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Questions and Answers About
the Functions and Diseases of the Liver
What are the Functions of the Liver?
The liver, located behind the lower ribs on the right side of your abdomen, weighs
about 3 pounds and is roughly the size of a football. This vital organ performs
many complex functions. Some of these are:
Your liver plays a key role in converting food into essential chemicals of
life. All of the blood that leaves the stomach and intestines must pass through
the liver before reaching the rest of the body. The liver is thus strategically
placed to process nutrients and drugs absorbed from the digestive tract into
forms that are easier for the rest of the body to use. In essence, the liver
can be thought of as the body's refinery.
Furthermore, your liver plays a principal role in removing from the blood
ingested and internally produced toxic substances. The liver converts them to
substances that can be easily eliminated from the body. It also makes bile, a
greenish-brown fluid which is essential for digestion. Bile is stored in the
gallbladder which, after eating, contracts and discharges bile into the
intestine, where it aids digestion.
Many drugs taken to treat diseases are also chemically modified by the
liver. These changes govern the drug's activity in the body. Your liver helps
you by:
What are the Diseases of the Liver?
There are many types of liver diseases, but among the most important are:
Liver diseases appear to be on the increase. Part of this increase may be
due to our frequent contact with chemicals and environmental pollutants.
Certain medications may also be hazardous to the liver in some individuals.
The liver, the detoxifying factory in the body, has become an increasingly
overworked organ. The present investment in liver research is scant in relation
to the magnitude, severity and destructiveness of these diseases.
Liver diseases are poorly understood. An adequate investment in effective
liver research has the potential of saving billions of dollars and preventing
untold human suffering. Experts estimate that more than half of all liver
diseases could be prevented if people acted upon the knowledge we already have.
Each year more than 25 million Americans are afflicted with liver and
gallbladder diseases and more than 51,532 die of liver and gallbladderdiseases
each year. There are few effective treatments for most life-threatening liver
diseases, except for liver transplants. Research has recently opened up
exciting new paths for investigation , but much more remains to be done to find
cures for more than 100 different liver diseases.
Meanwhile, patients and their families must cope with medical, financial and
emotional problems.
What are the Symptoms and Signs of Liver Disease?
If any of these signs or symptoms appear, consult your physician
immediately.
Is There Anything I Can Do to Help Prevent Liver Disease?
What are Some Of the Less Well Known Diseases That Can Affect the Liver?
Alagille Syndrome - an inherited disorder characterized by a
progressive loss of the bile ducts within the liver and narrowing of bile ducts
outside the liver over the first year of life. Symptoms include jaundice, pale,
loose stools and poor growth within the first three months of life.
Alpha 1 - Antitrypsin Deficiency - a hereditary disease that may lead
to hepatitis and cirrhosis. It is the most common genetic cause of liver
disease in children. The disease most often appears in the newborn period with
jaundice, swelling of the abdomen, and poor feeding. It may also appear in late
childhood or adulthood and be detected because of the appearance of fatigue,
poor appetite, swelling of the abdomen and legs or abnormal liver tests.
Autoimmune Hepatitis - a progressive inflammation of the liver
associated with an abnormality of the body's immune system and related to the
production of antibodies. Common symptoms include fatigue, abdominal
discomfort, aching joints, itching, jaundice, enlarged liver, and spider
angiomas (tumors) on the skin.
Biliary Atresia - a serious disease of the very young infant. This
disease results in inflammation and obstruction of the ducts which carry bile
from the liver into the intestine. Cirrhosis results when healthy liver cells
are destroyed, in this case by disease,and replaced with scar tissue.
Cystic Disease of the Liver - These include choledochal cysts,
Caroli's Syndrome, Congenital Hepatic Fibrosis, and Polycystic Liver Disease.
Fatty Liver - an accumulation of fat cells in the liver, common in
patients who are overweight or who have diabetes.
Galactosemia - a rare hereditary disease caused by elevated levels of
galactose (a sugar in milk) in the blood resulting from a deficiency of the
liver enzyme required to break it down. The disease usually appears in the
first few days of life following the ingestion of breast milk or formula. Vomiting,
liver enlargement, and jaundice are often the earliest signs of the disease,
but bacterial infections, irritability, failure to gain weight, and diarrhea
may also occur.
Gilbert's Syndrome - a relatively common and benign congenital
(probably hereditary) liver disorder, found more frequently in males. It is
characterized by a mild, fluctuating increase in serum bilirubin, a yellow
pigment excreted by the liver into bile.
