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.
