Medical Breakthroughs/First to Know
Reported November 2001
Web Article: Hereditary Hemochromatosis
By MICHELLE VAUGHAN, Ivanhoe Health Correspondent
LAKE MARY, Fla. (Ivanhoe Newswire) — Sandra Thomas says they could be the heroes
of society. "They" are patients with hemochromatosis, and Thomas should know.
She is the president and founder of the American
Hemochromatosis Society, and she dedicated her life to preventing and treating
the disease after her mother died of complications from hemochromatosis.
Although the U.S. Centers for Disease Control and Prevention in Atlanta have
labeled hereditary hemochromatosis the most common life threatening genetic
disease in the United States, few patients know they have the disease and even
fewer understand their treatment options.
Hereditary hemochromatosis, or iron overload disease, causes too much iron to
accumulate in the body, eventually poisoning vital organs. Although the process
could take many years, patients who are never tested for the disease
do not become aware of it until further complications, including cirrhosis of
the liver, heart disease and cancer, have already occurred. In fact, common
conditions associated with hemochromatosis, like chronic fatigue
syndrome and diabetes, often mask symptoms of the disease, making it more
difficult for doctors to diagnose.
According to New York hematologist Victor Herbert, M.D., "Nearly every American
with iron overload doesn't know it." And the numbers are large. An
estimated 12 percent of Americans, most of Northern European ancestry, have
hemochromatosis. Commonly known as the "Celtic curse," the disease strikes those
of Celtic, Irish, British, Scottish, Nordic or German ancestry.
"Every single man, woman and child should have the genetic test for hereditary
hemochromatosis to detect it as early as possible," says Thomas. "Those at risk
must be monitored."
Treatment for hemochromatosis is therapeutic phlebotomy, or bloodletting, the
same procedure used in blood donation. Patients initially diagnosed with
hemochromatosis need to have one unit of blood removed as often as once or twice
a week until serum iron levels drop to an acceptable level, and then
periodically throughout the rest of a patient's life. The never-ending need for
blood donations, combined with a large population of hemochromatosis patients
who need phlebotomy, seems on the surface to be a perfect combination.
However, says Thomas, the equation is not that simple. Only 15 blood donation
locations nationwide accept blood from hemochromatosis patients, forcing most
patients to pay for phlebotomy either in a hospital or doctor's
office setting, or at a blood bank that then discards the blood.
The Food and Drug Administration, in 1999, officially sanctioned hemochromatosis
blood as safe for collection purposes, but stopped short of mandating that
sources accept the blood free of charge as a donation. The FDA instead
instituted a variance system that forces blood banks and other medical
institutions to apply for the right to accept hemochromatosis blood and not
charge patients for the donation.
The reason only a small number of medical institutions have applied and received
the variance is vague. Thomas blames it on ignorance, or a fear that those with
hemochromatosis may also carry other blood-borne illnesses.
"In the past, hemochromatosis blood was considered not appropriate for the blood
supply. Even though there is nothing wrong with blood, and even though
hemochromatosis can not be transmitted, people worry that there might be other
conditions," says Thomas.
Dr. Herbert agrees that ignorance may be a factor, but also cites financial
considerations.
"If blood donation centers treat blood from hemochromatosis patients as
therapeutic phlebotomy, charge the donor and throw the blood away, it is all
profit," says Dr. Herbert. "The expense is in processing, testing, typing
and cross-matching the blood. Charging the donor and discarding the blood is
pure profit."
In fact, Herbert notes, blood banks that receive the variance must accept blood
from hemochromatosis patients free of charge, even if the patient is found to
carry another disease that disqualifies them from donating blood.
Both agree that while there may be a chance that some hemochromatosis patients
may have other blood-borne illnesses, it is no higher than are other patients
and their blood is tested just like regular donors. Moreover,
since hemochromatosis patients are under the care of a physician, they may
actually be better aware of their health. And, Dr. Herbert notes that blood
containing too much iron may actually be better to use in a transfusion.
"This is a win-win situation. The donor needs to get rid of excess iron and 85
percent of transfusion recipients need excess iron anyway," says Dr. Herbert.
"High iron blood is better than average blood for the average
recipient."
Dr. Herbert also notes that the blood shortage could be significantly reduced by
accepting hemochromatosis blood. In fact, Herbert calls these patients "walking
blood banks."
And, this is why Thomas insists that hemochromatosis patients may be the "heroes
of our society."
"They are highly motivated to donate blood because it saves their lives," said
Thomas. And it might save others as well.
If you would like more information, please contact:
American Hemochromatosis Society
4044 W. Lake Mary Blvd., Unit #104
Lake Mary, FL 32746-2012
(407) 829-4488
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