Hemochromatosis is primarily an inherited (genetic) condition that allows too much iron to be absorbed and stored throughout the body. It’s one of the most common genetic disorders in Canada.
1 in 9 Canadians carries the defective gene for this condition, and 1 in 300 Canadians is affected with the condition. Men and women are equally affected, although signs appear later in women.
Most people absorb only enough iron to meet their body’s daily requirements, and the excess is excreted. In hemochromatosis, however, iron continues to be absorbed and stored in different organs and tissues long after the body’s needs are met.
The liver is the first organ to store excess iron, after which it accumulates in the heart, pituitary gland, and elsewhere in the body. Left untreated, the resulting damage to the liver, heart, and pancreas may eventually lead to death.
In hemochromatosis, the total iron content in the body can reach as high as 50 grams, compared with the normal levels of about 2.5 grams in women and 3.5 grams in men. It’s been nicknamed "bronze diabetes" because it may be accompanied by diabetes mellitus and increased skin pigmentation (darkening of the skin).
Because women lose iron in their menstrual flow, they tend to be protected from getting hemochromatosis as long as they are having menstrual cycles. Thus it affects men earlier than women, usually when men are between 40 and 60 years of age. Rare cases have occurred in children.
Hemochromatosis is usually hereditary. It takes two defective copies of the same gene, with one defective copy inherited from each parent, to cause hemochromatosis. Siblings of those affected have a 25% chance of getting it. Both parents must be carriers of the defective gene, which means that a child of a person with hemochromatosis is less likely to have it than are the person’s siblings.
However, hemochromatosis may also be caused by multiple blood transfusions and certain liver diseases, especially cirrhosis. Chronic hepatitis C infection can also result in an increased storage of iron, although a genetic cause should always be ruled out first.
The good news is that hereditary hemochromatosis is treatable. The goal of treatment is quite simple: to remove excess iron from the body and to treat any damaged organs. Usually, the best treatment is to remove the excess iron by withdrawing blood (phlebotomy) – about a half-litre once or twice a week. This process is very similar to donating blood.
Phlebotomy should be started as soon as the person is diagnosed. This treatment may be required for 1 to 2 years until the iron level in the blood is normal. After that, blood won’t have to be removed as often to maintain normal iron levels.
Because people build up iron at different rates, some may be able to go without regular maintenance phlebotomy for quite some time (perhaps 6 to 12 months). Medications that chelate (bind) the iron (e.g., deferoxamine*) and remove it from the body are only necessary in patients with heart problems caused by hemochromatosis and in those who can’t have phlebotomy treatments because they have anemia.
Problems due to hemochromatosis are treated separately. For example, if a person has a reduced sex drive and a change in secondary sexual characteristics, such as atrophy of the testicles, testosterone therapy may be prescribed. Diabetes, arthritis, liver failure, and heart failure associated with hemochromatosis are treated as necessary.
All first-degree relatives (e.g., parents, siblings, or children) of a person with hemochromatosis should be screened for the condition. It’s important to detect the disease early so that treatment can be started before organ damage occurs.
All material copyright MediResource Inc. 1996 – 2025. Terms and conditions of use. The contents herein are for informational purposes only. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Source: www.medbroadcast.com/condition/getcondition/Hemochromatosis
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