Terrorism WHAT CLINICIANS NEED TO KNOW. and. Disaster. Smallpox: Recognition, Management, & Containment

Transcription

1 Terrorism WHAT Disaster and CLINICIANS NEED TO KNOW Smallpox: Recognition, Management, & Containment

2 and T errorism Disaster WHAT CLINICIANS NEED TO KNOW SERIES EDITORS Rush University Medical Center Chicago, Illinois Stephanie R. Black, MD* Assistant Professor of Medicine Section of Infectious Diseases Department of Internal Medicine Daniel Levin, MD* Assistant Professor General Psychiatry Residency Director Department of Psychiatry Gillian S. Gibbs, MPH* Project Coordinator Center of Excellence for Bioterrorism Preparedness Linnea S. Hauge, PhD* Assistant Professor Educational Specialist Department of General Surgery AUTHORS Rush University Medical Center Chicago, Illinois Stephanie R. Black, MD* Assistant Professor of Medicine Section of Infectious Diseases Department of Internal Medicine Daniel Levin, MD* Assistant Professor General Psychiatry Residency Director Department of Psychiatry Uniformed Services University Health Sciences Bethesda, Maryland David M. Benedek, MD, LTC, MC, USA* Associate Professor of Psychiatry Steven J. Durning, MD, Maj, USAF, MC* Associate Professor of Medicine Thomas A. Grieger, MD, CAPT, MC, USN* Associate Professor of Psychiatry Associate Professor of Military & Emergency Medicine Assistant Chair of Psychiatry for Graduate & Continuing Education Molly J. Hall, MD, Col, USAF, MC, FS* Assistant Chair & Associate Professor Department of Psychiatry Derrick Hamaoka, MD, Capt, USAF, MC, FS* Director, Third Year Clerkship Instructor of Psychiatry Paul A. Hemmer, MD, MPH, Lt Col, USAF, MC* Associate Professor of Medicine Benjamin W. Jordan, MD, CDR, MC, USNR, FS* Assistant Professor of Psychiatry James M. Madsen, MD, MPH, COL, MC-FS, USA* Associate Professor of Preventive Medicine and Biometrics Scientific Advisor, Chemical Casualty Care Division, US Army Medical Research Institute of Clinical Defense (USAMRICD), APG-EA Deborah Omori, MD, MPH, FACP, COL, MC, USA* Associate Professor of Medicine Michael J. Roy, MD, MPH, FACP, LTC, MC* Associate Professor of Medicine Director, Division of Internal Medicine Jamie Waselenko, MD, FACP** Assistant Professor of Medicine Assistant Chief, Hematology/Oncology Walter Reed Army Medical Center Washington, DC Guest Faculty Ronald E. Goans, PhD, MD, MPH* Clinical Associate Professor Tulane University School of Public Health & Tropical Medicine New Orleans, LA Sunita Hanjura, MD* Rockville Internal Medicine Group Rockville, MD Niranjan Kanesa-Thasan, MD, MTMH* Director, Medical Affairs & Pharmacovigilance Acambis Cambridge, MA Jennifer C. Thompson, MD, MPH, FACP* Chief, Department of Clinical Investigation William Beaumont Army Medical Center El Paso, TX Faculty Disclosure Policy It is the policy of the Rush University Medical Center Office of Continuing Medical Education to ensure that its CME activities are independent, free of commercial bias and beyond the control of persons or organizations with an economic interest in influencing the content of CME. Everyone who is in a position to control the content of an educational activity must disclose all relevant financial relationships with any commercial interest (including but not limited to pharmaceutical companies, biomedical device manufacturers, or other corporations whose products or services are related to the subject matter of the presentation topic) within the preceding 12 months If there are relationships that create a conflict of interest, these must be resolved by the CME Course Director in consultation with the Office of Continuing Medical Education prior to the participation of the faculty member in the development or presentation of course content. * Faculty member has nothing to disclose. **Faculty disclosure: CBCE Speaker s Core for SuperGen.

3 Smallpox: Recognition, Management, & Containment CASE AUTHORS: Deborah Omori, MD, MPH, FACP, COL, MC, USA David M. Benedek, MD, LTC, MC, USA DISCLAIMER This project was funded by the Metropolitan Chicago Healthcare Council (MCHC) through a grant from the Health Resources and Services Administration (HRSA). The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as representing the opinion of Rush University Medical Center, the Department of the Army, Department of the Navy, Department of the Air Force, Department of Defense, MCHC or HRSA. FDA Approved Drug and Devices Assurance Statement In accordance with requirements of the FDA, the audience is advised that information presented in this continuing medical education activity may contain references to unlabeled or unapproved uses of drugs or devices. Please refer to the FDA approved package insert for each drug/device for full prescribing/utilization information. INSTRUCTIONS The questions that appear throughout this case are intended as a self-assessment tool. For each question, select or provide the answer that you think is most appropriate and compare your answers to the key at the back of this booklet. The correct answer and a discussion of the answer choices are included in the answer key. In addition, a sign is provided in the back of this booklet for posting in your office or clinic. Complete the sign by adding your local health department s phone number. Design and layout 2005 Rush University Medical Center. The text contained herein falls under the U.S. Copyright Act of 1976 as a U.S. Government Work and is therefore considered Public Domain Information, however Rush University Medical Center reserves the right to copyright the design and layout of that information. 1

