by David S Bell, MD, FAAP*
August 17, 2011
This case history of a CFS patient whose symptoms include chronic pain is excerpted from Dr. Bell’s free e-book Faces of CFS - Case Histories of Chronic Fatigue Syndrome,* published in August, 2000, and downloadable at www.davidsbell.com. “Andrea’s Revolver” illustrates one aspect of cases he has followed since the mid-1980's. And the detective work continues. See the video of Dr. Bell’s recent 25-year follow-up on his patients.
Pain is a common problem in CFS, but it is also symbolic of the enormous challenge this illness presents to doctors and to patients. Patients can’t prove it, and doctors usually fail to treat it.
Despite the fact that pain occupies nearly all of their working day, doctors have a very poor understanding of it. Although we now have in our arsenal good medicines to relieve pain, we nevertheless try to avoid prescribing them.
• When a person who has just shoveled the snow off a long driveway complains of back pain, his doctor is likely to prescribe the same pain medications you can readily buy at your local Seven-11.
• If the snow shoveler’s back pain doesn’t abate in a few weeks, then the doctor realizes the problem might be worse than suspected. He refers the patient to a back specialist, which gets rid of the problem - for the doctor.
Most practitioners tell patients who have unexplained pain to seek help elsewhere.
Doctors are, as a rule, genuinely concerned about doing harm to the patient. This concern merges with another worry: losing one’s medical license by prescribing too many narcotics.
By law in New York state, doctors are required to write prescriptions in triplicate for narcotics and other potentially addictive drugs, a category of pharmaceuticals that just happen to be the most effective drugs for pain. These triplicate prescriptions help insure that powerful drugs won’t be over-prescribed, but the law is both time consuming and expensive - a nuisance for busy doctors.
More importantly, we doctors secretly suspect that someone spends their days counting the number of prescriptions we write, and worry that we could lose our medical licenses if there are too many carbons in our waste bins.
Aspirin presents none of these anxieties for us.
We never get into trouble from state regulators for offering aspirin to our patients in pain. The great irony is that aspirin may be more likely to kill the patient by causing a bleeding ulcer. But, ignoring that, we urge our patients to try aspirin or acetaminophen or ibuprofen. And if all of these fail, we usually suggest another mild medication, preferably one that the patient has never heard of.
Cancer pain, of course, is dire, and it is hardly surprising that oncologists (doctors who care exclusively for cancer patients) tend to have the most sophisticated understanding of, and compassion for, pain. They also do the best job of treating pain. When a cancer patient complains of pain, for the most part, the doctor opens his medical chest and says, in effect, take what you need to help with your pain.
Three weeks ago I had a toothache.
Like most men, I find this difficult to confess: My toothache hurt. By Friday, two days after it began, my toothache really hurt. On Saturday, it was unbearable. As I hobbled around my house holding my jaw, I found myself thinking, as if silently reciting a mantra: So this is pain.... so this is pain.
A friend gave me some serious pain medication, the kind that requires a prescription written in triplicate, and I was able to fall asleep at last. Even with the narcotic, however, the weekend was horrible. On Monday, I underwent the most wonderful root canal and my pain vanished.
The experience was an important lesson for me. I was reminded of the vast gulf between the subjective complaints of the patient and the objective doctor who listens to these complaints, the difference between hearing about someone else’s pain, their toothache, and my own.
Most important, I was reminded of the gulf many CFS patients must navigate every day.
I was able to see the end of my pain, even when the pain was at its height. I knew that on Monday my dentist would help me, not only because I can afford a dentist and have always paid my bills on time, but because my dentist understood completely that my particular condition was a painful one, and he had been nicely trained to alleviate it.
I see patients with pain every day in my practice. Pain steals in under many guises: the twisted ankle, the stomach ulcer, the spread of a malignant tumor. I try to respond given my best estimation of the severity of the pain.
I have studied CFS for years, and I still am unsure precisely where CFS fits along the spectrum of pain.
• Some of my colleagues accept as bona fide the pain that often accompanies the disease, and try their best to relieve it with medication.
• Sadly, most of them tell their patients that CFS is a minor illness; they advise them to get used to the pain until either the pain or the illness goes away. They may suggest aspirin.
Despite the fact that pain can be a prominent and even disabling aspect of CFS, it is the rare CFS sufferer who receives adequate treatment for it.
Andrea Thompson had pain, pain that surpassed virtually anything that I had seen before in my practice.
