The Medical Detectives Volume I

by Berton Roueche

  I understand now, having made some inquiries, how I happened to escape contracting Rocky Mountain spotted fever in spite of my unconcerned wanderings for so many years in an area in which the disease is historically endemic. It was, of course, in large part luck. But the odds were very much in my favor. It is estimated that even in heavily infested areas only about five per cent of the vector ticks are carriers, and it is a comforting fact that the dog tick (and its carrier cousins) is an indecisive feeder. It usually crawls around the skin of a human host for some little time before it chooses a place to eat. During those preliminary perambulations, it presents no kind of threat, and even when it settles down and thrusts its proboscis into the skin the migration of the R. rickettsii bacterium to its new host seems to take time—perhaps several hours. The moment for concern is at hand when the tick is not only attached but visibly enlarged, engorged. A well-fed dog tick is only too repellent a warning. I have never found an engorged tick on myself or on my wife or son, but I have removed any number from our dogs. They have varied in size, but the most gluttonous feeders have been about as big as a grape, and of a sickly leaden color.

  I was, as it turned out, far luckier than I knew in those years of imagined immunity. I may have been to some extent protected against Rocky Mountain spotted fever, at least in the earlier years, but its carrier is not the only toxic tick that finds a congenial home on eastern Long Island, and Rocky Mountain spotted fever is not the only tick-borne disease that is endemic in my neighborhood. This other tick is called Ixodes dammini, and it is host to the agents of two newly noted but otherwise entirely distinct diseases. A malaria-like disease called babesiosis is one of these. Babesiosis takes its name from that of the causative organism, a protozoan named Babesia microti, which, in turn, takes its name from that of its discoverer, the Rumanian bacteriologist Victor Babes. It is, in general, a relatively mild disease, and its victims usually recover without chemotherapeutic assistance, but it has a grave potential. Victims whose spleens have been removed or damaged, the elderly, and cirrhotic alcoholics are always at risk, and sometimes die. B. microti turns up as a tenant of I. dammini a little more often than R. rickettsii does of D. variabilis.

  The other disease that I. dammini may harbor is Lyme disease. Lyme disease is rarely fatal, but unless it is promptly diagnosed and appropriately treated its victims may recover from its acute assault only to give way later to a variety of debilitating chronic ills. I have encountered I. dammini only once, a couple of years ago, and, like my first tick, it was attracted to my wife. She found it settled on, but fortunately not attached to, her thigh after a walk in a weedy pasture. She lifted it off and showed it to me in the palm of her hand. It was a tiny thing. I. dammini is commonly described as about the size and shape and color of a poppy seed. That, to me, is an exaggeration. My—or my wife's—tick was about the size and shape and color of the period that ends this sentence. My wife supposed at first that it was a little scab or freckle. But it moved, and under a magnifying glass its eight arachnid legs were just visible. We twisted it up in a Kleenex and, on a hunch, took it the next day to a dermatologist in the neighboring village of Southampton—Bernard W. Berger, whom we knew to be an authority on Lyme disease and an active investigator into its nature. Dr. Berger gave it a glance, and identified it not only as I. dammini but also as an I. dammini nymph. It is primarily in the nymph, or middle, phase of its life cycle that I. dammini transmits Lyme disease to humans. It was well, as I say, that our tick had not yet attached itself: at least sixty per cent of I. dammini on eastern Long Island, Dr. Berger told us, are carriers of Lyme disease. That was bad enough. But I learned only the other day that ticks infested with the Lyme-disease organism can also be infested with the organism of babesiosis.

  The coincidence of the organisms of Lyme disease and babesiosis in a single obliging tick was first reported in 1983, in the New England Journal of Medicine, by a pediatrician named Edgar Grunwaldt, in general practice on Shelter Island. Shelter Island is a largely wooded island, some thirty square miles in area, that rises between the north and south forks of eastern Long Island. Dr. Grunwaldt was ideally situated to observe the comings and goings and the morbid proclivities of I. dammini. Although Lyme disease first caught the eye of science in 1976, in the little Connecticut community from which it takes its name, it has, like Rocky Mountain spotted fever, long since outgrown its regional origins. Lyme disease has now been reported in much of the continental United States, with deep and probably ineradicable roots along the upper Eastern Seaboard, in Minnesota and Wisconsin, and in California, Oregon, Utah, and Nevada. There is, however, no place yet known where its roots go deeper than in Dr. Grunwaldt's Shelter Island. In parts of the island, especially in some of its most idyllically pastoral areas, the incidence of infested I. dammini ticks approaches ninety per cent.