4 Smallpox: Recognition, Management, & Containment CASE AUTHORS: Deborah Omori, MD, MPH, FACP, COL, MC, USA David M. Benedek, MD, LTC, MC, USA INTENDED AUDIENCE Internal medicine, family medicine, and emergency medicine physicians, and other clinicians who will provide evaluation and care in the aftermath of a terrorist attack or other public health disaster EDUCATIONAL OBJECTIVES Upon completion of this case, participants will be able to: List the differential diagnosis of illnesses that present with a fever and a rash. Summarize key signs and symptoms that distinguish early smallpox from influenza or chickenpox. Explain the medical management for the treatment of smallpox. Describe infection control measures to ensure healthcare worker safety. Describe complications of the smallpox vaccine and issues surrounding an outbreak of smallpox, as well as methods for reducing concerns regarding vaccine availability and side-effects. Outline strategies for communicating to authorities and concerned others about a suspected outbreak of smallpox. CASE HISTORY A 37-year-old high school teacher calls your office one Monday morning in mid- January for an appointment that day complaining of the flu. She has a history of asthma, but otherwise is a healthy person. Her last visit to you was a year ago for her annual checkup. When you see her that afternoon, she looks fatigued and has difficulty sitting in a comfortable position, apparently due to her backaches. She reports that she returned from vacation in Germany 12 days ago. The patient did not receive a flu shot this year. She complains of a headache and backache that started suddenly the previous afternoon with fever and chills. Her temperature is F, heart rate 100, respiratory rate 20, and blood pressure 110/70. Physical examination is otherwise unremarkable except for several small erythematous macular lesions on the oral mucosa. 2

5 COMMENT: Given the time of year, the absence of a rash, and her constellations of symptoms, you may be thinking of influenza, not smallpox or chickenpox. However, the enanthem, lesions in the mucosa of the oro-pharynx where the virus has invaded the dermal blood vessels, would not be consistent with influenza. Besides a travel history, other questions to ask include recent contact with ill persons, an occurrence of or exposure to chickenpox in the area, a history of prior smallpox or varicella vaccination, a prior history of chickenpox or herpes zoster, new medications, and occupational history (eg, lab worker). 1 Smallpox is caused by the variola virus (genus Orthopoxvirus). Patients exposed to the most common form of smallpox, variola major, will have a symptom-free incubation period of 7-17 days, with an average of 12 days. 1,2 During this time, the variola (orthopox) virus is multiplying in the liver, spleen, and reticuloendothelial system. 2 The enanthem appears about a day before the rash, and patients are potentially infectious at this time. Initial symptoms appear as a febrile prodrome characterized by the abrupt onset of high fevers ( 101 F), chills, backache, headache, malaise, and a toxic-appearing patient. 1-4 Additional symptoms may include vomiting or abdominal pains. You should maintain a high level of suspicion if there are other clues that this is not the usual influenza, including severe prostration and mucosal lesions, since variola major smallpox has a case-fatality rate of 30% in unvaccinated patients. 2,3 The rash, beginning on the face and extremities, does not appear until 1-4 days after the prodrome of fever and constitutional symptoms, so the clinical picture initially may be mistaken for the flu or some other viral syndrome. Smallpox is spread through aerosol droplet transmission or infected clothing from person to person. 2,3 Smallpox is most infectious with the onset of the rash, especially for the first 7 to 10 days, but the risk of transmission persists until all the scabs have fallen off in about 3 weeks. The rash begins as small, red macules that evolve into 2-3 mm embedded papules in 1 to 2 days. The papules then turn into 2-5 mm vesicles in another 1-2 days before transforming into 4-6 mm pustules. After 5 to 8 days the pustules become umbilicated and crusted over. At the end of 2 weeks the crusts start to separate and by 3 weeks, they all fall off to leave pitted scars. 1-4 To ascertain an individual patient s risk for smallpox, review the criteria in Table 1. Table 1. Smallpox Risk Categorization and Diagnostic Criteria* Major Smallpox Criteria 1. Febrile Prodrome: occurring 1-4 days before rash onset; fever 101 F and at least one of the following: prostration, headache, backache, chills, vomiting, or severe abdominal pain 2. Classic smallpox lesions: deep-seated, firm/hard, round wellcircumscribed vesicles or pustules; as they evolve, lesions may become umbilicated or confluent 3. Lesions in the same stage of development: on any one part of the body (eg, the face or arm) all the lesions are in the same stage of development (ie, all are vesicles or all are pustules) Risk Categories Low Risk Moderate Risk High Risk 1. No febrile prodrome OR 1. Febrile prodrome and 2. < 4 MINOR smallpox criteria 1. Febrile Prodrome and 2. One other MAJOR smallpox criterion OR 1. Febrile prodrome and 2. > 4 MINOR smallpox criteria * Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. 1,5 Minor Smallpox Criteria 1. Centrifugal distribution: greatest concentration of lesions on face and distal extremities 2. First lesions on the oral mucosa/palate, face, or forearms 3. Patient appears toxic or moribund 4. Slow evolution: lesions evolve from macules to papules to pustules over days (each stage lasts 1-2 days) 5. Lesions on the palms and soles 1. Febrile prodrome and 2. Classic smallpox lesion and 3. Lesions in same stage of development 3

