Here are three myths about severity levels.
Even some of the best scientists, journalists, and advocates get tripped up by them.
Myth 1: range of severity
There is a misconception in some of the academic literature and mainstream media that there is a single severity level or a narrow range.
In reality, the range of severity is as wide as it is in HIV/AIDS.
The levels include almost asymptomatic, very mild, mild, moderate, severe, very severe, and dead.1
We know this because of disease progression, among other reasons.2 What disease you had when you were at the mild end, is what disease you still have.
Just as with HIV/AIDS, the disease starts to present its true colors after you worsen in severity. Just as with HIV/AIDS, unrecognizably different levels of severity are the same disease.3
You can start out at mild levels and progress to death. You did not switch diseases.
The range of severity is wide.
Related to myth 1: multisystem nature
A related misconception holds that the disease affects only one body system (or only a few symptoms). It implies that severe levels are "more of the same".
In reality, as you get sicker, the number and breadth of your symptoms increase. This increase covers a vast range and cannot be dismissed without dismissing the disease. It is salient and fundamental.
Just as with HIV/AIDS, many systems in your body are affected simultaneously. For example, it is not immune only, autonomic only, GI only, uro only, mitochondrial only, or vascular only (I could continue). It is all of those and more combined. 50 symptoms is not unusual.4
Multisystem nature is part of severity. Progressed levels are the same disease, but they are not "more of the same".
Most characterizations, even by sincere scientists, are of extremely mild levels at best and bear no meaningful resemblance.
The range of severity is wide. So is the range of multisystem nature.
Myth 2: severe vs. serious
There is also a misunderstanding about "severe" and "serious".
Just as with HIV/AIDS, sufferers at the mild end do not have a severe current presentation, but they have a serious disease.
Just as with HIV/AIDS, symptoms at the mild end provide false comfort and false direction to biomedical research.
Mild does not mean not serious.
Myth 3: the meaning of "mild"
Finally, there is a myth that mild levels are mild by normal standards.
Mild sufferers are mild by the standards of the disease, but they are often severe by a normal person's standards.
Consider the reverse
Consider the reverse. "Severe ingrown toenail" is severe by the standards of that disease, but it is usually trivial to any person with a sense of proportion.
As strange as it sounds, specialists in podiatry are right to call it severe. It is severe compared to mild levels. They need to acknowledge the range of severity (even though it is narrow in non-gangrenous cases).
You might also consider severe dandruff or severe paper cut. You can trek across Antarctica.
Now back to our case
Now back to our case. Mild sufferers of this disease are severe to any person who is not misopathic, but they are mild by the standards of the disease.
As strange as it sounds, we are right to call mild sufferers mild. They are mild compared to severe levels. We need to acknowledge the range of severity.
The range of severity is wide, so all of us have to call mild sufferers mild.
Otherwise, whoever we are talking to will assume that is as bad as the disease gets.
The range of severity is wide, so this means he or she will assume an extreme falsehood.
Many sufferers at the Very Mild level can't leave the house half the time. Very Mild is bad.
It is nothing in comparison to when they get sicker, but that does not mean that mild sufferers are mild by a normal person's standards.
Conclusion
I made three points:
- The range of severity is wide
- "Mild" does not mean not serious
- "Mild" means mild by the standards of the disease
Samuel
Footnotes:
They possibly include aysmptomatic also, just as HIV-infected people can be asymptomatic. They are readily distinguishable.
For example, co-occurrence in clusters or relapses/remittals at different severity levels can also suggest the range of severity.
Newcomers: just as with HIV/AIDS, you cannot tell from your symptoms at the earliest stages what disease you have. Only after you get sicker can you be more certain. When serious biomedical research starts being funded, we will get our widely-deployed equivalent of an HIV test. Just as with HIV/AIDS, this will not happen without far stronger and far more concerted political action than we have ever taken.
But not at mild levels. The surprise that many people show upon hearing about it illustrates the need for education.
No sufferer should ever be unaware of — or dismiss — other severity levels than his or her own, whether milder or more severe.
The need to break this disease into multiple subsets instead of recognizing the truth of what you are saying here--that there is just one disease--is misguided and ends up, whether purposeful or not, sustaining confusion about and mischaracterization of the nature of this disease.
ReplyDeleteInstead of seeking to break the disease into subsets, it would be greatly more rational to simply stage the disease by severity--creating perhaps four stages or "grades," as is done in cancer and other catastropic maladies. Someone who has been ill for 27 years, for instance, who has had heart attacks, strokes, exhibits multiple white matter lesions on MRI, has suffered cancer, and so on, might be stage 4.
