Scottish History and Culture

"The Celtic Curse"
Disease Strikes Persons of Celtic Origin

Continued from Page One
Contributed by Dan Laing , Webmaster Laing Family Website


Click for Larger Image Screening studies have shown that hereditary hemochromatosis actively occurs in one in two hundred to one in four hundred Europeans. The carrier rate is much higher however with ratios varying from one in seven to one in ten, depending on the population studied.

Blood tests that measure iron levels are used to diagnose hemochromatosis. It is most common among those above the age level of 35 after a lifetime in which iron is stored in various organs, but may also occur among teens with devastating results.

TREATMENT

Standard treatment, as medieval as it may sound, is bloodletting or bleeding (giving blood). This is necessary in order to force the body to use up the iron that it has stored in the various organs of the body. Blood is drawn once or twice a week, depending on the severity of the case, for the first year and a half. Thereafter, it is drawn every two to six months for the rest of the person's life. As the body regenerates the lost blood, it incorporates the stored iron.

Death usually occurs before age 55 and can happen as early as the teens.


Untreated,
HHC
is 100% fatal.

The hereditary hemochromatosis gene (HFE) was discovered in 1996, and causes what is known as the C282Y mutation. This mutation has been shown to be the cause of most of the hereditary hemochromatosis cases globally. Although how the basic genetic defect occurs is still under study, the gene discovery itself has led to major advances in studies of iron metabolism. The sequence of events leading up to iron overload is now well documented.

Hemochromatosis has now been recognized as one of the most common diseases in white populations, but much more study needs to be done before general population screenings are called for. The exception to this is of course Celtic derived populations due to the high incidence among this group. In the United States, one in 200 have been found to have the disease. Since any random sample in the US by definition includes members of other races, this ratio may be as high as one in 130 generally for whites.

In small towns and isolated communities originally settled by immigrants of celtic origin this ratio will approach the 2 in 29 statistical average found in purely Celtic populations. These areas include but are not limited to those settled by Scottish immigrants in the Altamaha Valley region of Georgia, the Cape Fear River Valley region of North Carolina, the Shenandoah Valley area of Virginia, and Eastern West Virginia. Also included are the areas settled by primarily Irish immigrants in Tennessee, Maryland, and New York.

In the US alone, there are an estimated 5,625,000 Irish-Americans and 8,230,000 Scottish-Americans who are potential carriers of the disease and an estimated 700,000(+) with the double mutation who will be affected by the disease. Total estimates for the white population of the US includes a potential 32 million carriers, and 1.5 million with the disease. Since both parents must carry the trait for their child to inherit the disease, HHC is difficult to genetically trace. An individual could still have HHC even if no one else in the family has ever had it.

Cartoon, Click for Larger Image In spite of the need for further studies, hemochromatosis already meets the criteria required by the World Health Organization for population screenings. Any competent medical lab or doctor's office can perform the necessary test to determine if one has the disease. To determine if one is a carrier of the gene requires genetic testing however. In the US, a genetics testing kit can be ordered from the Kimball Genetics Testing Lab which can be contacted at 1-800-320-1807. Knowing the you are a carrier and passing on this information may be the only way that you can protect your children and future generations from this slow killer.

If you are of Scottish or Irish descent, you may either have hemochromatosis or may be a carrier. For your sake and that of your children and grandchildren, don't delay, get tested today.

Back

Links:

"Are you Irish!? Celtic? New research finds high frequency of HH", American Hemochromatosis Society, Inc., http://www.americanhs.org/ah00009.html

"FAQ's about Hemochromatosis...Take This To The Doctor with You!", American Hemochromatosis Society, Inc., http://americanhs.org/faqs.htm

"Answers to your questions about hereditary hemochromatosis", College of American Pathologists, http://www.cap.org/html/public/hemo.html

"Hemochromatosis", Gastroenterology Consultants, PC, http://www.gastro.com/hemochro.htm

"Hemochromatosis", Jackson Gastroenterology, http://www.gicare.com/pated/ecdlv36.htm

Laing family Web Site http://members.spree.com/education/dlaing/Laings/Laing.html

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