One woman’s journey into medical hell and the lessons that will help cure millions.
This is Julia Hugo Rachel. Like millions around the world I was accused of having a mental illness, had my child taken away from me and spent years in mental and physical agony. Then I found I was not mentally ill but instead had a virus and was cured. There are many people like me and this book, Viral Assault, will show victims, families and friends that there is hope. This is my story.
Bruce Campbell’s fight with Chronic Fatigue Syndrome began in the late ‘90s when he caught something that seemed like flu: body aches and pains, difficulty sleeping, and other symptoms which resemble the common illness. But, when his symptoms didn’t subside, he went to his doctor for help.
Campbell’s initial diagnosis was that he had a simple virus which would soon go away, and he’d soon be back to normal. But as the days stretched into weeks and the lethargy, tiredness and aches did not subside, he began to worry. He found he was only able to work half days before exhaustion became overwhelming.
“For four months, I experienced symptoms without having a name for my suffering,” Campbell described in his blog. “I lived with tremendous uncertainty, wondering what was wrong with me and what my future would hold.”
Campbell was fortunate that his doctor, unlike many others, had the time and the patience to search for a diagnosis until he was eventually able to present his patient with some good news. The doctor explained that his illness had not one name but several: Chronic Fatigue Syndrome. Chronic Fatigue and Immune Dysfunction Syndrome. Myalgic encephalomyelitis or myalgic encephalopathy.
Campbell went on to describe the bitter sweetness of his diagnosis.
“Getting a diagnosis was both a relief and a shock,” Campbell described in his blog. “I was grateful to have a name for my strange and debilitating symptoms, but staggered by the recognition that I had a long-term illness.”
For Campbell, the next steps were to educate himself to learn just what living with CFS was going to be like. Learning about the illness was his way of coping with it and learning to survive, but for the millions of others who have yet to even get a name for their debilitating sickness, the path of pain continues.
According to the Centers for Disease Control, “Researchers estimate that at least one million Americans have ME/CFS, but only 20% are diagnosed with the illness. Patients, families, employers, and society endure significant costs associated with ME/CFS. These costs are estimated to be $18-51 billion annually in the United States. Although most common in women, ME/CFS affects people of all ages, including children, and all races and ethnicities. Scientists have not determined the cause or causes of ME/CFS.”
More than one million Americans have CFS which strikes more people in the United States than multiple sclerosis, lupus, and many forms of cancer. Real numbers are very hard to verify because doctors are so poorly educated that few can do an accurate diagnosis. Estimates of the number of people with the condition vary from 7 to 3,000 per 100,000 adults and enormous discrepancy.
Sufferers of Chronic Fatigue Syndrome (CFS) can empathize with the plight of people with Gulf War Illness described earlier in this book. They have experienced the same agony not just of their pleas for a diagnosis being ignored but of the months, even years, of pain and suffering.
"This is clearly an illness that's born from a biologic cause," said Nancy Klimas, MD, who directs CFS and Gulf War Illness research programs at Nova Southeastern University and the Miami VA Healthcare System. "It's a chronic, serious illness, and patients will only respect the providers that acknowledge that."
CFS is known by other names such as Chronic Fatigue Immune Dysfunction (CFIDS) or Myalgic Encephalomyelitis (ME). No matter the name you choose to use, the illness is one that affects the central nervous system, brain, blood, muscles, joints, GI tract and lymph system. Symptoms include disabling fatigue which is not significantly helped by rest, orthostatic intolerance (inability to stand for periods of time), muscle weakness and pain, joint pain, sleep disturbances and unrefreshing sleep, cognitive problems including memory loss and difficulty concentrating, gastrointestinal problems, headache, fevers and swollen lymph nodes.
Having three names has done little to grow awareness of this disease though, and in fact, its symptoms are some of the only data on the illness. Along with the name change, the focus on the fatigue portion led to a warped view of the disorder to being regarded as a psychiatric illness, thus looping it into the PTSD spectrum in misdiagnosed cases.
Judy Smith of EmpowHer explained the difficulty of the incorrect and multiple names of the disorder in her article Alternative Medicine Aids in My Ongoing Recovery From ME/CFS.
“Chronic fatigue syndrome (CFS) is one of its foolish names, a wastebasket diagnosis that some doctors hand out when they don't know what's wrong. And some toss this label your way when they don't care to take the time to try to find out. Myalgic encephalomyelitis (ME) is another name, which may or may not apply to me...For years, there has been ongoing controversy and attempts to settle on a name that properly acknowledges the severity of this condition. As with everything else that remains unresolved concerning this illness ... we cannot even get a name that works.”
Where naming the syndrome falters, so too does the CDC’s guidelines for even identifying it. In fact, according to the APA, “the CDC's criteria for patients to be diagnosed with CFS went from eight symptoms in 1988 to four out of eight in 1994. In 2005, the CDC broadened the criteria again, with efforts to assess symptoms, disability and fatigue.”
This inability to put a steady finger on just what patients and sufferers can expect from CFS is one of the reasons so few are diagnosed even though many more sufferers exist. As Campbell discussed in his story, sufferers of CFS show a variety of symptoms and severities of those symptoms through their time with the syndrome. A variety of factors can influence and impact the lives of with CFS (both identified and unidentified), including their lifestyles, climate and location.
The slippery nature of identification can almost be considered another symptom, a changing and morphing disease that just a fraction of sufferers know they have.
Further discussions of naming and identifying CFS from ME and CFIDS have spurred debate in the medical community when it comes to treatment and diagnosis. Some argue that all the different names actually fall into one bucket while others suggest they should be treated as wholly separate illnesses.
Dr. Byron Hyde, founder of the Nightingale Research Foundation described the difficult process of identifying either CFS or ME, and he says the classification of both in the same category was a huge misstep of the medical community.
“Do not for one minute believe that CFS is simply another name for Myalgic Encephalomyelitis. It is not. The CDC 1988 definition of CFS describes a non-existing chimera based upon inexperienced individuals who lack any historical knowledge of this disease process. The CFS definitions were written in such a manner that CFS becomes like a desert mirage: The closer you approach, the faster it disappears and the more problematic it becomes.”
Dr. Hyde went on to publish The Clinical and Scientific Basis of Myalgic Encephalomyelitis and Chronic Fatigue Syndrome with over 100 other collaborators, a book which was the first comprehensive and authoritative medical reference book on M.E. and CFS.
