy or those taking medications.

Even those without risk factors must exercise caution when drinking alcohol. Safe alcohol use is measured as one 12-ounce bottle of beer or wine cooler, one 5-ounce glass of wine, or 1.5 ounces of 80-proof distilled spirits. A glass of wine is likely to exceed 5 ounces when enjoying an evening with friends. It is easy to rationalize a second or third drink if you only drink on weekends. However, this excess puts extra load on the liver. Over time, this can amount to tissue damage.

Sadly, risk for cirrhosis is increasing along with our waistlines. Excess fat in the liver may lead to a fatty liver. This may lead to inflammation. Left unheeded, the inflamed liver may become scarred and hardened.

Those at risk for fatty liver disease tend to have other diet-related conditions, such as obesity, diabetes, or high triglycerides. However, someone without these problems may still develop a fatty liver. The movie Super Size Me is a good illustration. For thirty days, Morgan Spurlock ate only McDonald’s food. Simultaneously, he reduced his exercise level to the national average. Before the month was over, Spurlock was sick. Lab tests confirmed this, including abnormal liver enzymes. In one month, Spurlock developed a fatty liver.

So, if you are overweight, try to lose those extra pounds. Do this slowly and wisely. Don’t try to lose more than a pound or two weekly. Discuss this with your medical provider, particularly if you have diabetes or high triglycerides. Include regular physical activity in your fitness goals.

Drugs may cause liver damage. Not just illegal ones, but prescribed and over-the counter drugs. Best known for liver toxicity is excess acetaminophen or Tylenol®, although it is generally safe if taken as prescribed. This drug is often combined with other drugs, such as cold or pain medications. Drug-induced inflammation commonly occurs when a patient is unaware of the amount of acetaminophen he or she is taking. Discuss your medications with your medical provider. Always take medications as directed.

Dietary supplements may also cause liver damage or may interact with foods, drugs, or other supplements. Excess iron and vitamin A are noteworthy examples. Discuss dietary supplements with your medical provider. Spend some time doing research and choose supplements carefully.

For more information about dietary supplements and the liver, check out HCSP’s fact sheets such as HCV & CAM: Dietary Supplements to Avoid

Regular Checkups
While visiting his physician, a patient started to hyperventilate. “I’m sure I’ve got cirrhosis,” he exclaimed. The doctor asked, “Why do you think that? The liver is a non-complaining organ, so you wouldn’t feel any discomfort if you had cirrhosis.” “Exactly!” said the patient. “Those are my precise symptoms.”

The silent nature of liver disease is one of the reasons that regular medical check-ups are important. Each physician will tailor their recommendations for each patient, likely ordering regular blood tests and physical exams. Here are some general guidelines:

No cirrhosis – minimal fibrosis (Stage 0-early stage 2)

Annual physical exam
Yearly or twice-yearly lab tests, including liver panel and complete blood count (CBC); regular viral load tests are unnecessary unless you are undergoing treatment
Liver biopsy every 5 years

No cirrhosis – moderate to severe fibrosis (late stage 2-3)

Yearly or twice yearly physical exam
Every six months – lab tests, including liver panel and complete blood count (CBC); regular viral load tests are unnecessary unless you are undergoing treatment
Liver biopsy every 3 to 5 years
Late stage 3 fibrosis – ultrasound and alpha-fetoprotein (AFP) lab test may be ordered every 6 months

Cirrhosis (stage 4)

Regular, more frequent physical exams, depending on severity
Regular, more frequent labs, depending on severity – more extensive tests ordered including AFP; regular viral load tests are unnecessary unless you are undergoing treatment
Regular ultrasound; liver biopsy only if necessary; other diagnostic tests may be ordered

What You Can Do
There are measures you can take to help stave off cirrhosis or live with it if you are already there. Here are some recommendations:

Abstain from alcohol
Discuss all your medicines with your medical provider, including over-the-counter, herbs and other supplements
Do not exceed the recommended dose of acetaminophen (Tylenol®)
Maintain a normal weight
Do not eat wild mushrooms unless you are 100% sure of their safety
Avoid exposure to vibrio vulnificus, an organism found inraw or under cooked shellfish
Those with cirrhosis and open sores may need to avoid the ocean in areas where there is a risk of vibrio vulnificus exposure
Keep immunizations current
Consider HCV treatment

Drinking coffee may provide some benefit – specifically the caffeinated kind. The studies are small and although caffeine use is associated with liver tissue (histological) improvement, correlation is not the same as causation. Links about caffeine and the liver are included at the end of this article.

Cirrhosis is a scary subject but it does not mean that life is over. Some have used potentially life-threatening diagnoses as opportunities to make lifestyle changes. They turned their health around and lead full lives. Sometimes the end is actually the beginning.

Additional Information
Bennet Cecil, MD’s HCV Cirrhosis is a Life Threatening Disease published by HCSP’s Medical Writers’ Circle

Alan Franciscus’s Disease Progression: What is Cirrhosis?

Caffeine articles:
www.medpagetoday.com/MeetingCoverage
/AASLD/11647

http://www.hcvadvocate.org/news/newsLetter/2009/advocate0109.html#3

2008 Research