Adult sickle cell disease and SARS-CoV-2: an increasingly common comorbidity for a rare disease

Published: 27 August 2024
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Authors

Sickle cell disease (SCD) is a collection of genetic lesions that manifest in the diminished effectiveness of hemoglobin. We collected and reviewed the recent and extant literature on SARS-CoV-2 (COVID-19) and SCD. We posit an answer to the question associated with any adaptive responses to COVID-19 in individuals with SCD. We collected papers from MEDLINE and all available published papers on COVID-19 and SCD. Unlike a formal meta-analysis, given the early phase of this review in the pandemic, we did not seek unpublished papers. We found an emerging literature where case studies dominated, and traditional large N epidemiological studies were absent. Patients with SCD share many comorbid illnesses with an increased risk of mortality associated with contracting COVID-19. There is sufficient empirical justification to accelerate research on the impact of a viral pathogen like COVID-19 on individuals with SCD.

 

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List of all authors:

Michaela Boggan,1,2,3 Christopher L. Edwards,1,2,3 Jordan Meek,1,2,3 Mary Wood,4 W. Jeff Bryson,5 John J. Sollers,1,2,3 Debra O. Parker,1,2,3 Camela S. Barker,2,5 Jessica Miller,2,6 Brianna Downey,2,5 Asha Lockett,2,5 Jazmin Rosales,1,2,3 Courtney Munroe, Jr.,2 Noa Wax,2,5 Sharena Scott,1,2,3 Bridget Pittman,1,2,3 Merell Turner,1,2,3 Hillary T. Dietahin,1,2,3 Eric Smith,1,2,3 Alexandria McDougald,1,2,3 Samantha Beavers,1,2,3 Lakaiya Meadows,1,2,3 Maya Walker,1,2,3 Andrea Richardson,1,2,3 John J. Sollers, IV,2,8 Grace D. Donahue,2,8 Goldie Byrd,7 Malik Muhammad,1,2 Alvin Kidd,9 Walter Charles,1,2 Jonathan Livingston,1,2,3 Shiv Sudhakar10

 

1North Carolina Central University; 2NCCU Psychoneuroendocrine and Rare Diseases Laboratory; 3NCCU Debra O. Parker Research Incubator; 4Duke University Medical Center; 5Fielding Graduate University; 6Bridges Point Sickle Cell Foundation; 7Wake Forest Medical Center; 8Thomas Moore University; 9Livingston College; 10California Northstate University, United States