By the time Andrea walked into my clinic, she had already seen over 30 doctors. Her story was typical of most of the CFS patients who end up in the village of Lyndonville: Each of the doctors she had consulted over the long years of her illness were mystified when she recounted her symptoms, inexcusable now that CFS has been studied in this country for so many years.
Some of them performed studies and offered a multitude of conflicting diagnoses, from the common thyroid disease, which causes fatigue, to the more arcane reflex sympathetic dystrophy, an extremely rare and famously painful nerve disease. All of these diagnoses were wrong, since Andrea had the other symptoms as well: sore throat, headache, numbness and tingling in her legs and arms, as well as a kind of intellectual fogginess that made her feel perpetually “out of it.”
As months turned into years, and her doctors found it increasingly difficult to support their original diagnoses given Andrea’s wide array of symptoms, several of them began to suspect Andrea was a junkie who wanted pain medication to get high.
Talking to Andrea for ten minutes displayed the absurdity of these suspicions. Con artists have a certain talent, but Andrea was hardly a con artist. She was in pain.
Andrea was 37 when I saw her for the first time. Before falling ill in 1988, she had worked as an executive in a brokerage firm in New York state. Her education had been top-notch, and she had a Masters degree in business administration. She was married, had three children, and a nice house in the suburbs. Andrea and her family had been living the American dream - even if they didn’t have a Beamer.
Two years after her symptoms began, Andrea’s husband was promoted and transferred to Boston. Andrea was looking forward to taking a few months off work to rest, and to settle into her new home in Boston, after which she planned to look for a new job.
Once the move was complete, however, Andrea seemed to fall apart completely. In the beginning, at a loss to explain the problem, she and her husband blamed her rapid decline on stress. Yet, the move had been relatively stress-free; her husband’s company paid for the packing and unpacking of their possessions, for example.
Andrea’s dominant and most crippling symptom was pain, though she was hard pressed to pin-point its source.
Pain seemed to migrate through her limbs and even her face. But there were other symptoms, too.
• She had migraine headaches that were impervious to medication.
• She became hypersensitive to light, and took to wearing sunglasses even when she was inside her house.
• Curiously, odors of any kind made her feel nauseous.
• Mere sound, too, was often too much to bear. Andrea was an avid R & B fan and had a large library of recordings dating to the 1940s, but she could barely tolerate even the softest music.
It was as if every sense she possessed was too finely wired for life on planet earth.
Her first encounter with a Boston doctor ended as badly as her numerous encounters with doctors in New York. Andrea hadn’t expected a miracle – she just wanted something to take away the pain. Wearing sunglasses and asking for pain medication at the same time apparently made a poor impression.
She downplayed her pain when she met with a second doctor, with the result that he gave her a complete physical exam, ordered several laboratory tests and an MRI scan of her brain and neck, and performed nerve conduction studies. The lab tests were normal, and he prescribed mild pain medications and suggested meditation
The drugs didn’t touch the pain she felt, however, and Andrea was unable to meditate while in so much pain. She soon gave up trying.
She found some relief when a dentist prescribed codeine after she complained of facial pain, but he denied her additional prescriptions for the drug on the grounds that she might develop an addiction to it.
Doctors at a famous Boston pain clinic prescribed exercise and antidepressants, two therapies that simply ended up causing more pain. After two agonizing weeks, Andrea checked out of the clinic.
She had always been an attractive woman, but now Andrea looked ragged. She pleaded with several Boston doctors for more powerful pain killers, but her need for pain relief was proportionate to the degree of skepticism she encountered.
In fact, by now, no one she consulted believed she was in pain at all.
Her life was little more than misery compounded by the skepticism of the medical establishment and her friends. Not surprisingly, she began to doubt her own sanity.
• She became disheveled; her weight plummeted to 89 pounds. She had begun to look like a junkie, even to herself.
• She felt as if she barely knew her children anymore; they spent much of their time in day-care facilities.
• Her once-loyal husband began to display frustration and skepticism about her condition.
And always the pain - pain that doctors refused to treat. Worst of all, pain with no end in sight.
One day, Andrea loaded her husband’s revolver and put it in her mouth.
To this day, she has no explanation for why she didn’t simply pull the trigger.
The following day, she entered a psychiatric unit of a Boston hospital, where she remained for three weeks. The psychiatrists talked to her about “relocation stress,” childhood traumas, and “displaced” anger.
Andrea felt she was the only person who saw the problem clearly: Get rid of the pain, she kept telling the psychiatrists, and she would be okay.
Finally, her pleas were heard.