Hyde’s book was at the forefront of recognizing these syndromes as the first known cases of CFS which occurred in the early-mid 1980s. And just like GWI, the illness was originally only seen as a stress-related disorder, all in the head of the sufferer. But in 2010, further strides were made when the FDA put out an advisory not to take blood donors with chronic fatigue syndrome.
But if it’s all in their heads, then why would it affect their blood?
This dismissal was a huge first step to identifying that CFS and ME is not simply a psychosomatic disorders, but a real virus-based illness that can be transmitted to others.
At the time, the blood donor ban came because of report of CFS being linked to murine leukemia viruses. Though numerous studies showed this not to be the case, the publicity helped to have CFS thought of in a new light, as a biological disorder rather than a mental one.
“When an F.D.A. panel suggests that patients with CFS not donate blood, that’s going to impact the way doctors think about it,” said Mary Schweitzer , a former history professor at Villanova, who lives with the disorder and was originally misdiagnosed as having the flu when she first developed symptoms of CFS.
In Miriam E. Tucker’s article Chronic Fatigue Syndrome: Wrong Name Real Illness, she called for action from the medical community with specialists who can accurately identify CFS rather than simply treat it as a mental condition
"I think the most important thing for physicians to know is that while we don't have a diagnostic test or a proven treatment, there is now abundant evidence that in these patients there is an underlying biological process. Their symptoms are linked to problems of their biology and not imagined," said Harvard Health Publications Editor-in-chief and professor of medicine at Harvard medical School, Anthony L. Komaroff.
Further medical professionals backed up the sentiment, calling for recognition as a step towards finding the cure.
Dr. Jose G. Montoya, a professor of medicine at Stanford University Medical Center, who we will encounter again later on, reiterated Komaroff’s words, saying “obviously, the first thing you have to do is to see that it's real. That's not even a question for me anymore. Once you see that it's real, it's a matter of having the right technology...and a multidisciplinary approach," he said in an interview with Medscape Medical News.
But there are signs of hope. The Jackson Laboratory for Genomic Medicine in Farmington, Connecticut CFS has received a five year $3.3m federal grant
“to screen blood samples for potential immunological biomarkers of the disease.” Federal funding for research into CFS is expected to rise to around $7m in 2017.
The hope is that with research, CFS will become easier to understand and diagnose in patients.
"Of the many mysterious human illnesses that science has yet to unravel, ME/CFS has proven to be one of the most challenging," said Francis Collins, the Director of the National Institutes of Health.
NIH immunology specialist, Joseph Breen, described the importance of researching CFS at the molecular level "to figure out what's going haywire in these patients. Unfortunately, the symptoms mirror other symptoms. That makes it a very broad list.”
The recent commitment of funds and increased federal attention has given some hope to the millions of sufferers that not only may diagnosis of the illness become more routine but also that treatment may be found.
Clearly, there is a long way to go. Miriam Tucker’s research has uncovered
studies which demonstrated just how difficult it is for patients to even have CFS pinpointed by doctors and physicians.
In a survey of 256 patients, conducted by the Chronic Fatigue and Immune Dysfunction Syndrome Association of America (now called the Solve ME/CFS Initiative), 88% reported that they had been diagnosed by a physician. However, the majority saw more than four doctors before they received the diagnosis, with delays of 1-5 years for 36%, 5-10 years for 21%, and more than 10 years for 12%.
"This is a desperately long time to live with pain and impairment without validation," Carol Head, the association's president and CEO, said at an Institute of Medicine meeting in January 2014, the first of two open meetings held to discuss the forthcoming new diagnostic criteria.
Many ME/CFS patients—78%, in Dr Komaroff's referral population—are able to pinpoint the condition's onset to a flulike illness from which they never recovered. This differentiates ME/CFS patients from others who seek medical care for other fatiguing illnesses, including depression, whose responses are typically vague when asked when the condition started.
The distinction can also be elicited by asking the patient, "What would you be doing if you weren't ill?" Depressed patients typically won't have an answer, whereas ME/CFS patients will often respond with a laundry list of dreams deferred. "With depression, there is an apathy...People with ME/CFS are more angry and frustrated. They want to get better," Dr. Komaroff told Medscape Medical News.
The span of years and number of physicians consulted is staggering when the CDC estimates 1 million American currently have CFS, and this does not include those who are undiagnosed.
The road to recognition for CFS is only made more difficult when we consider conflicting studies and definitions which leave patients wondering what really is going on within their own bodies. The battle of names for the syndrome is one such struggle.
When CFS is misdiagnosed and misnamed as ME as Dr. Byron Hyde discussed, patients are once again lumped and pushed into categories where they don’t belong, denied proper care which can result in “long-term physical effects on many sufferers. In some cases this has resulted in death.”
Early in the article Chronic Fatigue Syndrome: Wrong Name, Real Illness by Miriam E Tucker, the author explains why patients with CFS are also calling for a name change.
“Many patients abhor the term "chronic fatigue syndrome" because they feel that it trivializes the condition,” Tucker explained, “which can render individuals severely debilitated, housebound, or bedridden.”
CFS and ME are often seen as interchangeable but PTSD is also an easy diagnosis ‘bucket’ for doctors to turn to, especially as some of the symptoms can seem very similar.
According to the General Psychiatry and JAMA/Archives journals, linking childhood stress or emotional instability to CFS does not make differentiation between PTSD and chronic fatigue any easier.
The studies suggest that if a person has undergone childhood stress such as abuse or extreme emotional changes at any point in their life, then they will likely develop CFS. Though there were no undisputed defining factors, the studies found a greater link between the trauma and later development of Chronic Fatigue Syndrome.
“The findings suggest that CFS and similar illnesses may result from the brain's inability to cope with challenging experiences,” the article reads. “Not all patients with CFS had experienced childhood trauma, but those who had tended to have worse symptoms than those who had not.”
But rather than make steps towards a clear definition, these findings only serve to muddy the waters between mental illness and virus for CFS.
The APA highlighted new research that says scientists are looking to the immune system as a starting point for CFS, arguing the poor immune health allows the disease to infiltrate the host with ease.
