INTRODUCTION

Cirrhosis of the liver is a chronic disease that causes cell destruction and fibrosis (scarring) of
hepatic tissue. Fibrosis alters normal liver structure and vasculature, impairing blood and lymph
flow and resulting in hepatic insufficiency and hypertension in the portal vein. Complications
include hyponatremia, water retention, bleeding esophageal varices. Coagulopathy, spontaneous
bacterial peritonitis, and hepatic encephalopathy.

Cirrhosis is a potentially life-threatening condition that occurs when scarring damages the liver.
This scarring replaces healthy tissue and prevents the liver from working normally. Cirrhosis
usually develops after years of liver inflammation. When chronic diseases cause the liver to
become permanently injured and scarred, the condition is called Cirrhosis. Cirrhosis harms the
structure of the liver and blocks the flow of blood. The loss of normal liver tissue slows the
processing of nutrients, hormones, drugs, and toxins bythe liver. Also, the production of proteins
and other substances made by the liver is suppressed. People with cirrhosis often have few
symptoms at first. The person may experience fatigue, weakness, and exhaustion. Loss of
appetite is usual, often with nausea and weight loss. As liver function declines, water may
accumulate in the legs and the abdomen (ascites). A decrease in proteins needed for blood
clotting makes it easy for the person to bruise, bleeding or infection. In the later stages of
cirrhosis, jaundice (yellow skin) may occur, caused by the buildup of bile pigment that is passed
bythe liver into the intestines. The liver of a person with cirrhosis also has trouble removing
toxins, which may build up in the blood. Drugs taken usually are filtered out bythe liver , and
this cleansing process also is slowed down by cirrhosis. People with cirrhosis often are very
sensitive to medications and their side effects. The doctor often can diagnose cirrhosis from the
patient’s symptoms and from laboratory tests. During a physical exam, the doctor could notice a
change in how your liver feels or how large it is. If the doctor suspects Cirrhosis, you will be
given blood tests. The purpose of these tests is to find out if liver disease is present. In some
cases, other tests that take pictures ofthe liver are performed such as the computerized axial
tomography (CAT) scan, and ultrasound. The doctor may decide to confirm the diagnosis by
putting a needle through the skin (biopsy) to take a sample of tissue fromthe liver. In some cases,
cirrhosis is diagnosed during surgery when the doctor is able to see the entire liver.
The liver is an important organ in the body. It performs many critical functions, two of which are
producing substances required by the body, for example, clotting proteins that are necessary in
order for blood to clot, and removing toxic substances that can be harmful to the body, for
example, drugs. The liver also has an important role in regulating the supply to the body of
glucose (sugar) and lipids (fat) that the body uses as fuel. In order to perform these critical
functions, the liver cells must be working normally, and they must have an intimate relationship
with the blood since the substances that are added or removed by the liver are transported to and
from the liver by the blood.

The relationship of the liver to the blood is unique. Unlike most organs in the body, only a small
amount of blood is supplied to the liver by arteries. Most of the liver's supply of blood comes
from the intestinal veins as the blood returns to the heart. The main vein that returns blood from
the intestines is called the portal vein. As the portal vein passes through the liver, it breaks up
into increasingly smaller and smaller veins. The tiniest veins (called sinusoids because of their
unique structure) are in close contact with the liver cells. In fact, the liver cells line up along the
length of the sinusoids. This close relationship between the liver cells and blood from the portal
vein allows the liver cells to remove and add substances to the blood. Once the blood has passed
through the sinusoids, it is collected in increasingly larger and larger veins that ultimately form a
single vein, the hepatic vein that returns the blood to the heart.
In cirrhosis, the relationship between blood and liver cells is destroyed. Even though the liver
cells that survive or are newly-formed may be able to produce and remove substances from the
blood, they do not have the normal, intimate relationship with the blood, and this interferes with
the liver cells' ability to add or remove substances from the blood. In addition, the scarring
within the cirrhotic liver obstructs the flow of blood through the liver and to the liver cells. As a
result of the obstruction to the flow of blood through the liver, blood "backs-up" in the portal
vein, and the pressure in the portal vein increases, a condition called portal hypertension.
Because of the obstruction to flow and high pressures in the portal vein, blood in the portal vein
seeks other veins in which to return to the heart, veins with lower pressures that bypass the liver.
Unfortunately, the liver is unable to add or remove substances from blood that bypasses it. It is a
combination of reduced numbers of liver cells, loss of the normal contact between blood passing
through the liver and the liver cells, and blood bypassing the liver that leads to many of the
manifestations of cirrhosis.

