Virginia T. Sherr, M.D.
Abstract reprinted permission from Practical Gastroenterology, 6:352-356
There has been both too much and too little written about Lyme and other tick-borne diseases. On the one hand (at least in the U.S.), they are seen by some as the source of a hundred unexplained ills; on the other hand, there is a dearth of published authentic narrative description, especially in regard to the complex and fascinating neurological and neuropsychological effects of borreliosis.
If physicians contract such diseases, experiencing the impact for themselves, while at the same time maintaining their medical and scientific knowledge and detachment, a unique "double" narrative can result. This is Dr. Sherr's situation: chance made her a victim of borreliosis, but her will and energy and intelligence have made her an expert on it too, and both voices—the voices of the patient and of the physician-investigator—are conjoined in this vivid and important personal narrative.
– Dr. Oliver W. Sacks
I stared at the strange red ring forming perfectly on the skin of my left leg, noting the tiny, glistening black spot in the ring's red center. What is this thing? I reviewed my risks calmly—it's too small to be a spider or tick, I thought. I had never seen anything like it. Perhaps a mite. Not likely to be dangerous or lethal. I'll watch it and see what it does. When I checked it again in a few hours, the tiny black speck was not there. Going about my business, I felt pleased with my scientific brio.
Fortunate genes and Health techniques learned as a psychiatrist had gifted me with a strong immune system. I had almost never had any antibiotics in my entire life. I stayed healthy. There was no idea then, in the mid-1980's, of what was to come a decade later.
The painless rash circle likely was a spider bite, one physician said; that was the first time I worried a little. In my experience as a physician and growing up around biologists and entomologists, this was not what I expected from the bite of the few local spiders that humans have to fear. And as a long-time gardener in a wooded area near my Pennsylvania home, I co-existed with multiple varieties of arachnids, all of which fled from my approach.
Unusual Things Begin To Happen
In March 1987, as I was preparing to conduct a big public event to be held in August, I endured the first head cold I'd had in 20 years—odd, I thought. In June, friends invited my husband and me to Cape Cod. I had known I was tired. I was surprised to find myself falling asleep while talking with patients in my office. I told myself I was lucky to have planned a vacation.
While driving northward, unusual things began to happen—I broke out in a generalized rash for the first time in my life. The itching was incredible. Suddenly my sinuses and throat began to get sore and then feel normal, then sore again in a seemingly rhythmic way. I felt weak and was exhausted on exertion. Returning to Pennsylvania, however, I gradually felt much better and was relieved when the daytime sleepiness tendency totally cleared up within two months. I guessed that I had come down with the newly described Chronic Fatigue Syndrome and recovered. By August 1987, 1 was back to full speed.
In the summer of 1989, 1 journeyed to Japan. Mid-flight there I experienced a splitting headache. Twenty-four hours later, I experienced a generalized rash and a sudden, complete, 3-hour duration, adynamic paralytic ileus. There was distention and a total loss of peristalsis and bowel sounds. This spontaneously resolved just in time to prevent my visiting an Emergency Room in Hiroshima. Then to my amazement, a second phenomenon occurred. Because I was thankful to be restored GI-wise and to be unafraid now to eat, I spent the next 2 weeks loading up on all the spaghetti that I could find in Japan. And nothing happened: no elimination, no bloating, no discomfort of any kind. I took Metamucil daily and even took some laxatives, just on principle. Nothing. Later, back home, a small normal elimination was my only souvenir of an experience that I would not have believed if someone else had told me. I consulted a proctologist who didn't laugh. He said, "I have only known one other person with such a history and he also was a physician." No other explanation has ever been forthcoming.
In 1990, there was another red ring, this time on the skin of my forearm. Again, there was a tiny, glistening black dot in the center of the bull's eye. More knowledgeable by now of Lyme disease and of the miniature forms of deer ticks. I sped to the nearest doctor, keeping my forearm outstretched. Once more I was told it was a spider bite, and that the black dot which lifted off easily was not a tick. My Lyme disease tests were negative and I felt well. But by the next occurrence, I thought I knew better than the tests. When in 1991 and 1992 the red circles reappeared, and although I still felt well, I turned to an infectious disease expert.
