The Medical Detectives Volume I
Page 21
The hosts preferred by the anthrax organism are horses, goats, cattle, sheep, and swine. Its human visitations are merely accidental. Man acquires the disease by contact with an ailing animal or from a contaminated animal product—skin, hide, hair, wool, bristle, bones. B. anthracis enters the body in the form of a spore excreted by an animal host. The nose or an open cut or abrasion are its usual portals of entry in man. Sometimes, though rarely, it enters by way of the mouth. Among grazing animals, on the other hand, the mouth is the customary portal. Anthrax spores, like those of most bacilli, are brutally resistant to extremes of heat and cold, and can exist for many years in the harshest natural environment. Once settled in an acceptable habitat, however, the spores discard their protective casing and rapidly proliferate. They generally make their presence felt within a couple of days. The signs and symptoms of anthrax reflect the initial focus of infection. Inhalation anthrax comes on like bronchopneumonia, and the manifestations of intestinal anthrax are those of any severe intestinal disorder. Cutaneous anthrax, as the other—and most common —form is called, is classically proclaimed by a lesion that, if left to mature, develops a black, scablike crust. (It is from the look of this distinctive blemish that the disease derives its name. Anthrax is the Latin transliteration of the Greek word for coal.) But these are superficial differences. Anthrax is essentially an acute intoxication. Its victims are overwhelmed, not by a concentration of B. anthracis at a certain site, but by a concentration in the blood and the lymph of a toxic substance that the organism elaborates as it grows and multiplies. B. anthracis is destroyed by massive doses of penicillin and other antibiotics, but drugs have no effect on its toxin. The prompt destruction of the organism is thus of vital importance in the treatment of anthrax. For once the accumulation of toxin has reached a certain level in the blood, the victim is beyond salvation. The immediate cause of death in anthrax is shock.
Anthrax has been a dreaded disease for at least five thousand years. It is probable that man became aware of its existence soon after he turned from hunting to a life of farming and animal husbandry. In the judgment of some medical historians, a reference to anthrax appears in the oldest pages of the Old Testament. They profess to recognize the disease in one of the curious plagues described in the Book of Exodus with which God punished Pharaoh for holding the Jews in bondage. "Behold," the verse they offer into evidence reads, "the hand of the Lord is upon thy cattle which is in the field, upon the horses, upon the asses, upon the camels, upon the oxen, and upon the sheep: there shall be a very grievous murrain." A murrain, however, is not necessarily anthrax. The word is a general term for any grievous disease of livestock. A more striking likeness of anthrax is found in the Georgics of Virgil. The pertinent passage reads:
To death at once whole herds of cattle go; Sheep, oxen, horses, fall; and heaped on high, The differing species in confusion lie, Till, warned by frequent ills, the way they found To lodge their loathsome carrion under ground; For useless to the currier were their hides; Nor could their tainted flesh with ocean tides Be free from filth . . .
Nor safely could they shear their fleecy store (Made drunk with poisonous juice, and stiff with gore), Or touch the web: but, if the vest they wear,
Red blisters rising on their paps appear,
And flaming carbuncles, and noisome sweat.
And clammy dews, that loathsome lice beget;
Till the slow-creeping evil eats his way,
Consumes the parching limbs, and makes the life his prey.
But Virgil's representation of anthrax is more than merely graphic. It is exceedingly precocious, as well. In his apparent understanding of the contagiousness of the disease and the manner in which it is spread, he seems to have been almost two thousand years in advance of his time. Some thirteen hundred years elapsed between the publication of the Georgics, in the first pre-Christian century, and the inclusion of anthrax (along with epilepsy, leprosy, and the itch) among the diseases thought to be contagious, and the nature of its contagiousness eluded rediscovery even longer than that. As late as the seventeenth century (when a pandemic in southern Europe killed sixty thousand people and uncounted thousands of livestock), it was considered only prudent that an animal dying of anthrax be slaughtered for food and its hide (or fleecy store) be salvaged and used. It was not until the early nineteenth century, little more than a generation before Koch's epochal depiction, that medical science could much improve upon the Virgilian view of anthrax.
