Part 1 of 2

Since the terrorist acts of Sept. 11, 2001, there have been many changes in our lives. From the federal government to our local communities, many efforts have been made to prevent additional attacks and to be prepared if another attack occurs. Hospitals and health care agencies provide a critical part of these preparedness efforts and as providers are expected to be prepared for victims of these attacks, whether they suffer from a traumatic injury or are infected with an exotic disease. Of these, no type of terrorist incident has the potential to generate more fear than a covert attack with a biological weapon. But no terrorist action could be hampered more by the vigilance of informed health care professionals.

Health care providers, particularly nurses, must increase their knowledge of the effects a biological attack would have on a population. With this knowledge, providers could be the first to notice an increase of consistent symptoms resulting from a covert attack. With rapid recognition and diagnosis, treatment can begin early and save many lives. Some hospitals, clinics, and physicians’ offices are connecting to regional web-based surveillance tools that will allow for even faster recognition of health syndromes that could be related to a biological attack. All health care professionals need to be armed with current knowledge. Additionally, each health care facility must have plans in place to provide isolation and personal protective equipment to ensure the safety of both their staff and facility from contamination and possible secondary infections from a contagious agent.¹

Biological weapons include bacteria, viruses, and biological toxins. Of the possible biological agents, the most likely or most hazardous include the bacteria Bacillus anthracis (anthrax) and Yersinia pestis (plague), the variola virus (smallpox), and the biological toxin botulisum (Clostridium botulinum). Both the U.S. and Soviet Union participated in weaponizing these and other agents after World War II. In 1972, many countries, including the U.S. and the Soviet Union, signed an agreement at the Biological Weapons Convention prohibiting the development, production, and stockpiling of bacteriological (biological) and toxic weapons. Despite this, many countries continued their research and development. Today, several of those, including state-sponsored terrorist organizations hostile to the U.S., are suspected of proliferating biological weapons for their use.²

Biological weapons are not new. In 1346, the Tatar army hurled the corpses of soldiers who had died of the plague over the Kaffa City walls, infecting residents who were defending the city. Some of those who left Kaffa may have started the Black Death pandemic that spread throughout Europe. Russian troops used this same tactic against Sweden in 1710.2 Biological warfare also occurred during the French and Indian War of 1754 to 1767 when an Englishman, Sir Jeffery Amherst, gave smallpox-laden blankets to Native Americans who were loyal to the French. The Native Americans sustained epidemic casualties as a result.²

With the possibility that such agents could be used again, heightened vigilance of health care providers is necessary. Early recognition of these illnesses could trigger earlier diagnosis and allow preventive measures that could save many lives. Caregivers need to learn the early signs and symptoms and the modes of transmission of biological agents, not only for the well-being of their patients, but also for themselves. The health of nurses who work on the front line of acute health care could be at risk. For example, those working in physicians’ offices, clinics, and EDs could be at risk of secondary infection from pathogens, such as plague and smallpox. And in the event of an attack, nursing challenges would not end with acute care. Large numbers of patients would require intensive care for weeks or even months. Entire wings of hospitals or clinics would be subject to quarantined isolation. Only education can reduce the risk.

Anthrax

After suffering through several years of anthrax letter hoaxes, real anthrax letters were mailed in 2001 to media and political offices. As a result, 22 people were infected with the bacteria. Inhalation exposure caused infection for 11 of the 22 victims, and at least the first nine were caused by exposure to anthrax found in letters.³ Although there has not been a repeated attack since then, the next episode may be right around the corner. The perpetrator of the 2001 attack has never been found, and it remains uncertain whether it was an act of domestic or international terrorism.

Bacillus anthracis is a spore-forming bacterium that causes a rapidly progressing infection. Once the infection is established, the victim is said to have anthrax. Bacillus anthracis derives its name from the Greek word for coal (anthrakis) because of the black, coal-like skin lesions it creates.⁴ Anthrax can develop from inhalation, ingestion, or exposure of nonintact skin to the bacterium. The spores are hardy and can remain viable for more than 40 years.⁵ At least 17 countries have developed an anthrax weapon program.⁴ In 1970, the World Health Organization (WHO) concluded that the release of 50 kg of aerosolized anthrax upwind of a population of 5 million could lead to an estimated 250,000 casualties and 100,000 deaths,⁶ substantiating the perceived threat from Bacillus anthracis. Because of the efficiency and durability of its spore, the bacterium’s use as a biological weapon has brought great concern to the U.S. military and, more recently, the public.