The specialist was barely polite. His attitude was dismissive and annoyed. He implied I was wasting his time. The ring had faded by the time I got an appointment. He said it was not possible for the same person to have had two Lyme disease bull's eye rashes over time. He said he would not treat non-symptomatic deer tick bites, even if he were convinced the rash was Lyme related. Anyway, "Only 10% of those bitten by infected ticks get Lyme disease."
I was embarrassed and angry. Early in life, I had been taught the ways of scientific observation, I knew I had recorded things carefully. I thought being a physician also would add to my strange data's credibility but it was not to be. I decided to take a wait-and-see attitude, resolving not to play doctor to myself nor subject myself again to such humiliation.
Symptoms Become More Severe
Several years later, I discovered yet another rash, a red blotch with the tiny black pearl in the center. The same night I went to bed feeling fine, but was awakened in the middle of the night by severe arthritic pains over my entire body. The pain was sudden, dramatic, and excruciating. I managed to return to sleep; miraculously the pain was gone when I woke the next morning! Nothing like this had ever happened to me before. Because of its brevity, the episode of joint pain was of little interest to the physicians whom I consulted. I noted it in my log in 1992 and, feeling well, nearly forgot about it myself. Later that summer and fall, however, I began to have episodes of major weakness and was exhausted on exertion. This lasted three months and I attributed it to my having discontinued the once daily 1/2 tab lorazepam 0.5 mg used to quell an arrhythmia during the previous six years.
In 1994, at age 62, 1 suddenly began to experience general arthritis. No one on either side of my family had experienced arthritis until extremely late in life. My father had lived to 101. Once again suspecting Lyme disease, I consulted a rheumatologist, only to learn that with no other obvious Lyme disease symptoms, he did not consider it a contender for the diagnosis. No one seemed to be interested in my repeated history of red bull's eyes (erythema chronicum migrans—the rash diagnostic of Lyme) or episodes of other transient, bizarre problems. Elisa and Western Blot tests done in conventional labs remained negative. I was not visibly sick and continued working. I was reassured by others that "we all experience stress in different ways." I felt patronized.
The cardiac arrhythmia present since the mid1980's suddenly intensified in August 1996 for no clear reason and didn't respond to any anti-arrhythmics. About this time, I started a support group for fellow sufferers of Restless Legs Syndrome. My legs troubled me at night, resulting in an escalating insomnia.
On return from a vacation in Costa Rica, in February 1997, 1 underwent a planned vaginal hysterectomy prompted by intermenstrual spotting. My hormone replacement therapy had no longer kept my menses regular. During the operation, I lost blood, which temporarily reduced my usually adequate hemoglobin from 14 to 8 gms. I recuperated normally for one week—hemoglobin back up to 10, when suddenly I was hit by a profound bone-deep chill, frigid hands, florid arrhythmias, and total weakness on exertion. I collapsed.
A Trip To The Emergency Room
On return from a vacation in Costa Rica, in February 1997, 1 underwent a In my first ever trip to an Emergency Room as a patient, I begged the doctors there to admit me—they refused because my "numbers were good." Next day, my cardiologist admitted me to another hospital for evaluation. I tried to explain my prostration in the presence of "good numbers" by rambling on to the admitting doctor about trivial stresses. I realized much later that I was trying to make sense of it myself, I was grasping at straws for an explanation of what was happening to me. I had extreme body weakness and severe multiple, specific muscle pains and tenderness that I only can describe as unearthly.