Someone who is showing abundant evidence of viral infection and sleeping 18 hours a day, but has been sick jsut 3 momths or 6 months, might be stage one.
These are NOT subsets--they are on a continuum not only of severity, but of progression of a specific, single disease. And, clearly, this is a progressive, degenerative disease. To call aspects of the progression "subsets" is folly and counterproductive.
Of course, if you don't believe this is one disease that manifests new and more serious symptoms as it progresses
, untreated, then I suppose the subset approach will appear reasonable.
But, in the case of the viral disease HIV, which exhibits marked changes over time when it goes untreated, the notion of breaking patients into subsets for the purposes of studying this single disease would be thought ludicrous.
CFS has marked similarities to AIDS, as you suggest. The central unifying principal behind AIDS is that a single agent has caused the disease and whatever symptoms emerge are due to the course of the virus at the heart of the disease.
Excellent point, Hillary.
ReplyDelete"Subsetting" and "heterogeneity" are indeed frequently an attempt to obscure the progressive nature of the disease.
This was thoughtful and well-written. Thanks, Sam.
ReplyDeleteI've often thought of the differences in MS - it seems some of us have a remission and relapse course, while others progressive. I've seen a lot of patients who were sick in their late teens, got better for a couple of decades, then relapsed into a progressive, very severe, disease. I'm not sure that's not my own history.
Then a lot of patients have a prodrome period when they are able to function, but they have symptoms that anybody familiar with the Disease would recognize. They don't get diagnosed until it explodes - and then it's a little late.
It is all so under-studied, so frustrating.
But mostly, I REALLY agree with your statement that what seems mild to us would be serious to an outsider.
Thanks.
Hi Mary,
ReplyDeleteThank you.
Yes, the different patterns of worsening are critical to study.
I agree with Hilary. We should weed out the patients with mental illness and not call them some ridiculous "subset" and then validate the various stages of the disease. I think the people harping on ME and CFS being different are buying into the CDC definition of CFS far too much,which needs to be thrown out altogether. CFS as we know it should be thrown out, but it is not a separate disease. Rather, we need to than accept the reality of various severity levels of the one disease.
ReplyDeleteI really appreciate your blogs Samuel, and thanks for breaking this down. I consider myself to be moderate, bordering on severe at times. I started out as mild and yet it was such a shock and I immediately could no longer work. Thank you for validating the severity of mild cases.
Also, you kind of lost me on "mild" versus Mild etc. Maybe you can clear that up better? I will try re-reading again. I have always appreciated your grasp on logic which usually makes perfect sense once I read and fully absorb what you're saying! :)
I am never suprised by the depth of knowledge of my patients. I learn an immense amount from them . I agree with Hillary and Mary that splitting the disease into different subsets will only lead to confusion. In complex disease there are different presentations. Let us take anterior poliomyelitis (polio) as an example. "Polio" when it was an epidemic scourge affected patients differently, some could not breath normally and were placed in an "iron lung", an encasing device that surrounded the body and by changing pressure and vacuum expanded the lungs. There were those patients who were partially but permanently paralyzed , President Roosevelt for example and then there were patients who were transiently afflicted. This was all the same disease in different presentations. It would be wrong to subset this disease, Salk and Sabine, both from Sinai, New York, may have been slowed if they thought they were researching different diseases.
ReplyDeleteSimilarly I think it is wrong to subset the disease which in turn will dilute and slow research into effective diagnosis and treatment.
Derek Enlander MD
Mount Sinai ME/CFS Center, New York
Misopathic agnotology driving Iatrophobia. It' a new paradigm!
ReplyDeleteWhenever I am bothered by symptoms of the disease, I simply walk away from my house and mortgage, have all my posessions taken to the dump, swap used cars and go live in the woods for two or three years while maxing out my credit cards. That usually clears me right up.
As one of those who "staggered" into see Cheney and Peterson in 1985, my illness has been obscured by the absolute 100% refusal of "CFS researchers" to investigate all of the circumstances involved in my outbreak of "mystery-malady".
ReplyDeleteFor this reason, and for purposes of clarity, I prefer to think of myself as part of the "Tahoe Mystery Illness" subset, as opposed to CFS.
Osler's Web: Inside the Labyrinth of The Chronic Fatigue Syndrome Epidemic
by Hillary Johnson
Crown Publishers, Inc.