The Japanese Aum Shinriko religious sect spent millions of dollars developing an anthrax weapon and on at least eight occasions released anthrax or botulism. Because of the difficulty encountered in the dispersion, the sect members failed to produce any infection and eventually concentrated their efforts on Sarin, a nerve agent. Sarin was used with only moderate success as a lethal agent in both Matsumoto and Tokyo.⁴

The largest incident involving inhalation anthrax occurred in 1979 in Sverdlovsk, USSR, when a military biological facility accidentally released spores into the air from a faulty laboratory ventilation system. The Soviet Ministry of Health stated that the deaths were due to contaminated meat, but in the summer of 1992, President Boris Yeltsin acknowledged that the deaths were due to an accidental release from a military microbiology facility, Compound 19. In total, 77 people developed anthrax, and 66 died.⁷ Although the majority of inhalation anthrax cases from Sverdlovsk developed between four and 14 days, cases continued to emerge up to 43 days after the release.⁴ Some argue that secondary aerosolization may be responsible for those infections that developed beyond seven days after the primary aerosolization.⁴

Cutaneous, ingested, and pulmonary anthrax

Cutaneous anthrax infection occurs when bacteria enter through cuts or breaks in the skin and cause a localized infection. In two to six days, the infection results in itching followed by papular lesions that turn vesicular and into depressed black scabs. At this point, a Gram stain and culture of vesicular fluid can confirm the diagnosis. Anthrax occurs most frequently on the head, hands, and arms of people who work with infected cows, sheep, or horses. Recent cutaneous infections have resulted from handling letters that contained anthrax spores and then touching open skin. Skin infections not promptly treated can progress into sepsis and have a mortality of 5% to 20%.⁷ Because cutaneous anthrax does not progress as rapidly as the pulmonary or gastrointestinal infection, it is usually not fatal if treatment is started before the onset of septicemia.

Ingested bacteria can also cause infection. Nonintentional infections are usually associated with the ingestion of meat from infected animals. Incubation is from one to seven days after ingestion. The bacteria invades the mucosa of the mesenteric region and infects the lymph nodes, resulting in nausea, vomiting, bloody diarrhea, abdominal pain, sepsis, and ascities. The fatality rate is 50%⁵ since it progresses to toxemia and sepsis. The only recorded case of ingested anthrax in the U.S. took place in May 2000, when an infected cow was butchered and eaten by a farm family. The infection was discovered early and treated successfully.⁸

The most dangerous form is pulmonary anthrax, also known as wool sorter’s or rag picker disease because livestock workers have inhaled spores and developed pulmonary infections from sorting hides of infected animals. The last naturally occurring case of pulmonary anthrax in the U.S. took place in 1978.⁹ An inhalation of 8,000 to 50,000 spores is needed to establish an infection.² Bacillus anthracis spores are only 3 to 5 microns in size, allowing easy implantation in fine bronchioles and alveoli. Once spores are inhaled, pulmonary macrophages carry them to the mediastinal lymph nodes, causing hemorrhaging and edema that can be seen on a chest X-ray as a symmetrically widened mediastinal area. Although blood cultures may be negative, the sputum is usually positive at this point.

In the first phase of pulmonary anthrax, victims experience flu-like symptoms, followed by a significant improvement or even apparent recovery that may stop them from seeking medical care.² Aggressive treatment with antibiotics during the initial flu phase can be successful. However, because many health care providers are unfamiliar with the disease, many patients receive supportive treatment then are discharged, subsequently dying. Providers who see unusually large numbers of normally healthy patients presenting with similar flulike symptoms should be suspicious of an intentional attack and alert local public health officials. There is no magic number that will trigger an investigation, but the astute recognition of an unusual chest X-ray combined with other consistent symptoms should be highly suspicious. Early detection is the key to saving lives.¹⁰

The second phase of pulmonary anthrax develops suddenly with severe respiratory symptoms, hypotension, and shock. The patient may have a fever and profuse sweating. Stridor and crepitant rales may also be present. Blood cultures will be positive, and bacteria may be visible on a Gram-stain smear. Specimens thought to be Bacillus anthracis should be confirmed through a state public health lab and the Centers for Disease Control and Prevention (CDC).⁵ This phase generally lasts less than 24 hours, with mortality approaching 100% even with aggressive antibiotic therapy.⁸

