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)