  "Shelter Island has been a laboratory for much of the investigation into Lyme disease," Dr. Grunwaldt told me in a talk we had at his home one Saturday afternoon—that being a time when he doesn't see patients. "We have the tick in abundance, and we have the disease. We also have babesiosis. As a matter of fact, it was babesiosis that brought me into the Lyme-disease investigation. A paper I wrote that was published back in 1977 in the New York State Journal of Medicine described three cases of babesiosis diagnosed here—the first cases reported in the state—and it came to the attention of Jorge L. Benach, of the state Department of Health and the department of pathology at Stony Brook. He was looking for a good source of ticks for research, and I told him he couldn't do better than here. It was through my interest in Lyme disease that I came to know Allen Steere, of the Yale Medical School. It was Steere and his associates at Yale who pioneered in Lyme disease and published the first report on it. And gave the disease its name. That was in 1976. You may remember the story. It's interesting. A woman in the Lyme area was the real pioneer. Back in the summer of 1972, her child developed a painful arthritis in the knee, and in talking with her friends and neighbors she discovered that a number of other children around there were suffering from the same thing. She apparently knew enough about arthritis to realize that a cluster of cases of a disease like that was unusual. She got in touch with the state health authorities, and Steere heard about the outbreak from them. Arthritis, of course, is only one of the forms that Lyme disease can take, but it was what Steere and his associates first observed. And at first they called it Lyme arthritis. I understand that the old-timers over in East Hampton had a disease they called Montauk knee. Lyme disease has probably been around for a long time. So has babesiosis. There was a disease along the New England coast that was known for many years as Nantucket fever.

  "It was chance that brought me here to Shelter Island. I was born and raised in Argentina. I studied medicine in St. Louis, at the Washington University School of Medicine, and trained and practiced in California for about ten years. I married a Long Islander, and we decided to settle in the East. It so happened that Shelter Island needed a doctor. I started practice here in the summer of 1975, and one of the first diseases I saw was what I'm now sure was Lyme disease. I had several patients with a specific rash called erythema chronicum migrans, which has been known and described, particularly in Europe, for a long time. Erythema chronicum migrans is a distinctive marker of Lyme disease. The rash begins with a lesion at the site of the tick bite and slowly spreads outward, in a circular pattern with a red rim. It can be quite large—fifteen or more inches in diameter. I saw the same rash again in the summer of 1976, and I took the trouble to search the literature. Most of the reports I found were Scandinavian, some of them going back to the nineteen-twenties. I found a 1962 paper by three researchers at the University of Helsinki which discussed a possible relationship between Ixodes ticks and the erythema chronicum migrans that was then associated with meningitis. But you can't count on the rash as an infallible clue. For one thing, as we know now, it isn't always present. For another, it only appears several days after the bite and the initial symptoms. In
any event, I started treating my cases with antibiotics, and that seemed to do some good. I followed Steere's work as it was published, and that's when I realized that what I was seeing was his Lyme disease. I remember a telephone conversation I had with Steere in 1978. He had decided that in certain cases Lyme disease was self-limiting—that it cured itself and after a while just vanished. That's true, of course, in a way. It seems to go away, but it really just goes underground, and then emerges in a much more serious way. These later complications usually take one of three forms. One, of course, is arthritis of the large joints—most often the knee. Another manifestation is neurological. It can resemble a form of meningitis or the facial paralysis called Bell's palsy. Those early Scandinavian investigators may very well have been seeing our Lyme disease. The third form affects the heart. When Steere assured me that the disease was self-limiting, I stopped using antibiotics. But then he dug deeper and changed his mind. We now know that prompt treatment with a penicillin can generally prevent the later manifestations.