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Citations

Kumar M, Al Khodor S. Pathophysiology and treatment strategies for COVID-19. J Transl Med 2020;18:353. DOI: https://doi.org/10.1186/s12967-020-02520-8
Huang C, Wang Y, Li X, et al. Clinical features of patients infected with 2019 novel coronavirus in Wuhan, China. Lancet 2020;395:497-506. DOI: https://doi.org/10.1016/S0140-6736(20)30183-5
Aziz M, Perisetti A, Lee-Smith WM, et al. Taste changes (dysgeusia) in COVID-19: A systematic review and meta-analysis. Gastroenterology 2020;159:1132-3. DOI: https://doi.org/10.1053/j.gastro.2020.05.003
Cascella M, Rajnik M, Aleem A, et al. Features, evaluation, and treatment of Coronavirus (COVID-19). StatPearls Publishing 2022.
Kato GJ, Steinberg MH, Gladwin MT. Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest 2017;127:750-60. DOI: https://doi.org/10.1172/JCI89741
People with Certain Medical Conditions. Centers for Disease Control and Prevention. Available from: https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/people-with-medical-conditions.html. Published 2022.
Conran N, De Paula EV. Thromboinflammatory mechanisms in sickle cell disease - challenging the hemostatic balance. Haematologica 2020;105:2380-90. DOI: https://doi.org/10.3324/haematol.2019.239343
Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med 2017;376:1561-73. DOI: https://doi.org/10.1056/NEJMra1510865
Appiah-Kubi A, Acharya S, Levy CF, et al. Varying presentations and favourable outcomes of COVID-19 infection in children and young adults with sickle cell disease: an additional case series with comparisons to published cases. Br J Haematol 2020;190:221-4. DOI: https://doi.org/10.1111/bjh.17013
Timeline: WHO's COVID-19 Response. World Health Organization. 2022. Available from: https://www.who.int/emergencies/diseases/novel-coronavirus-2019/interactive-timeline# (accessed July 24, 2022)
Udugama B, Kadhiresan P, Kozlowski HN, et al. Diagnosing COVID-19: the disease and tools for detection. ACS Nano 2020;14:3822-35. DOI: https://doi.org/10.1021/acsnano.0c02624
Singh SP, Pritam M, Pandey B, Yadav TP. Microstructure, pathophysiology, and potential therapeutics of COVID-19: a comprehensive review. J Med Virol 2021;93:275-99. DOI: https://doi.org/10.1002/jmv.26254
Menapace LA, Thein SL. COVID-19 and sickle cell disease. Haematologica 2020;105:2501-4. DOI: https://doi.org/10.3324/haematol.2020.255398
Boğa C, Asma S, Leblebisatan G, et al. Comparison of the clinical course of COVID-19 infection in sickle cell disease patients with healthcare professionals. Ann Hematol 2021;100:2195-202. DOI: https://doi.org/10.1007/s00277-021-04549-1
Takaoka K, Cyril AC, Jinesh S, Radhakrishnan R. Mechanisms of pain in sickle cell disease. Br J Pain 2021;15:213-20. DOI: https://doi.org/10.1177/2049463720920682
Madany E, Okwan-Duodu D, Balbuena-Merle R, et al. Potential implications of a type 1 interferon gene signature on COVID-19 severity and chronic inflammation in sickle cell disease. Front Med (Lausanne). 2021;8:679030. DOI: https://doi.org/10.3389/fmed.2021.679030
National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention. 2022. Available from: https://www.cdc.gov/ncbddd/sicklecell/data.html.
Kehinde TA, Osundiji MA. Sickle cell trait and the potential risk of severe coronavirus disease 2019-A mini-review. Eur J Haematol 2020;105:519-23. DOI: https://doi.org/10.1111/ejh.13478
De Luna G, Habibi A, Deux JF, et al. Rapid and severe COVID-19 pneumonia with severe acute chest syndrome in a sickle cell patient successfully treated with tocilizumab. Am J Hematol 2020;95:876-8. DOI: https://doi.org/10.1002/ajh.25833
McCloskey KA, Meenan J, Hall R, Tsitsikas DA. COVID-19 infection and sickle cell disease: a UK centre experience. Br J Haematol 2020;190:57-8. DOI: https://doi.org/10.1111/bjh.16779
Connors JM, Levy JH. COVID-19 and its implications for thrombosis and anticoagulation. Blood 2020;135:2033-40. DOI: https://doi.org/10.1182/blood.2020006000