On return from a vacation in Costa Rica, in February 1997, 1 underwent a Over the months which followed, I tried to explain that I was in serious difficulty to one physician after the other, as I gasped for breath on exertion, had painful muscle spasms in the thighs, face, and posterior shoulders, experienced deep, aching, burning pains in the hamstring muscles on sitting, had general weakness and malaise, ringing in the ears, super-sensitivity to touch, smell, and sound, icy hands and Restless Legs Syndrome. An overwhelming, general burning, sweating malaise began in the afternoons, peaked in the evenings, and was mostly gone by 10:00 p.m. There was seldom a headache and no fever. I asked my doctors if this diurnal pattern could be related to malaria. Now I no longer even had the energy to read. I was terrified but not depressed.
On return from a vacation in Costa Rica, in February 1997, 1 underwent a Finally, I could no longer sit in any chair, even soft cushioned ones unless I sat on the very edge of a seat so my tender hamstring muscles did not touch the chair pad. The doctors looked at me like a whiner—as if my story was that of The Princess and the Pea. "But you look fine," they said. "You know, hysterectomies are hard on a woman's emotions."
When physicians asked me how I felt, they could not identify with my description of the general malaise. When I explained that my muscles were weak and withered to the point of fragility, that my hands fumbled and were visibly shrinking, that there was loss of position sense, escalating hypersensitivity to touch, and that I had aged 10-15 years in four months, doctors repeatedly reassured me I was mostly OK and still looked just fine. "This isn't just about vanity," I explained to deaf ears.
My family doctor and I now thought I was suffering from the after-effects of the iron deficiency and of a bronchitis that I developed in April 1997. Escalating my iron intake gave some improvement in vitality and the RLS, but did nothing for the muscle wasting, malaise, and painful fibromyalgia-like symptoms, which were increasing daily. My immune system barely seemed to be working.
A Doctor For Each Symptom
I have a solo psychiatric practice, and as the illness rapidly weakened me to the point of near-helplessness and premature retirement I struggled to prevent that eventuality. Urgent medical help was sought from my cardiologist for the rampant exertional arrhythmia, my ophthalmologist for new cataracts and dimming vision, a sleep disorder specialist for escalating RLS with resultant insomnia, my family doctor for acute bronchitis, an internist for malaise and weakness, a physiatrist for fragile, easily tom tender muscles, Ear-Nose-Throat specialists for the whisper that my voice had become, an allergist for the same, and finally back to the rheumatologist. The latter saw me in crisis after a nightmarish experience.
In June 1997, just days prior to my youngest son's marriage, I began to develop blatant tremors and shakiness, felt painful bladder spasms and a diurnal, aching malaise to the extreme. I lost more of my sense of position, especially in my hands—grasping the air when reaching for familiar objects. There was and is a sense of being unaware of whatever was held in my left hand although I had no trouble feeling the object. The middle two fingers on my right hand intermittently twitched uncontrollably. Fingers on both hands fumbled and could not pick up small objects. The diurnal pattern to all this was clearly defined. From 1:00 PM to 10:00 PM, I felt as if my muscles and nervous system were on fire; a sense of panic playing around the back of my mind came forward. With all of this, I determined to make it through the wedding ceremony. I barely did. Stress does strange things, I was told.
By the following week, I could no longer sit down because of the escalation of burning pain in the thigh's hamstrings. I worried—how could a dynamically oriented psychiatrist possibly manage without sitting and talking?
Then occurred a dramatic lack of confidence in doing even the routine things I love to do and still was able to do—a daunting physical sense of psychologic timidity. Seeing the usual full day's number of patients was unthinkable because of this alone. I felt as if the self I knew was dissolving. I found myself impulsively irritable at inappropriate times.
Finally, A Diagnosis!
On one occasion, when I did not have the strength to open the door of the refrigerator, I realized that not only my future as a physician but my life was teetering on the brink. I implored my busy new family physician to hear me out. It took a lot of his time but he stayed the course, "I think you have Lyme disease," he said quietly. I was stunned. He knew nothing of the bull's eye rashes years before. Hearing all of my symptoms, he had recognized chronic Lyme disease clinically; he recommended I see a neurologist and embark on daily IV antibiotics.
