If you're reading this blog, chances are you or someone you know has already been diagnosed with an iron overload disease, such as hemochromatosis, sickle cell anemia, African siderosis, beta-thalassemia, porphyria, or juvenile hemochromatosis. The diagnosis of iron overload disease, however, is not based solely on the amount of iron in the blood. Let's take a look at how testing for iron overload works.
The first number to look at in determining whether someone has an iron overload is, as you might expect, serum iron. The term "serum" refers to the blood. Most of the body's stores of iron do not circulate in a form measured by this test. About 65 per cent of the body's total iron supply is used to make hemoglobin, the oxygen transport molecule found in red blood cells. About 30 per cent of the body's iron is stored in the liver, spleen, and bone marrow, for use in future manufacture of red blood cells. Another 4 per cent is found in the myoglobin, the oxygen "grabbing" molecule that allows muscles to operate with oxygen even when bloodstream oxygen levels are low.
This leaves only 1 per cent of the body's iron in a form that lab tests usually measure. This 1 per cent is bound to a transporter molecule called transferrin. The serum iron test measures the amount of iron bound to transferrin in the bloodstream. The concentrations of iron measured by the test are extremely low. Healthy levels of serum iron range from 60 to 170 millionths of a gram (micrograms) per deciliter of blood.
Hemochromatosis, beta-thalassemia, and other iron overload conditions, however, are not just about how much iron is in the blood. One reason is that the amount of iron in the bloodstream is variable. It's greater after eating foods that contain iron. That is the reason this test requires fasting. For women, it's lower after menstrual bleeding. Iron levels are increased by use of the contraceptive Pill, estrogen, or L-dopa. Iron levels are decreases by use of testosterone, drugs for gout, and the antibiotic methicillin.
And the major concern in iron overload is not just how much iron there is in the blood, but whether there is any excess capacity for transporting it where it needs to go. That is why doctors usually also order tests for serum ferritin and total iron binding capacity.
Ferritin is the "ferry" that transports iron in the bloodstream. Each molecule of ferritin can transport up to 4,500 iron atoms. Ferritin levels go up when there's cancer or infection, and also in iron overload disease. Total iron binding capacity (TIBC) measures the percentage of ferritin that is carrying iron. In healthy people, TIBC numbers range from 25 to 35 per cent. In iron overload disease, the TIBC numbers range from 45 per cent and up. Treatment to relieve iron overload, to ensure the bloodstream can still handle all the iron in circulation.
A liver biopsy used to be part of the diagnosis of iron overload disease. At least in the case of hemochromatosis, it not longer is. Taking out a minute sample of liver tissue to examine for excessive iron stores is an excellent way to find out if iron overload disease has already caused liver damage such as cirrhosis or liver cancer. It is not necessary for determining if the iron overload is caused by hemochromatosis.
Most people who have hemochromatosis carry two copies of a gene called C282Y. Some people who have hemochromatosis have one C282Y gene with another gene called H63D, and they usually have a much milder form of the disease. If you have these genes, and you have a TIBC over 45 per cent, you are assumed to have hemochromatosis.
Generally speaking, people with the genes for hemochromatosis who are under the age of 40 and who have ferritin levels under 1000 nanograms (billionths of a gram) per deciliter of blood are at very little risk for having already developed cirrhosis of the liver. People over the age of 40 who have the genes for hemochromatosis and ferritin levels over 1000 ng/dl are considered at high risk for developing cirrhosis of the liver and other symptoms, and are offered phlebotomy (blood withdrawal) or an iron chelating drug such as ExJade (deferasirox).
In future weeks, I'll have much more detailed information about the ways doctors diagnose other the other iron overload diseases. Remember, this blog is not itself a tool of diagnosis. Always discuss lab results with your doctor.
Monday, August 17, 2009
Iron Overload: The Disease That's Like the Guest Who Never Leaves
Why should anybody care about iron overload diseases? What is it about iron that makes it a special concern in human health? Shouldn't we all be taking Geritol?
Many of us who are of the age that we need to take serious care of iron overload disease are old enough to remember the slick television advertising campaigns for the alcohol-and-iron tonic Geritol. In the 1950's, you could open up the newspaper and see an ad featuring a woman sitting in a chair leaning against an ironing board. The caption read "After flu, cold, and sore throat, if you feel run down because of tired blood, take Geritol." In the next frame you could see the same woman with a beaming smile, holding her iron up in the air, with the caption, "Feel stronger fast in excellent condition!"