  "I was fortunate enough to have a role in the investigation of the cause of Lyme disease. Benach was involved, and Jeffrey Davis, of the Wisconsin Department of Health and Social Services. The laboratory work—the most significant work—was done at the Rocky Mountain Laboratories, in Hamilton, Montana, by a team headed by Willy Burgdorfer, of the National Institute of Allergy and Infectious Diseases. They collected their ticks here on Shelter Island and isolated a spirochetal bacterium that they were later able to demonstrate was the causative organism. It was named for Willy Burgdorfer—Borrelia burgdorferi. As you know, the cause of syphilis also is a spirochete. Not the same one, of course. And untreated syphilis, like Lyme disease, can later reappear, with very serious consequences.

  "The laboratory findings were published in Science and in the Journal of Clinical Investigation in 1982 and 1983. And that's about where we stand right now. We have the disease as an entity, we have the causative organism, and we have the vector—the tick. And we have an effective treatment. There are still some loose ends. The most important need is for an effective means of control or prevention. There is a search going on for an immunizing vaccine, but so far without much success. There is a growing demand in the endemic areas for a program to eradicate the tick, for a sanitizing spray. Many of the endemic areas are, of course, resort areas. There are good environmental reasons for opposing that approach. But there is another good, hardheaded reason. A safely selective spray is hard to imagine. And even if there were one it would hardly be worth the trouble. Advocates of a chemical attack on the tick don't seem to fully understand the nature of the tick and its life cycle. I. dammini is often called a deer tick. Its principal host is the white-tailed deer. That's where the ticks mate. Mating occurs in the fall. The males die after mating, but the females live on through the winter, and in the early spring they lay their eggs in the wild. Then they, too, die. The eggs hatch into larvae, and at some point in the summer the larvae, if they can, attach themselves to a host, usually the white-footed mouse—the field mouse—and help themselves to a big blood meal. Then they rest through the fall and winter. That one meal is all they need. In the spring—the second spring of the cycle—they develop into nymphs. It is in the nymph phase that the tick usually brings the disease to us—if it carries the spirochete, and if it chooses one of us for its meal. The nymph feeds like the larvae—once in a lifetime. But that meal is a big one—a long one, anyway. It gorges for several days. And it seems that only at the end of the meal is the spirochete transmitted to the host. Feeding time can be any time during the summer and early fall. Then the nymph matures into an adult, and mates. It's true that the nymphs can be found on your lawn. But the reservoir is the wild—the field mouse. The field mouse is a burrowing mouse, and you don't often find its burrows in your front yard. You find them out in the woods and scrub. A spray would have to be a powerful spray to penetrate the scrub and soak down into the burrow. It has been generally supposed that the tick finds a suitable host by sensing its animal warmth. It waits on a blade of grass or a shrub, feels the passing warmth, and drops. There is a feeling now, though, that more than heat is involved. It has been suggested that a preferred host exudes a scent, a chemical force of some sort, that incites the tick to drop. Maybe some of us are more attractive to ticks than others. My old dog has had Lyme disease three times. I've treated her just the way I treat my other patients. Of course, dogs range. Proximity to the tick is everything. I've had several cases of Lyme disease in elderly women—old ladies who never got any closer to nature than the front porch. They puzzled me for quite some time. Then I finally figured it out. They all had cats. The cats, being cats, ranged. And cats are mousers. My feeling is that those cats did their mousing at the source—at the burrow. Then they came back home and jumped up on an unsuspecting lap."

  I had been thinking about I. dammini's principal host and mating place—the deer. I wondered if the deer might be a crucial factor in any attempt at control. I wondered if the elimination of the deer here on Long Island, or even just on Shelter Island, would break the cycle and abort the disease. Dr. Grunwaldt thought for a moment, and shook his head. "I doubt it," he said. "I think the tick would probably find another host. And, besides, I can't see much public support for a deer-eradication program."

  I knew what he meant: What? Bambi?