A second reason for the problems caused by cirrhosis is the disturbed relationship between the
liver cells and the channels through which bile flows. Bile is a fluid produced by liver cells that
has two important functions: to aid in digestion and to remove and eliminate toxic substances
from the body. The bile that is produced by liver cells is secreted into very tiny channels that run
between the liver cells that line the sinusoids, called canaliculi. The canaliculi empty into small
ducts which then join together to form larger and larger ducts. Ultimately, all of the ducts
combine into one duct that enters the small intestine. In this way, bile gets to the intestine where
it can help with the digestion of food. At the same time, toxic substances contained in the bile
enter the intestine and then are eliminated in the stool. In cirrhosis, the canaliculi are abnormal
and the relationship between liver cells and canaliculi is destroyed, just like the relationship
between the liver cells and blood in the sinusoids. As a result, the liver is not able to eliminate
toxic substances normally, and they can accumulate in the body. To a minor extent, digestion in
the intestine also is reduced.
SIGNS and SYMPTOMS

Patients with cirrhosis may have few or no symptoms and signs of liver disease. Some of the
symptoms may be nonspecific, that is, they don't suggest that the liver is their cause. Some of the
more common symptoms and signs of cirrhosis include:

• Yellowing of the skin (jaundice) due to the accumulation of bilirubin in the blood
• Fatigue
• Weakness
• Loss of appetite
• Itching
• Easy bruising from decreased production of blood clotting factors by the diseased liver.

Patients with cirrhosis also develop symptoms and signs from the complications of cirrhosis that
are discussed next.

COMPLICATIONS OF CIRRHOSIS

Edema and ascites

As cirrhosis of the liver becomes severe, signals are sent to the kidneys to retain salt and water in
the body. The excess salt and water first accumulates in the tissue beneath the skin of the ankles
and legs because of the effect of gravity when standing or sitting. This accumulation of fluid is
called edema or pitting edema. (Pitting edema refers to the fact that pressing a fingertip firmly
against an ankle or leg with edema causes an indentation in the skin that persists for some time
after release of the pressure. Actually, any type of pressure, such as from the elastic band of a
sock, may be enough to cause pitting.) The swelling often is worse at the end of a day after
standing or sitting and may lessen overnight as a result of the loss of the effects of gravity when
lying down. As cirrhosis worsens and more salt and water are retained, fluid also may
accumulate in the abdominal cavity between the abdominal wall and the abdominal organs. This
accumulation of fluid (called ascites ) causes swelling of the abdomen, abdominal discomfort,
and increased weight.
Spontaneous bacterial peritonitis (SBP)

Fluid in the abdominal cavity (ascites) is the perfect place for bacteria to grow. Normally, the
abdominal cavity contains a very small amount of fluid that is able to resist infection well, and
bacteria that enter the abdomen (usually from the intestine) are killed or find their way into the
portal vein and to the liver where they are killed. In cirrhosis, the fluid that collects in the
abdomen is unable to resist infection normally. In addition, more bacteria find their way from the
intestine into the ascites. Therefore, infection within the abdomen and the ascites, referred to as
spontaneous bacterial peritonitis or SBP, is likely to occur. SBP is a life- threatening
complication. Some patients with SBP have no symptoms, while others have fever, chills,
abdominal pain and tenderness, diarrhea, and worsening ascites.

Bleeding from esophageal varices

In the cirrhotic liver, the scar tissue blocks the flow of blood returning to the heart from the
intestines and raises the pressure in the portal vein (portal hypertension). When pressure in the
portal vein becomes high enough, it causes blood to flow around the liver through veins with
lower pressure to reach the heart. The most common veins through which blood bypasses the
liver are the veins lining the lower part of the esophagus and the upper part of the stomach.