Health care providers must be alert for patients who present with symptoms consistent with early anthrax,¹¹ obtain appropriate diagnostic tests (e.g., blood cultures and chest radiograph),¹² and report suspicious illnesses to local or state public health authorities. Infected patients are generally no danger to health care workers. Anthrax is not contagious, and there are no known cases of person-to-person transmission. Only standard precautions for blood and body fluids should be taken. Unless a patient has had an intentional contamination with large amounts of visible spores, decontamination beyond carefully removing clothing and washing hands and other exposed skin with soap and water is not needed. Contaminated clothing should be placed in a bag for disinfecting or disposal.¹³

Prophylaxis is accomplished in two ways. First, there is a licensed vaccine that consists of a series of six doses — one given initially and others at two and four weeks, then at 6, 12, and 18 months, followed by annual boosters. At least three doses are needed for prophylaxis before exposure and should be followed by an additional three doses. The vaccine is now offered to all military personnel and civilians who are at risk of exposure. The vaccine is not routinely available to members of the public unless they are thought to be in high-risk environments. The vaccine is not recommended for pregnant women or people with depressed immune systems. Thirty percent of men and 60% of women who have the vaccine experience mild local reactions, but serious events occur at one per 200,000 doses.¹⁴

A second means of prophylaxis is with antibiotics, much like the treatment of an active infection. Because laboratories have produced penicillin- and tetracycline-resistant forms of the bacteria, initial postexposure prophylaxis with oral fluoroquinolones, such as ciprofloxacin (Cipro), or doxycycline over four weeks is recommended. If available, the vaccine may also be initiated. Although diagnosis and treatment may begin at the hospital, patients undergoing prophylaxis will be sent home to continue an antibiotic regimen, especially if a large-scale exposure has taken place. Treatment for inhalation anthrax may continue for up to 60 days because inhaled spores can remain latent for extended periods.¹⁰

Bubonic and pneumonic plague

The plague has a colorful history. In A.D. 541, the first great plague began in Egypt, spreading across North Africa, Europe, and Central and Southern Asia. During a period of four years, 50% to 60% of the population died. The second plague pandemic began in 1346 and spread throughout the Middle East, killing one-third of the European population and more than 13 million in China, where it earned the name Black Death.¹⁵

A childhood nursery rhyme encapsulates the ravages of the plague: “Ring around the rosie” (describing the red ring around the infected lymph node), “pocket full of posies” (the smell of death was so overwhelming that people carried pockets full of fragrant flowers to hold under their noses), “ashes, ashes, we all fall down” (“ashes” are related to burning the bodies and “falling” to victims dying). According to some texts, another lasting impression of the disease survives in a saying used even today. Because the number of deaths from the pneumonic plague overwhelmed the ability of priests to give last rights, they gave other church members the power to issue last rights by saying, “God bless you” after the victims coughed or sneezed.¹⁶

Although much has been done to improve the living conditions that contributed to plague outbreaks, about 13 cases occur annually in the U.S., most in New Mexico, Arizona, California, and Colorado.⁵ These occurrences are usually not related to living conditions but to flea bites from infected rodents commonly found in the Western states. The animals most commonly found to carry the bacteria include rock squirrels, ground squirrels, prairie dogs, wood rats, chipmunks, and their fleas.¹⁷ In these geographic locations, the disease is endemic in the rodent population and is passed from generation to generation through flea vectors. In recent years, most of the cases of primary pneumonic plague in the U.S. have been acquired through an exposure to domestic cats with pneumonic plague, and exposure has become an occupational hazard for veterinarians in the Western U.S.⁸ Health care workers should be highly suspicious of cases of plague outside these geographical areas, specifically if pneumonic plague is the first presentation. Terrorists could pose a serious threat by using the bacteria in an attack. Local and state public health authorities should be notified immediately if a case of plague is suspected.⁸

During the 1950s and 1960s, the U.S. developed the bacterium Yersinia pestis as a biological weapon. The Soviet Union reportedly had more than 10 institutions and thousands of scientists working to develop plague-based weapons.² These weapons were based on the spread of aerosolized bacteria upwind from the targeted population. Japan even investigated using infected fleas dropped in areas of enemy troops as a way of spreading the plague. In 1970, WHO assessed a worst-case scenario of a dissemination of 50 kg of Yersinia pestis in an aerosol cloud over a city of 5 million. The agency estimated the results to be 150,000 cases of pneumonic plague, with 36,000 deaths.⁶

Exposure to Yersinia pestis can cause three forms of the plague: bubonic, septicemic, and pneumonic. Bubonic plague is usually a flea-borne disease transmitted from an infected rodent. Direct contact of open skin with infected tissue or fluids can also cause a bubonic form of the disease.⁵ The bacteria migrates to the lymph nodes, where an infection is established typically between two to eight days after exposure.¹⁵ Because infected fleas usually bite the legs of a victim, inguinal lymph nodes are most often affected (90%).⁷ As the infection develops, the lymph nodes become painful, swollen, and hot to the touch before ulcerating. If left untreated, septicemic infection results in fever, chills, prostration, abdominal pain, shock, and bleeding into the skin and other organs. The death rate is 50% to 60%.⁷ In some cases, septicemia can spread the infection to the lungs, causing the pneumonic form of the disease.