“While most people host countless dormant viruses from childhood without getting ill, CFS patients may react to them since two types of their immune cells — known as natural killer cells and T-cells — seem not to function properly, Immune dysfunction, then, might be to blame for the complex mix of symptoms common to CFS.”
The hope for establishing CFS as a curable disease rather than a brain disorder comes in a few forms. One such study even hypothesizes a link between GWI and CFS.
GWI and CFS do have very similar symptoms, and one study by Georgetown University entitled “Studies on Chronic Fatigue Syndrome with Gulf War Illness” seeks to prove the connection. Researchers hypothesized that certain proteins seen in the spinal fluid of CFS and Gulf War Illness patients could be the cause of these two conditions. As was determined with the weak and strong versions of the protein coding gene PON1, the connection between these diseases may just lie in the proteins created in the body.
However, there are also some incomplete studies which seek to prove a disconnect between PTSD and CFS when it comes to stress level and stress affected jobs.
A study by Kang HK, Natelson BH, Mahan CM, Lee KY and Murphy FM published on PubMed.gov produced findings that seem to disprove the link between PTSD and CFS. Though acknowledging the two have similar symptoms, the results showed an external link not related to wartime activities as the origin of CFS.
In the study, scientists compared two groups of 15,000 people. The first set consisted of Gulf War Veterans while the second was non-veterans. The veterans, unsurprisingly, reported higher rates of both PTSD and CFS. But the interesting difference occurred when the veterans were tested for these illnesses depending on deployment-related stress level. While we would expect both PTSD and CFS to rise (if they were actually related), only PTSD elevated with stress level. CFS only inflated in low-stress jobs.
“While deployment-related stress could account for the higher risks of both PTSD and CFS, additional factor(s) unique to the Gulf environment may have contributed to the risk of CFS among Gulf War veterans.”
It’s kind of the like rectangle and square analogy. Every square is a rectangle, but a rectangle can never be a square. In this context, CFS is often an attribute of PTSD but PTSD is not an indicator of CFS.
A study from the University of Maryland Medical Center titled Chronic Fatigue Syndrome states the intention of the federal advisory committee to change the name of CFS to ME-CFS “to more accurately characterize the complex nature of the disease.”
Changing the name would mean that the CDC would have to revisit its already broad definition of the disease, and in turn, the VA would have to change its requirements for acknowledging and assisting its veterans who suffer from CFS.
Though the change seems like a bureaucratic ploy, one agreed upon definition, would help sufferers to understand their illness and find a path to recovery. For some this recovery may mean simply curing pain or resolving sleep difficulties, but for others, the symptoms are much more intense.
The increased suicide rate in people with CFS is a factor that should be monitored and is yet another connection to the numerous cases of misdiagnosis of people with CFS being told they have PTSD.
Unsurprisingly, people with ME and CFS also have higher suicide rates as the disorders are very closely connected in their symptoms, but it’s the nature of these diseases that sets them apart.
A study from the Lancet highlighting this suicide risk among people with CFS produced staggering results in the study published in February of 2016. The study took place following 2000 people with CFS over a seven year period, monitoring their risk of mortality in living with the disease.
Though rates of death among the patients were about on par with that of the regular population in terms of diseases such as cancer, one mortality risk among CFS sufferers stood out: suicide.
“...the findings for suicide deaths were striking—five people died during the 7-year period. Based on the suicide rate in the general population of England and Wales, the expected number would have been less than one death by suicide. In other words, suicide risk was increased almost seven-fold.”
It’s clear that the pain and apparently endless suffering of CFS sufferers who lack an effective diagnosis or treatment can cause the despair that leads to suicide. One such victim of CFS/fibromyalgia is Lisa Lorden Myers, a writer and producer of the website Living with CFS & Fibromyalgia. Myers documents her experiences with the disease and keeps up to date with the latest in research and news in hopes to reach out to other patients who suffer as she does.
“Patients with fibromyalgia and chronic fatigue syndrome have an exquisite understanding of the pain, both physical pain and emotional anguish, associated with having a poorly understood, incurable disease,” Myers wrote in in her story titled Killing Me Softly: FM/CFS & Suicide. “An illness like fibromyalgia or chronic fatigue syndrome, which is often doubted or neglected by the medical community, the public, and sometimes family and friends, can present unique problems. Patients with FM/CFS can become victims of isolation and despair.”
Paul Anderson suffered from CFS for three years and was driven to multiple suicide attempts.
Before his first suicide attempt, Paul left a note for his wife, saying “I'm so sorry. I just can't stand the pain any more.”
Though Paul’s wife, Jill, was able to rush her husband to the hospital in time to prevent his death, it did little to get Paul the help he needed. As he deteriorated, “suicide became part of the language of their relationship.”
Jill was left helpless to watch as her husband’s health declined.
"We would do five miles, then it was four, three, two, one, until he could only walk 100 yards before he was too tired to go on," Jill said. “His pain increased, and by the time he died, he was virtually bedridden.”
After returning home on the day of the fourth suicide attempt, Jill found her husband again having taken a handful of pills to try and kill the pain once and for all. After having seen all the suffering he went through in his years with the disease, she decided not to call an ambulance. She was arrested and charged with manslaughter although she was acquitted by a sympathetic jury.
Despite such suffering, there may, at last, be hope. Some doctors and researchers believe they may have inadvertently stumbled upon medication which they believe could be a long-term cure for CFS. The research is still in preliminary stages, but the preliminary results have been encouraging.
When Dr. Andy Kogelnik and Dr. Jose Montoya of the Stanford Medical Center were volunteering in the clinic one day, they stumbled upon a new discovery that could change the way we treat CFS.
Kogelnik joined the Stanford Medical Center to complete his residency and a fellowship after a path entwined with biology and computer science, a knowledge and interest in both brought him to the medical field.
“I came at medicine from a very data centric perspective and a solving problems kind of perspective,” Kogelnik recalled. “As I went through my training, I was actually kind of frustrated by the lack of proper use of data in medicine. It almost got me kicked out a couple of times.”
As part of his studies, Kogelnik used “molecular technologies to measure thousands of parameters in the body at once, on a molecular level.” He eventually began applying what he’d learned in clinical settings through his 8 years at the medical center.
When volunteering in the clinic with Dr. Montoya one day, the two encountered three patients who were referred there for viral infections. Initially, the three cases seemed unrelated, just as any other patients in the clinic.