The scientific comprehension of anthrax, though late in taking recognizable shape, was accomplished with dispatch. Few diseases have been so thoroughly riddled so fast. In addition to being the first disease irrefutably laid to a germ, it was the first of its kind to yield to total penetration and control. The control of anthrax was initiated by Louis Pasteur in 1881. In the spring of that year, before a gathering of scientists assembled near Paris by the Agricultural Society of Melun, he demonstrated (on a flock of sheep) that an animal inoculated with a culture of heat-attenuated B. anthracis was rendered immune to anthrax. He also showed, in another study, that the burial of infected animal carcasses (as originally recommended by Virgil) was not enough to check the natural spread of the disease. His recommendation was that the carcasses first be burned. Buried anthrax spores, he pointed out, are eventually disinterred by the peregrinations of earthworms and other subterranean creatures. A few years later, in 1895, an Italian investigator named Achille Sclavo, inspired by Pasteur's contributions to the control of anthrax in animals, developed an antianthrax serum for the treatment of the disease in man. Since then, the treatment of human anthrax has been further refined by the introduction of a succession of drugs to which B. anthracis is more or less susceptible—neoarsphenamine (in 1926), the sulfonamides (1935), penicillin (1944), chloramphenicol (1947), and (around 1950) the several tetracyclines. The impact of these prophylactic, hygienic, and therapeutic innovations has been decisive. Anthrax is no longer much of a problem anywhere in the Western world. It has almost entirely disappeared from Europe, and the biggest epizootic in the United States in recent years—an outbreak in Kansas and Oklahoma in 1957—felled fifteen hundred head of cattle, sixty-eight pigs, thirty- nine sheep, and fifteen horses. The incidence of human anthrax in this country—on the farm and in the animal products trades—has dropped from two hundred and two cases in 1917 (the second year for which accurate records are available) to five in 1964.
Nevertheless, the disease persists—not all susceptible animals are regularly vaccinated, not all infected carcasses are properly destroyed, not all processors of hides and hair and bristle and bone take the trouble to sterilize their products. And, in spite of the effectiveness of penicillin and other antibiotics, it is still, on occasion, fatal. It will probably always be so. For anthrax has now become such a rarity that the great majority of doctors have never seen a case, and when one turns up—as it did in Cincinnati in March of 1964—they only too often fail to recognize it in time. "The chances of making a correct early diagnosis of anthrax," Herman Gold, a staff physician at the Chester (Pennsylvania) Hospital and an authority on anthrax, has noted in a recent monograph, "are directly proportional to the physician's index of suspicion."
Dr. Brachman's telephone call to Dr. Decker on Friday the thirteenth of March was prompted by two considerations. One, of course, was to give the Ohio authorities the laboratory report from the Communicable Disease Center. The other was to express a personal interest in the case.
"Anthrax is something of a specialty of mine," Dr. Brachman says. "That's a little these days like specializing in botulism or rabies or smallpox. I mean, it's an interest one doesn't have much occasion to indulge. For which, of course, I'm duly grateful. But when a case does come along, I like to follow it up if I can. That was no problem here. Decker and I are old friends. So after I had given him our report and asked him to pass it on to Cockburn down in Cincinnati, I said I'd like to keep in touch with the investigation. Decker couldn't have been more obliging. In that
case, he said, he'd give the assignment to Peter Greenwald. I knew Peter. He was one of our own people. He was a young doctor who had chosen to do his military service in the U. S. Public Health Service instead of in the Army, and he was then on loan from CDC to Ohio as what we call an Epidemic Intelligence Service officer. I thanked Decker for his courtesy, and said I'd be glad to give Peter any help I could. On anthrax, that is. Not on the case. I knew nothing about that at this point. Except that it was a case of cutaneous anthrax—Cockburn had mentioned a skin lesion. And that the victim was a man named Hoffman.
