Peter Hibberd, M.D., is a doctor whose advice is based on more than 28 years of hospital outpatient and inpatient experience. He is an experienced emergency medicine physician, surgeon, and consultant. Dr. Hibberd is certified by the American Board of Emergency Medicine. He is also a fellow and active member of the American Academy of Family Physicians, an active member of the American College of Emergency Physicians, and a member and fellow of the American Academy of Emergency Medicine. Dr. Hibberd has earned numerous national and international professional certifications, memberships, and awards.
Tags: hemochromatosis | iron | liver | heart | phlebotomy

What is Hemochromatosis?

Monday, 01 October 2012 11:02 AM

Question: My daughter was diagnosed with hemochromatosis. Can you explain this condition? Few people seem to have heard of it.

Dr. Hibberd's answer:
Hemochromatosis is an inherited disease that is usually detected in the 3rd, 4th or 5th decade of life, though an occasional younger case is reported. This condition is most common in those of Celtish origin in whom 1 percent are affected and 10 percent are carriers of this recessive genetic disorder, and it affects 1 in 500 of those of European origin.
It is characterized by an excess absorption of iron. Without any efficient way to rid our body of excess iron, it builds up in your body. It is one of the most common genetic diseases in the United States. Your body normally absorbs about 10 percent of the iron in the food you eat. If you have hemochromatosis, you absorb more iron than you need, and it accumulates in body tissues, especially the liver, heart, and pancreas.
This extra iron can damage your organs. Without treatment, it will cause your organs to fail, inducing cirrhosis, arthritis, tanning of skin, cardiomyopathy, and diabetes.
The most common treatment is to remove some blood, usually on a weekly basis, just like when you donate blood. This is called therapeutic phlebotomy.
Medicines may also help remove the extra iron, but are usually reserved for those with other conditions that require blood transfusion and a secondary iron overload state occurs from the blood transfusion needs.
Your doctor might suggest some changes in your diet. Simple blood tests: a Hemoglobin (Hb) and a serum "ferritin" are usually used to monitor your total body iron stores, and are used to trend and adjust phlebotomy frequency.
Treating hemochromatosis can stop the progression of liver disease in its early stages, which leads to a near normal life expectancy, and minimal cardiac complications.
However, if cirrhosis, or scarring of the liver, has developed, the person's risk of developing liver cancer markedly increases, even if iron stores are reduced to normal levels. People with hemochromatosis should not ever take iron supplements. And those who have liver damage should not consume alcoholic beverages because they may further damage the liver. Treatment cannot cure the conditions associated with established hemochromatosis, but it will help most of them improve.

© HealthDay

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Hemochromatosis causes excess iron to build up in a person's body.
Monday, 01 October 2012 11:02 AM
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