None of the three patients mentioned in their meetings with the doctors that they each had Chronic Fatigue Syndrome. At the time, Stanford had a policy not to see patients with the syndrome. Kogelnik recalled that each patient concealed that they had CFS during their visit, and most wouldn’t have usually made it as far as the clinic had they disclosed their CFS.
In this case, Kogelnik and Montoya each treated the patients with an antiviral medication, a treatment meant for viral infections but never CFS, which was thought to be a mental condition.
To their surprise, a few months later, each of the original three patients called back with shocking news. The antivirals had cured each of their respective cases of CFS. One patient even admitted he’d been suffering with the disorder for 15 years, and by using the prescription from Kogelnik and Montoya, they were cured.
Kogelnik recalled when he learned the patients actually had been suffering from CFS, he was awestruck. The use of antivirals to treat these patients proved that Chronic Fatigue is not a mental disorder, but a viral infection, one which can be cured with simple medication.
“That made me start thinking, ‘well, you know, there's something more here.’ And so we started digging in more and more. The deeper I dug, I found a community and started talking about all kinds of docs who were dealing with these kinds of things,” Kogelnik said. “The deeper I dug, the more I realized, that really there is this larger epidemic of issues that mainstream medicine has really just decided to side step.”
Though the treatment in this case was relatively quick and painless, Kogelnik insists that isn’t the case with every instance of CFS. As Campbell discussed once more, everyone with Chronic Fatigue experiences the syndrome in a different light and is affected differently by the illness.
“That gets into a lot of complexity very quickly, and so the idea of precision or personalized medicine comes straight to the forefront with these diseases. Here you have a set of heterogeneous patients and they have some similarities, but they have multiple things going on in multiple systems and it's not necessarily one size fits all, in terms of treatment,” Kogelnik explained. “There are these syndromes, and Chronic Fatigue Syndrome's, one of them, that probably are grab bags of these. We can start to cluster different people together, but we also have to take a little bit of a different view here, in that there may be multiple things, multiple systems that are out of whack or out of sync or dysfunctional in some way that have been affected by these illnesses. They're compounding on one another. Sometimes you get lucky and you can treat with one pill or one thing, but other times you get somebody sort of 30% better or 50% better, and then you’ve still got this other percentage to go to get them back to normal.”
Basically, Kogelnik is saying that the antiviral treatment is not going to work 100% of the time for everybody. But it can help a percentage of the way, with the remaining percentage supplemented with other treatments. This is not to say that antivirals aren’t the place to start. With the antiviral medications prescribed by Kogelnik and Montoya, we’ve seen the start of a revolution in the CFS community.
"In medicine, we tend to like the one-answer solutions. We're great at diagnosing things that have one problem, like a clogged artery,” said Kogelnik “What we're really bad at are complex diseases that have a systemic issue going wrong. With ME/CFS it's the whole system that's broken down, so there may not be one pill to cure everybody. It's more a matter of figuring out the imbalance in the system. We're not good at thinking that way in medicine. I think that's something that we need to change, particularly around this disease."
While other medical professionals are not yet totally convinced of how CFS and ME should be approached in terms of treatment, Kogelnik said they do accept that other doctors should be looking past a simple mental medical diagnosis for CFS, saying those days are in the past.
"People do think it's a spectrum of disease. We've settled on that it's an immune-related disorder, and there is potentially a subset that's autoimmune, a subset that's virally triggered, a chronic viral infection, and perhaps other triggers or stressors,” he said. “People are still kicking around whether it's autoimmune or chronic low-grade infection."
Sufferers have turned to the federal government in an effort to raise awareness and perhaps create funding for more focused research. Dr. Mary Schweitzer had this to say in testimony before the Chronic Fatigue Syndrome Coordinating Committee (CFSCC) of the U.S. Department of Health and Human Services in Washington, DC on September 12, 2005:
On July 4, 2005, sometime between 2:30 am when his mother kissed him good night, and 6:30 am when his father came downstairs, Casey Fero died in his sleep. His heart simply stopped.
Casey Fero was 23.
Casey was a charming, friendly tow-headed kid with blue-green eyes. He was first diagnosed with CFS at the age of 9, then again at 15. He was plagued by headaches, cognitive difficulties, muscle weakness, and exhaustion. In addition to the symptoms of a serious illness, he had to endure doctors who did not “believe” he was “really” sick, and teachers who saw in him only a shirker.
By the end of his short life, however, he was happy. He had just completed two years of community college and was looking forward to beginning courses at the University of Wisconsin, and he had acquired a summer job.
He had many friends, who came to his home for days after he slipped away to mourn the loss together. It was his friends who decided that he would have liked a tissue and blood bank as his memorial, and I will distribute flyers about that in a minute.
In his mother’s words, “Casey had bull dog determination. In his mind, he had overcome all illness and if he just worked harder he could do anything. He died with so much enthusiasm to live.”
Most people in this room are acquainted with Casey’s mother, Pat Fero, President of the Wisconsin CFS Association; you may also have met his father, Bruce, when the Wisconsin group hosted the October meetings of the American Association for Chronic Fatigue Syndrome. Pat testified before this Committee about a year and a half ago.
Pat has had CFS since before Casey was born. She has also been diagnosed with neurally mediated hypotension; Casey did not want to undergo the tilt table test. Without a doctor of his own - because even at the University of Wisconsin, there were no doctors who “believed in” the disease - Casey carved his own therapy out of over-the-counter and mail-order supplements such as ImmunePro and Co-Q-10.
Seeing the supplements, the coroner originally told Pat that this would be their first “steroid” death. That turned out to be mistaken (of course) – but it shows, once again, that nobody really believes that “CFS” is a serious disease.
Last Thursday, September 8, Pat received the coroner’s report. The University of Washington forensic pathologist found that Casey died of myocarditis – that is, he had heart damage. There was inflammation, and the tissue was full of viral infection. Casey also had old fibrosis, indicating that the viral infection was not of a new onset. The pathologist was “shocked” at this finding.
Casey Fero died of Chronic Fatigue Syndrome.
I turned to the literature for an answer.
