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Started by Shawn Bishop (@Shawngbishop) · Jan 21, 2011

With Prompt Treatment, Hemochromatosis Can Usually Be Managed Effectively

With Prompt Treatment, Hemochromatosis Can Usually Be Managed Effectively

January 21, 2011

Dear Mayo Clinic:

I was diagnosed with hereditary hemochromatosis a year ago. I have had regular blood draws to remove iron from my body. My iron levels are stable for the moment. Will I eventually need a liver transplant?

Answer:

Hemochromatosis is a term used to describe diseases that cause iron overload in the body. If left untreated, hemochromatosis can, over a matter of years, cause iron to accumulate in organs such as the liver, heart and pancreas, triggering serious health problems like cirrhosis, liver cancer, heart disease and diabetes. With prompt treatment and regular monitoring, however, hemochromatosis that has not progressed to significant organ involvement can usually be managed effectively.

Hemochromatosis often results from a hereditary disorder. Hereditary hemochromatosis is fairly common, occurring in about 1 out of every 250 people of Northern European ancestry. Hereditary hemochromatosis causes the intestine to absorb more iron from food than is needed for normal bodily functions. Other causes of iron overload include blood diseases that make the body unable to incorporate iron into red blood cells.

Most people with hereditary hemochromatosis are diagnosed based on elevated levels of iron detected by blood tests. These tests are usually performed to investigate symptoms or to screen for the disease because a first-degree relative — a parent, sibling or child — has hemochromatosis. Many people with elevated iron levels do not have hemochromatosis. Instead, they may have a different liver disease or an inflammatory condition.

The majority of people with hereditary hemochromatosis begin experiencing symptoms in their 40s, 50s or 60s. Men tend to show symptoms earlier than women because women lose iron during menstruation and pregnancy. The most common and earliest symptom of hemochromatosis is fatigue. Some people with hemochromatosis may have arthritis affecting their fingers, wrists, ankles and knees. Others may have diabetes accompanied by a discoloration of the skin. Hemochromatosis may cause impotence in men. Patients with serious heart involvement may have symptoms of cardiac failure, including shortness of breath and fluid retention.

The outlook for people with hemochromatosis is generally good, unless diagnosis occurs after iron overload has begun to significantly affect organs. Those diagnosed before symptoms appear generally have a normal life span with successful treatment. But those who have advanced fibrosis of the liver can develop liver cancer even if iron levels are reduced. Involvement of the pancreas and joints is considered irreversible even with treatment of the iron overload disorder. Some heart problems due to iron overload can be corrected with treatment.

As you describe, treatment for hereditary hemochromatosis is removal of blood (phlebotomy). Phlebotomy involves periodically removing a unit of blood from a vein. The goal in using phlebotomy for hemochromatosis is to reduce iron levels to normal. When first diagnosed with iron overload, most people require removal of one unit (about 1 pint) of blood every one to two weeks. This may be necessary for up to two years. After that, phlebotomies are typically required much less often, perhaps every three to six months.

If there is no significant organ involvement, phlebotomies are often the only treatment needed to effectively manage hemochromatosis. However, advanced involvement of the liver or serious cardiac involvement could carry an increased risk of death even if iron is removed with phlebotomy.

It's unusual for hemochromatosis to require liver transplantation. A transplant may be considered for patients with advanced liver disease that's causing symptoms or in those who have liver cancer that cannot be treated by other means. But, in general, liver transplantation is not necessary for most people with hereditary hemochromatosis.

— John Poterucha, M.D., Gastroenterology, Mayo Clinic, Rochester, Minn.

Hemochromatosis

 

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