6 With her current signs and symptoms and lack of classic smallpox lesions, the patient is in the low risk category for smallpox, and you diagnose her with a viral syndrome. You recommend acetaminophen for fever and you tell her to stay home, push fluids and to follow up in 2 days. When she comes back to your office 2 days later, she reports that following your advice she went straight home after calling the school to report her illness, and has remained in bed with no contact with others since her appointment. Today she complains of a new rash and feeling so ill that she barely made it in. She noticed onset of the rash on her face and arms yesterday morning. Her fever has decreased to 101 F but she still complains of backaches, headache, and a slight cough. On evaluation, she looks toxic-appearing. Her lesions are all small vesicles with the majority on her face, forearms, palms, and legs. They appear to be deeply embedded and firm. A few lesions are on her trunk. COMMENT: At this point smallpox must be distinguished from other illnesses causing fever and a rash. 1,2,6 Chickenpox (varicella) is common and can be confused with smallpox. Because of the serious consequences of a smallpox outbreak, chickenpox must be differentiated from smallpox (Table 2). With chickenpox, the rash usually appears with onset of the fever and the lesions are in different stages of development with papules, vesicles, and crusting lesions appearing at the same time. 2 The pruritic rash of chickenpox is typically concentrated on the trunk and face and less so on the arms and legs (ie, centripetal distribution). In smallpox, the lesions are mostly concentrated on the face and extremities in a centrifugal distribution 3 (see Figure). The vesicles of chickenpox are superficial-appearing ( dewdrop on a rose petal ) unlike smallpox, where the vesicles are deep-seated and firm. 5 Lesions on the palms and soles are rare in chickenpox and patients do not appear very ill. Other illnesses that should be considered and excluded by history and/or physical examination include drug-induced rashes, erythema multiforme, disseminated herpes zoster or simplex, impetigo, contact dermatitis, molluscum contagiosum, rickettsial diseases, and enteroviral infections. 1,2,5 FIGURE. Child With the Typical Centrifugal Lesions of Smallpox. Slide courtesy of the World Health Organization. Table 2. Differentiating Chickenpox from Smallpox* Chickenpox Smallpox Prodrome None or mild Febrile prodrome 1-4 days before rash onset Appearance of lesions Superficial vesicles: Deep seated, firm/hard, round postules dewdrop on a rose petal Stages of development Different stages of development Same stage of development of lesions on any one part of the body Distribution of lesions Centripetal distribution: concentrated Centrifugal distribution: concentrated on the trunk, fewest on extremities on the face and extremities Rare on palms and soles Appear on palms and soles * Centers for Disease Control and Prevention. Poster: Evaluating Patients for Smallpox. 6 4

