BOB SISSON, Columbian staff writer

It's the most common genetic disorder in the United States, yet few people
even know they have it. And it can be deadly if not caught and treated soon
The disorder is hemochromatosis, also known as iron overload syndrome. It
affects as many as 1 in 200 American Caucasians, most of them of northern
European descent. Only an estimated 10 percent of people with the disorder
have been diagnosed.
Mention hemochromatosis to your friends and chances are good they' ll say
they've never heard of it.
"That's a common response," said Dr. Kris Kowdley of the Division of
Gastroenterology at the University of Washington School of Medicine and
director of the UW's new Iron Overload Clinic. "Most physicians think it's
a rare disease and most patients with it have no idea they have it."
People with hemochromatosis absorb too much iron. Normally, the digestive
tract screens out most of the iron people take in, allowing only a certain
amount to get to the blood. With hemochromatosis, something goes wrong in
that screening process and too much iron builds up in the body's organs.
Eventually that can cause heart failure, diabetes, arthritis, liver cancer,
cirrhosis of the liver, and bronze-colored skin.
Those complications, some of which are life-threatening, usually occur
between the ages of 40 and 60, although people as young as 20 have fallen
victim. By the time it's that severe, the damage has been done. Identifying
hemochromatosis as the actual cause is difficult because the complications
themselves mask it.
"It's very likely many people who die of old age have hemochromatosis and
never know it," Kowdley said. "It's also possible people with heart disease
and cirrhosis of the liver and diabetes have hemochromatosis as the
mechanism behind it and never know." Earlier symptoms, while the disease is
still treatable, are rather vague: impotence, joint pain, fatigue.
But that doesn't mean hemochromatosis can't be caught early. In fact, the
national Centers for Disease Control and Prevention recommends that
everyone 20 and older be screened for the disorder, especially considering
that 1 in 9 persons carries the recently-identified gene for hemochromatosis.
Screening involves going to a physician and having a blood test measuring
iron levels. A liver biopsy or a noninvasive genetic test might be
necessary to confirm a diagnosis.
"The key is to screen people early, before they have symptoms, and then the
majority can enjoy a normal life expectancy," Kowdley said.
Treatment is a procedure that goes back centuries: bloodletting. Generally,
a person with hemochromatosis has to have a unit -- about a pint -- of
whole blood removed once or twice a week to remove excess iron. Then, once
iron levels return to normal, a person usually needs to have blood drawn
only three or four times a year.
Patients with hemochromatosis also should reduce the amount of red meat
they eat, along with other foods high in iron, and avoid iron supplements
and vitamin C supplements, which can increase iron absorption in the body.
Alcohol also should be avoided.
The new UW Medical Center Iron Overload Clinic is designed to identify and
treat hemochromatosis and other types of iron overload. It also provides
genetic testing for the recently identified hemochromatosis gene, and
genetic counseling for patients and families. Call (206) 598-3339, or
e-mail lfoltz@u.washington.edu.
Bob Sisson covers health for The Columbian. He can be reached by calling
699-6006, or via e-mail at bob.sisson@columbian.com.