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Hemochromatosis

 
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Definition of Hemochromatosis

Hemochromatosis is a metabolic disorder that results in excess deposits of iron in the liver, pancreas, and other iron-storing organs.

Description of Hemochromatosis

Iron overload that does not cause tissue damage is hemosiderosis; that which does is called hemochromatosis.

Hemosiderosis can occur in hemolytic anemia, or when iron has been taken for an extended time when it is not necessary.

Unfortunately, some people have a tendency to absorb a relatively large amount of iron even if they do not need it. And since there is no way to get rid of it other than bleeding (via menstruation, injury or blood donations) their internal organs become iron overloaded and thereby cause complications such as liver damage.

The condition is estimated to afflict more than a million Americans, or about 1 in every 250 people, far fewer than suffer from an iron deficiency but still more than all those afflicted with cystic fibrosis, Huntington's disease, and muscular dystrophy combined.

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Causes and Risk Factors of Hemochromatosis

Hemochromatosis is uncommon and rarely occurs before middle age. One form is believed to be congenital. It can also result from blood transfusions and over consumption of dietary iron, especially among people with a genetic predisposition to accumulate iron.

Symptoms of Hemochromatosis

Hemochromatosis can cause cirrhosis of the liver, jaundice, diabetes, enlarged heart, congestive heart failure and irregular heartbeats, or arrhythmias. Loss of libido and testicular atrophy result from pituitary failure. Other, less common symptoms include abdominal pain, and arthritis.

Diagnosis of Hemochromatosis

One survey discovered that one in three people with iron overload had met with more than 11 doctors before receiving a proper diagnosis. The problem is that many of the initial symptoms (weakness, fatigue, achy joints, decreased sexual desire) can be attributed to other conditions.

That hemochromatosis frequently escapes early detection is very troubling, particularly in light of the simplicity of its treatment, which is periodic phlebotomy or blood-letting to get rid of excess iron stores.

Tragically, there are those who are not diagnosed correctly until somewhere is mid-life, when iron stores are five to 50 times the norm and the illness has ravaged their bodies.

Definitive diagnosis is made by blood tests. The serum ferritin test and the transferring saturation test provide a definitive answer. The full series of blood tests that check for iron overload can cost more than $100, but is worth considering for anyone who has a family history of hemochromatosis or who suffers from what might be the early symptoms of the disease.

Treatment of Hemochromatosis

Treatment involves the removal of 500 milliliters (about 15 ounces) of blood weekly until normal plasma iron levels are established. After that, blood is removed every 3 or 4 months to maintain normal iron levels.

Occasionally, anemia results from removal of the blood. When this occurs, the chelating agent deferoxamine is given to prevent anemia. Patients should adhere to a low-iron diet, avoiding foods such as liver, red meats, or iron-enriched breads and cereals.

Questions To Ask Your Doctor About Hemochromatosis

Is it hemochromatosis?

Has this been confirmed by blood tests?

What has caused this condition?

Are there any complications such as liver cirrhosis?

What treatment is recommended?

Is a chelating agent necessary?

Will treatment have to be continued permanently?

Is contracting anemia likely?

What changes in diet are called for?

What foods should be avoided?






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