Porphyria Educational Services
PORPHYRIA EDUCATIONAL SERVICES Bulletin Vol. 1 No. 14
April 4, 1999
The Liver & Gallbladder
The liver is a an important focus in the hepatic
types of acute porphyria as well as some of the cutaneous forms. Before focusing on the
porphyria types and the liver, let us look chiefly at the liver
itself. How much do we each know about our liver? The largest internal organ
in the human body.
The liver is the main organ that keeps you alive. It is said of the liver
that it performs over 100 separate bodily functions.
The sheer complexity of the liver makes it susceptible to almost
as many different diseases. Many of these diseases are
rare, or like some types of porphyria, not rare, but hardly
common either. Of the common liver diseases some are
"household names" that are all too common, including
hepatitis, cirrhosis, liver disorders in children,
alcohol-related disorders, and liver cancer.
In a report published in a medical
journal in 1998, it states that over 25 million people are
afflicted with liver and gallbladder disease each year.
Furthermore the report states that over 27,000 Americans die from
cirrhosis annually. With a mortality census like this, it
makes cirrhosis of the liver the third leading cause of death for
people between the ages of 25 and 59, and the seventh leading
cause of death overall in the United States today.
Viruses, hereditary defects such as porphyria, and reactions to
drugs and chemicals are among the known causes of liver
breakdown. Though
few treatments are effective for life-threatening liver disease,
avoiding alcohol and other substances known to cause damage can
do a lot to safeguard this important organ. Many drugs are
unsafe for use in people with liver disease, so it is vital to
read up on any pharmaceutical before taking it, even if your
doctor has presecribed it for you. This is especially true
in porphyria. If a drug is metabolized in the liver, you do
not want to use it. Porphyrins are manufactured in the liver. With
increased acute attacks or what some call chronic porphyria, the
liver may be damaged more readily.
Avoiding drugs is imperative. Seeking immediate
Intervention Therapy is also imperative to reduce the risk of
developing porphyrin crystallization in the liver. So where is this organ
that plays such a vital role in porphyria?
The liver is located behind the lower ribs, right below the
diaphragm on the right side of the abdomen. In an
average-sized man, it is about the size of a football, weighing a
little over three pounds. The liver can be compared to an oil refinery, or a
processing plant. The liver processes many chemicals necessary
for the body's overall functioning. For example, it
converts carbohydrates, fats, and proteins into chemicals
essential for life and growth. It manufactures and exports to other organs some of the
substances they need to function properly, such as the bile used
by the intestines during digestion. By now all of us know the word
"metabolizes". This is done in the liver.
It modifies drugs taken to treat disease so that they can
be used more easily by the body. And it cleanses the blood
of toxic substances either
ingested or produced by the body iself. But that's
only part of the picture. Below are some of the liver's
many other important functions:
-Regulates the blood's ability to clot
-Governs the transport of fat stores
-Stores extra vitamins, minerals, and sugars to prevent shortages
-Produces quick energy as needed
-Controls the production and excretion of cholesterol
-Breaks down alcohol Monitors and maintains the right level of
numerous chemicals and drugs in the blood
-Maintains and controls hormone balance
-Helps the body resist infection by producing immune factors and
cleansing bacteria from the blood
-fsStores iron
Viral hepatitis, a contagious infection of the liver, afflicts
more than 70,000 Americans each year. It is usually caused
by one of three different organisms.
Hepatitis A, formerly known as infectious
hepatitis, can be contracted by consuming contaminated water or
food, most notably shellfish. Since the virus is eliminated
in the stool, it also spreads through improper hand washing,
especially by restaurant workers or anyone else who handles food.
Although hepatitis A is seldom serious, in one percent of the
cases it can cause severe liver failure and death. It does
not cause chronic hepatitis and will not lead to cirrhosis or
other long-term liver problems.
Hepatitis B, formerly known as serum
hepatitis, is found in blood and other body fluids such as urine,
tears, semen, breast milk, and vaginal secretions. It is
usually transmitted in blood, via transfusions, or through
illicit injectable-drug use. But it also can be contracted
through a minor cut or abrasion, or during such everyday acts as
toothbrushing, kissing, or having sex. Infants can contract
the disease from the mother at birth, or from the mother's breast
milk. Dental work, ear piercing, and tattooing are other
ways people can get hepatitis B.