Pneumonic plague causes fever, chills, cough and difficulty breathing; rapid shock; and death. Talking, coughing, or sneezing spreads the disease in heavy droplets from person to person. The incubation period is one to four days,⁷ depending on the dose of inhaled bacteria. Pneumonic disease is characterized by overwhelming pneumonia, fever, bloody sputum, chills, and cough. The death rate is more than 50% and approaches 100% if treatment is not instituted within 24 hours of the onset of symptoms. Culturing or Gram staining lymph-node needle aspirate, sputum, or cerebrospinal fluid samples makes the diagnosis.⁵ Additionally, a complete blood count, urinalysis, and arterial blood gases (ABGs) should be obtained.

Test results that may indicate pneumonic plague are:

• A markedly elevated white blood count to levels of 20,000 or greater and a shift to the left. In late septic shock, the WBC count may be low.
  
• Gross hematuria, RBC casts, and proteinuria found on urinalysis.
  
• ABG evidence of hypoxia and/or acidosis.¹⁸
  
• Yersinia pestis is a nonmotile, Gram-negative bacillus that shows bipolar (looks like a safety pin) staining with Wright, Giemsa, or Wayson stain.¹⁵

Patients diagnosed with bubonic plague and a cough or those with pneumonic plague must be placed on droplet precaution in a private room or cohorted with similarly diagnosed patients.^15,19

Decontamination of these patients is not necessary, but caregivers in close contact with them must wear appropriate protective equipment, including masks and eye protection; to limit droplet production, patients should wear masks also. Patients are considered contagious until after 48 hours of antibiotic therapy.⁶ Antibiotic treatment for those exposed to the disease should continue for seven to 10 days. Streptomycin, tetracycline, chloramphenicol, gentamycin, and quinolone antibiotics are all effective.¹⁵

An attack involving infected mass casualties will rapidly deplete the pharmaceutical stocks in most hospitals. As a result, many area public health and state health agencies provide additional local stockpiles of antibiotics for treating mass casualties related to an intentional attack. The Center for Disease Control and Prevention also maintains a stockpile program called the Strategic National Stockpile. There are currently 13 SNSs in the United States, placed in different secret locations for rapid deployment anywhere in the country. They contain antibiotics and other materials to treat mass casualties from terrorist attacks. Typically, the initial stockpiles, called a “push pack,” contain the following antibiotics:²⁰

• More than 200,000 patient days of fluoroquinolone prophylaxis.
  
• More than 2,000,000 patient days of doxycycline prophylaxis.
  
• More than 20,000 patient days of pediatric fluoroquinolone prophylaxis.
  
• More than 40,000 patient days of therapeutic treatment.

The push pack can be followed by a “vendor managed inventory” that is more specific for the needs of the incident. In the case of a biological attack, the VMI may contain vaccines, additional antibiotics, or extra personal protective equipment.²⁰

The occurrence of either of these diseases requires a report to be filed with local public health agencies, and the CDC subsequently investigates all cases of inhalational anthrax. The CDC maintains the Health Alert Network, an around-the-clock Internet-based network of information about evidence-based practices and procedures for health care preparedness and response. When the CDC is informed about a possible biological attack, the Health Alert Network can be used to alert public health agencies in all 50 states to heighten their awareness to the threat. The network is also used for education and information sharing. In addition, The CDC uses Epi-X to alert public health officials across jurisdictions. Epi-X is a secure, Web-based communication system to enhance bioterrorism preparedness efforts by facilitating the sharing of preliminary information about disease outbreaks. The security of this system allows public officials to share information without generating fear among the public.²¹

Unfortunately, anthrax and plague are but two of four major biological agents that bioterrorists may use. The next module will discuss smallpox and botulism, as well as measures of emergency preparedness that may counter these threats.