The neurologist ordered a lumbar puncture, but scoffed at the possibility of Lyme disease. Throughout the years, my Elisa and Western Blot blood tests for Lyme done at local labs had remained negative. By now I knew that positive tests for blood or cerebrospinal fluid were not necessary or even typical for the diagnosis of Lyme disease, but other professionals still counted on them for the diagnosis, and sadly, still do.
If a lumbar puncture was the ticket to health, I was eager for it. However, the outcome of the test on my cerebrospinal fluid was as I had expected, negative. It has been observed that in disseminated Lyme, only 12% of verified cases have cerebrospinal fluid serologically positive for that disease.
A PICC venous catheter was re-installed in my arm under x-ray guidance. The first line had sparked a septic phlebitis and had been removed after only two doses of IV medication. The IV antibiotic Rocephin was restarted.
At first, things rapidly got worse. A sense of urgency, fear, and uncertainty tinged everything. Several times I reached out to other physicians by phone at night because I could no longer tell whether what was happening to me was important or was trivial. Routine noises elicited startled reactions. I had a nurse stay with me when I was alone because I was so unsure of what was happening neurologically. I later learned this flurry of symptoms was a Jarish-Herxheimer reaction to the kill-off of the spirochetes.
After Years Of Symptoms, Tests Turn Positive
At last I got "numbers." My Lyme IgM western blot blood test turned positive, and I had two strongly positive Lyme disease urine antigen capture tests.
Next, I sought help from a doctor who had special training in Lyme disease. Further blood tests revealed a positive test for ehrlichiosis and low positive antibody test for babesiosis, both also tick-borne diseases. At that time, six weeks of the 5 days a week IV antibiotic were considered the initial treatment for disseminated Lyme disease; in addition. I took doxycycline tablets by mouth for the ehrlichiosis. Months later, re-checks of blood work for ehrlichiosis and babesiosis turned "negative." Even later, however, testing in another laboratory revealed a high Liter for babesiosis antibody.
The Lyme specialist checked my previous brain MRI and wondered at the earlier analysis, "changes compatible with aging." It was hopeful to know that I might be able to stop the CNS Lyme injury in its tracks and that it wasn't Multiple Sclerosis.
Gradually, the IV antibiotics restored my vitality. I could read again. Some strength gradually returned; the RLS no longer robbed me of sleep. The medication costs of well over $ 1,000.00/week encouraged my own ambition to continue the work which I so much enjoy. However, I still could sit only on the very edge of a seat, not on my muscles that remained painfully hypersensitive to pressure.
The IV's were stopped at the end of the six-week period. Approximately 2-1/2 weeks later, the cardiac arrhythmias returned full force, along with the familiar intermittent exertional cardiac laboring, new areas of painful muscles, exquisite painful sensitivity on being touched. intermittent impulsive irritability, afternoon malaise, and general weakness.
IV therapy was restarted. Now I received IV Claforan 6 gms/100 ml throughout 12 hours of infusion via a portable CADD pump and experienced improvement of much of the pain and weakness with minimal side effects. My blood count and my liver studies were monitored weekly. The infusions had their own problems, but I found those were a welcome part of the price paid for a returning of strength as well as for salvation—of health and occupation.
Working with me then to maintain my functioning in conjunction with the generalist and the Lyme expert were visiting nurses, an exercise physiologist, and a masseuse who came to my home weekly as needed. I visited a physiatrist and a physical therapist whose twice weekly ultrasound and penetrating heat treatments were the only treatments which gave at least 24 hour relief from the painful hamstring misery. I consumed massive amounts of the appropriate nutrients and vitamins to help repair some of the damage done to multi-organ and immune systems by Borrelia burgdorferi, the microbial cause of Lyme disease, as well as the two other diseases. I rested between patients, lying flat. It was obvious that it would be incredibly more difficult to face this disease alone or with fewer support resources.