In 1985 in Incline Village on Lake Tahoe, Nevada, two physicians began noticing an unusually devastating illness with an array of symptoms never seen before.
Puzzlement at the first few cases turned into alarm when more and more patients staggered in with the same debilitating symptoms. Called variously the Lake Tahoe Disease, Chronic Epstein-Barr virus syndrome, Yuppie Flu, and finally Chronic Fatigue Syndrome, this new illness was also being noticed in Brigham and Women's Hospital in Boston, in various hospitals in Los Angeles and San Francisco, and in small towns in upstate New York as well as at other points around the United States., The majority of early cases reported in the press afflicted middle-class middle-aged women. Unable to find any one cause for this bewildering array of symptoms, the medical establishment attempted to convince these women that it was all in their heads.
http://www.amazon.com/Oslers-Web-Labyrinth-Syndrome-Epidemic/dp/051770353X
Hi Heidi,
ReplyDeleteThank you.
I think the Mild vs. mild footnote was confusing, so I removed it. Thanks for pointing it out.
Hi Derek,
ReplyDeleteInteresting about polio.
It is possible that we sometimes encourage "subsets" when we really are trying to address the politics of denialism, as in "stop evading biomedical research on the full range of severity" and "stop watering down cohorts with anti-scientific definitions". Those are not real subsets, but severity levels and diseases, respectively.
You are heading up a large research effort. Given resources, would you still do things like check to see if there is something different about outbreak cases, onset types, etc.? Or would that be of low value until other research results are returned?
Hi Jonathan,
ReplyDeleteAgnotology is a good word!
The study of induced ignorance or doubt, especially by bogus science.
Yup.
Hi Erik,
ReplyDeleteYes, all facts need to be accounted for.
From everything I've read about Coxsackie B, the Tahoe outbreak was exactly like the descriptions of a polio epidemic.
ReplyDeleteDr Peterson seems convinced that Coxsackie is ruled out and that flu was HHV6A.
It remains for the viral experts to decide. Having no viral lab, I never presumed to debate them on the issue.
But... there is one thing I did ask for.
Hillary Johnson described our outbreak perfectly in Osler's Web.
Particularly the Truckee teachers lounge incident that "shattered Cheney and Peterson's fragile equanimity", about whether there was an epidemic.
I asked for help in understanding what happened to us in that room.
As a prototype for CFS, I didn't think I would have to ask twice.
Researcher are supposed to "want" clues. Yet in over a quarter century, not a single CFS researcher has been willing to respond to evidence from "Ground Zero for CFS"
This circumstance has never been investigated, even though it was a major factor in Dr Peterson's decision to call the CDC for help.
If that kind of surrealistic super-insanity doesn't put the shattered-glass frosting on the Kafka-cake... I can't imagine what possibly can.
Then again, I couldn't have imagined any of this.
Paul Cheney pointed out decades ago that the disease goes through stages. 1) You are acutely ill; 2) You are chronically ill; and 3) Your body adjusts genetically to the illness.
ReplyDeleteThis means that at different stages of the illness you can manifest it in a variety of ways. My "acute" stage was "mild", and consisted of the classic viral symptoms (sore throat, low fever, etc.) Exercise, recommended by my physician, made it much worse. This stage was not diagnosed as CFS, but as a year-long flu. (With a question mark "flu?" on my records, made by my doctor.) I believe that many undiagnosed people with CFS/ME are at this stage.
My chronic stage, which was technically a relapse, was primarily neurological, but involved all systems - vascular, immune, nervous, and endocrine. This was classic "severe" CFS/ME with photosensitivity, pain, wasting, GI symptoms, cognitive problems, insomnia, post-exertional malaise, and so on.
Stage 3, also technically a relapse, involved profound orthostatic intolerance, a total collapse of the GI system, and an overwhelming hyper-response of the immune system - in short, autoimmunity.
Autoimmunity is the genetic end result of chronic CFS/ME. It will hit you where ever you are the weakest(gut, brain, heart). In my daughter's case, it hit her in the thyroid - Hashimoto's. In mine, the gut. In Sophia Mirza's case, it hit the spinal cord, the dorsal root ganglia of which were virtually destroyed by inflammation (i.e. immune system hyper-response).
This sort of systemic autoimmune response to an initial undiagnosed infection is not uncommon. MS is believed to be the result of an immune response to EBV infection in the brain. Chronic Lyme may also fall into this category.