As a result of the increased flow of blood and the resulting increase in pressure, the veins in the
lower esophagus and upper stomach expand and then are referred to as esophageal and gastric
varices; the higher the portal pressure, the larger the varices and the more likely a patient is to
bleed from the varices into the esophagus or stomach.

Bleeding from varices usually is severe and, without immediate treatment, can be fatal.
Symptoms of bleeding from varices include vomiting blood (the vomitus can be red blood mixed
with clots or "coffee grounds" in appearance, the latter due to the effect of acid on the blood),
passing stool that is black and tarry due to changes in the blood as it passes through the intestine
(melena), and orthostatic dizziness or fainting (caused by a drop in blood pressure especially
when standing up from a lying position).
Bleeding also may occur from varices that form elsewhere in the intestines, for example, the
colon, but this is rare. For reasons yet unknown, patients hospitalized because of actively
bleeding esophageal varices have a high risk of developing spontaneous bacterial peritonitis.

Hepatic encephalopathy

Some of the protein in food that escapes digestion and absorption is used by bacteria that are
normally present in the intestine. While using the protein for their own purposes, the bacteria
make substances that they release into the intestine. These substances then can be absorbed into
the body. Some of these substances, for example, ammonia, can have toxic effects on the brain.
Ordinarily, these toxic substances are carried from the intestine in the portal vein to the liver
where they are removed from the blood and detoxified.

As previously discussed, when cirrhosis is present, liver cells cannot function normally either
because they are damaged or because they have lost their normal relationship with the blood. In
addition, some of the blood in the portal vein bypasses the liver through other veins. The result
of these abnormalities is that toxic substances cannot be removed by the liver cells, and, instead,
the toxic substances accumulate in the blood.

When the toxic substances accumulate sufficiently in the blood, the function of the brain is
impaired, a condition called hepatic encephalopathy. Sleeping during the day rather than at night
(reversal of the normal sleep pattern) is among the earliest symptoms of hepatic encephalopathy.
Other symptoms include irritability, inability to concentrate or perform calculations, loss of
memory, confusion, or depressed levels of consciousness. Ultimately, severe hepatic
encephalopathy causes coma and death.

The toxic substances also make the brains of patients with cirrhosis very sensitive to drugs that
are normally filtered and detoxified by the liver. Doses of many drugs that normally are
detoxified by the liver have to be reduced to avoid a toxic buildup in cirrhosis, particularly
sedatives and drugs that are used to promote sleep. Alternatively, drugs may be used that do not
need to be detoxified or eliminated from the body by the liver, for example, drugs that are
eliminated by the kidneys.
Hepatorenal syndrome

Patients with worsening cirrhosis can develop the hepatorenal syndrome. This syndrome is a
serious complication in which the function of the kidneys is reduced. It is a functional problem in
the kidneys, that is, there is no physical damage to the kidneys. Instead, the reduced function is
due to changes in the way the blood flows through the kidneys themselves. The hepatorenal
syndrome is defined as progressive failure of the kidneys to clear substances from the blood and
produce adequate amounts of urine even though some other important functions of the kidney,
such as retention of salt, are maintained. If liver function improves or a healthy liver is
transplanted into a patient with hepatorenal syndrome, the kidneys usually begin to work
normally. This suggests that the reduced function of the kidneys is the result of the accumulation
of toxic substances in the blood when the liver fails. There are two types of hepatorenal
syndrome. One type occurs gradually over months. The other occurs rapidly over a week or two.

Hepatopulmonary syndrome

Rarely, some patients with advanced cirrhosis can develop the hepatopulmonary syndrome.
These patients can experience difficulty breathing because certain hormones released in
advanced cirrhosis cause the lungs to function abnormally. The basic problem in the lung is that
not enough blood flows through the small blood vessels in the lungs that are in contact with the
alveoli (air sacs) of the lungs. Blood flowing through the lungs is shunted around the alveoli and
cannot pick up enough oxygen from the air in the alveoli. As a result the patient experiences
shortness of breath, particularly with exertion.

