"CFS, and the patients who suffer from it, have a great deal to teach those of us who have made medicine our life. Once researchers unravel the physiologic mechanisms of this illness, the discoveries will help unravel basic mechanisms of unnumbered other diseases." Dr. David Bell MD
(Dr. Bell's case history of a patient, Maggie, with chronic fatigue syndrome and fibromyalgia plus panic attacks is excerpted from Dr. Bell’s book Faces of CFS - Case Histories of Chronic Fatigue Syndrome,* published in August, 2000.
It shows how a expert diagnostician can tell the difference between a panic disorder and ME/CFS and fibromyalgia. In Maggie's case her prior doctors had zeroed in on symptoms similar to those found in panic attack (heart racing, face flushing, lips quivering) while ignored symptoms that were associated with ME/CFS (exhaustion, cognitive problems, leaden feelings in limbs, sore throat, IBS symptoms). Their diagnosis incorrect Maggie received treatments that did nothing for her - until she Dr. Bell who correctly diagnosed with orthostatic intolerance and ME/CFS/FM and gave her appropriate treatments - Cort)
I do not think a hidden neurosis or other psychiatric condition was lurking in Maggie's subconscious. Her panic was organic in nature; it came on as a result of physiologic, not psychiatric, factors.
Maggie came into my office about two years ago. By then, she had been ill with chronic fatigue syndrome (CFS) for ten years. She was fairly classic for the disease: joint and muscle pain; night sweats; a headache that would not end; severe fatigue. Despite the latter, she could barely sleep at night.
Naturally, the doctors she had seen in the past were unable to explain the strange combination of symptoms that seemed to possess her like some demon from a haunted past. Maggie, in fact, had been to so many doctors, including two psychiatrists, that she had lost count.
Doctors grow suspicious when patients tell them they already have seen several doctors for the same problems; they assume such patients are looking for a medical opinion they like, as opposed to a medical diagnosis. This is called, in the trade, “doctor shopping.” Maggie knew she had been typecast as a shopper, but she was searching for a diagnosis that made sense.
The symptoms that distressed Maggie the most had to do with her heart.
If she took a walk, she developed chest pain and become short of breath. Once, she told me, after taking a very long walk, she had seen a doctor the next day. She looked ragged, as if she run a marathon and come in last. Her resting pulse was too fast - 110 beats per minute. She told the doctor that she was afraid she was having a heart attack, but the doctor surprised her with a diagnosis of panic attack, instead. Panic disorder has long been viewed as a bona fide condition in which the patient experiences panic at inappropriate times.
Chest pain and shortness of breath were just part of Maggie’s ‘heart’ symptoms, however.
- She often had episodes when her heart would race. Her heartbeat wasn’t merely rapid, it felt as if her heart was flopping around in her chest, as if she had drunk a gallon of coffee, but she had given up caffeine years ago.
- Her face would flush and her palms would sweat.
- Her lips would quiver, her hands would tremble, and one of her eyelids would twitch.
Most doctors, if not all, would diagnose what I have just described as panic disorder.
But Maggie also suffered from a constellation of symptoms that were diametrically opposed; in fact, they were remarkably akin to the state of being comatose.
- She was exhausted to the point of being unable to walk. She showered seated on a lawn chair because she could not stand, for instance; she slept on the couch in her living room because she was unable, at times, to climb the stairs to her bedroom.
- Another coma-like symptom had to do with the way her intellect was dulled. While driving, she sometimes couldn’t remember whether green meant stop or go. Nor could she recall the names of members of her family, or remember a seven digit telephone number - even if it happened to be her own. She found the dialogue on afternoon soap operas incredibly confusing, like a foreign language. Everyone spoke so fast!
- Her body felt leaden, and curiously toxic; she felt a certain kinship with Chernobyl’s radiation sickness victims.
- Her bowels couldn’t decide between coma or panic; they alternated between constipation and diarrhea.