Casey’s mother has NMH and passed out during the tilt table test. But there is nothing in the literature about NMH or POTS about sudden death, and the researchers I asked said they knew of no cases where someone died of those conditions. There are cases where a patient’s heart stops during the tilt table test but to my knowledge, all were revived. That is why the physician must be with the patient at all times during the test. And yet, Casey was lying down on a sofa. From what I know about NMH/POTS, if a heart stoppage were to occur, it would be while standing up.
There is a small body of literature in the United States on the possibility of cardiac complications from CFS.
Using an ultra-sensitive type of electrocardiogram [Halter monitor, making note of even the slightest abnormalities], Dr. A. Martin Lerner of Wayne State University has found evidence that EBV and cytomegaloviruses in CFS patients has caused heart damage. I do not know whether this research has been replicated, but under the circumstances, it takes on a new importance.
More recently, Peckerman et al published an article “Abnormal Impedance Cardiography Predicts Symptom Severity in Chronic Fatigue Syndrome,” in The American Journal of the Medical Sciences 326:2 (2003): 55-60. Unfortunately, Peckerman did not speculate on what would cause the abnormal impedance cardiography.
However, if you turn to the M.E. literature in the U.K., there is evidence of deaths in both adults and teenagers from sudden heart stoppage. Ramsay wrote about such cases, and in correspondence with Dr. Malcolm Hooper I was informed that he, too, was aware of sudden deaths in adolescents because of heart stoppage. Dr. Hooper has long been studying the hypothesis that M.E. is caused by an enterovirus (such as polio and coxsackie), because the illness behaves like enteroviruses do, it hides out and then pounces. There is no comparable research on enteroviruses in the United States (unless you count the sparse literature on post-polio syndrome).
Is this the price we have paid for detouring into CFS instead of accepting - no, welcoming - the robust research programs and existing literature in the M.E. community? If we had built on these studies, instead of squandering our time on measure of “fatigue” and a plethora of studies seeking “somaticization”, where might we be today? Had we been looking at this as a disease, instead of a psychological disorder, twenty years ago, would we now know enough to have prevented Casey’s death?
And if we hadn’t hidden what we already know from the public, would Casey have had a doctor of his own, perhaps even one that would have looked into the condition of his heart?
Pat Fero came down with CFS before Casey was born. His entire life was lived under the shadow of this disease - a shadow of widespread ignorance.
The pathologist was shocked to find heart damage. Why?
What is the virus that the pathologists found? HHV-6? Lyme? An enterovirus? Cytomegalovirus? EBV? Something completely new? Why is there no money for researchers studying these very questions?
We need the public to be told – NOW - that there is a serious disease out there, and that nearly a million people in the United States have it. They need to be told of the uncertainty about cause, prognosis, and cure. They need to be told of what they should be tested for to rule out other diseases. And they need to know that patients die.
Two months after Casey’s tragic, untimely death, most primary care physicians know little more about the disease than they did on the day Casey was born. That is criminal.
One year after this committee sent a list of suggestions (as is its task), to the Secretary of the DHHS, there has been no response. That is also criminal.
What does it take to impart a sense of urgency to this task?
Does it take the tragedy of a young man’s senseless death?
Carry Casey’s life and death like a banner. Take him to Congress, to the press, to the appropriate medical specialties. Let this be a turning point. Casey Fero died too young. Do not let his death be in vain.
While the medical community and federal institutions are still behind, Miriam Tucker, the author of the book on CFS, has uncovered a few studies which discuss treatments aimed to control CFS, but not necessarily cure it.
She writes that though there is not yet a cure, there are “numerous modalities for easing some of the common ME/CFS symptoms such as pain, disordered sleep, and gastrointestinal discomfort.”
Tucker recommends two videos which could be of use to those searching for more information about care and treatment called A Case-Based Approach to Chronic Fatigue Syndrome and Chronic Fatigue Syndrome: The Challenges in Primary Care.
Though these can be helpful, Tucker advises that there is a caveat.
“Use of both cognitive-behavioral therapy and graded exercise is highly controversial. The widely publicized 2011 PACE trial suggested that both modalities were beneficial, but those findings are rejected by most in the ME/CFS community, who cite numerous study flaws, including the patient selection criteria. The PACE trial was partly funded by the UK government agency that distributes disability benefits, and several of the investigators had ties to the insurance industry,” Tucker explains. “Some ME/CFS experts do recommend very gentle exercise, if the patient is able to do it, to prevent deconditioning, and may suggest counseling to help patients cope with the illness, but not with the expectation that either modality will improve the illness itself.”
This “very gentle exercise” must be little enough that no strain is caused to the sufferer which is pretty tough given that working out, by its very nature, puts strain on the muscles. In addition, a study by researchers at the University of Alabama at Birmingham and Johns Hopkins University School of Medicine shows the link between strain and inflammation in CFS.
In the study, 80 participants, 60 with CFS and 20 without the syndrome, completed an exercise of leg raises. Before the study, each reported their levels of pain, concentration abilities, lightheadedness etc. Then, the participants took to their assigned workouts. While some were completing real leg raises, others completed “sham” leg raises which did not put any extra strain on their bodies.
One day after their strain test, the participants were contacted by doctors to note their symptoms. The results were shocking. The results read as follows:
“Compared to those with CFS who underwent the sham leg raise, individuals with CFS who underwent the passive leg raise that actually strained their muscles and nerves reported significantly increased body pain and concentration difficulties during the procedure. After 24 hours, these same individuals who underwent the true strain also reported greater symptom intensity for lightheadedness and the overall combined score for symptoms. The individuals with CFS who underwent the true strain also reported more symptoms during, and 24 hours after, the true strain compared to individuals without CFS.”
To be able to prove such an obvious connect between strain and CFS was an interesting one because it further proved that CFS/ ME is not a mental abnormality, but that physical strain could affect a syndrome that is likely caused by a virus.
"These findings have practical implications for understanding why exercise and the activities of daily living might be capable of provoking CFS symptoms," said Kevin Fontaine, Ph.D., professor and chair of the UAB School of Public Health Department of Health Behavior and a co-author of the paper. "If simply holding up the leg of someone with CFS to a degree that produces a mild to moderate strain is capable of provoking their symptoms, prolonged or excessive muscle strain beyond the usual range of motion that occurs during daily activities might also produce symptom flares."
Because this study proved that even light exercise may be a difficulty for some sufferers of CFS, others patients may prefer an alternative option.