In the 1970's, the wildly popular Lawrence Welk Show featured a commercial in which a husband admires his Geritol-swigging wife's domestic prowess and then entones, with puppy dog eyes, "My wife--I think I'll keep her." Geritol became a staple for every aging sitcom character from Archie Bunker to Fred Sanford. Hundreds of millions of people heard the iron supplement slogan, "When you've got your health, you've got just about everything."
Unfortunately for 2.5 million or more of us who are becoming the twenty-first century's Geritol Generation, iron has not helped us keep our health. For those of us who have various conditions that make our bodies absorb or retain unusual amounts of iron, the decades of iron excess are leading to literal rust. And like the houseguest who never realizes it's time to get packing, iron builds up in our parenchymal organs, the brain, colon, liver, lungs, pancreas, and skin in particular, causing an astonishing array of health problems.
There are dozens of diseases that cause excessive accumulation of iron. The most common of these in the USA, although it's often not diagnosed, is hereditary hemochromatosis. In this genetic disease, the colon is unusually permeable to iron.
Iron overload is often a complication of sickle cell disease, occurring at sickle cells are destroyed and release their iron. It's a complication of another blood disorder, beta-thalassemia. Iron overload results from African siderosis, alcohol abuse, and viral hepatitis. It is among the sequellae of aceruloplasminemia, atransferrinemia, dysmetabolic iron overload syndrome, fatty liver, juvenile hemochromatosis, neonatal hemochromatosis, and enzyme diseases. It can be caused by inhalation (working with asbestos products, grinding steel, mining iron, and inhaling tobacco smoke), ingestion (eating iron-fortified foods, eating excessive amounts of red meat, alcohol used in excess, and iron supplements), injection (walking over rusty metal, multiple blood transfusions), and decompartmentalization (destruction of red blood cells or destruction of liver tissue).
Iron overload can overwhelm the endocrine glands, the joints, the heart, and the liver. It can cause arthritis, diabetes, and bronzing of the skin. It can cause erectile dysfunction, infertility, shrinking of the external sex organs, hypothyroidism, and early menopause. It can lead to cancer, heart attacks, and fatal infections.
Some people with iron overload disease are never diagnosed. Some people, like my father, die of fatal infections just a few weeks after diagnosis in their ninth decade of life. Symptoms may appear at age 25 or age 55. The course of iron overload diseases differs from person to person. All iron overload diseases, however, require medical treatment.
That does not mean there is nothing you can do. Diet makes a difference. A number of nutritional supplements augment what your doctor can do to help you manage the symptoms of iron overload disease. Two nutritional supplements even seem to act as chelators, although they should be used with caution.
The critical first step, however, is getting an accurate diagnosis. The US Centers for Disease Control found that in a group of nearly 3000 people who had hereditary hemochromatosis, over 67 per cent had been given multiple misdiagnoses before being properly diagnosed as suffering iron overload disease. They saw an average of three doctors and waited an average of 9 years before getting the right diagnosis. So, in my next post, let's take a look at testing to know whether you have iron overload disease.
Many of us who are of the age that we need to take serious care of iron overload disease are old enough to remember the slick television advertising campaigns for the alcohol-and-iron tonic Geritol. In the 1950's, you could open up the newspaper and see an ad featuring a woman sitting in a chair leaning against an ironing board. The caption read "After flu, cold, and sore throat, if you feel run down because of tired blood, take Geritol." In the next frame you could see the same woman with a beaming smile, holding her iron up in the air, with the caption, "Feel stronger fast in excellent condition!"
In the 1970's, the wildly popular Lawrence Welk Show featured a commercial in which a husband admires his Geritol-swigging wife's domestic prowess and then entones, with puppy dog eyes, "My wife--I think I'll keep her." Geritol became a staple for every aging sitcom character from Archie Bunker to Fred Sanford. Hundreds of millions of people heard the iron supplement slogan, "When you've got your health, you've got just about everything."
Unfortunately for 2.5 million or more of us who are becoming the twenty-first century's Geritol Generation, iron has not helped us keep our health. For those of us who have various conditions that make our bodies absorb or retain unusual amounts of iron, the decades of iron excess are leading to literal rust. And like the houseguest who never realizes it's time to get packing, iron builds up in our parenchymal organs, the brain, colon, liver, lungs, pancreas, and skin in particular, causing an astonishing array of health problems.
There are dozens of diseases that cause excessive accumulation of iron. The most common of these in the USA, although it's often not diagnosed, is hereditary hemochromatosis. In this genetic disease, the colon is unusually permeable to iron.