7 QUESTION 1 Your patient meets criteria for being at high risk of having smallpox with her febrile prodrome, the classic smallpox lesions, and lesions (vesicles) in the same stage of development. She also has 4 minor smallpox criteria. What should you do immediately? (Select all answer choices that are appropriate.) a. Send the patient home for home quarantine. b. Obtain fluid from the skin for a PCR test for varicella. c. Call the CDC immediately to report an outbreak. d. Immediately call your Infectious Disease consultant. e. Place the patient in a private room with the door closed. Reminder: You can find the Answer Key & Discussion on page 9. You contact your Infectious Disease (ID) consultant, who immediately comes to your office, evaluates your patient, and agrees with your assessment. Your local health department official is contacted and listens to the ID consultant s description of your patient s symptoms and her skin findings. The health department official determines that your patient is in the high risk category and a probable case. (A confirmed case of smallpox is one that is confirmed by laboratory testing or satisfies the clinical case definition and is epidemiologically linked to a laboratory confirmed case. 1 ) The CDC s definition of a clinical case of smallpox is: an illness with acute onset of fever 101 F (38.3 C) followed by a rash characterized by firm, deep-seated vesicles or pustules in the same stage of development without other apparent cause. 1 The CDC s definition of a clinical case of smallpox is: an illness with acute onset of fever 101 F (38.3 C) followed by a rash characterized by firm, deep-seated vesicles or pustules in the same stage of development without other apparent cause. 1 The health department official recommends immediate isolation of your patient, preferably at a Type C (contagious) facility with non-shared ventilation systems that exhaust air through a HEPA filter to the outside. If not available, then the patient should be isolated at a hospital facility with strict airborne and contact precautions, and in a negative air pressure room in which the air is vented externally. She recommends that healthcare personnel who will be in contact with the patient wear personal protective equipment (PPE), which includes an N95 respirator, a gown, and gloves. While arrangements for admission are being made, she advises isolating your patient immediately in a private room (preferably one with negative airflow), closing the door, placing a surgical mask on the patient, and covering the patient prior to transport. The health department official confirms that you have called your infection control department to apprise them of the situation. She will in turn report the case to the CDC immediately, who will provide further instructions and recommendations for testing your patient. Infection Control, in collaboration with the health department, will also begin the process for tracking down, prioritizing, and vaccinating exposed individuals (contacts) of this probable case. QUESTION 2 Which of the following actions should be taken next? (Select all answer choices that are appropriate.) a. Immediately release all patients and office personnel from your office. b. Take digital photos of the patient s rash. c. Provide your office personnel with fitted N95 respirators, gloves, and gowns. d. Vaccinate everyone in your office. 5

8 SMALLPOX VACCINATION CLINICS 9 As soon as possible after the first case is confirmed, state and local health officials, in collaboration with the CDC, will determine the appropriate scope of the vaccination response, based on size of the initial outbreak, the amount of vaccine available, and the possibility that additional new and epidemiologically related cases will be identified in subsequent days. It is important to develop your hospital s plan for operating an internal smallpox vaccination clinic for employees and/or patients, before an outbreak occurs. Potential sites for the vaccination clinic should be considered. A vaccination team should be identified and trained. Team members do not need to receive the smallpox vaccine in the pre-event setting, but should be pre-screened for eligibility. For more information on planning your vaccination response, see the CDC Smallpox Response Plan and Guidelines. 1 Those at greatest risk of contracting smallpox, and who should be vaccinated first, are household contacts of the case and those with face-to-face exposure. COMMENT: Smallpox vaccine is a live-virus vaccine and is available through the CDC. It is made from live vaccinia virus (from the same genus Orthopoxvirus as variola virus) that multiplies in the epithelium and develop into vesicles in 5-8 days that become grayish-white pustules about 1-2 cm in size. 2 Within a week, redness, swelling, and soreness develop around the pustule. Patients develop fever, myalgias, and lymphadenopathy. Crusting begins in the center and spreads outwards in another 3-5 days developing into a scab. In 2 to 3 weeks, the scab falls off leaving a pitted scar. If the smallpox vaccination is given within 3 days after exposure, it will prevent or lessen the severity of smallpox symptoms. 4 Even vaccination 4 to 7 days after exposure can lessen the severity of symptoms. 9 Those at greatest risk of contracting smallpox, and who should be vaccinated first, are household contacts of the case and those with face-to-face exposure, since smallpox is spread by direct transmission of infected droplets. In an outbreak, the CDC recommends surveillance and containment of smallpox by identifying and isolating smallpox cases and vaccinating close and potential contacts in a Ring Vaccination strategy. 2,9 In this strategy, groups are prioritized and vaccinated starting with the high-risk groups and working down the risk groups (Table 3). 4,9 Primary and secondary contacts need to be identified, vaccinated, and monitored. Healthcare workers who will be taking care of patients with smallpox will need to be vaccinated as well as other people who might come into contact with cases, such as emergency response teams and law enforcement personnel (see sidebar). 9 Table 3. Prioritization of Risk Group Categories for Vaccination* Priority Risk Group 1 Face-to-face or close-proximity contacts (< 6.5 feet or 2 meters) or household contacts of smallpox patient after onset of patient s fever 2 Persons exposed to initial release of virus 3 Household members of contacts to smallpox patients (if case contacts develop smallpox while under surveillance at home) 4 Persons involved in direct medical care, public health evaluation, first responders, law enforcement, or transportation of confirmed or suspected smallpox patients 5 Laboratory personnel involved in collection and/or processing of clinical specimens from suspected or confirmed cases 6 Others with high likelihood of exposure to infectious materials (hospital laundry, waste disposal, disinfection) 7 Those involved in contact tracing and vaccination or quarantine/isolation or enforcement, or law enforcement interviews of suspected cases 8 Those essential personnel allowed to enter facilities designated for the evaluation, treatment, or isolation of confirmed or suspected cases 9 Those present in a facility with a smallpox case if fine-particle aerosol transmission was likely during the time the case was present and not yet isolated * Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. 4,9 Starting with highest risk. Contraindications for receiving the smallpox vaccine include women who are pregnant, those with immunodeficiency disorders or undergoing immunosuppressive treatments, skin diseases such as severe eczema, atopic dermatitis, active shingles, recent burns, or a serious allergy to a vaccine component. 4,9,10 These contraindications would apply in the absence of any exposure pre-event. There are no contraindications post-event. 6