Smith of EmpowHer offers a simple yet effective alternative treatment that she came across when trying to treat her CFS.
“The only things that have helped me on my road to recovery have come from the arena of alternative medicine. If not for alternative medicine, I would not be writing this article, I would not be writing at all. I also would not be walking and talking like a normal person. I went low-carb in 2002 and that was life-changing for me. Within weeks my sensation of vibrating had diminished, my head fog had decreased, my sense of panic and anxiety became manageable, and my stamina had increased.”
While the change in diet did something to help Smith, she still reported other severe symptoms, at times so bad that she thought she was having a stroke. For that reason Smith continued with alternative treatments, most of which were prescribed by a new doctor.
Smith took various vitamins, including B12, vitamin D, omega-3 oil and a tincture with her doctor called “Deep Immune.” She reported other worthwhile treatments such as acupuncture which “kept a chronically inflamed arm functioning from month to month,” as well as castor oil, which she applied to painful spots on her skin.
Natural supplements also became a favorite of Smith’s during the time, and she recalled that she “used ear seeds and acupressure, and juiced vegetables. I drank liquid chlorophyll, and assorted types of teas.”
To work on her severe cases of vertigo, Smith used nasal lavage, and “no longer felt like I was going to bump into things, no longer felt like I was vibrating. Visually, the walls and floors no longer seemed to threaten to move in and rise up on me. My ability to concentrate and comprehend rose significantly.”
This return of focus is one aspect which many sufferers of CFS desperately seek when it comes to looking for treatment. The feeling of normalcy returns when they are able to walk in a straight line and focus on a single point without becoming dizzy.
For further inflammation, Smith saw chiropractors and continued to stay on her low-carb diet, “avoiding sugar and grains in particular, has helped to prevent a resurgence of debilitating symptoms such as cognitive fog, parasthesia (fancy name for mysterious physical sensations), weakness, fatigue, anxiety and panic attacks. I don't feel like I am starved for oxygen and don't have to suck in great gusts of air anymore.”
The results for Smith were near incredible. She felt her sense of normalcy returning and continued to find new treatments which could help her cure each symptom piece by piece. When a new one would arise, she turned to her doctor for another alternative treatment to keep the pain, fatigue and other symptoms at bay.
“I no longer felt like I had brain damage. No, let me correct that. I felt like I had less brain damage than previously. I can't say that this sensation is completely gone. But my cognitive difficulties have shrunk to a much more manageable size, and it's much easier to work around it than it used to be,” Smith wrote in her article.
The next possible treatment is one that may cause you to think twice, as it’s one that we mentioned in another chapter but for it’s negative effects for that group. Mestinon pyridostigmine bromide (PB) had been used to treat patients of CFS and has shown very few side effects, but a great deal of positive ones.
The article A Mestinon Miracle: Vagus Nerve Stimulating Drug Helps Long Time ME/CFS Patient Exercise from ProHealth examines the cases of four women from around the world, all of whom suffered from CFS. The main focus was a former lifelong athlete who was forced to stop working out when the pain induced by Chronic Fatigue Syndrome made exercising too much of a pain to bear.
But when her doctor, David Systrom of the Brigham’s Women’s Hospital in Boston, prescribed PB, her life changed dramatically.
The results reported after the subject began taking PB meant that after years of suffering, she was able to lead a normal life.
Although her treatment wasn’t focused upon a totally natural regime like Smith had used, PB was the path for her and numerous other women who suffered from CFS and turned to the pill.
“She hasn’t been able to exercise without paying for it for almost three decades but it was clear within a couple of weeks that something was different. She described suddenly feeling like “going for a run” – a feeling she hadn’t had in decades. She was tired afterwards but the dreaded PEM never materialized,” the article from ProHealth reads. “Then she went cross-country skiing – one of the most energy intensive exercises there is. She’s now running 3 plus miles a couple of days a week and working out in the gym. She has one side effect that’s apparently caused by the medication; after exercise sometimes she feels lightheaded. Other symptoms improved. She does take sleeping pills but now they seem to be working better and she was able to cut down her sleep by an hour or two a night. She’s able to work full days. Much of her alcohol intolerance has disappeared. Her very high resting heart rate has returned to normal. She’s not healthy but she’s improved enormously.”
The turnout was nothing short of miraculous for this patient, and it lead to the decision to attempt the same treatment for three Japanese women who exhibited symptoms and tests very close to that of the main subject.
Upon treatment, one woman’s three year case of ME/CFS vanished and as her pain faded, her muscle strength resurged. The second subject benefitted from the same results, as her “ten year problem with fatigue improved significantly with her “muscle-action amplitude” more than doubling.” The final Japanese woman who tried the treatment, who had been suffering for a decade and a half, finally felt the relief she had for so long been missing. These subjects noted great changes in the way their bodies responded to PB and noted the return of strength in each of the cases.
But why does PB work?
The disorder which PB is usually used to treat features symptoms quite similar to those of CFS. So, it makes perfect sense that similar treatments would work across the board for each of these disorders.
The other illness with similarities to CFS, called myasthenia gravis (MG) is an autoimmune neuromuscular disease and is a result of antibodies latching onto and blocking acetylcholine receptors at the neuromuscular junction. This stoppage of the receptors stop the acetylcholine from sending messages to the muscles therefore stopping any directions for how to move or handle or contract the muscles. This stoppage leads to (what also happens to be the most common of the symptoms of CFS) fatigue.
Though any variety of treatment can be the key for certain patients, the one that many, including Campbell, find to be the most important is the support of others both inside and outside the CFS community.
Campbell uses personal testimony to describe the importance of having those steadfast people in his life and notes that they come in a variety of forms:
“Support is another one of the key ingredients to living well with long-term illness. I see support as having three elements. One is practical help, such as grocery shopping, housecleaning or driving. Luckily, I did not need help of this kind very often. Even though there were times when I was so sick I could not go out of the house, for the most part I could provide for myself. ”
Campbell goes on to discuss other types of support including acceptance and understanding. He describes the various people in his life, including friends and family, doctors, fellow patients and role models who provided him with all different sorts of support to help him through CFS. Even if he never met them in person, the various kinds of help these people provided was instrumental in his recovery.