At night, when she awoke drenched in sweat, her heart pounding, it was as if panic was awakening her from her coma. There seemed to be no middle ground. Her doctors told her all these symptoms were 'due to stress.' To one such doctor, Maggie commented that, aside from the fact that she had lost her job and her husband, that she was bankrupt, and that she was often too weak to climb the stairs of her house, she really had no stress at all.
Our bodies respond to stressful events with the release of adrenaline from the adrenal glands. Adrenaline activates the body’s primal “fight or flight” response. It is a natural stimulant, resulting in a sudden rush of energy to deal with the crisis at hand. Panic is appropriate in times of danger, and adrenaline will give someone the strength to run, if that’s the best choice, or fight. A caveman confronted by a saber tooth tiger might choose to run; the student faced with final exams might choose to knuckle down and study.
Of course, after the adrenaline rush, we are left exhausted and weak, but the crisis is over and with a little rest we are good as new. This, in fact, is the key difference between panic disorder and CFS. Patients who have panic disorder may have relatively normal activity and energy when there is no panic. CFS patients never achieve a normal level of activity, no matter how much they rest or what measures they take to relieve stress. Take a person with CFS and put them on a sunny beach for a day, and their fatigue will not go away, nor will their panic-like symptoms.
Doctors and researchers have long experienced difficulty differentiating CFS [or the dysregulated autonomics of fibromyalgia (1,2,3)] from simple anxiety.
"Whatever else this disease does to the human brain, it includes agitation in its repertoire"
It is true that many persons with CFS experience anxiety. But because CFS patients tend not to have obvious, diagnosable psychiatric illnesses, I suspect that when panic occurs in CFS patients, the cause is physiological, not psychiatric. Whatever else this disease does to the human brain, it includes agitation in its repertoire.
That first time I evaluated Maggie, I realized her medical history was consistent with both CFS and panic disorder, however. In fact, if I were to follow the strict guidelines that the government has laid down regarding CFS, I would not diagnose Maggie with CFS. That’s because research criteria in use today are designed to create a homogeneous population of CFS patients for research purposes.
From clinical experience, however, Maggie’s tender lymph nodes, sore throat, endless headache, and intellectual difficulties were not consistent with a diagnosis of panic disorder. From a clinical point of view the most accurate diagnosis I could render for Maggie was both panic disorder and CFS.
Maggie didn’t really care about the label; she wanted to get at the basis of her symptoms.
Together, we decided to try some of the newer tests for CFS, tests involving the autonomic nervous system. In particular, we would look at the blood vessels and the heart’s ability to pump blood throughout the body. By the time I met Maggie, I had already learned that many CFS patients had low levels of circulating blood in their bodies, an abnormality sometimes accompanied by pulse and blood pressure changes when standing.
In many medical illnesses, the patient will feel ill regardless of whether he or she is lying down or standing up. With CFS, patients will say, "I feel so exhausted I have to lie down." For 15 years, nearly every patient with CFS has said this sentence, and I, as well as most other doctors, have misinterpreted it. No wonder this illness is so poorly understood. People should not be tired from standing up for 15 minutes.
The terms "fatigue" or "exhausted" don't even really describe the unpleasant sensation experienced. "Collapse" is more accurate. What the patient with CFS is really saying is "If I don't lie down, I am going to collapse." Medically, the appropriate term is "pre-syncopal," meaning the state which occurs prior to fainting.
Maggie agreed to allow me to test her orthostatic tolerance, her ability to function during quiet standing.
During a physical exam, she had two abnormalities:
- Fibromyalgia tender points in her muscles
- Difficulty maintaining her balance standing with her eyes closed, a common finding in patients with CFS.
The first test we did was very simple, a test of concentration done on two different days, once while standing for 15 minutes and once after lying down for the same length of time.
One of the most prominent symptoms of CFS is the cognitive impairment. Patients will have difficulty with confusion, short term memory, doing simple calculations, and remembering names or telephone numbers. Sometimes this is so severe that concern is raised about dementia or senility. The hardest thing for a patient with CFS is "double tasking."