"I had a call from Peter that afternoon. He was leaving for Cincinnati in the morning and wanted to talk things over. All he knew about anthrax was what the textbooks say. The best I could do was suggest some questions to ask. First on the list was Hoffman's occupation. Did he work with animals or animal products? Did he ride? Did he often visit the zoo? Had he done any digging or gardening lately? Grubbing around in the soil can bring up buried spores. Did he use a shaving brush? How about new rugs at home, any new upholstered furniture, new clothes? Some of those items may sound a little fanciful. I think they did to Peter. But they're not in the least far-fetched. You can find them all in the literature. Take shaving brushes, for example. There was a serious epidemic in this country around the time of the First World War that was traced to shaving brushes made from contaminated horse hair. As for clothing as a source of infection, I can vouch for that myself. I worked on such a case back in 1956. It happened in Philadelphia. The victim turned up with a classic anthrax lesion on his left forearm—so classic that the doctor couldn't help but recognize it. But there wasn't a clue where he might have got it. He was a grocery clerk and he lived in a downtown apartment—a completely sterile environment. Then we learned two things. He had recently bought a rough woolen mackinaw, and he usually wore it with a short-sleeved shirt. And not long before the appearance of the lesion, his wife had accidentally scratched him on the left forearm. We got the coat and took some samples of the wool and cultured them, and two of them were positive for anthrax. But the strangest I ever heard of was a fatal case somewhere up in Connecticut in 1946. The victim worked for a piano manufacturer and his job was making keys out of ivory—raw ivory. It turned out that the ivory he used was contaminated.
"I didn't hear from Peter again until Tuesday. He called me Tuesday morning from Columbus, and he seemed to have the matter pretty well in hand. He had followed the trail from Cincinnati out to Oxford and back to Cincinnati again, and he had talked to a number of people—Cockburn, Mrs. Hoffman, a doctor in Oxford, several Cincinnati doctors, the company that Hoffman worked for, and even a friend of Hoffman's on the job. The answers to all but one of my questions were negative. That one affirmative answer, however, was extremely interesting. Hoffman was an insulation man, and the materials he worked with included hair felt. The hair in hair felt is animal hair. Not only that. He had been working with hair felt insulation at the time he took sick. The company—I'll just call it that—showed Peter the invoice for the felt that Hoffman had been working with, and he copied down the specification number. It was number 303. It came, they said, like all their felt, from a manufacturer I'll call the Ajax Corporation. Ajax was an old concern, with headquarters in Chicago and plants at Milwaukee and Newark. Well, that was even more interesting.
It changed the whole complexion of the case. It was no longer just a state or local problem—it was now a national affair. I told Peter I'd probably be seeing him soon, and hung up.
"But first I had to have a word with Decker. That was only common courtesy. Decker was expecting my call. He had naturally seen Peter's report and could guess what I had in mind. He supposed that I would want to stop off in Ohio and go over the evidence before calling on the Ajax people. If so, he said, come ahead. He would clear it with the Department. I told him I planned to leave that afternoon, and we arranged to meet in Columbus. The next call I made was to the headquarters of the Ajax Corporation in Chicago. I identified myself and described the case and asked permission to visit their plants. They were stunned. They simply couldn't believe it. Ajax had been in business since before the Civil War and this was the first time it had suffered even a suspicion of anthrax. However, they said, they were glad to cooperate, and would instruct the managers of the Milwaukee and Newark plants to expect me. After that, I made a couple of routine for-your-information calls—to the state epidemiologists in Wisconsin and New Jersey. Then I got my working kit together—a set of plastic bags for the samples I proposed to collect, a couple of marking pens, and a camera. Then I went home and packed my suitcase, and left.