Hypersplenism

The spleen normally acts as a filter to remove older red blood cells, white blood cells, and
platelets (small particles that are important for the clotting of blood.). The blood that drains from
the spleen joins the blood in the portal vein from the intestines. As the pressure in the portal vein
rises in cirrhosis, it increasingly blocks the flow of blood from the spleen. The blood "backs-up"
and accumulates in the spleen, and the spleen swells in size, a condition referred to as
splenomegaly. Sometimes, the spleen is so swollen that it causes abdominal pain.
As the spleen enlarges, it filters out more and more of the blood cells and platelets until their
numbers in the blood are reduced. Hypersplenism is the term used to describe this condition, and
it is associated with a low red blood cell count (anemia), low white blood cell count (leucopenia),
and/or a low platelet count (thrombocytopenia). The anemia can cause weakness, the leucopenia
can lead to infections, and the thrombocytopenia can impair the clotting of blood and result in
prolonged bleeding.

Liver cancer (hepatocellular carcinoma)

Cirrhosis due to any cause increases the risk of primary liver cancer (hepatocellular carcinoma).
Primary refers to the fact that the tumor originates in the liver. A secondary liver cancer is one
that originates elsewhere in the body and spreads (metastasizes) to the liver.

The most common symptoms and signs of primary liver cancer are abdominal pain and swelling,
an enlarged liver, weight loss, and fever. In addition, liver cancers can produce and release a
number of substances, including ones that cause an increased in red blood cell count
(erythrocytosis), low blood sugar (hypoglycemia), and high blood calcium (hypercalcemia ).

Common causes of Cirrhosis

• Alcohol is a very common cause of cirrhosis, particularly in the Western world. The
development of cirrhosis depends upon the amount and regularity of alcohol intake.
Chronic, high levels of alcohol consumption injure liver cells. Thirty percent of
individuals who drink daily at least eight to sixteen ounces of hard liquor or the
equivalent for fifteen or more years will develop cirrhosis. Alcohol causes a range of
liver diseases; from simple and uncomplicated fatty liver (steatosis), to the more serious
fatty liver with inflammation (steatohepatitis or alcoholic hepatitis), to cirrhosis.

• Nonalcoholic fatty liver disease (NAFLD) refers to a wide spectrum of liver diseases
that, like alcoholic liver disease, ranges from simple steatosis, to nonalcoholic
steatohepatitis (NASH), to cirrhosis. All stages of NAFLD have in common the
accumulation of fat in liver cells. The term nonalcoholic is used because NAFLD occurs
in individuals who do not consume excessive amounts of alcohol, yet, in many respects,
 the microscopic picture of NAFLD is similar to what can be seen in liver disease that is
due to excessive alcohol. NAFLD is associated with a condition called insulin resistance,
which, in turn, is associated with the metabolic syndrome and diabetes mellitus type 2.
Obesity is the most important cause of insulin resistance, metabolic syndrome, and type 2
diabetes. NAFLD is the most common liver disease in the United States and is
responsible for 24% of all liver disease. In fact, the number of livers that are transplanted
for NAFLD-related cirrhosis is on the rise. Public health officials are worried that the
current epidemic of obesity will dramatically increase the development of NAFLD and
cirrhosis in the population.

• Cryptogenic cirrhosis (cirrhosis due to unidentified causes) is a common reason for

liver transplantation. It is termed cryptogenic cirrhosis because for many years doctors
have been unable to explain why a proportion of patients developed cirrhosis. Doctors
now believe that cryptogenic cirrhosis is due to NASH (nonalcoholic steatohepatitis)
caused by long standing obesity, type 2 diabetes, and insulin resistance. The fat in the
liver of patients with NASH is believed to disappear with the onset of cirrhosis, and this
has made it difficult for doctors to make the connection between NASH and cryptogenic
cirrhosis for a long time. One important clue that NASH leads to cryptogenic cirrhosis is
the finding of a high occurrence of NASH in the new livers of patients undergoing liver
transplant for cryptogenic cirrhosis. Finally, a study from France suggests that patients
with NASH have a similar risk of developing cirrhosis as patients with long standing
infection with hepatitis C virus. (See discussion that follows.) However, the progression
to cirrhosis from NASH is thought to be slow and the diagnosis of cirrhosis typically is
made in patients in their sixties.

