Varicella-Related Deaths -- Florida, 1998
During 1998, the Florida Department of Health (FDH) reported to CDC six fatal cases of varicella (chickenpox). FDH investigated all death certificates for 1998 with any mention of varicella as a contributory or underlying cause (1). Eight deaths were identified; two were reclassified as disseminated herpes zoster and six were related to varicella, for an annual varicella death rate of 0.4 deaths per million population. Two deaths occurred in children and four in adults; none had received varicella vaccine. The infection source was identified for three cases; two adults acquired varicella from children in the home, and one child acquired varicella from a classmate. One infection source was known to be unvaccinated; the other two were presumed to be unvaccinated. This report summarizes these varicella deaths and recommends prevention strategies.
Case 1. On February 19, a healthy, unvaccinated 6-year-old boy developed a varicella rash, abdominal pain, malaise, and loss of appetite following exposure to a classmate with varicella. The child had asthma and intermittently had been on inhaled steroid therapy but had not received steroids within the previous month. On February 22, he was hospitalized with hemorrhagic skin lesions, tachycardia, tachypnea, and a platelet count of 89,000 (normal range: 150,000-350,000). Several hours after admission he developed pulmonary edema and respiratory insufficiency and required mechanical ventilation. He died on February 23. Tissue samples of multiple organs had a positive polymerase chain reaction for varicella zoster virus (VZV).
Case 2. On March 27, a healthy, unvaccinated 58-year-old woman developed a varicella rash. She was born in Cuba and had moved to the United States in 1995. She did not have a history of or known exposure to varicella. On April 3, she was hospitalized with a 5-day history of increasing shortness of breath and productive cough and was diagnosed with varicella pneumonitis. She was treated with intravenous acyclovir and ceftriaxone, but developed adult respiratory distress syndrome (ARDS), disseminated intravascular coagulopathy, renal failure, and coma. She died on April 20.
Case 3. On April 27, a healthy, unvaccinated 29-year-old man developed a varicella rash. In early April, his children had contracted varicella. On April 29, he sought care at a local emergency department for chest pain and respiratory distress. Chest radiographs showed bilateral pulmonary interstitial infiltrates. On April 30, he began coughing up blood, was intubated because of increasing respiratory insufficiency, and was treated with intravenous acyclovir and antibiotics. He developed sepsis, ARDS, and multiorgan failure, and died May 12.
Case 4. On May 5, a 21-year-old unvaccinated female employee at a family child care center developed a varicella rash after exposure to a child with varicella. The employee had a history of asthma and was treated with 5 mg prednisolone per day. She was hospitalized on May 7 with varicella pneumonitis and received intravenous acyclovir on May 8, but she died the same day.
Case 5. On July 11, an 8-year-old unvaccinated boy developed a maculopapular rash diagnosed clinically as varicella and confirmed by direct flourescent antibody test on July 23. He had acute lymphocytic leukemia (ALL) and had been on immunosuppressive therapy since receiving a bone marrow transplant on May 15. He had not had varicella and had no known varicella exposure. He was treated with varicella zoster immunoglobulin on July 16 and acyclovir on July 23. He died on July 25 after recurrence of leukemia with a graft-versus-host reaction complicated by disseminated varicella, cellulitis, ileus, and hypertension.
Case 6. On October 3, an unvaccinated 45-year-old man with diabetes mellitus, asthma, and cirrhosis of the liver developed a varicella rash. He was born in Cuba and had resided in the United States for 35 years. He had no history of varicella and no known exposure. He was not receiving steroids or immunosuppressive drugs. He was admitted to the hospital with varicella on October 5 and on October 6, treatment was initiated with oral acyclovir. He died on October 8; pathologic evidence from the postmortem examination revealed VZV in all major organs.
Reported by: B Shelton, E Uribarri, M McCullom, S Heller, V Logsdon, B Keith, S Noll, P Molina, Florida county health depts; P Yambor, H Janowski, MPH, Bur of Immunizations, Florida Dept of Health; S Wiersma, MD, RS Hopkins, MD, State Epidemiologist, Bur of Epidemiology, Florida Dept of Health. SA Hall, MS, Association of Schools of Public Health, Atlanta, Georgia. Varicella Activity, Child Vaccine Preventable Diseases Br, Epidemiology and Surveillance Div, National Immunization Program; State Br, Div of Applied Public Health Training, Epidemiology Program Office; and an EIS Officer, CDC.
Editorial Note: Deaths continue to occur from varicella, a disease that is now vaccine-preventable. In Florida in 1998, the death rate was similar to the crude national varicella death rate of 0.4 per million population for 1990-1994, the 5 years preceding vaccine licensure (2). During this period, approximately 100 varicella-related deaths occurred yearly in the United States. Similar to Florida in 1998, in the rest of the United States 55% of varicella-associated deaths occurred among persons aged greater than or equal to 20 years (CDC, unpublished data, 1998).
Varicella vaccine has been available since 1995 and is recommended for all susceptible persons aged greater than or equal to 12 months (3,4). During July 1997-June 1998, the coverage level among children aged 19-35 months in Florida was 31%, slightly lower than the national coverage rate of 34% (CDC, unpublished data, 1999). In February 1999, the Advisory Committee on Immunization Practices (ACIP) recommended that all states require varicella vaccine for child care and school entry; implementation of this requirement should increase vaccine coverage dramatically. ACIP also strengthened recommendations for the vaccination of susceptible adults at high risk for exposure, including men living in households with children (5). ACIP continues to recommend that vaccination be considered for all susceptible adolescents and adults.
Five of the six case-patients who died because of varicella were eligible for vaccination. The sixth, a child with active ALL (case 5), was ineligible for vaccination. Under a special protocol, children with ALL who meet inclusion criteria may be vaccinated (3). Although one case-patient was receiving systemic steroids when she contracted varicella, the dose was not large enough to be a contraindication; varicella vaccine can be administered to adults receiving less than 20 mg prednisone per day or its equivalent, and to children receiving less than 2 mg per kg body weight per day or a total of less than 20 mg per day (3).
Two case-patients (2 and 6) were aged greater than 30 years and were born and raised in Cuba. The epidemiology of varicella in tropical regions differs from that in temperate regions. VZV is heat labile and may not survive and transmit well in warm climates. In the tropics, age distribution of cases and VZV seroprevalence data have indicated a higher proportion of cases occurring among adults (6,7). Clinicians should be aware of the greater susceptibility of adults to varicella when evaluating persons from tropical countries.
Widespread implementation of ACIP recommendations will protect healthy children and adults, thus protecting persons with contraindications to vaccination from exposure to VZV. This includes infants aged less than 12 months, pregnant women, persons with cancers or other immunocompromising conditions, and persons on high-dose systemic steroids (3). Efforts to increase varicella vaccination of susceptible children, adolescents, and adults should include educating health-care providers that severe morbidity and death from varicella are preventable.
Varicella-related deaths became nationally notifiable on January 1, 1999. A standard form for reporting varicella-related deaths is available through state public health departments. Detailed investigations of these deaths, including history of varicella, presence of immunocompromising conditions, and initiation and progression of rash, will assist state health departments in differentiating between varicella-related and disseminated herpes zoster-related deaths. Varicella death surveillance data will be used by state health departments and CDC to improve prevention efforts.
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