GUEST BLOG BY ERIK JOHNSON
The matter of “What is CFS?” is of utmost controversy and bitter debate.
But I know EXACTLY why the syndrome was coined, and the reasons for it. My presence at this conference is to explain the circumstances under which I was asked to serve as a prototype.
This is the backstory of why the syndrome was coined, and is the basis of why there is a syndrome called CFS.
Raggedy Ann Syndrome
In short, during the early 1980’s there was an illness sweeping the USA called “adult mono”, a strange and unknown reactivation of Epstein-Barr virus. . This “adult mono” was life-destroying by causing chronic fatigue. It was called the “yuppie flu”.
But it was not spectacular enough or devastating enough to capture the imagination of doctors, and was largely ignored. Many people tended to recover on their own if they took better care of themselves.
Then my town of Incline Village was hit with a savage flu-like illness.
It had elements in common with “adult mono”, but also many contradictions – one of which was a condition of such weakness and inability to control the limbs that it was virtually paralytic.
The CDC objected that with its long incubation period, it was nearly impossible for EBV to result in huge outbreaks, and initially refused to investigate for this reason.
A local teacher described it as like a “Raggedy Ann doll with the stuffing knocked out”. So this “not quite like yuppie flu” illness was called “Raggedy Ann Syndrome”. Due to the Raggedy Ann Syndrome being spectacular and devastating to our community, it captured the attention of the world.
Ted Van Zelst
A wealthy Chicago businessman named Ted Van Zelst, who was trying to get help for his daughter with the “adult mono”, saw an opportunity to use the greater severity of the Raggedy Ann Syndrome to force the CDC out of its disinterest and inaction toward the “Yuppie Flu”. Van Zelst believed that in such a serious and spectacular outbreak such as ours, surely some immune abnormalities would be found. These could be used to force the CDC to do something.
From CFIDS Association of America:
Timeline of Key CFIDS* Events
The term “myalgic encephalomyelitis” is coined for a CFIDS-like illness, investigated by Dr. Melvin Ramsey and published in the Lancet.
First documented cluster of approximately 200 CFIDS cases occurs in Incline Village, Nev. This outbreak focused attention on the condition in the United States. Dozens of children and adults in Lyndonville, N.Y. begin reporting a severe flu-like illness. Suspected cause of their symptoms: CFIDS.
Name “chronic Epstein-Barr syndrome” begins to be used in U.S., based on preliminary studies by Dr. Stephen Straus of the National Institutes of Health (NIH); he later revises his theory linking the illness to Epstein-Barr virus (EBV) following intense scientific criticism.
The Charlotte CEBV Association (named following speculation about EBV as cause of the illness), founded by Marc Iverson and Alan Goldberg, begins to meet. This group later becomes the CFIDS Association of America (TCAA).
Ted Van Zelst, whose adult daughter has CFIDS, becomes the first person to testify before Congress requesting research funds for the illness. His congressman, John Porter, becomes a strong supporter of CFIDS issues.
The Centers for Disease Control (CDC) publishes the first official case definition in the Annals of Internal Medicine.
The National Institute of Allergy and Infectious Diseases (NIAID) reports that 25 percent of the calls taken by their communications office are about CFIDS. Only AIDS has a higher volume of calls.
Van Zelst had given funding to a Harvard doctor in Boston (Dr. Anthony Komaroff) to generate compelling evidence from his yuppie flue patients to validate the “adult mono”, but aside from abnormal neurological testing results, nothing concrete had shown up. Van Zelst contacted Dr. Paul Cheney of our town and made him the same offer. He had Dr. Komaroff come to Incline to collaborate in the search for some evidence.
The CDC had made a quick investigation of our outbreak and found that we did not match the adult mono illness, and left, planning no further study. All they did was look for EBV and nothing else.
The CDC had no interest in our Raggedy Ann Syndrome and was going to carry on treating yuppie flu as they had been – as a trivial condition of people who didn’t take care of themselves.