Self-support was another key aspect which Campbell describes and numerous other patients agree. The idea of simply believing that they possess the capability to triumph over the illness can be enough of a motivator to make real progress.
“I started looking for a self-help program for people with CFS almost as soon as I was diagnosed. My work at Stanford had shown me that self-help could be a powerful complement to medical care. I had seen people gain greater control, improve their quality of life and sometimes even change the course of their illness by taking responsibility for those things under their control. I thought that a self-help program for CFS could be even more valuable than the ones I had known for other illnesses. I reasoned that if self-help could be useful for illnesses with well-established treatments, it should be even more helpful for a condition with no standard or widely effective treatment. When I couldn’t find any such groups for people with CFS, I decided to start one. I thought that by getting together with other patients to talk about what each of us had found helpful and by supporting one another, we could gain some control over our illness.”
As Campbell progressed with his illness, he learned what worked for him and incorporated those practices into the group he led. He broke the ideas into easy steps to make recovery from CFS more like a to-do list than a long and grueling process (though it was). His outlook and strategies helped him to continue learning about Chronic Fatigue and how it affected the lives of those in his group.
The course relied on three ideas which have remained in the guidelines for the course to this day. Self help is the first of these pillars “the belief that how one lives with chronic illness can change its effects and perhaps its course.”
The next pillar is what Campbell calls an energy envelope which focuses on the “idea that illness limits energy, but that patients can reduce symptoms and the severity of relapses by accepting the limits imposed by the illness”
The final piece is to use various strategies to cope with the disorder. He stresses that because multiple symptoms are common with CFS, multiple and various techniques might be necessary for any given patient.
With an illness that is so diverse and has so far defied accurate diagnosis, there are still multiple treatments some of which work for one person but faill utterly for the next. That suggests the illness itself needs to be broken down into many further sub-categories, each of which will require a particular set of treatments. But for Andy Kogelnik, the process has to start with better education for doctors so that diagnosis – even of the general illness of CFS – can at least start a process of treatment.
“They're invisible to the medical system because we don't deal with them in the medical system. We say, 'Oh, you're sick, go see the specialist.' The specialist hones in on one little thing, and so you're left with this hodgepodge of diseases,” Kogelnik said. “There's a fair amount of infection disease drivers in there, but it's on the chronic side that we miss these things.”
“We're at a point in medicine we're we've just got the tools to be able to start to look at these things and change that conversation,” Kogelnik said.
New and puzzling clues are emerging from the dark maze that is CFS as more and more studies reveal new complexities.
For example, a study by Cornell researchers claimed to have found biomarkers which signify “chronic fatigue syndrome (CFS) in gut bacteria and inflammatory microbial agents in the blood.”
Because many CFS sufferers complain of stomach and other gastro- related issues, the findings fit in well with what some researchers had expected. In fact, this same microbe has been linked to other health issues including neurological and autoimmune issues.
The study was conducted when the researchers diagnosed ME/CFS in 83% of the total 48 patients with CFS and 39 controls without the syndrome, using blood and stool samples. The results showed:
“Overall, the diversity of gut bacteria was significantly reduced. In addition, there were fewer bacterial species known to have anti-inflammatory properties in ME/CFS patients, a similar observation seen in patients with inflammatory bowel disease. Furthermore, researchers identified specific markers of inflammation in the blood, which were likely due to intestinal permeability. Bacteria in the blood will trigger an immune response which can exacerbate symptoms and, thus, increase the severity of the condition.”
Though it is not known how the microbe works into the CFS equation, either as a result or cause, the presence of the microbe shows an abnormality and again shows the link of CFS to a disease either autoimmune or viral.
Another study by Julia Newton had the goal to research and explore possibilities of cardiac issues in CFS be researching red cell mass and volume of plasma in the body. Forty-one of the patients were those affected with CFS while 10 control subjects were used in the case. The study found that the CFS patients had lower end-systolic (the time at which ventricular contraction occurs) and end-diastolic (the minimum arterial pressure during relaxation and dilatation of the ventricles of the heart when the ventricles fill with blood) volumes together with reduced end-diastolic wall masses.
Though the results themselves might mean little to a layman, the conclusion section of the study lays the figures out to show a correlation.
“This study confirms an association between reduced cardiac volumes and blood volume in CFS. Lack of relationship between length of disease, cardiac and plasma volumes suggests findings are not secondary to deconditioning. The relationship between plasma volume and severity of fatigue symptoms suggests a potential therapeutic target in CFS.”
Targeting plasma is now just another area of research which medical professionals are recommending doctors explore. The possibility of plasma levels affecting CFS patients is not one that would have been thought of 30 years ago when CFS first appeared but progress is being made on multiple fronts.
When the average doctor spends between four and ten minutes with a patient, it is hardly surprising that many illnesses get dumped into convenient buckets, especially if they can be labeled ‘psychological’ because then the doctor can pass the patient along to someone else. But doctors and researchers like Kogelnik are calling for a medical system that can look below the surface and use the full range of scientific tools available to do a better job of diagnosis.
“This isn't just about chronic fatigue, but chronic fatigue happens to be a great poster child for why we need to do this, but there's many other illnesses out there that also have some of that complexity to it,” Kogelnik said. “We just ignored it. We act like diabetes is one disease or maybe two. That's how it’s learned in medical school. The reality of it is diabetes is probably a hundred different diseases when you break it down into molecular mechanisms, biochemical mechanisms. You really got to treat them. Each one of those should have a different pathway.”
In May 2016, supporters gathered under the Millions Missing banner from around the world “to demand greater recognition and research funding for ME/CFS.”
Protesters in Melbourne, Australia, London, England, Belfast, Ireland, Ottawa, Canada and in Boston, Washington DC, Seattle, San Francisco, Dallas, Raleigh, Atlanta and Philadelphia, U.S.A. showed their support with protest, in what was called the largest of it’s kind in the disease’s history. The protests were “coordinated by a group of dedicated advocates in order to highlight the long neglect of patients with ME and CFS. Millions Missing represents the missing millions of dollars of research funding that should have been spent to find a cause and a cure. Millions Missing also represents the millions of people who are missing their careers, their families, their friends, and their lives due to the ravages of a disease that can disable a sufferer for decades.”
