Primary hemochromatosis is a genetic disorder passed down through families. It occurs at birth. People with this condition absorb too much iron through their digestive tract. Iron builds up in the body, especially the liver. You are more likely to get this disease if someone else in your family has or had the condition.
Secondary (acquired) hemochromatosis is due to other blood-related disorders (such as thalassemia or certain anemias) or many blood transfusions. Sometimes it occurs in people with long-term alcoholism and other health conditions.
Hemochromatosis affects more men than women. It is particularly common in Caucasians of western European descent.
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Hepatomegaly is enlargement of the liver beyond its normal size. Certain conditions such as infection, parasites, tumors, anemias, toxic states, storage diseases, heart failure, congenital heart disease, and metabolic disturbances may all cause an enlarged liver.
The goal of treatment is to remove excess iron from the body and treat any organ damage.
A procedure called phlebotomy is the best method for removing excess iron from the body.
One-half liter of blood is removed from the body each week until the body iron level is normal. This may take many months or even years to do.
After that, the procedure may be done less often to maintain normal iron levels.
How often you need this procedure depends on your symptoms and your levels of hemoglobin and serum ferritin, and how much iron you take in your diet.
Testosterone hormone therapy can help improve the loss of sexual desire and changes in secondary sexual characteristics. Diabetes, arthritis, liver failure, and heart failure should be treated as appropriate.
If you are diagnosed with hemochromatosis, you should follow a special diet to reduce how much iron is absorbed from your digestive tract. Your doctor or nurse will recommend:
Do not drink alcohol, especially if you have liver damage
Do not take iron pills or vitamins containing iron
Do not use iron cookware
Do not eat raw seafood (cooked is fine)
Do not eat foods fortified with iron, such as 100% iron breakfast cereals
Untreated, iron overload can lead to liver damage.
Extra iron may also build up in other areas of the body, including the thyroid gland, testicles, pancreas, pituitary gland, heart, or joints. Early treatment can help prevent complications such as liver disease, heart disease, arthritis or diabetes.
How well you do depends on the amount of organ damage. Some organ damage can be reversed when hemochromatosis is detected early and treated aggressively with phlebotomy.
Screening family members of a person diagnosed with hemochromatosis may detect the disease early so that treatment can be started before organ damage has occurred in other affected relatives.