Part 2 of 2

Almost daily, some type of terrorist activity is taking place in the world. Because of terrorist activity here in the United States, preparedness activities have reached beyond federal, state, and county agencies into our everyday lives. Nursing is one of the professions that has been affected the most dramatically with these preparedness efforts. Remember the obscure diseases like anthrax and plague that as a student nurse you never thought you’d see beyond a textbook? Chances are greater than ever that you might need to be adept at recognizing their symptoms. And you may have to confront other diseases that could emerge through the acts of terrorists. For example, what do you remember about botulism and smallpox? This module, Part 2 of a two-part series, will discuss botulism and smallpox and the nurse’s role in emergency preparedness in the event of an attack.

Botulism

Botulinus toxin, produced by Clostridium botulinum, is the most toxic substance known. A single gram of crystalline toxin evenly dispersed for inhalation over a population could kill more than 1 million people.²² Research found that only 0.001 mcg/kg of body weight was a lethal dose for 50% of a test animal population. To put this in perspective, botulinus toxin is about 100,000 times more toxic than the military nerve agent Sarin, which was used in the terrorist attack in a Tokyo subway.²³

During the Cold War, the Soviet Union extensively researched botulinus toxin as a biological weapon because of its extreme toxicity. In 1991, Iraq admitted to a United Nations inspection team its research in the use of botulinus toxin as a biological weapon. Four years later, it was discovered that Iraq had also filled and deployed more than 100 munitions containing the toxin. Here in the U.S., a religious extremist group, the Bhagwan Shree Rajneesh, gained recognition by cultivating Salmonella bacteria to contaminate restaurant salad bars and affect the outcome of a local election. Although the attempt to influence the election was unsuccessful, 751 people became ill and 45 were hospitalized. Authorities later found that the group intended to develop Clostridium botulinum for other malicious purposes.²³

Clostridium botulinum is a spore-forming bacillus that produces seven types of toxin. These toxins, types A through G, create similar effects through three presentations of botulism — food-borne, wound, and intestinal.²⁴ Naturally occurring cases are rare, and usually improperly prepared or canned food cause disease.²⁵ Once colonized in the body, the bacterium systemically releases the botulinus toxin, resulting in signs and symptoms that ranging from visual difficulty to flaccid paralysis.²⁴ To prevent botulism, all food must be heated to more than 240 degrees F or boiled for 10 minutes to destroy both bacterium and toxin.^23,26 Botulism is somewhat rare, but has occurred. The largest outbreak in the United States happened in 1977 when 59 people were identified and treated after eating poorly preserved jalapeño peppers.²³

Botulinus toxins enzymatically block acetylcholine release at the terminal end of presynaptic motor neurons,²² halting the conduction of stimulus across the synaptic junction in cholinergic autonomic sites. The interruption of neurotransmissions results in descending flaccid paralysis of motor and autonomic nerves, causing double or blurred vision, drooping eyelids, slurred speech, difficulty swallowing, dry mouth, and muscle weakness.²⁷ The early signs and symptoms include marked fatigue, weakness, and vertigo. These are followed by blurred vision, dry mouth, and difficulty swallowing and speaking. The early prominent signs can be easily remembered as the “4Ds” — diplopia, dysarthria, dysphonia, and dysphagia.²² The initial signs and symptoms are easily confused with Guillain-Barre syndrome or myasthenia gravis, and paralysis of the respiratory muscles can progress to respiratory failure. Definitive treatment may include fluids, nutritional support, and mechanical ventilation lasting from weeks to months.²² The presentation of this toxin-related clinical syndrome is known as “botulism.”

The onset of signs and symptoms is generally quicker after ingestion of the bacterium than after inhalation exposure. Depending on the dose, the onset of symptoms from ingestion can be two hours to 10 days, averaging 12 hours to 36 hours.^26,28Inhalation symptoms generally occur between 24 hours and 36 hours. Although symptoms progress to respiratory failure slower than with food-borne exposure,²⁸ one reported case resulted in respiratory failure in only 24 hours after the onset of symptoms.

Diagnosing this illness is difficult because symptoms can mimic many other diseases. Generally, patients will not have a fever, and cerebrospinal fluid will be normal. Paralysis is symmetrical, and there are no changes in mental status. The standard test for the toxin is to use serum or fecal specimens in a mouse bioassay. This test is time-consuming, and clinicians should not wait for definitive results from the bioassay to begin treatment. Instead, the decision to treat should be based on history and clinical findings. Therapy consists of passive immunization with an antitoxin and supportive care.²²