It is likely that what many people describe as severe CFS/ME is either stage 2 or 3. It is also likely that what doctors describe as CFS is either stage 1 or 2.
If you add to this model the longitudinal studies of John Richardson, in which he catalogs the long-term effects of entero viral infections, you will have a complete picture of the extent, severity, and progression of the illness.
The confusion experienced by the research community when test results are "inconsistent" among CFS/ME patients is not only the failure to understand that the illness can be progressive - as per the above - but is a reflection of misconceptions and attitudes towards the illness maintained and perpetuated by the CDC.
ReplyDeleteI know a thing or two about how Dr Cheney came by some of his concepts.
Back when he and everyone else was fixated on the virus that went through town as the "cause", I asked him, "If so many people got that weird flu but recovered, then wouldn't the actual "cause" actually be whatever difference existed in those who became chronically ill?"
He became very thoughtful.
I could tell that I really got him going with that idea. But when I asked him to look at a particularly strange thing that happened, he couldn't bring himself to believe me. Very doubtful.
If what I said were true, that would mean the "Thing" I pointed at must be doing something absolutely unprecedented on a global scale.
This seemed far too unlikely to give the notion any serious consideration.
The only power I have to draw anyone's attention to it is by reminding them that the "Raggedy Ann Syndrome" is the subset that caused this new "CFS" syndrome to be created.
---------------------------------
sler's Web: Inside the Labyrinth of the Chronic Fatigue Syndrome Epidemic
by Hillary Johnson
Crown Publishers Inc. NY
Epilogue
page 675-676
The process of CFS was like falling dominoes, or the chain reaction of nuclear fusion. Once the slide began, Cheney increasingly believed, the instigator was irrelevant to the outcome, which was inevitably a momentous and intricate constellation of "multiple injuries" to the body. "All great avalanches start with something very small," he said. "It is not unlike the theory of chaos in which a butterfly flaps its wings in Japan and starts a hurricane in Florida. It becomes like a snowball going downhill. One doesn't necessarily care what the the little pea was that caused all this shit. You can find the pea, but what do you think that is going to do? Nothing."
Thank you, Erica.
ReplyDeleteDo you by chance have links at hand to the context of Paul Cheney's comments or the most relevant parts of John Richardson's studies?
By present-day definition, there are 29 separate and distinct types of AIDS patients (everything from malaria, TB, cervical and lung cancers). Only every 29th patient is alike.
ReplyDeleteThere have to be 29 separate & distinct types of CFIDS patients.
"Chapter Forty-one
ReplyDeleteAs It Progresses, CFS Can Be Measured In "Stages"
One of the problems researchers encounter in studying CFS is that, like AIDS, there is no good indication of when a patient actually becomes infected with the disease-causing agent; therefore, it is difficult to assess the progression, or "natural history," of the syndrome.
A Long Island physician, Dr. Perry Orens, has devised a set of criteria by which to "stage" CFS, in terms of the progression of illness.
Dr. Orens's criteria range from what he has called Class I, in which the patient meets the CDC criteria (50 percent reduction in activities lasting at least six months), to Class IV, in which patients are completely disabled and bedridden.
The system used to classify AIDS patients' disease in stages, called the Walter Reed Classification System (developed at the Walter Reed Army Hospital), is no more specific than Dr. Orens's CFS stages.
In Walter Reed Stages 0 through 2, the patient is essentially healthy; antibodies for HIV and chronically swollen lymph nodes are the only symptoms noted.
In Walter Reed Stage 3, T4 cell counts drop to below 400; in Stage 4, there is a partial failure of the ability to respond to skin tests (i.e., cell mediated immunity begins to fail, as it does in CFS patients). In Stage 5, there is a complete failure of cell-mediated immunity (called "anergy," which is also seen in CFS patients) andor the development of thrush, a yeast infection of the mouth. (CFS patients, of course, can also develop thrush.)
In Stage 6 -- the stage at which patients are said to have AIDS -- opportunistic infections occur.
CFS patients can also develop "opportunistic infections." In his February 1993 testimony to a Food and Drug Administration Scientific Advisory Committee, Dr. Paul Cheney pointed out that of his five CFS patients who'd died during the preceding six months, three died as a result of overwhelming opportunistic infections.