• Chronic viral hepatitis is a condition where hepatitis B or hepatitis C virus infects the
liver for years. Most patients with viral hepatitis will not develop chronic hepatitis and
cirrhosis. For example, the majority of patients infected with hepatitis A recover
completely within weeks, without developing chronic infection. In contrast, some
patients infected with hepatitis B virus and most patients infected with hepatitis C virus
develop chronic hepatitis, which, in turn, causes progressive liver damage and leads to
cirrhosis, and, sometimes, liver cancers.
• Inherited (genetic) disorders result in the accumulation of toxic substances in the liver
which lead to tissue damage and cirrhosis. Examples include the abnormal accumulation
of iron (hemochromatosis) or copper (Wilson's disease). In hemochromatosis, patients
inherit a tendency to absorb an excessive amount of iron from food. Over time, iron
accumulation in different organs throughout the body causes cirrhosis, arthritis, heart
muscle damage leading to heart failure, and testicular dysfunction causing loss of sexual
drive. Treatment is aimed at preventing damage to organs by removing iron from the
body through bloodletting (removing blood). In Wilson disease, there is an inherited
abnormality in one of the proteins that controls copper in the body. Over time, copper
accumulates in the liver, eyes, and brain. Cirrhosis, tremor, psychiatric disturbances and
other neurological difficulties occur if the condition is not treated early. Treatment is with
oral medication that increases the amount of copper that is eliminated from the body in
the urine.

• Primary biliary cirrhosis (PBC) is a liver disease caused by an abnormality of the

immune system that is found predominantly in women. The abnormal immunity in PBC
causes chronic inflammation and destruction of the small bile ducts within the liver. The
bile ducts are passages within the liver through which bile travels to the intestine. Bile is
a fluid produced by the liver that contains substances required for digestion and
absorption of fat in the intestine, as well as other compounds that are waste products,
such as the pigment bilirubin. (Bilirubin is produced by the breakdown of hemoglobin
from old red blood cells.). Along with the gallbladder, the bile ducts make up the biliary
tract. In PBC, the destruction of the small bile ducts blocks the normal flow of bile into
the intestine. As the inflammation continues to destroy more of the bile ducts, it also
spreads to destroy nearby liver cells. As the destruction of the hepatocytes proceeds, scar
tissue (fibrosis) forms and spreads throughout the areas of destruction. The combined
effects of progressive inflammation, scarring, and the toxic effects of accumulating waste
products culminates in cirrhosis.

• Primary sclerosing cholangitis (PSC) is an uncommon disease found frequently in

patients with ulcerative colitis . In PSC, the large bile ducts outside of the liver become
inflamed, narrowed, and obstructed. Obstruction to the flow of bile leads to infections of
 the bile ducts and jaundice and eventually causes cirrhosis. In some patients, injury to the
bile ducts (usually as a result of surgery) also can cause obstruction and cirrhosis of the
liver.

• Autoimmune hepatitis is a liver disease caused by an abnormality of the immune system

that is found more commonly in women. The abnormal immune activity in autoimmune
hepatitis causes progressive inflammation and destruction of liver cells (hepatocytes),
leading ultimately to cirrhosis.

• Infants can be born without bile ducts (biliary atresia) and ultimately develop
cirrhosis. Other infants are born lacking vital enzymes for controlling sugars that leads to
the accumulation of sugars and cirrhosis. On rare occasions, the absence of a specific
enzyme can cause cirrhosis and scarring of the lung (alpha 1 antitrypsin deficiency).

• Less common causes of cirrhosis include unusual reactions to some drugs and
prolonged exposure to toxins, as well as chronic heart failure (cardiac cirrhosis). In
certain parts of the world (particularly Northern Africa), infection of the liver with a
parasite (schistosomiasis) is the most common cause of liver disease and cirrhosis.