If botulism is suspected or diagnosed, it must be reported to the local public health department. To obtain the botulism antitoxin, the Centers for Disease Control and Prevention (CDC) must be contacted. Antitoxin administration is indicated as soon as possible after clinical diagnosis has been made. Severe cases of botulism require additional supportive treatment that may include the use of ventilators over a period of weeks or months.²⁹ Many communities have prepared for possible large-scale attacks by increasing the availability of ventilators for mass casualties. In addition, the CDC has available large quantities of ventilators through Strategic National Stockpiles.³⁰

Although the CDC has an antitoxin, it might not be used during a mass casualty event related to botulism exposure because up to 9% of those treated experience hypersensitivity.²² Any use of the antitoxin requires prior testing for an allergic response. This licensed antitoxin contains neutralizing antibodies against botulinus toxin types A, B, and E, the most common causes of human botulism. Only experimental antitoxins are available for the other toxin types.²² A vaccine has also been developed, but it is not available to the public unless an identified occupational risk is established. This vaccine is associated with many adverse effects, including some serious ones, such as anaphylaxis in as much as 2% of the population. To be effective, the vaccine must be given before exposure. After the initial dose, the vaccine is given at two and 12 weeks.²² Antibodies produced after the third dose slowly diminish, necessitating an annual booster.

People with botulism do not require decontamination and present no risk to health care workers. Botulism is not contagious and has never been reported to cause a person-to-person infection. There is no reason to isolate these patients; universal precautions are sufficient. Patients who survive the illness may have shortness of breath and fatigue for years, and complete recovery occurs only after all affected nerve endings are regenerated.²³

Smallpox

Exposure to variola virus causes the disease known as smallpox. Although the World Health Organization declared smallpox eradicated from the world population in 1980, fear remains that a state-sponsored terrorist group could use it as a biological weapon.²⁹ The high fatality rate and easy transmissibility of the variola virus makes smallpox the most serious terrorist threat from a biological weapon. And the virus still exists in laboratories in at least two locations: the CDC in Atlanta and at Vector in Novosibirsk in the former Soviet Union. Specimens may also exist in other locations, including North Korea, which may have acquired it from the Soviet Union.²²

Smallpox received its name at the end of the 15th century in England. Its original name, small pokes (poke means sac), distinguished it from syphilis, which was then called great pokes. Before vaccinations were available, almost everyone contracted the disease, but an aggressive vaccination program dramatically reduced the incidence of smallpox by the 1970s.³¹ The last naturally contracted case of smallpox occurred in October 1977 when Ali Maow Maalin, a hospital cook in Somalia, broke out with the world’s final case of variola, which resulted in the vaccination of 57,000 people and control of a possible outbreak. Ali survived the disease. In 1978, a British medical photographer, Janet Parker, died after an accidental exposure at the University of Birmingham in England.24 Her mother and father contracted the disease from her. Her father died of a heart attack early in the disease process, and her mother subsequently recovered from smallpox and became the last person on earth to publicly have the disease.³¹ There have been no subsequent cases. It has been reported that when several World Health Organization doctors told Ali Maow Maalin about the deaths in the Parker family, he wept, stating, “I’ll no longer be the last case of smallpox!”³²

Variola occurs in at least two principal forms: variola minor, which has a fatality rate of about 1%, and the more serious variola major. Both variola major and minor progress in a similar fashion, with variola minor having lesser signs and lasting for a shorter period of time.³¹

The incubation period after exposure is about 12 days. Symptoms begin with malaise, fever, rigors, vomiting, headache, and backache, with some patients developing delirium. Lesions in the mouth and throat appear early in the illness and release large amounts of virus into the saliva. This earliest period of infectivity occurs before any outward signs are noted. The smallpox rash appears several days after the other symptoms and progresses from macules to papules, which then become pustular vesicles. The progression of variola vesicles differs from varicella (chickenpox) in that they begin primarily on the face and extremities and in only small numbers on the trunk. This is the opposite of varicella, which usually begins on the trunk.²³ The illness remains contagious until all scabs separate, after about three weeks.

Although at least 90% of the cases follow the pattern described above, there are two other variations of the disease that present very differently: hemorrhagic and malignant. The hemorrhagic form of smallpox is always fatal. The illness begins with a shorter onset and rapidly progresses into severe prostration, high fever, and head, back, and abdominal pain. The skin produces an erythema followed by petechiae and frank hemorrhages on both the skin and mucous membranes. Death occurs within about five to six days. The malignant form of the disease is frequently fatal. The onset of symptoms is similar to the hemorrhagic form, but the confluent lesions develop slowly and never progress to the pustular stage. The lesions remain soft and flattened. If the patient survives, the lesions gradually disappear without forming a scab.³¹