An article in ProHealth showcased some of the major cities which participated in the Millions Missing campaign
“SAN FRANCISCO: More than one hundred people gathered in front of the Federal Building in San Francisco. Carol Head, CEO of Solve ME/CFS Initiative, exhorted the federal government to live up to its promises. “We're all here today, because it's time for our government to increase funding and support for this disease,” she said. Ron Davis, scientific director of Open Medicine Foundation, talked about the research he is doing. “We are very close to finding a biomarker,” he said. “We do NOT want to wait another thirty years.” And as his wife, Janet Dafoe, spoke about their severely ill son, Whitney, the crowd chanted “Whitney ... Whitney ... Whitney.”
BOSTON: Demonstrators assembled at the JFK Federal Building. They handed out information and gathered signatures on a petition to support more funding for ME/CFS. Members of the group met separately with senior officials from the Boston branch of Health & Human Services to ask for their support. Charmian Proskauer, President of the Mass CFIDS/ME and FM Association was upbeat about the meeting. “They were very well informed, understood our issues, and asked great questions. We see this as the beginning of an on-going relationship,” she said.
MELBOURNE, AUSTRALIA: In Melbourne, hundreds of shoes were placed on the lawn in front of the Victoria State Library.
LONDON, ENGLAND: In London, demonstrators held a silent protest at the Department of Health. Many stretched duct tape across their mouths to symbolize government neglect of ME/CFS patients. Ollie Cornes, a former software engineer, said, “The treatments the NHS provides are so ineffective as to be a waste of time and can be very harmful, which is why we are protesting at the Department of Health.” Like so many other patients, Cornes was unable to attend the demonstration in person. He was represented by a pair of red baseball shoes.
Sufferers with PTSD, GWI and CFS/ME have all struggled for recognition from the medical establishment. Dismissed as ‘mentally ill’ it is now more generally appreciated that each illness has a different diagnosis and the latest research suggests that even those generalizations do not accurately reflect what is happening to the body and the mind.
What we do know is that women are more affected with CFS/ME than men and most people don’t develop the illness until they are out of childhood, usually between 19 and 40 years old. And although most argue that genetic or environmental factors are to blame, no scientist or doctor have been able to select the specific gene or virus as the true cause.
What is not known but is suspected by some researchers is that the same viruses could provide a link to other illnesses such as Epstein-Barr, Human Herpes Virus-6 and mouse leukemia. Though a precise link between CFS or ME and Epstein-Barr, HHV6 and mouse Leukemia has not yet been identified, the results of current research will be discussed at length in Chapter 8.
Although it has taken only (!) 30 years to get to the state of knowledge we have today, it is clear that the medical establishment is at last beginning to take notice of the deaths and the pain and suffering of millions. The bureaucracy has responded as bureaucracies often do by first suggesting a new name for the problem. Instead of CFS or ME, a committee convened by the Institute of Medicine (IOM) has proposed instead Systemic Exertion Intolerance Disease, or SEID.
After reviewing more than 9000 scientific studies, hearing testimony from experts, and soliciting input from the public, the committee concluded that “the name ‘chronic fatigue syndrome’ has done a disservice to many patients,” calling it “stigmatizing and trivializing.” Myalgic encephalomyelitis (ME), they noted, “does not accurately describe the major features of the disease.”
At least 20 sets of diagnostic criteria exist, the committee noted, which has confused patients, clinicians, and their families, as well as researchers studying the disease. The proposed diagnostic criteria are more focused on “the central symptoms” such as a reduced or impaired ability to work and study, malaise after exertion, and “unrefreshing” sleep.
The study was sponsored by the US Department of Health and Human Services and the Social Security Administration.
“The essence of this disorder is that if patients with this disorder engage in exertion—cognitive, emotional, physical, whatever—that their symptoms are made much worse and often for a prolonged period of time,” said Ellen Wright Clayton, a law professor at Vanderbilt University in Nashville and chair of the IOM committee. The name, she said, reflects this. “We want to name it for what it is,” she said. “This is what the patients experience.”
“My son is sick with it, and when I tell people, they say, ‘I had that once,’ because they were tired once,” said Ronald Davis, IOM committee member and a biochemist who heads the genome center at Stanford University in Palo Alto, California, “ME is a better name, but there are no real data that fit the name.”
Davis hopes the report will convince all clinicians that they can diagnose the disease and that it is real. “I hope it will get rid of those who may not believe it,” Davis said. “They’ll have to keep it to themselves. It’s incompetence and it’s malpractice.”
Following the IOM report, the National Institutes of Health announced that it is refocusing research efforts on CFS and ME.
The attitude among many researchers has been “maybe this is an unsolvable problem, let’s just work on something else,” said Francis Collins, the NIH Director. “I’m happy to say we’re countering that attitude rather strongly here.”
Although no additional money is being thrown at the challenge, the problem is being given extra focus and oversight is being shifted to a more front line division.
“For the patient population this is huge,” says Robert Miller, a former coal miner who lives in Reno, Nevada, and developed the disease in 1982 after a bout with the flu. “One of the really key things is that we’re basically being moved out of Siberia.”
But there is a long way to go. Despite an intense international lobbying effort, a death toll that climbs every year and the agonizing suffering of millions, the amount of money devoted to the problem of CFS remains tiny. CFS/ME strikes more people in the US than Multiple Sclerosis, Lupus and many forms of cancer and yet funding from the NIH means that CFS get $7m, MS $98m and Lupus $93m Cancer gets quite a bit more, as it should. Understandably, CFS sufferers and their families are outraged at the puny sums NIH devotes to their problem and indeed the allocation of resources seems completely out of line with the amount of suffering involved.
But, with a higher profile and a new name, CFS/ME is finally coming out of the darkness and into the light where both money and additional research will undoubtedly follow. The work will be too late for many who are suffering today but I’m betting we’ll look back on 2016 as the year the tide turned. And in another 30 years when CFS and ME have been subdivided into perhaps dozens of specific illnesses, our heirs will look back on this period as one of the Dark Days of modern medicine. But there is hope. I’m confident that many of us who have suffered so needlessly for so long will at last have the prospect of a successful treatment.
This is Julia Hugo Rachel. Next week, I’ll be continuing my journey with my son as we struggle to find treatment for the illness that looks like it will kill him.
Julia Hugo Rachel
CFS and ME – Ignorance and Tragedy