Dr. Cheney and Dr. Dan Peterson persevered and did MRI scans, using the first MRI to be set up in Nevada. These showed punctate lesions that were similar to those found in AIDS patients. This was the first evidence that made it look bad for the CDC to ignore us.
Then Dr. Peterson sent blood to the Gallo lab and found a newly discovered virus, Human Herpes Virus Six, Variant Alpha (NOT Beta, which is the roseola virus and is a completely different animal). The CDC had no choice but to be drawn back into the investigation, as now we looked like a new disease of “HHV6A”.
North Lake Tahoe Bonanza, Friday October 10 1986
Tahoe patients tested for new virus
by Jean Lamming NLTB Staff Writer
An Incline Village resident stricken with Chronic Epstein-Barr Virus said Wednesday that the national medical researchers believe a new virus they have discovered is partly to blame for the syndrome.
Bill Rulle said researchers from the National Institutes of Health in Bethesda, MD. interviewed him last week in Incline and took samples of his blood. They will test it for a virus they discovered a year ago and think might be a partial culprit in the fatigue syndrome that struck some 200 North Shore and Truckee residents in the last two years.
“They think there might be a new virus and that what it is doing is triggering the Epstein-Barr Virus reaction on a constant basis.” Rulle said of the National Institutes of Health (NIH) researchers.
The researchers are calling the new virus HBLT and B-cell lymphoma, Rulle said. New tests for the virus are inconclusive.
Its symptoms include a positive test for Chronic EBV and the fatigue syndrome that accompanies it, said Rulle, who calls himself an “interested patient.”
Doctors, including Incline internist Paul Cheney who treated many area fatigue patients, and officials at the NIH have been vague on the subject of a new virus.
Cheney said in September that news of a medical discovery that might eventually be linked to Lake Tahoe would be announced in a prestigious medical journal this year.
A spokesman for Science magazine, a definitive professional research journal, said Monday that the magazine would carry two research articles from the NIH’s cancer research division in its Oct. 24 edition.
The Science spokesman declined to comment on the content of the articles and said researchers involved are bound not to release information on articles before they are published.
The NIH researchers, who have reportedly tested about 72 blood samples from area residents for presence of the new virus, took another 90 samples from residents during a visit last week, Rulle said.
As many as 90% of the 70-some Lake Tahoe CEBV patients tested for the new virus show positive signs of carrying it under the new test, Rulle said. Results of blood tests from people who are not infected with CEBV showed none were affected with the new virus either, he said.
– “They are relatively sure that the new virus is in fact activating the EBV and not the other way around.” – Bille Rulle Incline Village fatigue illness victim. –
The test is too new to be conclusive, but supports the hypothesis that CEBV, which most adults carry latently, was activated in many area victims by the new virus, Rulle said.
“That was a scientific basis that what we have here is a new virus and all we can see as a symptom is CEBV,” said Rulle.
“They are relatively sure that the new virus is in fact activating the EBV and not the other way around.” he said of researchers.
The symptoms of the new virus fall under the umbrella of symptoms CEBV victims suffer from in varying combinations and degrees, Rulle said. These include chronic fatigue, upper respiratory-tract infections, headaches, tingling and loss of feeling in extremities, dizziness, memory loss, sleep disturbances and more.
Rulle, who has been sick with CEBV for about two years, said if people at Lake Tahoe have the new virus, they might be among the first to develop antibodies to it.
“That is why we have two groups from NIH very interested – the scientific group that discovered it (new virus) and the people who recently visited who study viral groups in populations.
It was at that juncture that Ted Van Zelst appeared and made his pitch to Dr. Cheney about working together to battle the CDC.
Dr. Cheney wanted to use a brand new cell flow cytometry lab – “Cytometrics” – that was doing sophisticated blood cell analysis, but this was very expensive. Van Zelst paid to cover the cost. Since the goal at this point was to overcome the CDC’s fixation on EBV reactivating for trivial reasons, Dr. Cheney set out to find people with the same signs and symptoms of the Raggedy Ann syndrome (more severe than yuppie flu), but who were EBV negative.