My son has amazing powers of concentration. He can be in his room doing his homework, listening to rock-and-roll music while the TV, without sound, shows a football game. I made many vain attempts to inform him that it is not possible to do his homework that way, but his grades were good, and studying this way seemed to do little harm. He was able to "multiple task," just like I do when I talk on the car phone, drive, and find a quarter for the toll booth, all at the same time. For my son, he probably used 80% of his brain studying, 19% enjoying the music, and 1% watching the football game. Most shows on TV, the sitcoms, can be enjoyed with even less dedicated brain function.
Double-tasking is one of the first things to go in CFS.
The test we gave to Maggie was the coding test of the WISC, a standard intelligence test. In this test, a series of simple codes are made for numbers between 0 and 9, the code printed at the top of the page. The test involves finding, or remembering, the codes for a list of numbers, and scoring is done by adding up the correct coding accomplished in two minutes. It is an annoying little test.
After lying down, Maggie did the test, getting 55 right in two minutes, a normal amount. A week later we repeated the test after standing, and she was able to get only 40 correct, a 20% drop. We have been testing this with healthy persons and found that, in general, they do as well standing as after lying down.
Maggie's cognitive problems are related to the position she is in before the test. When horizontal, she does well; after standing, she is dull as a melon. This correlates to another curious detail often found in the history-taking of someone with CFS - they read lying down. Most healthy persons prefer to read sitting up.
We measured Maggie's circulating blood volume at a nearby hospital, and like 80% of our CFS patients, she was low, or as she would later say, "down a quart."
This finding seems to mesh with the difficulty standing; gravity makes it difficult for blood to get up to the brain, and if the circulating blood volume is low, it should affect brain blood flow. And the mechanism of CFS may be that simple: decreased blood flow to the brain. We don't know if animals can get CFS, but if they do, giraffes would be in big trouble.
The next test was the most exciting for me, the most unpleasant for Maggie. We call it "orthostatic testing" and it can be done in any doctors office.
It is a simple test, perfected by Dr. David Streeten (4), and described in Andrea Thompson's case.
The patient lies down for 10 minutes during which the pulse and blood pressure are taken. Then during a half hour of quiet standing the pulse and blood pressure are measured every five minutes along with observations of what the patient is experiencing.
Maggie's blood pressure and pulse were normal while lying down, and she was feeling relatively well - it was a good day for her. We measured the amount of adrenaline in her blood while lying down and it came back at 350, a normal level. The first ten minutes standing were not bad for Maggie, although she started feeling tired. Then she described pain in her legs, lightheadedness, and spiraling fatigue. Her palms were sweaty, and I saw her grow visibly pale. Most significantly of all, she became agitated, and, eventually, she became panicked.
As soon as Maggie lay down, we drew a second blood sample to measure her adrenaline again. I also gave her an infusion of saline water. Normally, saline is the last thing you want to give someone with high blood pressure, but I already knew Maggie had abnormally low circulating blood volume.
The elevation of her blood pressure had to be due to a constriction of the blood vessels, a compensation mechanism that would help circulate the blood to her brain. The fast pulse reflected the same problem: Her autonomic nervous system was trying to make her heart beat faster in order to circulate blood.
While standing, Maggie began to fall apart because her brain was deprived of blood.
A healthy person has several compensatory mechanisms that automatically keep blood flowing to the brain indefinitely. Maggie might look healthy to a casual observer, and the private disability company she had paid into for many years prior to becoming ill said that she was just fine.
But imagine Maggie trying to work an eight-hour day when she had such dramatic changes in pulse and blood pressure after just 20 minutes standing. I would definitely not want her to be my bank teller trying to make change after standing for two hours.
Maggie’s compensatory mechanisms were impaired. All but one, that is: adrenaline production.
The body produces adrenaline to compensate for impaired blood flow to the brain. In fact, it is one of the body’s most effective ways of improving circulation, which is why it is called the "fight or flight" mechanism.