Hemochromatosis - a genetic condition that causes the body to absorb
and store too much iron. While many individuals with this disease have no
symptoms, injuries to the liver can slowly lead to cirrhosis if the illness is
not treated.
Neonatal Hepatitis - inflammation of the liver that occurs only in
early infancy, usually between one and two months after birth. Symptoms include
jaundice, failure to grow or gain weight, and an enlarged liver and spleen.
Porphyria - a disease in which porphyrins, a chemical compound in the
body, do not successfully perform their task of forming heme (the substance
that makes blood red) in human beings. When they do not function properly,
porphyrins build up in the body, causing a variety of symptoms ranging from
abdominal pain and weakness to blisters on the skin.
Primary Biliary Cirrhosis - a chronic liver diseases that causes
slow, progressive destruction of bile ducts in the liver. The disease is 10
times more frequent in women than men, and is usually diagnosed in people 30 to
60 years of age. Many patients have no symptoms and are diagnosed through the
appearance of an abnormality on routine liver blood tests.
Primary Sclerosing Cholangitis - a disease in which the bile ducts
inside and outside the liver become narrowed due to inflammation and scarring.
It usually begins in the 30's, 40's or 50's and is commonly associated with
fatigue, itching and jaundice.
Reye's Syndrome - a rare complication of childhood respiratory
infections characterized by vomiting that begins three to seven days after the
onset of flu or chickenpox. It is believed that aspirin may contribute to the
development of Reye's Syndrome. Other symptoms include listlessness, staring,
and drowsiness.
Sarcoidosis - a systemic disease of unknown cause, in which nests of
cells appear in many tissues, including the lung, lymph nodes and liver.
African-Americans are affected about 15 times more often than whites in the
United States, with the highest incidence in the southeastern states.
Tyrosinemia - a genetic inborn error of metabolism associated with
severe liver disease in infancy. Children may have either the acute form, in
which symptoms appear in the first month of life, or a more chronic form. In
both cases, liver transplantation is required.
Type I Glycogen Storage Disease - a deficiency of the enzyme glucose
- 6 - phosphatase which helps in maintaining a normal blood glucose (sugar
concentration) during fasting. Symptoms include growth failure, a greatly
enlarged liver, and a distended abdomen.
Wilson's Disease - An inherited disorder
of copper secretion by the liver. Copper accumulates in the liver and nervous
system leading to severe liver and neurological disease. Penicillamine is a
successful treatment for patients with early Wilson's Disease; liver
transplantation
Questions&Answers about
Liver Transplantation
What diseases are treated by liver transplantation?
Although the first human liver transplant was done in 1963, the procedure
did not gain widespread acceptance in medical practice until the 1980s. With
this acceptance, questions from patients, relatives, healthcare workers and the
public have increased.
A large number of diseases are capable of interfering with the liver's
function sufficiently to threaten the life of the patient and most are
potentially treatable by liver transplantation.
In adults, cirrhosis, the death of liver cells due to a variety of causes,
is one of the most common reasons for which liver transplantation is done. In
children, the disease most often treated by liver transplantation is biliary
atresia, a failure of the bile ducts to develop normally to drain bile form the
liver.
People who develop cirrhosis of the liver due to excessive use of alcohol do
not need a liver transplant. Abstinence from alcohol and treatment of
complications will usually allow them to live for prolonged periods without a
transplant. For patients with advanced liver disease, where prolonged
abstinence and medical treatment fails to restore health, transplantation is a
consideration.
Most cancers of the liver begin somewhere else in the body and spread to the
liver. These are not curable with a liver transplant. Likewise the tumors which
start in the liver have usually spread to other organs by the time they are
detected and are rarely cured by liver transplantation. Transplantation at an
early stage of liver cancer may result in long-term survival for some patients.
Is liver transplantation a treatment of last resort, when everything else
has failed?
Yes and no. If medical treatment is likely to allow prolonged survival with
good quality of life, transplantation would be reserved for the future.
However, ideally the surgery is undertaken before the terminal stage of the
disease when the person is too ill to withstand major surgery and will not
survive until a suitable donor is available.
There are effective medicines for some liver diseases, while for others only
treatment for complications is available. Treatment of complications may be all
that is required if the liver is not failing. Frequently medical treatment
delays, but does not eliminate, the need for transplantation.
How is the decision made to transplant?
This is a decision made in consultation with all individuals involved in the
patient's care, including the patient and/or family. The patient and family's
input is vital and they must clearly understand the risks involved with proceeding
to transplantation.
What are the major risks?