ANATOMY AND PHYSIOLOGY:

The liver is located in the upper right-hand portion of the abdominal cavity, beneath the
diaphragm and on top of the stomach, right kidney and intestines. The liver, a dark reddish-
brown organ that weighs about 3 pounds, has multiple functions.

There are two distinct sources that supply blood to the liver:
 • oxygenated blood flows in from the hepatic artery
• nutrient-rich blood flows in from the portal vein

The liver holds about one pint (13 percent) of the body’s blood supply at any given moment.

The liver consists of two main lobes, both of which are made up of thousands of lobules. These
lobules are connected to small ducts that connect with larger ducts to ultimately form the hepatic
duct. The hepatic duct transports the bile produced bythe liver cells to the gallbladder and
duodenum (the first part of the small intestine).

Cirrhosis is known in three major forms. In Laennec’s (alcohol-induced) cirrhosis, fibrosis

occurs mainly around central veins and portal areas. This is the most common form of cirrhosis
and results from chronic alcoholism and malnutrition. Postnecrotic (micronodular) cirrhosis
consist of broad bands of scar tissue and results from previous acute viral hepatitis or drug-
induced massive hepatic necrosis. Biliary cirrhosis consists of scarring of bile ducts and lobes of
the liver and results from chronic biliary obstruction and infection (cholangitis), and is much
rarer than the preceding forms.

Diagnostic Evaluation:
 1. Elevated serum liver enzyme levels, reduced serum albumin.
2. Liver biopsy detects cell destruction and fibrosis of hepatic disease.
3. Liver scan shows abnormal thickening and a liver mass.
4. CT scan determines the size of the liver and its irregular nodular surface.
5. Esophagoscopy determines the presence of esophageal varices.
6. Percutaneous transhepatic cholangiography differentiates extrahepatic from intrahepatic
obstructive jaundice.
7. Paracentesis examines ascitic fluid for cell, protein, and bacteria counts.

Pharmacologic Interventions:
 1. Provide asymptomatic relief measures such as pain medications and antiemetics.
2. Diuretic therapy, frequently with spironolactone, a potassium-sparing diuretic that
inhibits the action of aldosteroe on the kidneys.
3. I.V albumin to maintain osmotic pressure and reduce ascites.
4. Administration of lactulose or neomycin through a nasogastric tube or retention enema to
reduce ammonia levels during periods ofhepatic encephalopathy.

Surgical Intervention:

1. Transjugular intrahepatic portosystemic shunt may be performed in patients whose

ascites prove resistant. This percutaneous procedure creates a shunt from the portal to
systemic cisculation to reduce portal pressure and relieve ascites.
2. Orthotopic liver transplantation may be necessary.

Nursing Interventions:
1. Observe stools and emesis for color, consistency, and amount, and test each one for
occult blood.
2. Monitor fluid intake and output and serum electrolyte levels to prevent dehydration and
hypokalemia, which may precipitate hepatic encephalopathy.
3. Maintain some periods of rest with legs elevated to mobilize edema and ascites. Alternate
rest periods with ambulation.
4. Encourage and assist with gradually increasing periods of exercise.
5. Encourage the patient to eat high-calorie, moderate protein meals and supplementary
feedings. Suggest small, frequent feedings.
6. Encourage oral hygiene before meals.
7. Administer or teach self-administration of medications for nausea, vomiting, diarrhea or
constipation.
8. Encourage frequent skin care, bathing with soap, and massage with emollient lotions.
9. Keep the patient’s finger nails short to prevent scratching from pruritus.
10. Keep the patient quiet and limit activity if signs of bleeding are evident.
11. Encourage the patient to eat foods high vitamin C content.
12. Use small gauge needles for injections and maintain pressure over injection site until
bleeding stops.
13. Protect from sepsis through good handwashing and prompt recognition and management
of infection.
14. Pad side rails and provide careful nursing surveillance to ensure the patient’s safety.
15. Stress the importance of giving up alcohol completely.
16. Involve the person closest to the patient, because recovery usually is not easy and
relapses are common.