At the very least, it seems that many CFS patients could be classified as being permanently or semi-permanently in Stage 4 of AIDS. "
America's Biggest Cover-Up: 50 More Things Everyone Should Know About The Chronic Fatigue Syndrome
Epidemic And Its Link To AIDS
by Neenyah Ostrom
The book, in its entirety, is transcribed here:
http://fms-help.com/aids.htm
When is it going to dawn on people that the FDA has no plans of helping???
ReplyDeleteNever has, never will.
Just google "WHO OWNS THE FDA?"
This is an excerpt from a book PUBLISHED IN 1993!
"Chapter Forty-six
An Experimental Drug That Appears Promising For CFS And AIDS Is Being Evaluated By The FDA
In February 1993, the Food and Drug Administration's Anti-Viral Drug Advisory Committee met to consider the merits of an experimental drug, Ampligen, that appears to have promise for treating CFS (and, in earlier trials, AIDS). So far, no drug has been approved for the treatment of CFS by the FDA.
Ampligen appears to correct a defect in a very important natural anti-viral pathway. When this pathway isn't working properly, viruses aren't attacked by the body's natural defenses. In CFS patients, as in AIDS patients, this anti-viral pathway is defective; furthermore, Ampligen appears to correct the defect in both sets of patients.
At the February meeting at the FDA, Kim Kenney of the CFIDS Association of America (in Charlotte, NC), told the FDA that approving a drug to treat CFS is extremely important to patients, many of whom are desperate to get better. She told the FDA that "the reactions of our constituents range from impatience to desperation, depending on the severity of their condition. Impatient to get back to work, to enjoy their families again. And for some, desperation to reverse the severely debilitating effects of CFIDS, including dementia, unrelenting muscle and joint pain, severe encephalitis, not to mention the problems that accompany a severely impaired immune system."
Dr. Daniel Peterson, one of the two physicians who identified the original outbreak of CFS in Incline Village, Nevada, in 1984, told the FDA committee that "a significant number" of his patients who fell ill in 1984 "never recovered." Dr. Peterson also said that, over the last eight years, 20 percent of the patients he'd examined had become completely disabled. Dr. Peterson also cautioned that the CDC's "crude incidence" of four per 100,000 "most certainly vastly underestimates the true incidence of the disease."
Dr. Peterson told the FDA that, although Ampligen "appears to have great potential in this disease process, it has been bogged down in a corporate and bureaucratic quagmire, and yet the disability and anguish of the patients and treating physicians remains unaddressed."
....
Perhaps most compellingly, however, Mr. Crum described for the FDA what happened to him when he stopped taking Ampligen because, as he said, "Receiving I.V. infusions three times per week is not pleasant." But, within six weeks of discontinuing Ampligen therapy, he once again began having seizures and his IQ again plummeted.
"In short, I regressed to the same seriously ill condition I was in prior to receiving Ampligen," Mr. Crum told the FDA Committee.
Similar testimony from other patients, or their families if they were too impaired to deliver or prepare testimony themselves, was also presented to the FDA committee.
The FDA, however, has yet to grant approval to Ampligen or any other drug to treat CFS. "
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
The book, in its entirety is published, here:
America's Biggest Cover-Up: 50 More Things Everyone Should Know About The Chronic Fatigue Syndrome Epidemic And Its Link To AIDS
by Neenyah Ostrom
http://fms-help.com/aids.htm
or just google "NON HIV AIDS"
FDA's resistance to Ampligen reflects the fact that efficacy and safety have come to be defined as something that can only be proven in FDA terms by methods that involve spending at least one billion dollars and take at least 10 years; this fits the economic model of creating a patent-derived and FDA enforced monopoly from which extraordinary economic rents are derived for the elite group of giant corporations capable of fulfilling FDA protocols. This in turn preserves the economic rents to be gained from progress in medicine for the corporate oligopoly.
ReplyDeleteIt is necessary to obtain all information about the NIH funded biobank project in order to apply pressure appropriately should it seem to be going off the rails. The information needs to be presented in a communal forum such as this or Co Cure or perhaps a special new blog so that we know what we know and what we haven't been able to find out. I think we should start by identifying the people involved in getting this grant written and approved in both Britain and America. Any suggestions?
ReplyDeleteHi Deborah,
ReplyDeleteYes, it does seem that we are dealing with regulatory capture.
[my post on the draft agenda].
Hi Deborah,
ReplyDeleteI really like your idea of a site or blog for taking community submissions for watchdog purposes. I think it should be dedicated and cover all events and studies. To keep administrative overhead low, maybe a blog with one post per topic and people can comment?