9 Possible complications of the smallpox vaccine include: accidental inoculation (comprise 50% of all complications and usually due to viral transfer from patient s hand to other parts of the body) generalized vaccinia (caused by viremia with development of a vesicular rash on the trunk and abdomen, usually a benign self-limiting condition in patients with no serious medical illness) erythema multiforme eczema vaccinatum, in patients with a history of eczema or atopic dermatitis postvaccinal encephalitis (with a 40% case fatality rate) progressive vaccinia (a potentially fatal illness especially if immunosuppressed) bacterial infections at the vaccination site (usually Staphylococcus aureus or Group A Beta Hemolytic Streptococci). 2,4,9,10 Vaccinia Immune Globulin (VIG) can be given for the eczema vaccinatum, progressive vaccinia, and for severe complications of generalized vaccinia and accidental inoculation. 9,10 VIG should not be given to treat postvaccinal encephalitis (not effective) or vaccinia keratitis (it can cause corneal scarring). 10 The safety of the smallpox vaccine was examined in 450,293 US military and Department of Defense civilian workers given the smallpox vaccine from December 2002 through May There were 37 (.008%) cases of acute myopericarditis, 36 (.008%) with generalized vaccinia, 48 (.01%) who experienced autoinoculation and 21 who had contact transfer, as well as a single case of encephalitis. 11 This experience demonstrated that large-scale smallpox vaccinations can be administered safely with low rates of adverse reactions. 11 QUESTION 3 Your patient is hospitalized in a Type C (contagious) facility with strict airborne and isolation precautions. How should you medically manage your patient at this point? (Select all answer choices that are appropriate.) a. broad-spectrum antibiotics b. supportive care c. VIG d. smallpox vaccine e. anti-viral agent Cidofovir The practice s CEO, under the guidance of the CDC, decided to close the clinic for the remainder of the week to take time to recover from the scare and to allow public health officials to ensure that the clinic is cleared for further patient care. Your home phone has been ringing constantly over the course of the week. Apparently, some word about a potential smallpox case has leaked out since other recent patients have been calling with concerns for their own safety. You realize that patients seen in the office before the teacher presented are not at any risk from merely being in your office, but you recognize that worry and fear are understandable responses to the news of an outbreak of a lethal and contagious disease. You have changed your answering machine message to advise concerned parents from the teacher s school to contact the board of administration or local or state health officials with questions. When you return to the office the following Monday morning, you find that the normally calm waiting room is filled with a sense of tension and anxiety. The local television station has sent a news reporter to gather information. There is a crowd outside the waiting room. The office manager re-opened the clinic this morning as 7

10 scheduled, although the receptionist and the morning nurse have not shown up to work. You answer the ringing phone. Your patient s school nurse is calling with concerns over student safety. On another line the principal wants to know when the teacher is expected to return to work, and if she can receive visitors. Employees from non-medical offices within the building, as well as parents and students (some wearing face masks) are crowding the waiting room. Some believe they are infected, some demand vaccination, while others carry signs announcing the dangers of vaccination. COMMENT: The above scenario highlights the importance of effective risk communication, both for the public as well as within the healthcare community. Recent experience with outbreaks of infectious disease (eg, SARS), as well as surveys of hospital employee behavior in the aftermath of terrorist activities, suggest that healthcare worker absenteeism may pose serious challenges to the delivery of care during a crisis You can enlist the media to help deliver clear and concise information to the public. The principal should be reminded of patient confidentiality with medical information. The crowd in the waiting room should receive information to address their concerns. Delivery of clear and concise information through redundant channels, including enlisting the media to help deliver the message, may reduce the burden on the medical system. QUESTION 4 At this point, preventive measures that may allow for continued delivery of care to those in need and restore a sense of normalcy for others include which of the following: (Select all answer choices that are appropriate.) a. Call for law enforcement personnel to assist in removing the television reporter from your office. b. Provide appropriate assurance to the crowd regarding the safety of the clinic environment. c. Close the clinic and advise building employees and others in the waiting area that unless they have already scheduled appointments they should contact their own physicians or the CDC for more information. d. Print information sheets that describe signs and symptoms of smallpox, and prompt referral, as well as sheets describing the indications, benefits, and risks of smallpox vaccination to all concerned parties. The CDC quickly set up a smallpox vaccination clinic at the county hospital s Infectious Disease Clinic. Besides helping the local health department with disseminating updated information to the public, the CDC has begun screening and vaccinating people in your area using the Ring Vaccination strategy. Vaccination of emergency responders and healthcare workers in the city has also begun. The local health department is contacting your other patients and people who had potential contact with your patient so they can be vaccinated. Your patient, still in isolation, is currently stable with supportive care and has developed pustules mainly over her arms, legs, and face. Her variola DNA test result was positive for smallpox. Fortunately, no new cases have surfaced thus far, but doctors offices in your area are being inundated with anxious patients. The local police helped with dispersing the crowd that gathered around your office building, but your office is still closed because of the publicity that has attracted national media attention. Besides taking care of your smallpox patient with the help of the Infectious Disease consultant and the CDC, you volunteer your time at the smallpox vaccination clinic. It is still unclear how your patient acquired smallpox, but the CDC is investigating her trip to Germany. 8