Perhaps the most significant finding we made that day had to do with Maggie’s adrenaline. Her adrenaline level had risen to 1450, way above normal, when she had been forced to stand for 20 minutes. Maggie’s body was pumping out adrenaline like mad just to compensate for the crisis of quiet standing.
Adrenaline has one drawback: It causes anxiety.
Her panic was organic in nature; it came on as a result of physiologic, not psychiatric, factors
I believe now that Maggie’s panic was simply due to elevation of her adrenaline levels in an attempt to compensate for impaired blood flow to the brain. I do not think a hidden neurosis or other psychiatric condition was lurking in her subconscious. Her panic was organic in nature; it came on as a result of physiologic, not psychiatric, factors. Panic is appropriate during a crisis, but not during quiet standing.
The surge of adrenaline has several drawbacks. First, it doesn't work very well. If it had, Maggie would feel wonderful, like the burst of energy after having a cup of coffee in the morning after a good night's rest. It may be that the adrenaline keeps her up longer, but the price is fatigue. A healthy person will notice that after a crisis, a near car accident for example, the surge of adrenaline is followed by profound exhaustion. Chronic stress will leave a person drained and tired, but recovery occurs quickly once the stress is removed and good rest is supplied.
Maggie's fatigue is due to a combination of decreased blood flow to the brain and the surge of adrenaline her body produces to attempt to remedy the problem.
Suddenly, there were several medical diagnoses I could give Maggie.
Technically speaking, she had:
- chronic fatigue syndrome
- fibromyalgia pain and tender points
- idiopathic hypovolemia (decreased blood volume)
- postural orthostatic tachycardia
- orthostatic narrowing of the pulse pressure
- orthostatic diastolic hypertension
- hyperadrenergic orthostatic intolerance
Maggie was not displeased; literally overnight, she had gone from being a hypochondriac to having seven medical diagnoses.
Research into blood volume and brain blood flow is in its infancy. Nevertheless, while we are still in the dark as to what initiates these problems, breaking CFS down into its many parts may allow us to develop specific diagnostic tests for the disease.
Moreover, Maggie's testing results inspire theoretical treatment options. If we could somehow increase blood flow to the brain and deliver more oxygen to brain tissue, the symptoms of impending collapse should improve. The need for the adrenaline surge would be removed, so the postadrenaline exhaustion would also disappear. In concept, that is.
The co-existence in CFS patients of simultaneous coma and panic symptoms is no longer paradoxical.
The concept that a presumably psychiatric symptom - panic - would be helped by a drug which increases circulating blood volume is hard to believe.
Doctors and researchers who assume panic disorder is caused by psychiatric problems exclusively have been looking at the phenomenon in an overly simplistic way. The same chemical mediators that are present in emotionally stressful situations are also the same chemical mediators that help circulate the blood. A test would be to find a way to improve the blood circulation in the brain.
We started with a drug to expand her blood volume, and Maggie's response to treatment made me so excited that my socks started rolling up and down all by themselves.
Peter Rowe and his colleagues at Johns Hopkins made an important discovery in 1996 after discovering that patients with CFS would faint when tilted upright on a tilt table. They treated their patients with a drug called fludrocortisone (Florinef™), which works by increasing salt and water retention by the body, thus raising blood volume.
I had tried Florinef in many of my patients, and it improved their symptoms about one-third to one-half the time. In my experience, adolescents and those with milder illness responded best to this treatment. The drug helped Maggie: her rapid pulse slowed down, her sleep improved, and she felt slightly more energy. Her panic nearly disappeared.
The concept that a presumably psychiatric symptom - panic - would be helped by a drug which increases circulating blood volume is hard to believe. But it happened to Maggie. Her panic was clearly caused by CFS and its concomitant blood volume deficits, not the other way around. I was encouraged by these changes, but my socks had not yet started rolling, the surprise was yet to come.
Maggie and I talked almost every day during this trial, and we decided to push the therapy a little further by adding salt tablets, hopefully to augment the response.
Two days after adding salt tablets, Maggie returned to “normal.”