Before surgery, the risks are mainly the development of some acute
complication of the disease which might render the patient unacceptable for
surgery. With transplantation there are risks common to all forms of major
surgery, as well as technical difficulties in removing the diseased liver and
implanting the donor liver. One of the major risks for the patient is not
having any liver function for a brief period. Immediately after surgery,
bleeding, poor function of the grafted liver, and infections are major risks.
The patient is carefully monitored for several weeks for signs of rejection of
the liver.
What are the overall chances of surviving a liver transplant?
This depends on many factors but overall 60 -75% of adult patients and 80 -
90% of children survive and are discharged from the hospital.
How long does it take to recover?
In part this depends on how ill the individual was prior to the surgery.
Most patients should count on spending a few days in an intensive care unit and
about two to four weeks in the hospital.
Initially in the intensive care unit there is very careful monitoring of all
body functions including the liver. Once the patient is transferred to the
ward, the frequency of blood testing, etc. is decreased, eating is allowed and
physiotherapy is used to regain muscle strength. The drug or drugs to prevent
rejection are initially given by vein, but later by mouth. During the
transplantation, frequent tests are done to monitor liver function and detect
any evidence of rejection.
If a transplanted liver fails to function, or is rejected, what can be
done?
There are varying degrees of failure of the liver, however, and even with
imperfect function, the patient will remain quite well. Occasionally, when
circumstances and time permit, a failing transplanted liver can be replaced by
a second (or even third) transplant. Unfortunately, there is no dialysis
treatment for livers as is possible with kidneys. Researchers are experimenting
with devices to keep patients with failing livers alive while waiting for a new
liver.
What side-effects do patients commonly experience from the drugs used to
treat or prevent rejection?
All the drugs used for rejection increase the person's susceptibility to
infections (and possibly to the development of tumors). Various medicines are
used, and each has its own effects. Cortisone-like drugs produce some fluid
retention and puffiness of the face, risk of worsening diabetes and
osteoporosis (a loss of mineral from bone). Cyclosporine produces some tendency
to develop high blood pressure and the growth of body hair. The dose of this
medication must be very carefully regulated. Kidney damage can occur from
cyclosporine but this can usually be avoided by monitoring the drug levels in
the blood. Common side effects for FK-506 include headaches, tremor, diarrhea,
increased tension, nausea, increased levels of potassium and glucose and kidney
dysfunction.
Do recipients of liver transplant have to take these medicines for the
rest of their lives?
Usually. However, as the body adjusts to the transplanted liver, the amount
of medicine needed to control rejection is reduced. There are patients who have
been successfully taken off these drugs. Researchers are attempting to
determine why this has been successful in these cases.
Routine follow-up consists of monthly blood tests, measuring of blood
pressure by a local physician with annual or semi-annual checkups at the transplant
center.
Recipients should avoid exposure to infections as the immune system is
depressed. Illness should be reported to the doctor immediately and medicines
taken only under medical supervision.
What about physical activity after a liver transplant?
Most patients are able to return to a normal or near-normal existence and
can participate in fairly vigorous physical exercise six to twelve months after
a successful liver transplant. As with other physical activities, sexual
activity may be resumed.
Is it safe for women to become pregnant after transplantation?
Studies have shown that women who undergo liver transplantation can conceive
and give birth normally, although they have to be monitored carefully because
of a higher incidence of premature births. Mothers are advised against nursing
babies because of the possibility of immunosuppressive drugs being ingested by
the infants through breast milk.
What about diet?
Transplant patients have a tendency to gain weight because of their
retention of water. They are advised to lower their intake of salt to reduce or
eliminate this water retention. Otherwise patients should maintain a balanced
diet.
Can there be a recurrence of the original disease in the transplanted
liver?
If the disease was caused by hepatitis B or C viruses then recurrence is likely.
Other types of liver disease do not recur. There is every indication that those
who are well after one year remain so indefinitely.
Where do the donor livers come from?
Livers are donated, with the consent of the next of kin, from individuals
who have brain death, usually as a result of a head injury or brain hemorrhage.
When such a donor is identified, transplant centers are contacted by a computer
network and arrangements are made to retrieve whatever organs may be donated.
Frequently this involves a team from a transplant center flying to the donor
hospital to remove the organs, and returning with them for the transplant
operation.
For liver transplants, the only requirements are that the donor and
recipient need to be approximately the same size, and of compatible blood
types. No other matching is necessary.
How can I donate my organs?
If you wish to be an organ donor, carry an
organ donor card and place an organ donor sticker on your medical
identification card. It is important to discuss organ donation with family
members since they will have to give consent. An organ donor card is available
from the American Liver Foundation.