I was the first one.
As told in “Surviving Mold”, when Dr. Cheney asked me to volunteer to be in his project to break the CDC impasse, I refused, and told him that since the mold was making me sick, I was not a perfect representation of the new viral hypothesis of “HHV6A Disease”.
Dr. Cheney said this didn’t matter. The HHV6A virus was the main point of interest, and my mold problems were a secondary issue which would not interfere. Here is where I saw my chance to get research into the mold, which was ALSO being ignored by the medical profession. So I agreed, on the basis that when researchers came to investigate, I could tell them about the mold that had very obviously been having an effect where clusters of “HHV6A disease” emerged.
The cell flow cytometry found high red cell debris, an inverted CD4/CD8 ratio, and reduced B cell population – which was very scary to the CDC’s herpesvirus division, headed by Dr. Carlos Lopez. Lopez now took a personal interest in the Raggedy Ann “outbreak”, as the new HHV6A had been found by the Gallo lab to be a major problem in AIDS patients – and the similarity of MRI scans spoke to a common pathway.
At the same time, the funding Van Zelst had provided to Dr. Komaroff for the purpose of finding immune abnormalities in our outbreak as a demonstration to the CDC had paid off. Low NK cell function was found.
North Lake Tahoe Bonanza, November 16 1987
“Incline Victims Show Cell Abnormalities”
Tests Reveal New Clue in Fatiguing Illness
by Chris Fotheringham NLTB Managing Editor
Laboratory results published this week in a prestigious medical journal confirm that over 50% of Incline Village chronic fatigue patients tested have suffered “dramatic” abnormalities in their immune systems.
Calling it the “most significant finding yet” in efforts to unravel the mystery of the widespread fatigue illness, Harvard researcher Dr. Anthony Komaroff said Monday the report published Sunday in the Journal of Immunology is the first scientific study that confirms “something is wrong with these people.”
“It really is dramatic,” said Komaroff, who is chief of general medicine at a Harvard teaching hospital in Boston.
The article, which underwent nearly 11 months of peer review before being published, was authored by Komaroff, Incline Village internist Dr.Daniel Peterson, and former Incline internist Dr Paul Cheney.
Dr Michael Caligiuri, an immunologist with the Dana-Farber Cancer institute of the Harvard Medical Center, was the lead author for the article which was originally submitted for review in January.
Komaroff says test results reveal an attack on the immune system’s “natural killer cell” which is the body’s primary means of killing virus-infected cells or cells that become cancerous.
Komaroff said “There is a substantial reduction in the number of natural killer cells in patients tested.” He said the study has determined that this “major defense against virus infection and cancer” is damaged in over half of the test cases involving Incline Village patients.
Komaroff first brought his team of researchers to Incline Village in February of 1986 after Incline doctors Cheney and Peterson had documented an outbreak of approximately 200 cases of mononucleosis-type illnesses in the North Tahoe and Truckee area beginning in the fall of 1985.
While the Incline Village cluster of fatigue cases has drawn primary attention in the national media, researchers have found widespread occurrence of the illness throughout the country.
See TESTS on page 9. http://www.ncbi.nlm.nih.gov/pubmed/2824604
This body of evidence scared the CDC to death and got their full attention. Dr. Gary Holmes, the CDC epidemiologist who did the original 1985 investigation, was directed by Dr. Carlos Lopez to draw up a paper of his findings. Since the evidence was disproving the CDC’s “adult mono/EBV Syndrome”, the working title of Holmes’ paper was “Chronic Mononucleosis-Like Syndrome” – CMLS.
Since this was hard to pronounce, the CDC took to calling it “Raggedy Ann Syndrome”, to distinguish it from the yuppie flu. Dr. Lopez called for a meeting do discuss what to do about the Raggedy Ann Syndrome. This is the famous “Holmes committee”.
The champions of the EBV hypothesis, Stephen Straus and James Jones, showed up ready to fight to preserve their “EBV dominant” paradigm. In essence, they didn’t know what the Lake Tahoe “Raggedy Ann Syndrome” was, and they didn’t care.