11 ANSWER KEY & DISCUSSION QUESTION 1 Your patient meets criteria for being at high risk of having smallpox with her febrile prodrome, the classic smallpox lesions, and lesions (vesicles) in the same stage of development. She also has 4 minor smallpox criteria. What should you do immediately? (Select all answer choices that are appropriate.) a. Send the patient home for home quarantine. b. Obtain fluid from the skin for a PCR test for varicella. c. Call the CDC immediately to report an outbreak. d. Immediately call your Infectious Disease consultant. e. Place the patient in a private room with the door closed. ANSWER: The correct answers are d and e. If your patient meets criteria for high risk of smallpox, contact your Infectious Disease consultants for an immediate evaluation. If your patient is deemed to indeed be high risk, report the case immediately to your local or state health department. The local or state health department will then evaluate the case and contact the CDC Emergency Operations Center ( ) if your patient is determined to be at high risk. The state and local health authorities will also start the mobilization of resources for outbreak control. If you suspect your patient has smallpox, you must immediately isolate the patient to prevent further exposure to other individuals and patients in your office. Smallpox is transmitted primarily from person-to-person and from contaminated clothing and bedding. Infection control measures start with contact and airborne precautions. 1,7 In a patient who meets criteria for high risk of smallpox (clinical case definition), testing for variola DNA case confirmation of smallpox should be done at a laboratory within the CDC Laboratory Response Network. 1 In the absence of an outbreak, testing only those who fit the clinical case definition lowers the chance of a false positive test. 1 If your patient is in the moderate risk category, then you would perform the rapid testing for varicella-zoster virus after you have contacted your Infectious Disease or Dermatology consultant. 1 Home quarantine is not appropriate in the case of smallpox. Patients suspected of having smallpox need to be isolated at a Type C facility, if available, or a hospital with strict airborne and contact isolation precautions in place, while evaluation and testing proceeds. 7 QUESTION 2 Which of the following actions should be taken next? (Select all answer choices that are appropriate.) a. Immediately release all patients and office personnel from your office. b. Take digital photos of the patient s rash. c. Provide your office personnel with fitted N95 respirators, gloves, and gowns. d. Vaccinate everyone in your office. ANSWER: The correct answers are b, c, and d. The CDC recommends taking digital photos of the high risk patient to aid in consultation. 1 To decrease the transmission risk while awaiting further instructions, the CDC recommends airborne and contact precautions. Smallpox is transmitted primarily from person-to-person and from contaminated clothing and bedding. Infection control measures start with contact and airborne precautions. 1 If your patient is deemed to be a suspect or probable case of smallpox, then your patient and contacts (office personnel and exposed patients) will need to be vaccinated against smallpox. 1,2 Vaccination of persons exposed to a case within 2-3 days after exposure 9