  I know of no one—friend or acquaintance or neighbor—in my part of Long Island who has had Rocky Mountain spotted fever. Nor do I know anyone who has had babesiosis. Lyme disease is a different matter, and this is not surprising. Dr. Grunwaldt told me that he had seen only one case of Rocky Mountain spotted fever in his thirteen years of practice on Long Island, and only twenty cases of babesiosis. But he has seen and treated at least four hundred cases of Lyme disease. I have a number of friends who have suffered its protean rigors. One of them, and one of the local pioneers in this morbid respect, is a woman named Priscilla Bowden (Mrs. Jeffrey Potter), an artist, an amateur flutist, and a knowledgeable gardener. Miss Bowden, a slim, dark-haired woman in her forties, lives with her husband on a verdant acre in the village of East Hampton, with expanses of lawn and many shade trees and flowering shrubs in a pleasant surround of woods. They have—or had at the time she took sick—a small brown dog and a large white cat. Her illness, she told me in a talk we had at her home, had its beginnings just after the Fourth of July weekend in the summer of 1982.

  "It was July 7," she said, glancing at a sheaf of papers. "A Wednesday—I keep a diary. It's not a 'Dear Diary' diary, it's just a kind of social record, but if something interesting happens I make a note of that, too. Well, I'd been feeling mean for a couple of days. Not actually sick, but just not feeling well. I blamed it on the big weekend of the Fourth. Summer weekends here are always a strain. Too many parties. So I dragged around, and then, all of a sudden, it struck. It started with a headache—a horrible, terrible headache. And I felt burning up. I took my temperature. It was 103.5 degrees. That was around midafternoon. At around seven, I took it again: 104 degrees. That was pretty frightening. I went to bed and spent a miserable night. I was still burning up the next morning. Jeffrey called Dr. Medler—Raymond Medler, in East Hampton. We were given his first available appointment: one o'clock. When I got there, I didn't have to say much. Dr. Medler took my temperature. It was 104.6 degrees. He said he didn't know what was wrong with me, but with a fever like that the only place for me was the hospital. And right away. I said can't I even go home and get my toothbrush? He said no, that I should go straight to the hospital—Southampton Hospital. So I went.

  "I was fortunate enough to get a private room. My headache was horrible. They got me into bed, and Dr. Medler arrived and started me on aspirin. It was amazing. In a couple of hours, my fever was down to 99.9 degrees, and my headache had practically vanished. I felt well enough to ask for some paper and a pen; and I started making these notes. Then the aspirin wore off and my headache came back and my fever went up to 102.5 degrees. They seemed to go together. Fi
rst the fever, then the headache. Or maybe it was the other way around. My memory is a little hazy about those next few days. I made my notes, but the details are a little dim. Dr. Medler and the nurses kept asking me if I remembered having a tick bite. I said no—not as far as I knew. It was somewhere around that time that they began to speak of Lyme disease. I don't think I'd ever even heard of Lyme disease. I wrote that down in my notes, only I wrote '1-i-m-e,' and 'arthritis.' That's what they called it then. They asked me about a rash. I hadn't noticed any kind of rash, and I didn't have a rash then. Not at that moment. That was Thursday and Friday and Saturday. My fever went up and down, and so did the headache. Then, on Sunday, there it was—a rash. It was a circular red rash, about three or four inches in diameter. It was on my leg, my thigh. Then I saw two more circles, also on my leg. One of the nurses found another one, on my back. So now it was established. I had 'lime arthritis.'

  "On Monday morning, July 12, a man walked into my room carrying a camera and a black bag. He stopped at the foot of the bed, and said, 'I can tell you one thing that's the matter.' I stared at him, and he said, 'Your kimono is not tied the right way. I've been to Japan, and the Japanese are very careful about whether the fold is to the left or the right.' My way—I forget which way it was—meant I was moribund or something. Well, that was my introduction to Dr. Berger—Bernard W. Berger, a dermatologist and, I later learned, an expert on Lyme disease. Dr. Medler had called him in as a consultant. Anyway, he made me laugh with his kimono joke. We talked, and I told him my sad story, and he opened up his camera and took some photographs of my rash. He told me what there was to tell about Lyme disease, and put me on a course of penicillin. Maybe I was already on it; I don't remember. One thing he told me was that in four weeks or so I might have a strange reaction. We talked about the tick bite. He said it could have been a few days before I got sick, or it could have been two weeks. There were certainly plenty of opportunities for me to pick up a tick—the garden, the shrubbery, the woods, the cat. I felt much better after talking with Dr. Berger. I asked Jeffrey to bring me some drawing paper, and I did a little drawing—a self-portrait of me sitting up in bed. There's a color print of it on the wall over there."