NOTE:
Dayton, Ohio is my birthplace. I was
pleased that I was able to arrange
for an article on iron overload/hemochromatosis
to be written by Valryn Bush who lives
in Dayton. She has written an excellent
article and it will surely save some
lives!
Sandra Thomas, President, American Hemochromatosis Society (AHS)
Iron
Overload
by
Valryn Bush
It's a medical paradox, involving one of the most common genetic
disorders you never heard of and death by a mineral necessary
for life. It's hereditary hemochromatosis, (HH), a metabolic
disorder that causes the body to absorb too much iron from
a normal diet.
Over
time, the excess iron lodges in
vital organs, especially the liver,
where it promotes other diseases
and eventually death. Once considered
a rare disease, it's now considered
the most common abnormal genetic
defect in North America. The list
of symptoms is long, but some of
the most common are cirrhosis,
diabetes (sometimes with a bronzed
skin pigmentation), heart disease,
chronic fatigue, arthritis, impotence,
and infertility.
The
treatment is simple-blood is drawn
to deplete the body's iron stores,
usually at least once a week. Detected
and treated while the person is
still healthy, there are no adverse
effects. When found later, some
conditions improve but others are
irreversible. "The key is
to remove the iron as quickly as
possible." said local gastroenterologist
Rajeev Mehta.
Recent
studies have turned up roughly
the same rate of incidence, about
1-200 with the double gene and
the potential for iron overload,
and 1-10 as carriers. In the Dayton
metro area, there should be almost
5,000 people with the disease,
but they, and their doctors, probably
don't know who they are.
"There's
an old tradition among doctors,
that it always causes bronze diabetes,
that it's rare and only affects
Caucasian males." said Dr.
Virgil Fairbanks, from the department
of pathology at Mayo Clinic. "What's
important to understand is that
it's really quite common, the commonest
single gene hereditary disorder. "
Tom
Bidlack, who lives near Defiance,
Ohio, has HH. He says he feels
isolated. "It just boggles
my mind." He said. "If
this is true then why don't I know
anybody around who has this? Why
am I all alone?"
Sandra
Thomas, a native Daytonian who
now lives in Florida, works as
the national public education director
for the Iron Overload Diseases
Association, a non-profit organization.
Her mother, Josephine, has HH,
and the frustration of getting
to the correct diagnosis made her
determined to raise awareness of
this condition. "If people
were diagnosed with it, thought
their kids might inherit it, they'd
be beating down the doors to get
every bit of information they could
on it." Thomas said. "But
because people aren't being diagnosed,
because no one is being screened,
there is apathy and lack of interest."
The
proper tests for iron overload
are called serum iron and total
iron binding capacity, used together
to measure saturation. Mercator
Genetics Inc. discovered the gene
for HH, last summer, and a genetic
test became commercially available
this month from SmithKline Beecham
Clinical Laboratories. The Southern
California Division of Kasier Permanente,
which insures over 7 million people,
did a screening of 30,000 people
in conjunction with the Center
for Disease Control in Atlanta.
"From
a practical standpoint, if one
is going to diagnose, it needs
to be done by screening." Said
Dr. Vincent Felliti, who heads
the department of preventative
medicine at Kaiser. "If you
do it by the symptoms, you find
these people too late. If someone
in your family has it, it would
be crazy not to be screened. All
the manifestations of this disease
are totally preventable."
Those
who work to bring attention to
HH are hoping that the CDC will
soon make an announcement recommending
routine screening for people over
18. The CDC and the National Institutes
for Health are holding a joint
meeting on iron overload March
3-5, in Atlanta.
"If
you look, you will find it." Said
Dr. Sharon McDonnell, a master
of public health in the CDC's division
of nutrition and physical activity. "The
more we looked into it, the more
compelled we were. Right now we
have a genetic disorder and physicians
aren't really ready. My own training
was that it was rare and only affected
middle-aged white males."
The
CDC announcement, originally planned
for spring and now expected in
June, might make a difference.
Dr. James Barton, of the Southern
Iron Disorders Center in Birmingham,
Alabama, says he isn't so sure. "I
sit on that advisory committee-and
it will be a long time before any
recommendations are made." he
said. "It's going to take
government cooperation and they
don't have any sense of expediency.
I think it's going to be years
away."
Besides
HH, there is an African-American
variety caused by a different and
as yet undiscovered gene. The symptoms
are similar but the iron test results
are typically a little different
and secondary factors, such as
anemia, alcohol consumption, and
viral hepatitis, seem to be involved.
Dr. Barton, who specializes in
this type of iron overload, says
they need more studies but it looks
like it might affect 1-2% of the
African-American population. "Most
people in either category remain
undiagnosed." he said.
Iron
overload more rarely can be acquired,
in cases of repeated transfusions
or massive doses of iron supplements.
Dr. Rajeev Mehta, a local gastroenterologist,
said he once treated a patient
who made himself sick with large
amounts of iron loaded supplements
and preparations. He feels that
iron supplements are dangerous
unless prescribed by a physician
who has first determined the cause
of the anemia. "I'm surprised
the FDA hasn't taken them off the
market." he said. "Remember-an
iron heart is a weak heart."
(c)
1997 Dayton Daily News
----------------------------------------------------------
This
story also appeared in the May
28th, 1997 issue of the Portland
Oregonian.