All Symptoms Related To Tick-Borne Diseases
Every bizarre symptom I personally have experienced, including many not even mentioned here, have been identified in some way by others to be associated with chronic, multisystem, disseminated Lyme disease, ehrlichiosis, and for the babesiosis which flowered seven months into the IV treatment for Lyme disease when I developed more severe malaria-like symptoms. The IFA (IgM) antibody test then was positive for babesiosis at a titer of 1:512. The IV antibiotics were stopped. Zithromax, Suprax, and episodic month-long bouts of Mepron were begun with each round leading to stepwise improvement in all symptoms. (Two and one half years into the acute phases of the diseases, my Lyme and babesia PCR's both are positive and my antibodies for Human Granulocytic Ehrlichiosis (HGE) are positive at the 1:160 dilution. While they are not curative for me yet, the antimicrobials allow me to function, assist my memory and give me enough energy to continue my office practice.)
At the time that symptoms of tick-borne disease appeared, they didn't seem to be a part of a pattern. Afflicted patients whom I see in my office often label themselves as stressed, neurotic, or hypochondriac. They are slow to believe, even with positive tests, that Lyme disease could be the underlying cause of disparate patterns of symptoms stirred up by normal life events—long plane flights, stress, even joy.
Unlike the Princess of the story, I already have a husband—one whose swollen sore knee, previously thought to he caused exclusively by a tom ligament, and recent surges of impulsive irritability are now relieved by treatment for Lyme and babesiosis diseases. He was one of the lucky ones. The diagnosis was confirmed and treatment begun via our family doctor's finding a positive Lyme Western Blot IgG test before the planned surgery was carried out. And finally, 2-1/2 years into my treatment with antibiotics, my blood Lyme disease polymerase chain reaction (PCR) test turned positive for the presence of DNA of live spirochetes.
I understand tick-borne pathogens to be remarkably psychic organisms. They can flourish in situations where any kind of excitement (mental or physical, positive or negative) exists. They wax and wane, submerge and then ambush as if they have minds of their own. I am not surprised that tick-borne diseases have been called fiendish.
My heart goes out to the Princess whose authenticity was questioned but who steadfastly maintained that a pea was under her mattress. Lyme borreliosis, ehrlichiosis, and babesiosis-inspired sensitivity to touch as well as exquisite muscle tenderness might have explained her situation as it does mine. The deer tick has made us sisters in and under the skin. Maybe what we both needed was not a prince, but an openminded physician. A healer who would listen carefully, consider infectious causes and prescribe any necessary long-term antimicrobial treatments for Lyme disease and its nefarious companions, including the rickettsia of ehrlichiosis and the red cell infecting protozoa, babesia.
The GI Symptoms Of Tick-Borne Diseases
Returning to more time in my practice, I began to evaluate people with the usual panic attacks, depression and anxiety, but often now these symptoms were in tandem with bizarre gastrointestinal complaints as well. This constellation ultimately was traced, to the great surprise of the patients and their doctors, to the new Great Imitators of the 90's—Lyme and other tick-borne diseases (TBD). The first such patient had undergone an exhaustive GI work-up which offered no clues to explain his intense abdominal pain.
Ronald is a Protestant minister. He: chose to see me, psychiatrist, because he had become certain that his unexplained epigastric pain resulted from psychologic identification with mother who died an agonizing death stomach cancer. was furious at my idea we could be looking infective process. It took considerable effort separate him self-applied label of "hypochondriac" and get accept advanced Lyme Western Blot antibody testing. These tests were done positive. then eager for the appropriate antibiotic treatment previously unsuspected disease. Antibiotics alone relieved gastric completely. Less irascible then, phoned new home in Alabama say efforts, "Nice call."