Suspicion of the illness is based on the clinical presentation. Either electron microscopy used on a culture or Gispen’s modified silver stain then viewed under a microscope can yield clues to a microbiologist, but none that conclusively differentiate among variola or other orthopoxviruses, such as those causing monkeypox or cowpox. Suspected cases of variola must be reported immediately to local public health authorities. Because smallpox no longer naturally occurs, even one case will stimulate a national response from the CDC and experts from the Department of Homeland Security and other national law enforcement authorities. To isolate any outbreak, a strict quarantine with respiratory isolation of all people in direct contact with the index case will probably be implemented for at least 17 days.²³

Smallpox spreads directly from person to person through droplets and airborne virus during coughing, sneezing, or talking. It is more contagious during the pre-eruptive period. About 30% of those exposed through close contact will develop the disease, and 30% of those will die five to seven days after the onset of symptoms.²⁴ Health care providers must protect themselves from all body fluids, and extensive efforts to protect from respiratory exposure should be taken using N95 respirator masks, which are designed specifically for use in health care settings. Care should be taken when handling bed linen because contaminated clothing and linen can also spread the virus.³¹ All bed linen should be autoclaved or incinerated. Because of variola’s infectivity, the challenges of finding large isolation areas to provide care to numerous contagious patients may be overwhelming to hospitals and clinics. There is no drug available to directly treat smallpox, so care is supportive.²²

Late in 2002, the federal government released a plan to once again vaccinate the American public. The plan consisted of three phases. During Phase I, smallpox health care responders were vaccinated. These included hospital and health department personnel who would respond to care for patients infected with smallpox. These responders would also become the vaccinators to limit or stop an outbreak. Phase 2 provided vaccinations to emergency medical services personnel, firefighters, and law enforcement officers who would be the first responders to an outbreak. Phase 3 was for members of the public who requested the vaccine. Phase 3 was never initiated because of the possible side effects of the vaccine. These vaccines were all voluntary.³³

States initially expected that they would administer 450,000 doses to health workers during Phase 1. Though the CDC shipped almost 300,000 doses, fewer than 40,000 were given. The same situation took place during Phase 2. The low number of volunteers was related to the contraindications and general fear of receiving the vaccine.³⁴ The smallpox vaccine was not recommended for people who were immunocompromised. This included anyone on steroids, receiving chemotherapy, pregnant or expecting to become pregnant, or who has had an organ transplant. The vaccine was also not recommended for anyone who lived with a person in any of these categories. Additionally, individuals who had various heart conditions were not recommended to receive the vaccine. These included three or more of the following factors: high blood pressure, high cholesterol, diabetes, a first-degree relative with a heart condition before the age of 50, and/or a cigarette smoker.³⁵

It was determined that any person who had previously had a smallpox vaccine, no matter how many year prior, would have many fewer incidents of reactions.³⁶ Currently, research continues into developing a new and safer vaccine to protect the American public.

Even one case of smallpox would be considered an outbreak and treated as an act of terrorism. If an outbreak occurs in the U.S., the CDC plans to institute ring vaccinations. Simply stated, ring vaccinations are an effort to vaccinate every person who was in contact with a confirmed case of smallpox. When feasible, double ring vaccinations will be instituted to stop an outbreak. Double ring vaccinations include vaccinating all contacts of the contacts to a confirmed case of smallpox. This can include hundreds of thousands of people surrounding just one confirmed case.³⁷

If health care providers suspect that any patient has been infected with either botulism or smallpox, they must immediately call their local public health agency. Both botulism and smallpox are reportable diseases requiring immediate action within the community. If bioterrorism is suspected, the CDC immediately becomes involved, and a federal response will follow. Nurses as frontline health care providers must be able to recognize signs and symptoms of these diseases and know what needs to be done to protect themselves and others if the disease is contagious.

Unlike the immediate effects of bombings or chemical incidents, the effects of a clandestine use of a biological agent could progress over hours, days, and weeks, rapidly becoming a public health emergency capable of causing mass anxiety and even panic. To remain safe and offer the best care, providers must understand the implications and clinical issues resulting from the intentional heinous use of a biological agent.