"Peter met me at the Columbus airport. We spent the evening with Decker and Charles Croft, the chief of the Division of Laboratories up there, reviewing the case. They didn't like it much better than the Ajax people. Hoffman was the first case of anthrax in Ohio in a good many years. Since the 1952 epizootic, I think. That was an outbreak that began with swine and then moved on to cattle and sheep, and Ohio had two human cases. The trouble was traced to contaminated bone meal that had originally come from Pakistan and India. It was also arranged at the meeting that Peter would drive me down to Cincinnati in the morning. I spent the night at a motel somebody recommended, and we made an early start. Our first stop was the hospital where Hoffman died. I wasn't checking up on Peter. It was just that I wanted to get my own feel of the case. I talked with Schmidt, the bacteriologist, and the attending physician and his consultants, but I didn't learn anything that I didn't already know. Then they had a question for me. What about the people who had been in contact with Hoffman —the nurses and the orderlies and his wife and children? Were they in any danger? That was an interesting question. It gives you a pretty good idea of what a mystery anthrax has become. But I was able to reassure them. Human-to-human transmission of anthrax is unheard of. It has never happened. Besides, if anyone had been infected, he would certainly be showing some signs of it by now.
"The next stop we made was the office of the company. I wanted to see the hair felt that Hoffman had worked with. The management were as nice as could be—Peter had broken the news very smoothly—and they took me into the warehouse and showed me several big rolls of the stuff. I cut and bagged a couple of samples, and then I noticed something. All these rolls carried the specification number 318. 'Wait a minute,' I said. 'The invoice that Dr. Greenwald saw here yesterday had a different number. It was number 303.' Oh, no, they said. That was impossible. There must be some mistake. The only hair felt they ever used was number 318, and they would show me the invoice to prove it. Which they did—and it was just as Peter had said. It was marked number 303. They were flabbergasted. The invoice covered a shipment of twenty-five rolls of 303 felt—each of them fifty feet long by three feet wide by one inch thick—and it was dated December 2, 1963. That would have been in plenty of time for the hospital remodeling job at Oxford. Work began there on January 8. When the management had recovered from the shock of the invoice, we went back to the warehouse and checked out every roll of hair felt in stock. All were number 318. Then somebody suggested the shipping room. We went down to the shipping room, and there, back in a corner, was part of a roll—maybe a dozen feet—marked number 303. It was all that remained of the shipment. I took a sample for analysis. It looked exactly like the other felt, but, of course, that didn't mean a thing. I asked them to cover it up and leave it alone until further notice. Then, like the people at the hospital, they had a question to ask. Only this was a natural question. They wanted to know if their other employees were in any danger. A lot of them had handled or worked with felt. I had to admit that the danger was there. I doubted, though, if it amounted to much by now. Too much time had passed. In any event, that was something for the city health authorities to look into. They would, I was sure, do a properly thorough job.
"The people at the hospital in Oxford were also worried about infection. They had every reason to be. Peter introduced me to the administrator there, and he showed us around. The rem
odeling included insulating some water pipes in the kitchen ceiling and enlarging the newborn nursery and installing air conditioning there. The pipes in the nursery were hidden behind a false ceiling of acoustical tile, but those in the kitchen were exposed. All of the insulated pipes were wrapped in a protective canvas sheathing, and except for the finishing touches, the job was done. The kitchen was already back in use, and the nursery was scheduled to reopen in about a week. I wanted a sample of the installed felt, and the administrator called over Hoffman's working mate, a man named Jensen, and he climbed up and cut me a little piece from one of the pipes in the nursery. I was glad to meet Jensen, and we talked for a moment. He was terribly depressed about Hoffman. They had been good friends. But otherwise he felt fine. No lesions, no symptoms of any kind. I asked him about the roll of felt—was there any of it left? He thought there was, and took me down to a basement hall where they kept their supplies and showed me the remains of the roll, and I got myself a sample. I suggested that the rest of the roll be boxed and returned to the company in Cincinnati. And it might be a good idea to disinfect this part of the hall. That was when the administrator asked about the danger of infection. Did I think they ought to close the hospital and rip out all the insulation? I was prepared for the question and I told him what 1 thought. There was certainly no reason to close the hospital. Nor was there any reason to take out the insulation—not at this point. And I thought it was safe enough to reopen the nursery as planned. The insulation was well enough covered. But I advised them not to use the air-conditioning. Not until they heard from me it was safe.