At first, I didn’t know what Maggie meant by “normal.” Did she feel a bit better? No, she was reporting complete recovery, normal for the first time in ten years. It was as sudden as a switch being thrown; she described her transformation as “awakening from a dream.” Her thinking was crystal clear. She walked for two miles that first day, smelling the flowers of spring. The whole feeling of illness, the malaise, was gone.
Unfortunately, her recovery was brief. Thirty-six hours later, she began slipping back to her former state, minus the panic, and she has remained in that particular jail cell since then.
We have tried 20 different treatments, raising and lowering doses of this and that, but she has not regained her freedom. In retrospect, the memory of 36 hours of freedom remains a cruel reminder of how nice it feels to be healthy.
The importance of Maggie's experience to me is a confirmation of a long-held belief that CFS is a reversible state, even after ten years.
Not just treatable by reducing some of the symptoms, but reversible. Her return to health was short-lived, but it occurred. If she had permanent damage to her brain, I do not think the 36 hours would have been possible.
What is so frustrating is the fact that this state could not be maintained, even though we tried treatments that pushed so much blood into her brain that her skull was ready to explode. Maggie has been a good sport about all of this. Each treatment attempt was discussed in detail and she was eager to take the steps.
While disappointed that we have not been successfull I see the problem a little differently now. By expanding blood volume it is possible to make some improvements, but not all the time. Even with Maggie's dramatic 36 hours, the response cannot be maintained even if the blood volume is returned to normal or even elevated levels.
The problem must be in the blood vessels themselves. They must be constricted so tight in the brain that little blood gets through. Perhaps it is one of the hormones that constricts blood vessels. Perhaps an infection of the blood vessels. Perhaps it is an excessive sensitivity of the blood vessels to adrenaline.
Hopefully, this story will soon have a new and more encouraging ending. CFS is a devastating physiologic process that undermines the body’s energy and the brain’s cognitive ability, but I believe that full recovery will someday be possible.
We await the next step, the step that will make Maggie’s precious 36 hours stretch into days, months and years. There is so much to study in this illness, and so much to be gained. Science needs to recognize the importance of CFS, embrace its difficult paradoxes, and get to work.
- CFS is not an isolated, rare illness nor is it an illness behavior for lazy people.
- The consequences of this illness weigh heavily on not only the victim, but also on family, community and society.
- Its tentacles seem to reach across boundaries, blurring the lines between other neurological and immunological disorders.
- Dr. David S Bell, MD, Lyndonville, New York, August 15, 2000
- Take the Orthostatic Intolerance Poll
- Dr. Bell on the Low (Low) Blood Volume in ME/CFS
- Dr. Bell's Home Orthostatic Intolerance Test
* This article is excerpted with kind permission from Dr. Bell’s classic book Faces of CFS - Case Histories of Chronic Fatigue Syndrome; © David S Bell, MD, 2000.
1. A major internet survey by Dr. Robert M Bennet, et al. in 2007 found that 45% of fibromyalgia patients suffer balance (orthostatic/dizziness) problems; 38% suffer chronic anxiety/panic; 42% suffer concentration problems/'fibrofog' and 40% also have chronic fatigue.
2. For information on fibromyalgia-related autonomic problems & management, see FM specialist Dr. Mark Pellegrino’s article “Those Dysfunctional Autonomics – Can Cause a Number of Conditions that are Part of Fibromyalgia.”
3. Autonomic nervous system dysfunction appears to explain many if not all nonpain features of fibromyalgia, according to a 2009 study by Cala Solano, et al.“Autonomic Dysfunction in Fibromyalgia Assessed by the Composite Autonomic Symptoms Scale (COMPASS)”
4. Dr. David Streeten, author of the book Orthostatic Disorders of the Circulation, was a pioneer of orthostatic disorder research & treatment.
Note: This information has not been reviewed by the FDA. It is for general information and is not intended to prevent, diagnose, treat or cure any illness, condition or disease. It is very important that you make no change in your healthcare plan or health support regimen without researching and discussing it in collaboration with your professional healthcare team.