12 should prevent or decrease the severity of smallpox. 8 Persons vaccinated up to 4-7 days after exposure may also benefit, most often by experiencing a less severe illness. Patients who were seen at your office when your patient with smallpox came in 2 days ago will also need to be contacted. Although patients with smallpox are not infectious until the rash appears, the CDC recommends vaccinating people who had contact with cases when they were febrile in the event that the case had a faint or unrecognized rash at that time. 1 The CDC and local health authorities will provide recommendations on the scope of the vaccination response, as well as distribute the smallpox vaccine. It is important to develop your hospital's plan for operating an internal smallpox vaccination clinic for employees and/or patients, before an outbreak occurs. Your office personnel and patients may have been exposed to smallpox. A primary contact is one who has contact with a confirmed, probable, or suspected case who is believed to have been infectious at the time of contact. The risk of smallpox increases the longer one has face-to-face contact at a distance of 2 meters or 6.5 feet. 1 It has been reported that one case of smallpox may result in 5-7 secondary cases. 1 After your patient has been isolated in a private room, your office personnel and patients need to be gowned, gloved, and fitted with N95 respirator masks while awaiting further instructions from the CDC and your local or state health department. 7 If your office personnel were not wearing any personal protective equipment (PPE) when they initially encountered the patient, they may need to be furloughed from work until it is clear they have not been infected. The local public health department will assist in making this decision. QUESTION 3 Your patient is hospitalized in a Type C (contagious) facility with strict airborne and isolation precautions. How should you medically manage your patient at this point? (Select all answer choices that are appropriate.) a. broad-spectrum antibiotics b. supportive care c. VIG d. smallpox vaccine e. anti-viral agent Cidofovir ANSWER: The correct answers are b and d. Treatment consists mainly of supportive care, monitoring fluids and electrolytes, maintaining nutrition, providing good skin care, and monitoring for complications. Severe fluid and electrolyte losses leading to shock or hypotension can occur because of fever, vomiting, poor oral intake from painful mucosal lesions, fluid shifts, and skin desquamation. 2,8 The vesicles and pustules should not be ruptured and scabs should be allowed to separate and fall off. Secondary skin infections should be treated when they occur. 2,8 Other complications of smallpox include subconjunctival hemorrhage or other signs of bleeding, corneal keratitis and ulcers, arthritis (most commonly involving the elbow), bronchitis, pneumonitis, pneumonia, pulmonary edema, encephalitis, diarrhea, nausea, vomiting, and orchitis. 8 If the patient has not yet been confirmed to have smallpox by laboratory testing and will be hospitalized with other confirmed or suspected smallpox patients, he/she should be given the smallpox vaccine to protect him/her from contracting smallpox from these patients in the event of misdiagnosis. 2,8 Unless the patient shows evidence of a concomitant bacterial infection or secondary bacterial infection, treatment consists only of supportive care. Vaccinia Immune Globulin (VIG) is given for complications from the smallpox vaccine (eczema vaccinatum, progressive vaccinia, and for severe complications of generalized vaccinia and accidental inoculation). 2,8 Animal studies show in-vitro and in-vivo activity against the orthopoxviruses. 2,8 However, at present, it is not known if Cidofovir will be effective in treating smallpox or the complications of the smallpox vaccine, 8 and the drug has significant toxicities including nephrotoxicity and bone marrow suppression. 10

13 QUESTION 4 At this point, preventive measures that may allow for continued delivery of care to those in need and restore a sense of normalcy for others include which of the following: (Select all answer choices that are appropriate.) a. Call for law enforcement personnel to assist in removing the television reporter from your office. b. Provide appropriate assurance to the crowd regarding the safety of the clinic environment. c. Close the clinic and advise building employees and others in the waiting area that unless they have already scheduled appointments they should contact their own physicians or the CDC for more information. d. Print information sheets that describe signs and symptoms of smallpox, and prompt referral, as well as sheets describing the indications, benefits, and risks of smallpox vaccination to all concerned parties. ANSWER: The correct answers are b and d. Given the nature of the infectious spread of smallpox and the stage of illness in your index patient when she was transferred to the hospital, those in your outpatient clinic can be assured that they do not require face masks. Unless your facility s employees or patients have specific knowledge of close contact with the infected individual after her return from Germany, they have very low risk of infection. Clear and concise written guidance is often reassuring and can be easily distributed. Fact sheets for patients are available at Because office personnel and other healthcare workers may be unavailable (if infected, quarantined, or absent despite lack of clinical rationale), having templates ready in advance that can be modified to fit your specific circumstance will reduce the burden for employees who respond to a crisis. Information regarding the vaccine may reduce unnecessary demand and alleviate some protest over its administration when necessary. Closing the clinic will do little to diminish fear and anxiety. Moreover, dispersed crowd-members will pose an unnecessary burden on their own healthcare providers and misguidance may prompt them to tell others to unnecessarily present for care. Delivery of important information often can be best accomplished through effective use of the media. In consultation with public health officials, a briefing indicating that this patient s illness was detected promptly, before she returned to school and most likely before she became infectious, will help to calm the situation. 11