The CDC, recognizing that local health care providers cannot do it all, has developed nine Strategic National Stockpiles to counter large chemical or biological attacks. These stockpiles are strategically located around the country to be mobilized during a terrorist disaster. When brought to a community, they provide large supplies of antibiotics, chemical antidotes, and other medical supplies to help in the health care management of mass casualties. It would take less than 12 hours for a stockpile to arrive in any region of the U.S., but the community must manage the logistical dispersal of medication and supplies.38 If plans don’t already exist for dispersal and personnel and transportation resources haven’t already been identified, the stockpile’s use will be limited during the disaster. Health care providers must take immediate, active steps to ensure that the plans and needed resources for the dispersal of this stockpile have been addressed and identified in their community or region.³⁸

Preparedness efforts for terrorism are widespread and include many disciplines. Most hospitals have extensive plans for dealing with mass casualties related to trauma. Accreditation agencies require hospitals to practice these plans at least annually to ensure proficiency during a disaster. However, acts of terrorism have consequences for health care organizations that differ from the typical mass casualties of trauma. In these cases, contaminated or contagious mass causalities as well as larger numbers of psychologically injured victims will confront already taxed health care systems. The Joint Commission on Accreditation of Healthcare Organizations (JCAHO) recognizes that this type of preparedness is different and recently issued a 24-page advisory emphasizing the need for a new level of preparedness. The level at which hospitals prepare for terrorism and mass emergencies will be a key part of JCAHO’s quality-rating process, and deficient organizations may risk losing accreditation. Hospitals and other health care providers must react with education for the clinical staff, better and more comprehensive plans, effective personal protective equipment, and unprecedented coordination among local, state, and federal agencies.³⁹

Health care employees need assurances that they will be protected first if public health representatives determine that vaccinations or post-exposure prophylaxis is needed. Without these assurances, a health care agency runs the risk that staff will leave or refuse to come to work. Health care agencies should increase their stock of pharmaceuticals so that assurances made to employees can be carried out if a biological attack takes place. Planning for terrorism must extend beyond traditional boundaries to involve cooperation among competing health care organizations so that ideas, best practices, and resources can be assessed and consistently used.³⁹

Emergency preparedness efforts are under way in many acute health care facilities. These efforts should include moving triage outside of the hospital during a disaster to lessen the possibility of contaminating waiting rooms and emergency departments. Hospitals and clinics are increasing decontamination capabilities for mass casualties of biological or chemical attacks. Plans for satellite treatment facilities to reduce the burden and risk of overwhelming hospitals and clinics are being discussed and developed in most urban communities. Hospitals and clinics are developing disaster plans that complement each other and draw on each other’s resources. These health care disaster plans are working more urgently with other local resources, such as emergency medical services, law enforcement, and fire departments. An act of terrorism is not just a concern of hospitals, but of communities, states, and the nation.³⁹

Recently, we’ve witnessed horrible acts of violence around that world that have caused thousands of deaths and injuries to innocent people. We watched as suicide bombers have created death and fear throughout the world. We’ve seen our own troops in foreign counties attacked as they act as peacekeepers for other counties. And in the fallout of these terrible events, health care providers have been diligently preparing for events such as these to occur in the United States. Both JCAHO and OSHA have developed guidelines for training and preparedness for health care providers dealing with mass casualties from terrorist incidents.^39,40

The intentional outbreaks of anthrax in New York; Washington, DC; and Boca Raton, Fla., brought hundreds of worried well into local emergency rooms wondering whether their flu symptoms were related to a hidden intentional exposure to anthrax.⁴¹ The outbreak of severe acute respiratory syndrome (SARS) that occurred in 2003 caused serious concern for the American public. Although this was a naturally occurring event, it became an eye-opening experience that demonstrated how our ability to rapidly travel the world could contribute to an uncontrolled spread of a contagious disease.

The biological agents that terrorists could use have the capability to generate casualties in the thousands. Anthrax, plague, botulism, and smallpox are all examples of these. Probably the biggest challenge in the health care environment is to find room to care for large numbers of infected patients and to do so safely. To make matters worse, hospitals across the nation are suffering with emergency department overcrowding.⁴² Additionally, there has been a loss of 38,000 hospital beds (4.4%) nationwide since 1996. This includes a 20% decrease of ICU capacity from 1995 to 2001.⁴³

Planning for mass casualties goes beyond separate health care facilities. Planning must occur on community, regional, and state levels. Planning for patient surge must be scaled and flexible to allow for the care of large numbers of patients on any given day. Help has come from several departments of the federal government in the form of grant funding.44 The Health Resources and Services Administration, a division of the U.S. Department of Health and Human Services, has been one of the most active in offering funds for hospitals to prepare through planning and training and to purchase equipment needed to treat mass casualties.⁴⁴

But the buck stops here. Health care providers represent the last stop for victims needing medical care from acts of terrorism. We must take on that challenge and continue to prepare for the worst while providing the best care available.