Imported Human Rabies — New Jersey, 2011

On July 8, 2011, the New Jersey Department of Health and Senior Services (NJDHSS) contacted CDC about possible rabies in a hospitalized Haitian woman aged 73 years. Rabies was included in the differential diagnosis because she had acute, progressive encephalitis of unknown etiology. No history of animal exposure had been reported at the time of hospitalization. On July 18, CDC confirmed rabies virus infection, later identified as a canine rabies virus variant present in Haiti. The patient's neurologic status continued to deteriorate, leading to her death on July 20. This report summarizes the patient's clinical course and the associated public health investigation. This is the third report of human rabies in the United States acquired in Haiti since 2000 and highlights the importance of obtaining a detailed history for patients who have traveled from a rabies-endemic country and the value of consultation with medical and public health professionals regarding any animal bites.

Case Report

On June 30, the patient went to a New Jersey emergency department (ED) with right shoulder pain, chest pain, headaches, and increased blood pressure (157/100 mm Hg) despite hypertension medication. Based on her signs and medical history of hypertension, high cholesterol, and diabetes mellitus type II, pulmonary embolism and ischemic heart disease were suspected. A complete blood count (CBC), routine chemistries, a troponin level, an electrocardiogram, chest radiographs, and a chest computerized tomography (CT) scan were normal. When given pain medication, the patient had difficulty swallowing, but she declined testing for dysphagia and was released with pain medication and directions to follow-up with a primary-care physician.

The next day, she visited two different EDs, reporting shortness of breath, spasms, hallucinations, and difficulty maintaining balance. A drug reaction was suspected. A CBC showed a slight monocytosis, and a urinalysis was positive for leukocytes and blood with many bacteria on sediment examination suggestive of a urinary tract infection. A chemistry panel and head CT scan were normal. Treatment with antibiotics and anxiolytics was begun. While in the ED, the patient became progressively combative and incoherent and was admitted on July 2 for evaluation of altered mental status. The next day she became febrile with a temperature of 101.3°F (38.5°C), which was attributed to a urinary tract infection. On July 5, her respiratory secretions increased, her temperature was 102.8°F (39.3°C), and she experienced several episodes of upper extremity tremors.

The patient was transferred to the intensive-care unit, where she was intubated and a nasogastric tube placed. A video electroencephalogram revealed subclinical seizures and possible status epilepticus. Cerebral spinal fluid (CSF) was unremarkable except for a slight increase in the number of white blood cells (7/µL [normal: 0–5/µL]), predominantly lymphocytes. Magnetic resonance imaging of the brain revealed chronic periventricular white matter changes. Encephalitis was diagnosed, and infectious disease consultation was sought on July 7. To rule out infectious etiologies, CSF was tested for herpes simplex virus 1 and 2, West Nile virus, Eastern encephalitis virus, and St. Louis encephalitis virus; all were negative. Viral, bacterial, and fungal cultures also were negative. A nuchal skin biopsy was obtained on July 12 for rabies diagnostics. The patient became hypotensive and hypothermic and exhibited hypothyroidism; she was subsequently diagnosed with central diabetes insipidus and hypopituitarism. By July 14, she was comatose and had a high-grade atrioventricular block.

Samples of serum, CSF, saliva, and a nuchal skin biopsy were sent to CDC on July 15 for rabies testing. Rabies virus antigens were detected in the skin biopsy by direct fluorescent antibody testing on July 18. Rabies virus RNA was detected in the biopsy and saliva by reverse transcription–polymerase chain reaction testing. Sequencing of viral amplicons revealed a rabies virus variant with an RNA sequence closest to that found in a 2004 Florida human rabies patient, associated with a canine rabies virus variant from Haiti. On July 18, the patient was considered brain dead, and she was declared dead 2 days later.

Public Health Investigation

On July 7, NJDHSS was notified of a woman hospitalized with acute encephalitis and recent residence in a rabies-endemic country but no history of animal exposure. NJDHSS consulted CDC, and a decision was made to continue to rule out other etiologies while collecting specimens for rabies testing. Delays in specimen collection and shipment occurred, and specimens were not received by CDC until July 15. Rabies was diagnosed on July 18. On July 19, the patient's daughter informed NJDHSS that a telephone call to a cousin in Haiti revealed that in April the patient had been bitten by a dog she adopted. She did not consider the bite severe and did not seek medical attention. This was confirmed by family member interviews conducted in Haiti by the Haitian Ministry of Health.

Her daughter reported that the patient experienced intermittent right arm numbness and headaches starting approximately June 25. Family members and members of the patient's church congregation were identified as potential close contacts of the patient in the 2 weeks preceding illness onset. Three family members and a frequent house guest received rabies postexposure prophylaxis (PEP) in accordance with Advisory Committee on Immunization Practices guidelines (ACIP) (1). An educational talk about rabies and an informational flyer were provided to church members prior to services on July 24. No church members were identified as being exposed.