The psychiatrists prescribed methadone, a synthetic form of morphine that is used to manage morphine addiction, but has the same pain-deadening effects as morphine. The drug worked. Slowly, Andrea began to reconstruct her life.
What was causing Andrea Thompson’s extraordinary pain?
Usually, pain is associated with tissue damage from injury. The pain sensation serves as a warning, preventing further damage to the victim. This sensation travels on nerve pathways from the injured tissue to the brain.
Sometimes, pain is due to damage in the pain receiving areas of the brain itself. In this case, the disease is to the actual pain processing centers, even though the body mistakenly interprets the sensation of pain as if it were coming from somewhere else.
Malignancies involving the thalamus, a part of the brain that helps to process sensory impulses, can cause this kind of pain. But Andrea Thompson had no malignancies, and the MRI scan I ordered of her brain proved to be normal.
A third cause of pain is known as “sympathetically mediated” pain - pain that derives from damage to the autonomic nervous system. The autonomic nervous system controls our breathing, body temperature, blood flow, and heart rate - all those functions we can’t control by sheer will; body functions we barely even notice.
One of the diseases associated with this kind of pain, “reflex sympathetic dystrophy,” was the nerve ailment proposed by at least one doctor as the cause of Andrea’s pain.
Usually, “RSD” is the a result of an injury to a leg or an arm, or even a single nerve in that extremity. What is most curious about RSD is that the pain may be delayed for months or years, long after the specific injury had healed.
RSD is often accompanied by changes in blood flow, sweating, and, curiously, atrophy of the fingers. In 1988, I heard Dr. Paul Cheney, who had studied CFS among Nevada patients, describe what he called the “disappearing fingerprints” of CFS sufferers. The surface of his patient’s fingertips had grown completely smooth, entirely lacking the normal, distinctive whorls.
Dr. Cheney, a true disease detective, even remanded a few of these patients to a police station to find out whether they could be fingerprinted, but their fingerprints left no distinctive pattern.
When I heard about this bizarre discovery, I wondered whether it was possible to experience RSD as a body-wide disease instead of one confined to an extremity.
Though it remains controversial, some doctors believe that sympathetically mediated pain can be eliminated by blocking the nerve pathways, or even severing the nerve itself. In truth, doctors argue about the causes - and the treatment – of sympathetically mediated pain every day. Some of my colleagues believe it to be a psychological condition, although new research suggests otherwise.
I began to learn about the relationship between CFS and another puzzling condition called orthostatic hypotension, a failure of the body to adequately pump blood while standing in the upright position, from Dr. Peter Rowe and colleagues at Johns Hopkins.
Soon afterward I began working with Dr. David Streeten, an endocrinologist who had worked extensively with "orthostatic intolerance" - the inability of the body to tolerate the upright position.
There are only a few truly great doctors who stand out from the thousands of ordinary ones; they are the true masters of medicine. [The late] David Streeten was one of these masters. He had both a brilliant scientific mind and the perception and intuition of a superb clinician. If I were 30 years younger and fresh from medical school, I would have become his intern and really learned medicine.
But working with a master has its drawbacks. When I sent my CFS patients to see him for a consultation, they returned to my clinic with the news that he was much nicer to them than I ever was.
I met Dr. Streeten for the first time in February 1994 when I accompanied Andrea Thompson to his offices in order to observe his evaluation of her first hand. Until this experience, I was under the mistaken perception that I understood CFS. Dr. Streeten proceeded to teach me some of the basics that I had overlooked for 15 years.
I had always viewed chronic fatigue as the result of the chemicals released by a viral infection, and had concentrated solely upon looking for the infection that might have caused the condition.
Now I was seeing what could be the mechanism, regardless of cause, which might be causing the symptoms to persist.
The myriad of symptoms revolving around exhaustion, weakness, lightheadedness, and inability to concentrate were likely due to the inability of the body to circulate blood to the brain when standing, or sometimes even sitting.
When the CFS patient is lying down, the symptoms are improved, although not gone entirely. The disability of CFS has to do with the fact that we spend our non-sleeping lives in the upright position.
Dr. Streeten utilized MAST trousers in his experiments. The acronym stands for Military AntiShock Trousers. These inflatable pants can be blown up like an inner tube in order to create a veritable body cast from the toes all the way to the mid-chest. When paramedics need to transfer trauma patients suffering from fractured legs, MAST trousers are very handy because they become an instant cast and compress the lower body.
Dr. Streeten performed a deceptively simple experiment with his patients.