14 REFERENCES 1. Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. Version 3.0. Guide A: Smallpox Surveillance and Case Reporting; Contact Identification, Tracing, Vaccination, and Surveillance; and Epidemiologic Investigation. Available at: Accessed January 19, Breman JG, Henderson DA. Diagnosis and Management of Smallpox. N Engl J Med 2002;346: Henderson DA, Inglesby TV, O Toole T, eds. Bioterrorism: Guidelines for Medical and Public Health Management. JAMA & Archives Journals: AMA Press; 2002; Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. Version 3.0. Executive Summary. Available at: Accessed January 19, Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. Version 3.0. Annex 5: Generalized Vesicular or Pustular Rash Illness Protocol. Available at: Accessed January 19, Centers for Disease Control and Prevention. Poster: Evaluating Patients for Smallpox. Available at: Accessed January 19, Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. Version 3.0. Guide C, Part 1 and 2: Infection Control Measures for Healthcare and Community Settings and Quarantine Guidelines. Available at: Accessed January 19, Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines. Version 3.0. Annex 1: Overview of Smallpox Clinical Presentations, and Medical Care of Smallpox Patient. Available at: Accessed January 19, Centers for Disease Control and Prevention. Smallpox Response Plan and Guidelines Version 3.0. Guide B: Vaccination Guidelines for State and Local Health Agencies. Available at: Accessed January 19, The US Department of Health and Human Services and Center for Disease Control and Prevention. Pocket Reference Guide for Smallpox Vaccine Adverse Events. Available at: Accessed January 19,

15 11. Grabenstein JD, Winkenwerder W. US military smallpox vaccination program experience. JAMA 2003;289: Bai Y, Lin C, Lin C, et al. Survey of stress reactions among health care workers involved with the SARS outbreak. Psychiatr Serv 2004;55: Maunder R, Hunter J, Vincent L, et al. The immediate psychological and occupational impact of the 2003 SARS outbreak in a teaching hospital. CMAJ 2003;168: Petterson, JC. Perception vs. reality of radiological impact: the Goiania model. Nuclear News 1988;31: SUGGESTED READING 1. Benedek DM, Holloway H, Becker SM. Emergency mental health management in bioterrorism events. Emerg Med Clin North Am. 2002;20: Glass T, Schoch-Spana M. Bioterrorism and the people: how to vaccinate a city against panic. Clin Infect Dis. 2002;34: Inglesby TV, Grossman R, O Toole T. A plague on your city: observations from TOPOFF. Clin Infect Dis. 2001;32: Centers for Disease Control and Prevention. Fact sheet for your patients. Available at: Accessed January 19, Centers for Disease Control and Prevention. Poster of the algorithm for evaluating patients for smallpox. Available at: Accessed January 19, Centers for Disease Control and Prevention. Risk evaluation algorithm. Available at: Accessed January 19, Centers for Disease Control and Prevention. Worksheet to collect patient information in assessing the risk. Available at: Accessed January 19, Centers for Disease Control and Prevention. CDC Interim Recommended Notification Procedures for Local and State Public Health Department Leaders in the Event of a Bioterrorist Incident. Available at: Accessed January 19,

16 YOU ARE THE FIRST LINE OF DEFENSE. Recognize agents of terrorism and emerging infections. Respond by immediately contacting your local health department at ( ) - Phone number of local health department You may also contact the Centers for Disease Control (CDC) Emergency Response Hotline (24 hours/day) at

17 and T errorism Disaster WHAT CLINICIANS NEED TO KNOW Rush University Medical Center faculty, in collaboration with faculty from the Uniformed Services University of the Health Sciences (USUHS) authored a case series to provide continuing medical education (CME) for terrorism preparedness and other public health emergencies. A series of 14 case studies was developed to provide innovative learning opportunities for health professionals to problem-solve issues related to terrorism or other public health emergencies. Due to the complicated and volatile nature of a terrorist event, the case studies were designed to expand outside the clinicianpatient interaction and involve: deploying outside resources notifying appropriate officials coordinating a response team dealing with media and concerned public initiating emergency/disaster plans Each case provides the CME user with decision-making challenges within his or her discipline, along with scenarios that address broader interdisciplinary issues. This interdisciplinary approach is particularly important in disaster preparedness, when health professionals will likely be called on to work outside their day-to-day experiences. Authored by experts in the field, each self-paced case includes a thorough case history, questions to test your knowledge, a resource list of additional readings and relevant websites. One-hour CME and CEU credit is available for each case, following the successful completion of the CME questions included with each case. The cases in the series include: MEDICINE The medicine cases address recognition of the agent, diagnosis, treatment, and medical case management. Pneumonic Plague Radiation Attack Sarin Smallpox: Recognition, Management, & Containment Staphylococcal Enterotoxin B Viral Hemorrhagic Fevers PSYCHIATRY The psychiatry cases address issues of disaster psychiatry. Emergency Mental Health After a Suicide Bombing Psychiatric Sequelae in a Survivor of 9/11 Psychosocial Management of a Radiation Attack INTERDISCIPLINARY The interdisciplinary cases address basic medical management,general disaster planning, communicating with the media and concerned public, and psychosocial case management. Anthrax Chemical Attack: Airway and Anxiety Management SARS Smallpox Attack: Assessment, Communication, & Coping Pandemic Influenza For more information or to order your free copy of any of the cases in this series, please contact: Office of Continuing Medical Education Rush University Medical Center Suite 433 AAF Chicago, Illinois Telephone: (312) Facsimile: (312) cme_info@rush.edu

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