Some patients who later were proven to have had Lyme disease, had episodes of sudden, complete inability to swallow, or of a loss/gain in weight, or of having unexplained nausea and vomiting. I have seen three cases presenting only as a chronic morbid dread of the latter without actual emesis. I have seen several cases of Lyme hepatitis presenting as panic disorder. A few patients who think they are "somatosizers," have had bizarre bowel bulging which was easily palpable but which was gone by the time the patient reached the specialist. Patients have reported sudden changes in stool consistency from normal to, for example, immutably putty-like. The resulting obstipation defied any method of relief short of "digging" by people who never before had constipation. Abdominal pain with or without nausea and loss of appetite, sometimes was traceable to Lyme-inspired abdominal wall muscle spasms or 10th rib tenderness, or episodic difficulty in swallowing, or outright but temporary esophageal paralysis or diarrhea with or without bloody stools or episodic non-gaseous abdominal bloating—all frequently have been seen in cryptically infected patients. Except for the bloating, these are among the first symptoms to resolve when patients are properly diagnosed and treated for the underlying disorders. In approximately 60% of these people, there is no awareness of being tick-afflicted. Interestingly, in the three years since I have become Lyme-literate, I have only run across one other person who had an experience such as I had during the two weeks following my own adventure with adynamic paralytic ileus. She was a physician who, like myself, was reluctant to talk of the seemingly impossible disappearing act that happened within her.
The clinician is likely to note that the patient under his/her care for Lyme and associated TBD has abnormal liver function studies prior to the use of antibiotics. In a study in Hepatology 1996 Jun; 23 (6): 1412-7 in which all common causes of hepatitis were ruled out, 40% of Lyme-diagnosed patients had at least one abnormal LF test. GGT and ALT were the most frequently elevated LF studies. These abnormalities usually resolved after three weeks of the appropriate antibiotic therapy although the spirochetes were not eradicated in that period of time. Likewise, gastroparesis, gastritis, duodenitis, and swollen spleens are not uncommon findings in Lyme disease patients. Crohn's disease and Irritable Bowel Syndrome are potential hazards for people with chronic Lyme. Lyme spirochetes have been harvested and cultured from the bowel mucosa of such patients.
Child victims of Lyme are particularly vulnerable gastroenterologically. They usually have been labeled as school-avoidant whiners, truants, or chronic complainers. A child's academic future may be jeopardized at an early age by the stomach distress of tick-borne disease, the resultant inability to focus cognitively and frequent absenteeism from school. The classic paper here is "Gastrointestinal Pathology in Children with Lyme Disease" by Drs. M. Fried, P Duray, and D. Petrucha, Joumal of Spirochetal and Tick-borne Diseases, Vol. 3, No. 2 June 1996.
Of course, close follow-up of CBC and of liver function enzymes for those stabilized on long-term antibiotics is essential. Laboratory testing is important at the outset but is not diagnostically definitive for Lyme and other TBD. Since a percentage of patients are seronegative to antibody tests, polymerase chain reaction (PCR) and other DNA and RNA based tests are warranted. These tests, urine antigen tests, and the soon likely-to-be-certified blood culturing studies will help pin down the offending microbes. Local laboratories are unlikely to be geared to sensitive testing for Lyme borreliosis, ehrlichiosis (HME and HGE types) or babesiosis. I recommend a research quality laboratory be utilized. IGeneX Reference Laboratory of Palo Alto, CA and BBI Laboratories of CT and Boston are recommended. However, the diagnosis of TBD is a clinical one and is made by the Lyme-aware physician in the absence of positive lab reports only after a careful evaluation of the sufferer. Lyme and related tick-carried illnesses present with every possible symptom in the medical lexicon. The symptoms shift in kaleidoscope fashion from one hour to the next in the same patient and seldom present identically in two different individuals. Lyme is a multi-system disease. It was once said of another spirochetal disease, that "to know syphilis is to know all of medicine." It is apparent that to know all of medicine, one needs to get the GIst of all tick-borne diseases.
Fortunately for science, Oliver W. Sacks, Professor of Neurology at Albert Einstein College of Medicine and later adjunct professor of Psychiatry at NYU Medical Center relocated himself to these shores from his native England. He proceeded to combine his medical expertise with an investigative curiosity. This led to writings that shared with the public beneficial results of his considering each of his patients as a challenge to be fully understood and well served. Such teachings have enriched American medicine and delighted his readers world-wide. He is said to be the modern master of the case study.
Virginia T. Sherr, M.D. is a psychiatrist in private practice in Holland, PA.