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Zika and Sickle Cell Disease
Ward Hagar June 14, 2016
What’s Known about Zika Virus (ZIKV)
Family:Flaviviridae Genus:Flavirirus Single stranded postive RNA genome “Usual” symptoms febrile illness,
exanthem, arthralgia's; not fatal Reports of Guillain-Barré 618 U.S. cases (195 in pregnancy)
Rare Fatal Zika Virus Infection 15 year old from Columbia with SC for “5 years” Four days of fever > 40⁰ C, arthalgias, retro-
ocular pain, abdominal pain, myalgias, and jaundice
Tachycardic, tachypneic, but no elevated WBC, no lymphadenopathy, no rash, no petechiae; alert then stuporous; hemgloglobin 8 mg/dl, platelets 54
Died of evolving respiratory failure (pulse oximetry was initial 93%) after 37 hours
Hemothorax, splenic sequestration, hepatomegaly and panacinar necrosis, erythrophagocytosis
Dengue Yellow Fever
West Nile Japanese Encephaliti
s
Zika
Reported with Sickle Cell?
Yes No No No Yes
Fatalities Worse
For SCD?
125 vs 4.1/ 1000
SC vs SS (OR 4.4)
Not Known
Not Known Not Known One case,rare
death
Retro-orbital pain
Yes No No No Yes
Lymph Nodes No No Yes No NoPlatelets Low Low Normal Normal 85% Low
Neuropathy Brachial neuritis,myelitis,
encephalitis
Minimal in 1% including meningitis
and encephaliti
s
Hemiparesis,convulsions
,mental retardation
Micro-encephal
y, Guillain-
Barré
Reasons for Possibly Increased Mortality
Immunosuppression Endothelial dysfunction from inflammation SC vs SS cells dehydrate more quickly SC vs SS patients may have more neo-
angiogenesis
Questions What’s the natural history? 80% of persons seem
to be asymptomatic. Little is really known. How long are people infectious? Incubation time? How long is virus in the blood? Does one infection confer immunity or does one
infection increase the risk of another? Will the inflammation of viral infection induce VOC
or ACS? If like dengue infections, half would die with 24 hours of presentation
Should the Olympics be moved?
Selected References Arzuza-Ortega, L., A. Polo, G. Perez-Tatis, H. Lopez-Garcia, E. Parra, L. C. Pardo-Herrera, A. M. Rico-
Turca, W. Villamil-Gomez and A. J. Rodriguez-Morales (2016). "Fatal Sickle Cell Disease and Zika Virus Infection in Girl from Colombia." Emerg Infect Dis 22(5): 925-927.
Bravo, J. R., M. G. Guzman and G. P. Kouri (1987). "Why dengue haemorrhagic fever in Cuba? 1. Individual risk factors for dengue haemorrhagic fever/dengue shock syndrome (DHF/DSS)." Trans R Soc Trop Med Hyg 81(5): 816-820.
Haug, C. J., M. P. Kieny and B. Murgue (2016). "The Zika Challenge." N Engl J Med 374(19): 1801-1803.
Moesker, F. M., F. D. Muskiet, J. J. Koeijers, P. L. Fraaij, I. Gerstenbluth, E. C. van Gorp and A. D. Osterhaus (2013). "Fatal dengue in patients with sickle cell disease or sickle cell anemia in Curacao: two case reports." PLoS Negl Trop Dis 7(8): e2203.
Petersen, L. R., D. J. Jamieson, A. M. Powers and M. A. Honein (2016). "Zika Virus." N Engl J Med 374(16): 1552-1563.
Rankine-Mullings, A., M. E. Reid, M. Moo Sang, M. A. Richards-Dawson and J. M. Knight-Madden (2015). "A Retrospective Analysis of the Significance of Haemoglobin SS and SC in Disease Outcome in Patients With Sickle Cell Disease and Dengue Fever." EBioMedicine 2(8): 935-939
McGinty, Jo Craven (2016). “Zika Draws U.S. Researchers Into a Race for Understanding.” Wall Street Journal , http://www.wsj.com/articles/zika-draws-u-s-researchers-into-a-race-for-understanding-1464956736 (accessed June 7, 2016).
Wikipedia contributors, "Zika virus," Wikipedia, The Free Encyclopedia, https://en.wikipedia.org/w/index.php?title=Zika_virus&oldid=724122092 (accessed June 7, 2016).
SCDC Updates
! New name: Sickle Cell Data Collection ! New logo in the works
! All state data submissions approved or in review ! Medicaid data arriving soon ! Hospital discharge, ED, vital records to follow ! NBS pending
! Clinical data requests to begin this summer
SCDC Updates
! New publication: Paulukonis ST, Eckman JR, Snyder AB, Hagar W, Feuchtbaum LB, Zhou M, Grant AM, Hulihan MM. Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004 through 2008. Public Health Rep. 2016 Mar-Apr;131(2):367-75.
! Coming: papers on ED utilization (2) and maternal outcomes among women with SCD, and abstracts for SCDAA meeting, ACEP meeting, ASH meeting
SCDC Updates
! Next Webinars: ! September: Dr. Julie Kanter and South Carolina’s
SCD Plan ! November: Dr. Jean Raphael and Center for Child
Health Policy and Advocacy at Texas Children’s Hospital
! New SCD Clinic at MLK Jr. Outpatient Center in Los Angeles – PR and Outreach ! World Sickle Cell Day (6/19/16): CA Messaging
! CDC Video Projects – Transition and Hispanics
Items of Interest " American Journal of Preventive Medicine:
Developing a Unified Approach for Sickle Cell Disease (06.16.2016)
" World Sickle Cell Day (06.19.2016) " Association of Public Health Laboratories:
Hemoglobinopathies Current Practices for Screening, Confirmation, and Follow-up modular webinar (July 2016)
" PCORI Funding Announcement: Management of Care Transitions for Emerging Adults with Sickle Cell Disease (08.15.2016)
" Multidisciplinary SCD Conference: Be A Part of the Change (09.17.2016)
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