Starting on July 18, the risk for rabies virus exposure to hospital staff members was assessed through a questionnaire by NJDHSS and the hospital's infection prevention program. Risk levels ranging from nil (for no exposure at all) to high (for definite exposure to fluids without use of personal protective equipment) were assigned. A total of 246 hospital staff members were identified as having possible contact with the patient during ED visits and hospitalization, of whom 10 (4%) received PEP. Of these 10 staff members, five had not followed standard infection control procedures, two had potential exposure to patient saliva at an open wound or mucous membrane, and three received PEP despite assessments of infection risks which were nil to low.

Reported by

Dori Prasek, Romeo Mamon, Infection Prevention, Overlook Medical Center, Summit; Olena Stepanyuk, MD, Infectious Diseases Svcs of New Jersey LLC, South Orange; Faye E. Sorhage, VMD, Colin T. Campbell, DVM, Christina G. Tan, MD, Miranda Chan, MPH, New Jersey Dept of Health and Senior Svcs. Charles E. Rupprecht, VMD, PhD, Div of High-Consequence Pathogens and Pathology, National Center for Emerging Zoonotic Infectious Diseases; Danielle M. Tack, DVM, EIS officer, CDC. Corresponding contributor: Danielle M. Tack, dtack@cdc.gov, 404-639-5278.

Editorial Note

The most recent case of human rabies caused by a canine rabies virus variant circulating within the United States was in 1994, and no U.S. canine rabies virus variants have been identified in dogs since 2004 (2,3). Since 2000, eight human rabies cases associated with dog bite exposures have been reported in the United States, all acquired abroad. Three, including the case described in this report, were acquired in Haiti (4–6). Since 2000, approximately 96% of all domestically acquired human rabies infections in the United States have been associated with bat rabies virus variants. In 1983, Pan American Health Organization (PAHO) member countries, including Haiti, began consolidated efforts to prevent dog-transmitted rabies in humans. During the past 20 years, the number of human rabies cases has been reduced by approximately 90% (7). Before 2006, five to 13 human rabies cases were reported annually in Haiti, where the dog and cat population is estimated at 1 million, and less than 50% are vaccinated against rabies. National canine rabies vaccination campaigns were interrupted by the 2010 earthquake, but CDC, PAHO, and other partners are working closely with the Haitian government to improve rabies surveillance as well as diagnostic and animal control capabilities (8). Rabies education and canine vaccination campaigns based on the Global Alliance for Rabies Control Blueprint for Rabies Prevention and Control* are planned.

As is typical of human rabies cases in the United States, rabies was not considered early in the patient's clinical course because animal contact history had not been elicited. Although no standard treatment for rabies exists once symptoms begin, experimental intervention may be considered if the disease is detected early (1,9). Early identification also can limit secondary exposures to medical personnel and patient contacts, minimizing the need for PEP. Standard infection control practices, as outlined by the Hospital Infection Control Practices Advisory Committee, should prevent most health-care worker exposures (10). Goggles, masks, and gloves should be worn during high-risk activities, such as intubation and suctioning. Human-to-human transmission has not been documented in a health-care setting; nevertheless, transmission of rabies virus could occur if open wounds or mucous membranes were contaminated with saliva or neural tissue (1). In the case described in this report, several exposed personnel had not adhered to standard infection control procedures. Prompt and thorough education of employees was critical for assessing exposure risk and minimizing unnecessary PEP.

Rabies should be considered as a differential diagnosis for any severe, progressive, unexplained encephalitis. This case illustrates the importance of clinicians obtaining complete animal exposure histories, as well as the need for prompt medical and public health evaluation of all animal bites, regardless of perceived severity. Rabies is preventable if PEP is administered soon after exposure (1). In countries where canine rabies is endemic, all dog bites should be managed as a rabies exposure until the dog's disease-free status can be confirmed.

Acknowledgments

Charles Glagola, MPH, Borough of Roselle Health Dept, Roselle; Megan Avallone, MS, Westfield Regional Health Dept, Westfield; Jan Schwarz-Miller, MD, Rashmi Kaura, MD, Ayodeji Olarewaju, MD, physicians and occupational health staff, Overlook Medical Center, Summit, New Jersey. Richard Franka, DVM, PhD, Ivan Kuzmin, MD, PhD, Andres Velasco-Villa, PhD, Jesse Blanton, MPH, Felix Jackson, MS, Pamela A. Yager, Div of High-Consequence Pathogens and Pathology, National Center for Emerging Zoonotic Infectious Diseases, CDC.

References

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7. Pan American Health Organization. Elimination of dog-transmitted human rabies in Latin America: situation analysis. Washington, DC: Pan American Health Organization; 2005. Available at http://www.paho.org/english/ad/dpc/vp/rabia-sit.htm. Accessed December 22, 2011.
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10. Siegel JD, Rhinehart E, Jackson M, Chiarello L; Healthcare Infection Control Practices Advisory Committee. 2007 guideline for isolation precautions: preventing transmission of infectious agents in healthcare settings. Atlanta, GA: US Department of Health and Human Services, CDC, Healthcare Infection Control Practices Advisory Committee; 2007. Available at http://www.cdc.gov/hicpac/2007ip/2007isolationprecautions.html. Accessed December 22, 2011.