Type C hepatitis virus is the cause of a
disease known as "non-A, non-B hepatitis," which is
also contracted through contact with contaminated blood, or
through household or sexual contact with an infected
person. It affects approximately 170,000 Americans each
year.
The problem with hepatitis B is that five to 10 percent of those
who become infected with this disease become chronic carriers who
can spread it to others for an indefinite period of time.
At present there are more than a million of these silent carriers
in this country, and their number is growing by two to three
percent annually. Consequently, authorities recommend that
all children and anyone with a high risk of exposure be
vaccinated against this dangerous virus.
Chronic carriers usually do not develop chronic hepatitis.
If it does develop, however, cirrhosis and primary cancer of the
liver can be long-term consequences. An estimated 4,000
people in the United States die from hepatitis B-related
cirrhosis annually. Carriers are many times more likely to
get liver cancer than are non-carriers.
Treatment for acute hepatitis
consists of rest and small, nourishing meals; fluids; and
sometimes anti-nausea drugs such as trimethobenzamide (Tigan).
Chronic cases of hepatitis B and C are now being treated with
interferon, a biotech medicine derived from the human immune
system.
THE GALLBLADDER The gallbladder stores
bile, a substance the liver produces to aid digestion. The
most common disorder of the gallbladder is the formation of
gallstones. These stones often get stuck in the bile ducts
that lead from the gallbladder to the first part of the small
intestine, causing the gallbladder to become inflamed.
Gallbladder surgery, in which the entire organ is removed, is one
of the most common operations in this country. Laparoscopic
cholecystectomy, a new procedure that requires only a small
incision, has cut down stress and recovery time
dramatically. Chenodiol, a recently available drug that
dissolves gallstones, is an alternative to surgery.
However, due to limited success and troublesome side effects, it
is not widely used.
Other Diseases of the Liver Return to top In cirrhosis, liver
cells are damaged and replaced by scar tissue which, as it
accumulates, hardens the liver, diminishes blood flow, and causes
even more cells to die. The loss of liver function that
accompanies this degenerative condition results in
gastrointestinal disturbances, jaundice, enlargement of the liver
and spleen, emaciation, and accumulation of fluid in the abdomen
and other tissues. Over half of the deaths related to
cirrhosis are due to alcohol abuse, hepatitis, and other viruses.
Chemicals, poisons, too much iron or copper, and blockages of the
bile duct also may cause the disease.
Treatment of cirrhosis usually consists of eliminating the
underlying cause, if possible, to avoid further damage, and
preventing or treating complications. Care is mostly
supportive, often including a specialized diet, diuretics (water
pills), vitamins, and abstinence from alcohol. For some
patients, a liver transplant is now a feasible option.
Liver abscesses are caused by bacteria,
such as Escherichia coli (E. coli) or staphylococcus
(staph), or by Entamoeba histolytica, the parasite that causes
amebic dysentery. In either case, the offending organisms
destroy liver tissue, leaving a cavity that fills with other
infectious organisms, white blood cells, and liquefied liver
cells. Common symptoms include shoulder and abdominal pain,
fever, weight loss, chills, nausea, vomiting, anemia, and, if
there is severe liver damage, jaundice. If the offending
organism can't be determined, liver abscesses are treated with
long-term antibiotics such as aminoglycosides, cephalosporins,
clindamycin, or chloramphenicol. If E. coli is
causing the infection, treatment includes ampicillin; for
Entamoeba histolytica, chloroquine (Aralen), or metronidazole
(Flagyl) are included.
Pediatric liver diseases afflict tens of
thousands of children in this country annually, and kill hundreds
each year. More than 100 different liver diseases are found
in infants and children. Most of these disorders are
genetic. Among the more common are:
Biliary atresia, an inadequate bile
duct, often fatal but sometimes relieved by surgery; Chronic
active hepatitis, in which scar tissue forms and destroys the
liver; Wilson's disease, in which an abnormally large buildup of
copper in the liver is treated with vitamin B6 and
d-penicillamine, or, in some cases, corticosteroids such as
prednisone; Reye's syndrome, an acute, often fatal disease
secondary to flu or other infections in which fat accumulates in
the liver and the patient lapses into coma.
Other serious diseases of the liver, fortunately seen less
frequently than these discussed above, include fatty liver,
hepatic coma, and liver cancer.