We have yet another acronym for the disease which David S. Bell called The Disease of A Thousand Names (1991) in one of the first books published on this illness. Bell is a well known medical doctor in Lyndonville NY, the site of one of the first CFS epidemics in the 1980's.
Since Bell's publication, little has been done to define the etiology (cause), pathophysiology (what's not working and why), and worthwhile treatment protocols to manage or cure this disease. About the only thing that has happened is that the US Govt. Center for Disease Control (CDC) settled on a name for the illness: Chronic Fatigue Syndrome. This unpopular name insults patients suffering from severe neurological, endocrine, and immune disorders and misleads physicians and public into thinking that the disease is all about being tired. No wonder much energy has been spent over the past two decades looking for substitute names such as CFIDS, XAND, or non-HIV AIDS.
Now we have a new acronym: CIRS, which stands for Chronic Inflammatory Response Syndrome. The name has been coined by Ritchie Shoemaker, physician in Pocomoke City, MD, whose interest in a cluster of patients made sick by an outbreak of Pfisteria in the estuaries of the Chesapeake Bay catapulted him into research on the etiology, pathophysiology, and treatment of illness with symptoms similar to, but not identical to, the disease we call CFS.
Kudos to Shoemaker for getting beyond the hand-holding and the palliative pills. When he used cholestyramine to control diarrhea in a very sick patient, after no other symptomatic relief worked, and found that all the other symptoms disappeared, he got curious enough to pursue the issue like a real scientist. His discovery was popularized in his 2001 book, Desperation Medicine. Soon he became known as the doctor who gives cholestyramine for illness associated with fat-soluble biotoxins. This grew to include Lyme disease, ciguatera poisoning, and mold illness.
His understanding of this cluster of biotoxin-induced illnesses has expanded immensely over the last decade. He has solved the issue of etiology for several of these illnesses, but CFS is not one of them. His identification of CFS as a subset of CIRS rests on his belief, and his unpublished evidence, that the pathophysiology and treatment is the same as for those biotoxin illness he has successfully treated due to dino-flagellates, blue-green algae, and Lyme unresponsive to antibiotics.
His most recent book, Surviving Mold, summarizes his discoveries in language somewhat accessible to lay people, at least to those willing to carry and work their way through a 750 page tome with rants and repetition and no index. I was. I found it fascinating. My yellow highlighter kept busy. At the end, my understanding of what I've been going through for 25 years was radically changed.
More user-friendly is his recent lecture to physicians in the Hopkinton Drug Series, currently available free online at http://www.hcam.tv/videos/specials-and-unique-programs. Click on the link to Shoemaker's lecture on the sidebar on the right. This boils the information in the book down to 80 minutes, including a few jokes and personal anecdotes. It would have been interesting to hear the questions and comments from the large audience, physicians interested in learning how to use a hormone called VIP. But the archived talk ends with Shoemaker's final sentence.
So is CFS CIRS? Shoemaker thinks it is, whether or not XMRV turns out to be a major player, an opportunistic infection, or a laboratory contaminant.
Is he right? I don't know. But there is one way to find out: Let hundreds of people with CFS be tested for the six principle 'biomarkers' Shoemaker has identified.
Perhaps someday Shoemaker will get funding for a study. In the meantime, I'd love to hear from anyone who does these tests. It was a real eye-opener for me to get the results. I saw that I fit the pattern quite nicely.
Six biomarkers for CIRS (patient must have 3 of the 6)
1. VCS (visual contrast sensitivity) diminished. The test can be done online (for a small fee) on Shoemaker's site at http://www.chronicneurotoxins.com/VCSTestInformation.cfm
2. A susceptible HLA DR. This acronynm describes genes on the white blood cells known as lymphocytes which determine whether you inherit the ability to eliminate different kinds of biotoxins from the body. About 25% of the US population has one or two of the deficient genes. Even people without these genes can get the illness if they get a large enough or long-term exposure. This test is available thru Labcorp, and is covered by Medicare and most health insurance with code 012542, Dx 279.10; 377.34; 279.8, (yellow, refrig)
3. MSH below 35 pg/ml. MSH stands for melanocyte-stimulating hormone. This important molecule helps regulate the immune system, sleep (through melatonin) and pain/pleasure (through endorphins). Shoemaker claims it is low in everyone with CFS. My deficiency of it explains a lot of symptoms I've had over the years. MSH should be tested through Labcorp with code 010421 DX 253.2 (Lav-freeze). Medicare covers it.
