An Arizona emergency physician, Dr. Ray Malone, recently telephoned me and asked how I would diagnose a case of smallpox. He thinks that all medical personnel, especially paramedics, ought to be able to give, instantly, a better answer than I did.
Test yourself: Think about what you would have said, before you read on.
Dr. Malone wrote a novel about a scenario that he thinks is quite likely: an al-Qaida terrorist deliberately becomes infected, enters the U.S. during the incubation period, and infects another suicidal holy warrior, plus everyone he can manage to contact, once the rash breaks out. The plot is foiled by an intrepid physician, who is called to Washington to work for Homeland Security just after he teaches personnel at his hospital.
Malone says the key is in the nursery rhyme: “Ring around the rosie/ Pocket full of posies/ Ashes, ashes/ We all fall down.” This rhyme, which first appeared in print in 1881, has been called a coded reference to the Black Plague, a suggestion debunked on www.snopes.com. Smallpox is a much more plausible source. The “ring” is the red or white ring that tends to appear around the lesions. The posies were for disguising the odor. And the victims generally died and were cremated.
The ring is not mentioned in the Wikipedia article on smallpox, in the 1997 Textbook of Military Medicine, nor other fairly recent sources. However, The Reference Handbook of the Medical Sciences, copyright 1903, states: “In smallpox all of the pocks have a prominent red base, while in varicella the red base is slight if at all present.”
This handbook notes other features that distinguish smallpox from chickenpox. Smallpox generally begins with a chill and a fever that reaches 102 F and persists for two days. If there is an initial fever in varicella, it tends to be slight and lasts only 24 hours. Intense lumbar pain and vomiting are constant features early in smallpox, and absent or rare in chickenpox. The rash of smallpox begins on the uncovered parts of the body, especially the face, and involves the palms and soles. Varicella begins on covered parts, as the trunk. In smallpox, lesions progress through four distinct stages–macule, papule, vesicle, and pustule–each lasting from one to several days. All smallpox vesicles become pustules and most umbilicate. In varicella, the rash may vesiculate within 24 hours, and most of the vesicles do not become pustules, though they sometimes umbilicate. Other sources note that there is one crop of lesions in smallpox, all in the same stage of development, while varicella may produce several crops in various stages.
Malone describes the odor of “rotting flesh” in smallpox victims. My mother, Phyllis Orient, a survivor of the 1928 outbreak of smallpox in St. Joseph, Mo., mentioned the odor first, when asked how she would recognize a case of smallpox. The odor was said to be distinctive and unforgettable, and may be noticed quite early, perhaps even before the rash.
Our pediatrician did not believe that my mother could have had smallpox because she has no scars. He insisted on vaccinating her. She said, “Go ahead, but it won't take.” It didn't. Her father had been vaccinated annually at the bakery where he worked, because the vaccine never took and no one believed his history either. Grandpa, she said, had been covered with lesions everywhere, including the soles of his feet, except eyeballs and ear canals. To prevent scarring, the family physician prescribed rubbing with camphorated oil, which was banned by the FDA in 1980 because of reports of poisoning by accidental ingestion or even topical application. (Some products such as Ben-Gay and Vicks VapoRub still contain up to 11% camphor.) There are no controlled studies of this remedy!
The horrors of introducing smallpox in a population now almost totally susceptible have been discussed here previously (November 1998; May, July, September 2002; and Civil Defense Perspectives, January and November 2002). Fortunately, vaccination is effective for a short time after exposure (Malone says three days, and others say up to two days, or four days for previously vaccinated persons)–Grandma, who was vaccinated post exposure, did not get smallpox.
A mathematical model predicts that the most important factor by far in the extent of of spread is the length of time before people withdraw to their homes, followed by delay in detection. Both of these factors are more important than vaccination strategy (Eubank S, et al. Nature 2004;429:180-184).
Widespread public knowledge of how to recognize smallpox could stop biological suicide bombs. Airport security screeners should never allow anyone to cover her face or hands at the security checkpoint, and should be alert for unusual odors, ringed skin lesions, or signs of pain, prostration, or fever.
Malone's novel, Ring Around the Rosie: This Is Not a Nursery Rhyme, is available at www.raymalonemd.com
Vaccines. The efficacy of current vaccines, the mainstay of preventive efforts, is seriously questioned in the summer and fall 2006 issues of the Journal of American Physicians and Surgeons (www.jpands.org). In addition, Dr. Tom Jefferson of The Cochrane Collaboration called for an urgent reevaluation of the UK vaccination program (BMJ 2006; 333:912-915). Not only was evidence of efficacy weak, but there is surprisingly little data concerning safety. (See AAPS News of the Day 10/31/06, www.aapsonline.org). The search for a “universal” vaccine, which targets a protein that doesn't mutate from year to year, is underway (Science 2006;312:380-382). The merits of vaccinating poultry are internationally debated. Because of incomplete effectiveness, vaccine can increase the risk of between-flock transmission before an outbreak is detected (Nature 2006;442:17).
Antivirals. Viral resistance to neuraminidase inhibitors oseltamivir (Tamiflu) and zanamivir (Relenza) carries a “fitness cost” of lessened transmissibility and pathogenicity. Resistant strains, however, have recently been isolated in 18% of Tamiflu-treated children. Up to 92% of isolates have been resistant to M2-protein inhibitors, amantadine and rimantidine (JAMA 2006;295:891-894), with little fitness cost. Prophylactic use should decrease the incidence of resistance if transmissibility of the mutated virus is less, but increase the incidence of amantadine resistance (Science 2006;312:389-391). Some strains of avian flu, however, are apparently sensitive to amantadine, so that some health authorities recommend stockpiling this far less costly drug in addition to Tamiflu (WSJ 3/15/06).
Other Drugs. In 1918, healthy young adults with robust immune systems accounted for more than half the deaths (EID, January 2006, www.cdc.gov/eid), possibly because of overproduction of proinflammatory cytokines. Statin drugs could help by damping down an extreme immune reaction (Times [London] 10/4/06)–or by activating Vitamin D receptors (Lancet 2006;368:83-86). See DDP Newsletter, July 2006. Cardiac glycosides, especially digitoxin, also target mechanisms crucial for the pathogenesis of influenza A and might be of value (Haux J, “Digitalis for Bird Flu?” 1/10/06: www.cancerwire.com).
UV. A collection of articles from the 1970s on air disinfection by ultraviolet light is available from DDP on request. This could possibly interrupt airborne spread.
DDP, 1601 N. Tucson Blvd. Suite 9, Tucson, AZ 85716, (520)325-2680, www.oism.org/ddp