He bid them don deflated MAST trousers, then asked them to stand next to a wall of his examining room. As they stood upright in these somewhat peculiar circumstances, he monitored the patient’s blood pressure and pulse. When the CFS patient would become ill during standing, he would inflate their MAST trousers.
Upon meeting Andrea, Dr. Streeten measured the amount of blood in circulating in Andrea’s body and discovered that the level was strikingly low. Next, he tested Andrea’s blood pressure and pulse for 10 minutes while she was lying down, and they were normal. Blood tests drawn at that time for adrenaline and other "catecholamines" were drawn and later shown to be normal.
Then Andrea stood quietly with the plastic MAST trousers around her body but not inflated. Then the problems began. Within minutes she began to feel ill, her pulse began to climb, and the pain began to grow. By 10 minutes of standing her speech was slurred and she appeared a little drunk, swaying back and forth and her feet began to look purple. Her blood pressure dropped and heart rate increased.
Then the pain really kicked in.
She was confused. All she could do was to say over and over, “The pain...the pain...” While still standing, a second sample of blood was drawn from the line in her arm, again tested for adrenaline.
At that point, while Andrea was still standing, Dr. Streeten inflated the MAST trousers, causing symmetrical pressure around her legs, thighs and abdomen. I had often seen Andrea in this state before, a state where she looked as if she had been on a binge for days. But my teeth nearly fell out when seven seconds later she straightened up and looked around and said with a clear voice, “What happened? The pain is gone.”
I was as astonished as Andrea. Even Dr. Streeten was surprised by the magnitude of this response. He hypothesized that the pressure on Andrea’s legs and abdomen by the MAST trousers had forced a more vigorous blood flow through Andrea’s brain, and that this increased blood flow stopped the pain.
Andrea wanted to buy a pair of MAST trousers for home use. (Later on we actually tried this, but it turns out to be impossible to walk around with these things on which sort of cancels out the purpose.)
Dr. Streeten was suitably impressed by Andrea’s case. He submitted his MAST trouser experiment on Andrea and about 20 other CFS patients to a scientific journal. The editors rejected the paper, initially, because the patient was not sufficiently “blinded” in this experiment. In the world of science, of course, it is optimum for patients in experiments to be ignorant of the researcher’s modus operandi.
My question to the journal editors is this: How could we sneak Andrea into a pair of MAST trousers without her finding out about it? I can think of only one way: Render her unconscious with a general anesthetic. The problem with that, of course, is that it is very difficult for people to describe their pain, or lack of it, while unconscious. Sometimes modern medicine is bewildering.
The temporary stoppage of Andrea’s pain in Dr. Streeten’s office that day offers up no simple explanations or conclusions, just theories.
One simplistic theory is that because Andrea had too little blood circulating in her body, her blood pressure dropped dramatically when she was forced to stand for any length of time. When her blood pressure dropped, so did the flow of blood to her brain, which houses the mechanism that mediates both the sensation of pain and blood flow: the autonomic nervous system.
By inflating Andrea’s MAST trousers, Streeten was able to rescue, so to speak, blood that was trapped in her legs, and send more to her brain.
Still, the theory has by no means been proven. In the time that has elapsed since Andrea’s visit with Dr. Streeten, however, it has become apparent to me and to other doctors who study CFS that problems with blood flow to the brain, and the regulator of that flow - the autonomic nervous system – is responsible for both the exhaustion and the migrating pain of the disease.
Of course, myriad questions remain. Might the cause of pain in CFS be a result of a decreased blood flow through the thalamus? Is there an inherent abnormality of the blood vessels that causes the decreased blood flow through the brain?
A different theory of CFS pain centers on hormones. Studies have demonstrated that a number of different hormones are in insufficient supply in CFS patients. One of these is called growth hormone. The pituitary gland of the brain produces this particular hormone, and besides its primary job of making children grow, it has numerous functions in the body.
Scientists have discovered that there is also a second hormone, called vasopressin, that is deficient in CFS patients.
This hormone is essential to regulate the concentration of water in the body, as well as to regulate the size, or diameter, of the blood vessels. I had already determined that Andrea had levels of vasopressin that were much too low to be considered normal. A deficiency of vasopressin could predictably cause excessive thirst as well as an insufficient supply of circulating blood in the body.
As things have turned out, many patients with CFS experience both of these problems.
And this is where the trail leads us to endorphins, hormones produced by healthy people that act as the natural pain killers for the body. Interestingly, vasopressin is a substance that reflects the production of the natural pain-killing endorphins.