4. ADH and osmalolity tested at the same time to show dysregulation. ADH determines how often we pee and how concentrated our urine is. Osmality measures the concentration of electrolytes and other ions in the blood plasma. My ADH was abysmally low, almost non-existent at 0.8 (normal is 1.0 - 13.3 pg/ml) while my osmolality was normal 290 (280-300 is normal). Both tests are done through Labcorp. ADH is not always covered. Specs are Lav-freeze, 046557, Dx 253.5 and SST-refrig, code, 002071, Dx 253.5
5. MMP-9 is elevated, above 332 ng/mL. Mine was a whopping 578! MMP-9 stands for matrix metallopeptidase 9. This is an enzyme involved in the breakdown of the extracellular matrix. Without getting too technical, it's an inflammatory marker involved in many degenerative processes. My score is proof that I'm breaking down.....aagh. As if I didn't know already. MMP-9 should be done through Quest with SST-freeze, code 41865, Dx 340. Medicare paid.
6. ACTH/Cortisol measured simultaneously to show dysregulation. ACTH stands for AdrenoCorticoTropic hormone which is released from the pituitary to stimulate the adrenals to release cortisol. First the hypothalamus has to secrete corticotropin-releasing hormone to the pituitary. Hundreds of studies have found problems in the HPA axis in CFS patients. This illness transforms our ability to cope with stress, so it becomes very important to minimize physiological and emotional stress and learn skills to deal with stress. ACTH and cortisol are both done at Quest with Lav-freeze, 21420P, Dx 255.4 and SST-freeze, 11281X, Dx 255.4. (Actually the tests can be done at any lab, but Shoemaker believes, after working with many patients, that the lab he specifies gives more accurate results. Hence the need for your doctor to bother with more than one lab -- which many practitioners will not do.)
For the record, my scores were both in the normal range, although my ACTH level was quite low relative to my cortisol level, and I have lots of other tests showing problems with "the adrenals."
How many of us have been told we have adrenal issues? We've done blood tests and saliva tests. We've taken herbs, glandulars, hormones, and vitamins to correct the problem, but don't get the promised results, or find we can't tolerate the treatment. Perhaps the poor gland has gotten exhausted and can no longer produce what is required. But more likely, with a chronic inflammatory process going on, the adrenal gland is just responding to the alarm signals. Taking cortisol adds fuel to the fire, for there is a sensitive feedback between the hypothalamus, pituitary and adrenals. So if the adrenal hormone level goes up, the hypothalamus will tell the pituitary to produce less; the pituitary will tell the adrenal to produce less. In the end, we don't correct the problem.
Since publishing this case definition, Shoemaker has added a lot of other tests that tend to be abnormal in people with mold and neuro or biotoxin illness such as elevated C3a, elevated C4a, elevated TGF-beta 1, decreased VEGF, decreased VIP, and elevated leptin (in those with weight issues).
A full list of the tests he recommends and monitors during treatment can be found at http://www.survivingmold.com/diagnosis/lab-tests
If you have CFS and have had some or all of these tests done, I'd love to know if you meet the criteria for CIRS. If you haven't tested, but have a physician willing to work with you, the tests will  demonstrate that you do have serious, measurable abnormalities (very useful if you're trying to qualify for disability) and  help you to understand why you feel like crap and why your symptoms and energy seem to change from day to day.
Given the preponderance of viral onset CFS, for all CFS patients to meet the CIRS criteria, some viruses or certain immune irregularities would have to set up this pattern. I believe that most CFS doctors are not yet interested in Shoemaker's research because he hasn't demonstrated that such a link exists. The high inflammatory cytokines and other immune system chemicals have been found by researchers in Klimas' lab, and while various regulatory peptides have been found to be diminished in this illness, there isn't much overlap with Shoemaker's findings yet. It will be very interesting to see what develops, for CIRS describes a pattern of immune, endocrine, and neurological dysregulation that makes sense for the predominance of symptoms experienced by patients with CFS.
Have you been tested? Let me know in a comment or a private e-mail
Is ME/ CFS CIRS?
Blog entry posted by JanisB, May 2, 2011.
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