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Hemochromatosis
Learn how the new prebiotic soluble fibers benefit bowel health and many GI disorders
The Liver
The liver is the largest organ in the body. It is found high in the
right upper abdomen, behind the ribs. It is a very complex organ and
has many functions. They include:
- Storing energy in the form of sugar (glucose)
- Storing vitamins, iron, and other minerals
- Making proteins, including blood clotting factors, to keep the
body healthy and help it grow
- Processing worn out red blood cells
- Making bile which is needed for food digestion
- Metabolizing or breaking down many medications and
alcohol
- Killing germs that enter the body through the intestine
The liver shoulders a heavy work load for the body, and almost
never complains. It even has a remarkable power to regenerate itself.
Still, it should not be taken for granted. The liver is subject to
illnesses that can lead to permanent damage. One example is
autoimmune hepatitis, a condition in which the body fights against
its own liver.
What is Hemochromatosis?
Hemochromatosis is commonly known as iron overload disease. A
malfunction in the way iron is absorbed from the intestinal tract
causes the body to absorb and store too much iron. Certain vitamins
and minerals are normally stored in the liver, and iron is one of
them. However, too much iron stored in the liver can damage it. With
hemochromatosis, excess iron is also deposited in other organs and
tissues, especially the heart and pancreas, causing damage to these
organs, too. This disease occurs more commonly than was originally
thought. It is estimated that hemochromatosis affects about one in
every 300-400 people. Occasionally, a secondary type of iron overload
(called hemosiderosis) occurs in people who take large amounts of
iron supplements, or in people who must have repeated blood
transfusions, or who have certain other diseases. However, the body
usually returns to normal processing of iron when the excess intake
is eliminated.
Cause
Exactly what makes the body absorb and store too much iron is not
completely understood. It is known that hemochromatosis is inherited.
The gene for the disease is a very common one; about one in every 10
people carry it. However, a person can carry one gene and not get the
disease. It is only when a person gets a hemochromatosis gene from
each parent -- a double dose -- that the disease develops. Therefore,
people who have parents, brothers, or sisters with this disease
should be closely monitored by a physician.
Symptoms
The disease can develop at any age, but symptoms usually do not occur
until middle age (between the ages of 40 and 60). Women often develop
the symptoms at a later age than men, probably because women lose
significant amounts of iron through menstruation and pregnancy. The
damage to the liver and other organs comes from long-term storage of
excessive iron.
When the liver is badly damaged, scarring or hardening develops.
This is called cirrhosis. Damage to the liver may also cause jaundice
(yellow color to skin and eyes). Excess iron deposited in the
pancreas causes it to stop making insulin, and diabetes mellitus
develops. Hemochromatosis can also cause enlargement of and damage to
the heart. Too much iron can be harmful to the pituitary gland (in
the brain), and this may result in a number of hormone problems.
Another common problem is the development of arthritis.
Hemochromatosis can be a perplexing disease, because there are so
many symptoms that can vary from patient to patient.
Diagnosis
Early diagnosis is important, because treatment can prevent long-term
injury and damage to body organs. When there is a family history of
hemochromatosis, and especially if there are symptoms present, the
physician will order certain blood tests. One of the tests is a
measure of iron in the blood. Another is for blood ferritin, a
protein that carries iron. With hemochromatosis, these tests will be
abnormally high. When the blood tests are repeated and the results
are consistently high, the physician usually diagnoses
hemochromatosis. A liver biopsy is then performed to determine how
much iron is stored in the liver and if there has been any liver
damage. Liver biopsy is done under local anesthesia. A slender needle
is inserted through the right lower chest to extract a small piece of
liver for microscopic analysis.
Treatment
Red blood cells are rich in iron, so their removal will reduce the
amount of iron in the body. Treatment begins with one to two pints of
blood being removed each week or so, until stored iron is reduced to
normal levels. It may take several months or longer to accomplish
this. After iron stores are reduced to normal, the patient should
continue to have blood drawn periodically.
Complications of hemochromatosis, such as arthritis, hormone
deficiency, heart failure, and diabetes, can be treated successfully
in most cases. If these complications have already occurred in the
patient, regular treatment with blood withdrawal should still be
done. At this stage, it can prevent further harm and may even improve
some of the damage.
How Can the Patient Help?
Alcohol seems to make the problem worse, so people with
hemochromatosis should avoid alcohol or have only an occasional
drink. A regular balanced diet is recommended, but patients should
avoid large quantities of iron rich foods, such as red meats
(especially liver) and iron fortified food products. Iron supplements
should never be taken. Even small amounts of iron present in some
multivitamin preparations can be harmful.
Liver Transplantation
Liver transplantation is now an accepted form of treatment for
chronic, severe liver damage. Advances in surgical techniques and the
use of new drugs to suppress rejection have improved the success rate
of transplantation. The outcome is excellent for hemochromatosis
patients, and with the slow progression of this disease, it is
possible to plan elective transplant surgery. Survival rates at
transplant centers are well over 90 percent with a good quality of
life after recovery.
Summary
Hemochromatosis is a common inherited disease that causes the body to
absorb and store too much iron. People with a family history of the
disease should be tested early, because symptoms which can signal
organ damage usually do not occur until late in the disease.
Hemochromatosis can be controlled, and complications can be avoided
or treated. Those patients who are diagnosed early can look forward
to a completely normal and active life.
Learn how the new prebiotic soluble fibers benefit bowel health and many GI disorders
Related Diseases
Cirrhosis
Related Procedures
Liver
Biopsy | Liver
Transplant
This material does not cover all information and is
not intended as a subsitute for professional care. Please consult
with your physician on any matters regarding your health.
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Chek Med Systems®, Inc., All Rights Reserved.
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