They said it was either lacking evidence, or that the evidence was inconclusive as it did not point at any known disease. Their goal was to set aside the Tahoe outbreak and keep everything at “CEBV Syndrome” level, so they could pursue their “fatigue illness” studies (with sizeable CDC/NIH grant funding, of course).
Actually, Straus showed up with a new name: “Neuroasthenia”.
At this “Holmes committee” meeting, Straus and Jones battled until the three physicians present who diagnosed our outbreak as “a typical outbreak of myalgic encephalomyelitis” – Byron Hyde, Gordon Parish and Alex Shelokov – got disgusted and walked out. David Purtilo of Colorado was isolated and outgunned. Cheney and Peterson were not invited, as their evidence base was in Dr. Gary Holmes’ hands.
Straus and Jones moved into the lead and it appeared certain that all of our Tahoe evidence was going to be set aside. Someone was keeping Senator Harry Reid, D. Nevada, apprised of what the Holmes committee was doing. He personally intervened and said it made no sense for the committee to set aside the very object of investigation which was its purpose and reason for convening.
This could not be denied, but Straus and Jones could still make it APPEAR that whatever new study was designed by the CDC would be for THEIR syndrome. Straus used his seniority and power to put pressure on junior epidemiologist Gary Holmes to extract everything from his CMLS definition which tied it to the Tahoe outbreak.
Gary Holmes complied. We do not know how he felt about this, but his actions would seem to show he was very much aware of the implications, and was not on board with this. For this is what he did: Holmes handed out his “Tahoe Study” CMLS paper in the middle of a meeting – even without anything IN the paper to focus on the Tahoe evidence, this still pointed AT the Raggedy Ann Syndrome as central to whatever “entity” was being discussed.
Upon receipt of this “Holmes paper”, Stephen Straus went absolutely ballistic and had a wild screaming fit. This took the focus out of his hands and put it squarely on the Tahoe outbreak.
At this point, the Holmes committee should have designated the Raggedy Ann Syndrome and associated evidence as the object and purpose of the new “research tool”.
But Straus was not done. He used his influence to freeze the evidence base at the evidence level of Holmes’ “Chronic Mononucleosis-Like Syndrome” paper, and allow no more into the new syndrome that the CDC agreed to create. And again, Straus tried to keep mentions of Tahoe out of it.
The most prestigious member of the Holmes committee, Dr. Elliot Kieff of Brigham and Women’s Hospital, wrote several angry letters of complaint to Gary Holmes about a new syndrome DEVOID of objective measures and without a clearly stated target. He was not yet aware that Stephen Straus was directing Gary Holmes to omit specific neurological signs and symptoms of the Tahoe outbreak from his definition. Exasperated at this ambiguity, he asked,
“Is the intention to create a new psychiatric classification?”
But Straus cast too large of a shadow and got his way.
Our fate hung in the balance.
Would people see through the deceptively ambiguous CFS definition and look at why it was created? Or would they take the lazy way out, and just think that the few stated symptoms were all that was known?
Straus and Jones embarked on a campaign to say that CFS was nothing more than a new name for the old EBV syndrome. Since the many thousands who had been diagnosed with CEBV Syndrome did not know about our evidence or about the battle that occurred in the Holmes committee, the patient groups accepted this as fact, and worked to spread the word that this was indeed what CFS is. In this way, the real reasons for the existence of this new syndrome were almost completely made to disappear.
If it were not for a few books and perceptive doctors, Stephen Straus’ plan to revert the paradigm to a trivial fatigue illness and subvert the evidence would have worked flawlessly. The “founding evidence” that was KNOWN to the CDC/NIH and under scrutiny by the Holmes committee, and even acknowledged by the AT THE TIME – that the Raggedy Ann Syndrome was why there is a “Chronic Fatigue Syndrome” – would have been gone forever.
And along with it, my story of “Mold at Ground Zero for CFS”.