For Rare Blood Disorder, Pumping Iron Is the Cure

Tag : iron

But there's at least one disease left where bloodletting is stillthe preferred means of treatment.
Hemochromatosis is an inherited disease in which the body becomesoverloaded with iron. When that occurs, the iron builds up inorgans and tissues, causing slow damage from within that prompts amultitude of symptoms and illnesses.
"Until people get sick, they don't usually know it's there," saidGerald Koenig, director of the Iron Disorders Institute. "We thinkit's the most underdiagnosed disease in the country."
There are about one million people in the United States with agenetic predisposition for contracting hemochromatosis, Koenigsaid. "About 150,000 can expect to get sick, some very much so," hesaid.
Hereditary hemochromatosis is one of the most common genetic disorders in the United States,most often affecting Caucasians of northern European descent,although other ethnic groups are also at risk, according to theU.S. National Institutes of Health.
Iron is an important micronutrient for the human body and is foundin many foods, mainly in red meat and iron-fortified breads andcereals. In the body, iron becomes part of hemoglobin , a molecule in the blood that transports oxygen from the lungs toall body tissues.
"Most people maintain just the right amount of iron in theirbodies," Koenig said. "It varies by a gram or two between people."
But hemochromatosis causes the body to absorb too much iron fromfood, with the metal collecting over a period of years in suchorgans as the heart, liver and pancreas. Depending on how eachperson's body deals with the excess iron, the symptoms ofhemochromatosis can vary widely.
And this makes it very hard to diagnose the true disorderaccurately, said Eugene Weinberg, professor emeritus of biology andmicrobiology and immunology at the Indiana University School ofMedicine, in Indianapolis.
"It takes a while for even an astute physician to detect," Weinbergsaid. "It's interesting how different the presentation is frompatient to patient. It's such a jumble, and that's what puts aburden on the general practitioner."
Blood tests, combined with a thorough family medical history,usually are used to narrow the diagnosis down to hemochromatosis.
But, iron levels aren't usually tested during normal bloodscreening, Weinberg said. Patients must request the testing,although some experts are working to make it part of medicalcheckup screenings.
"We're tying to get an iron test put in there, so we can get atiron loading," he said.
Cirrhosis of the liver is one of the most common diseases resulting from hemochromatosis,Koenig said. "Ninety percent of the iron absorbed will go to theliver," he said, adding that the disorder can also cause enlargedliver, cancer of the liver or liver failure.
Joint pain and severe arthritis in the fingers are other very common symptoms, Weinberg said. "Ifa person comes in and can't open their fist, that's referred to as'iron fist,' " he said.
Undiagnosed and untreated, hemochromatosis increases the risk fordiseases and conditions such as diabetes , irregular heart beat or heart attack , arthritis, cirrhosis of the liver or liver cancer , depression , impotence , infertility , hypothyroidism , and some cancers, according to the NIH.
The NIH lists other common symptoms and diseases related tohemochromatosis, including: Fatigue or lack of energy. Loss of sex drive or impotence. Early menopause . Abnormal pigmentation of the skin, making it look gray or bronze. Thyroid deficiency. Damage to the adrenal glands.
Some people might not suffer any problems at all. Weinberg saidthere's a 100-year-old retired faculty member he knows who has avery high iron load but no ill effects from it.
"Here's a person who was able to tuck away the iron without itcausing destruction of the organs," Weinberg said. "And yet thereare other people who can't handle it."
The best means of treating hemochromatosis is phlebotomy, Koenigsaid — bloodletting, one of the most ancient, and mostlydiscredited, forms of medical treatment. But, in this case, thedraining of blood forces the body to process its excess iron.
"When you take blood out of the body, iron stored in body tissue isused to make new blood," Koenig said.
Some hemochromatosis sufferers can give blood as frequently astwice a week to stay healthy, very often to blood banks. "Mostpeople only are allowed to give blood once every seven weeks,"Koenig said.
Oral medications that would help the body better rid itself of ironare being tested, but, for the time being, bloodletting is the mostsimple and pain-free way to help sufferers lead normal lives, hesaid.
SOURCES: Gerald Koenig, director, the Iron Disorders Institute,Greenville, S.C.; Eugene Weinberg, Ph.D., professor emeritus ofbiology and microbiology and immunology, Indiana University Schoolof Medicine, Indianapolis; U.S. National Institutes of Health,Bethesda, Md.
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