Iron overload is often a complication of sickle cell disease, occurring at sickle cells are destroyed and release their iron. It's a complication of another blood disorder, beta-thalassemia. Iron overload results from African siderosis, alcohol abuse, and viral hepatitis. It is among the sequellae of aceruloplasminemia, atransferrinemia, dysmetabolic iron overload syndrome, fatty liver, juvenile hemochromatosis, neonatal hemochromatosis, and enzyme diseases. It can be caused by inhalation (working with asbestos products, grinding steel, mining iron, and inhaling tobacco smoke), ingestion (eating iron-fortified foods, eating excessive amounts of red meat, alcohol used in excess, and iron supplements), injection (walking over rusty metal, multiple blood transfusions), and decompartmentalization (destruction of red blood cells or destruction of liver tissue).
Iron overload can overwhelm the endocrine glands, the joints, the heart, and the liver. It can cause arthritis, diabetes, and bronzing of the skin. It can cause erectile dysfunction, infertility, shrinking of the external sex organs, hypothyroidism, and early menopause. It can lead to cancer, heart attacks, and fatal infections.
Some people with iron overload disease are never diagnosed. Some people, like my father, die of fatal infections just a few weeks after diagnosis in their ninth decade of life. Symptoms may appear at age 25 or age 55. The course of iron overload diseases differs from person to person. All iron overload diseases, however, require medical treatment.
That does not mean there is nothing you can do. Diet makes a difference. A number of nutritional supplements augment what your doctor can do to help you manage the symptoms of iron overload disease. Two nutritional supplements even seem to act as chelators, although they should be used with caution.
The critical first step, however, is getting an accurate diagnosis. The US Centers for Disease Control found that in a group of nearly 3000 people who had hereditary hemochromatosis, over 67 per cent had been given multiple misdiagnoses before being properly diagnosed as suffering iron overload disease. They saw an average of three doctors and waited an average of 9 years before getting the right diagnosis. So, in my next post, let's take a look at testing to know whether you have iron overload disease.
More About Me
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Both my mother and my father came from families that suffered the tragic consequences of hemochromatosis. Seven members of my family died of liver cancer. My father was misdiagnosed with Parkinson's disease, a condition that is improved by increasing the brain's supply of L-dopa. For over 20 years a perceptive doctor gave him medications that conserved L-dopa without adding to it. That doctor went into research. Only a few weeks before Dad died, a hurried doctor he had never seen before looked at the chart and "corrected" the omission of L-dopa. Less than three months later, Dad was dead. It turned out he had had Lewy body disease all along. This is a condition related to iron overload that is made worse by L-dopa.
I was tested and found to have the genes for hemochromatosis back in 1999. I did not get serious about doing something about my own iron overload, however, until I started having "clots that weren't clots" in major arteries. Stress tests and various scans have revealed that my arteries are as clean as the proverbial whistle. Despite having no detectable atherosclerosis, I started having the symptoms of a heart attack--without the blood clot--and extensive damage to my colon--withour the clot in the inferior mesenteric artery. Only when my own iron levels got to the point of toxic sludge did I realize that, yes, I really did need treatment.
It's not like I shouldn't have known better. I'm the author or co-author of 11 books natural medicine. I've formulated natural products, written television scripts, helped companies deal with the FDA and FCC and FTC, and worked for law firms that specialize in truth in labeling issues. I managed to earn a reputation as a "conservative" commentator on natural health because I am a stickler for facts. That doesn't mean I was eager to get my blood drawn 3 times a week.
Fortunately, there are more treatments available today than ever before. Back in the day when doctors did not know to look for iron overload and the diagnosistic distinctions it required, many people suffered needlessly. Many people died of preventable disease. The Iron Disorders Institute and the Iron Overload Diseases Association have been busy getting excellent information to physicians. Doctors now know what to do. But there are still, according to one estimate, at least 2.5 million people just in the United States who have iron overload diseases and aren't getting treatment.
This blog is about what I have learned about living well despite iron overload, and about my journey to normal iron levels. Some posts will deal with tehcnical issues. Some posts will deal with personal issues. Some posts are about what I've already learned the hard way and some are about what I am still learning. But every post is about turning back the clock on this still-poorly-publicized disease.
Thank you for visiting my blog. Perhaps you are an unintentional expert in living well with iron overload disease like me. Perhaps you are a physician or a researcher, or a concerned family member or friend. Please feel free to share your experiences, comments, corrections, and questions. And please come back often to this site.
All content © Robert Rister 1998-2009.
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