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Irish descendants at higher risk for disease

By John Hejkal
Staff writer
March 10, 2000

As St. Patrick's Day draws near, Irish blood may be a cause for pride in many people. But Irish livers may be a cause for problems.

Those of Celtic origin are at a particularly high risk for hereditary hemochromatosis, or HH, said Sandra Thomas, founder and president of the American Hemochromatosis Society in Delray Beach, Fla. She has dubbed the disease the Celtic Curse.

HH is a gene mutation that causes the body to store abnormally high levels of iron. Iron gets stored mostly in the liver, pancreas or pituitary gland in the brain. If too much iron accumulates, these organs can become damaged.

Dr. Mark Hutchins of the Nebraska Hematology-Oncology center in Lincoln said HH usually won't be detected until the disease manifests itself in noticeable damage to the body.

Thomas, whose mother died from liver cancer because of HH in 1999, said the key to making sure people didn't suffer from the disease was getting the word out to the nearly 32 million people who could be at risk.

"People say, 'How can this be so prevalent, and I've never heard about it?'" she said. "Well, that's what we want to know and what we're trying to change."

If found early, HH is easily treated, but if left undetected, liver cancer or heart failure could be the eventual result, Hutchins said.

"Iron is one of the metals in the body that we don't have a normal way of excreting," Hutchins said. "When you absorb iron into your system, it's there until you bleed."

The normal treatment for HH is regular blood-letting. The bleeding is done just like a donation to blood bank, Thomas said.

"Diet is not the way to manage hemochromatosis," she said. "Usually it's the blood-letting. That's the treatment for this disease."

The blood-letting creates a deficit of blood in the body. People with HH can draw iron out of the large stores in their bodies to make more blood.

Blood from HH patients is acceptable for donation to blood banks, Thomas said, so increasing awareness about HH could have dual benefits.

"That would be a great way to end the blood shortage in this country, if we could diagnose the patients and get them to donate the blood," she said.

Researchers believe Celtic people formed the HH mutation in their genes as long as 40,000 years ago in the area that is modern day Ireland, Thomas said.

The diet of the people may have been low in iron because of famine conditions at the time. To compensate, people developed this way to store as much iron as possible in their bodies.

Irish people are not the only ones who may inherit the disease. Those of French, Scandinavian or German descent also are at high risk.

"I know the Celtic curse sounds a little ominous," Thomas said. "But diagnosed early, this disease is curable. Diagnosed late, it's deadly."

Hutchins said people usually don't notice problems from HH until age 40 or 50, but he recommended testing between the ages of 20 and 30 for people with family histories of liver damage or heart failure.

HH rarely causes problems in young women, he said, because menstruation gives iron a ready place to go as women replace the lost blood.

Thomas said the HH mutation is easily detectable with a simple lab test.

Those who want more information should call the American Hemochromatosis Society at 1-888-655-IRON.  end of article dingbat


Irish descendants at higher risk for disease
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