Could it be that CFS patients have low levels of the endorphins, explaining in part, why they experience so much pain?
The fact remains: Regardless of why it exists, or what causes it, pain that is both intense and chronic is a daily fact of life for a great many patients who suffer from CFS.
I began testing CFS patients in my office for changes in pulse and blood pressure when they stand quietly, and found that the vast majority have abnormalities. This is the reason that people with CFS may look healthy to outsiders, but they are unable to stand at the check-out counter of the grocery store. In fact, on occasion, some of them faint, or come very close to fainting, presumably because they cannot maintain good blood flow to the brain.
One day, I showed the blood pressure graphs to one of my best friends, David Lieberman, a substance abuse counselor. I pointed out to him that in addition to their blood pressure abnormalities, these patients often experience a racing pulse. David made the observation that these physiological signs are picture identical to the body’s reaction during withdrawal from narcotics.
Could it be that CFS sufferers are in a constant state of ‘narcotic withdrawal’ because they fail to produce enough of the body’s natural pain killers, the endorphins?
I remember a patient with CFS that I had seen almost 15 years ago. This woman had taken doses of codeine three times a day, every day, for nearly 20 years. She told me it boosted her energy, and provided relief from her symptoms of muscle and joint pain, headache, and trouble thinking. Although she had never increased her dose, I privately suspected she was addicted to codeine.
To see if she was addicted, I asked her to stop taking codeine for a period of two months, and for reasons I will never understand, she actually agreed to do so upon my request. During those two months, her symptoms of pain and fatigue were prominent and troublesome, but she bravely stuck it out.
What fascinates me, in retrospect, is that after 20 years of taking codeine, this particular patient displayed absolutely no signs of withdrawal or even a craving for the drug. She never developed tolerance to codeine, where it would become ineffective without increasing the dose.
Could it be that for these 20 years she was replacing her own body's deficient endorphins with daily codeine?
Many scientists would tend to dismiss this particular observation. The skeptics among them would probably assume that the woman was sneaking codeine on the side without my knowledge, and, frankly, I would be unable to prove otherwise, since I did not perform regular tests on this woman for the presence of codeine in her system. But, after years of practice, I believe that there is one factor in clinical medicine to which scientists rarely give credence: the bond of trust between doctor and patient. I tend to believe what my patients tell me. I may just be gullible.
Andrea has been taking methadone for four years now, and the synthetic morphine provides her with relief from her pain.
Curiously, she, too, has not required any increase in her dose, which typically occurs when people take drugs like morphine and methadone over the long haul. David Lieberman and I often talk about the possibility that CFS patients require narcotic drugs the way a diabetic needs insulin. The myriad symptoms of diabetes are caused, after all, by a deficiency of insulin, a natural substance that healthy people produce in adequate amounts.
I have wondered, as well, if blood volumes that are far too low are a clue to the cause of the major symptoms of CFS, particularly pain.
I frankly consider it embarrassing that so little research has been done on blood volume, blood flow, endorphins and other brain hormones in patients with CFS. If one tenth of the money that has been applied to psychological studies of people suffering from CFS had been used to study endorphins instead, we would have seen some progress in understanding this terrible disease.
One problem, of course, is that CFS continues to be so controversial throughout medicine that earnest investigators eager to study it are often stymied by the failure of government granting agencies to help.
This phenomenon is particularly unfortunate when one considers how many CFS patients are in pain each day. According to some researchers, more people in this country have CFS than all the people with AIDS, lung and breast cancer combined.
Currently, the disease is approximately 15 times more prevalent than paralytic polio was at the height of the polio epidemic of the late 1940s.
Research barriers have not only impeded my colleagues from understanding the disease, these barriers have prevented doctors from taking a hard look at the pain CFS patients experience, and from treating that pain appropriately.
Eventually, some patients just give up. The foundation of the Hippocratic oath is, “First, do no harm.”
By failing to treat her pain, doctors caused Andrea Thompson to spend half an hour with a loaded revolver in her mouth.
- Dr. David S Bell, MD, Lyndonville, New York, August 15, 2000
* 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. It may be downloaded as a free eBook at Dr. Bell’s website www.davidsbell.com. This information has not been evaluated by the FDA. It is general information only and is not meant to prevent, diagnose treat or cure any condition, illness or disease. It is very important that you make no change in your healthcare plan or health support regimen without researching and reviewing it in collaboration with your professional healthcare team.