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 ranging from visual difficulty to flaccid paralysis.²⁴ To prevent botulism, all food must be heated to more than 240 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 U.S. 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.^26,28 Inhalation 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 years 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 casualties 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 the 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.