Gris JC, Perez-Martin A, Quere I, Sotto A. COVID-19 associated coagulopathy: The crowning glory of thrombo-inflammation concept. Anaesth Crit Care Pain Med 2020;39:381-2. DOI: https://doi.org/10.1016/j.accpm.2020.04.013
Carnethon MR, Pu J, Howard G, et al. Cardiovascular health in african americans: a scientific statement from the American Heart Association. Circulation 2017;136:393-423. DOI: https://doi.org/10.1161/CIR.0000000000000534
Clark LT, Ferdinand KC, Flack JM, et al. Coronary heart disease in African Americans. Heart Dis 2001;3:97-108. DOI: https://doi.org/10.1097/00132580-200103000-00007
Barnes KC, Grant AV, Hansel NN, et al. African Americans with asthma: genetic insights. Proc Am Thorac Soc 2007;4:58-68. DOI: https://doi.org/10.1513/pats.200607-146JG
Inciardi RM, Adamo M, Lupi L, et al. Characteristics and outcomes of patients hospitalized for COVID-19 and cardiac disease in Northern Italy. Eur Heart J 2020;41:1821-9. DOI: https://doi.org/10.1093/eurheartj/ehaa388
Williamson EJ, Walker AJ, Bhaskaran K, et al. Factors associated with COVID-19-related death using OpenSAFELY. Nature 2020;584:430-6. DOI: https://doi.org/10.1038/s41586-020-2521-4
Gupta S, Hayek SS, Wang W, et al; STOP-COVID Investigators. Factors associated with death in critically ill patients with coronavirus disease 2019 in the US. JAMA Intern Med 2020;180:1436-47. DOI: https://doi.org/10.1001/jamainternmed.2020.3596
Cummings MJ, Baldwin MR, Abrams D, et al. Epidemiology, clinical course, and outcomes of critically ill adults with COVID-19 in New York City: a prospective cohort study. Lancet 2020;395:1763-70. DOI: https://doi.org/10.1016/S0140-6736(20)31189-2
Laurencin CT, McClinton A. The COVID-19 pandemic: a call to action to identify and address racial and ethnic disparities. J. Racial Ethnic Health Disparities 2020;7:398-402. DOI: https://doi.org/10.1007/s40615-020-00756-0
Gould E, Shierholz H. Not everybody can work from home: black and Hispanic workers are much less likely to be able to telework. Economic Policy Institute. 2020. Available from: https://www.epi.org/blog/black-and-hispanic-workers-are-much-less-likely-to-be-able-to-work-from-home/
Schmidt H, Shaikh SJ, Sadecki E, et al. US adults’ preferences for race-based and place-based prioritisation for COVID-19 vaccines. J Med Ethics 2022;48:497-500. DOI: https://doi.org/10.1136/medethics-2021-107741
Centers for Disease Control and Prevention. COVID Data Tracker. Atlanta, GA: US Department of Health and Human Services, CDC; 2022. Available from: https://covid.cdc.gov/covid-data-tracker
Wells L, Gowda A. A legacy of mistrust: African Americans and the US Healthcare System. Proceedings. 2020. Available from: https://proceedings.med.ucla.edu/index.php/2020/06/12/a-legacy-of-mistrust-african-americans-and-the-us-healthcare-system/
Hammonds, EM, Reverby, SM. Toward a historically informed analysis of racial health disparities since 1619. Am J Public Health 2019;109:1348-9. DOI: https://doi.org/10.2105/AJPH.2019.305262
Hoffman KM, Trawalter S, Axt JR, Oliver MN. Racial bias in pain assessment and treatment recommendations, and false beliefs about biological differences between blacks and whites. Proceed Nat Acad Sci 2016;113:4296-301. DOI: https://doi.org/10.1073/pnas.1516047113
Staton LJ, Panda M, Chen I, et al. When race matters: disagreement in pain perception between patients and their physicians in primary care. J Natl Med Assoc 2007;99:532-8.
Trawalter S, Hoffman KM, Waytz A. Racial bias in perceptions of others' pain. PLoS One 2012;7:e48546. DOI: https://doi.org/10.1371/journal.pone.0048546
Waytz A, Hoffman KM, Trawalter S. A superhumanization bias in whites' perceptions of Blacks. Soc Psychol Personal Sci 2014;6:352-9. DOI: https://doi.org/10.1177/1948550614553642
Alsan M, Wanamaker M. Tuskegee and the Health of Black Men, Q J Econ 2018;133:407-55. DOI: https://doi.org/10.1093/qje/qjx029
Volscho TW. Sterilization racism and pan-ethnic disparities of the past decade: The continued encroachment on reproductive rights. Wicazo Sa Rev 2010;25:17-31. DOI: https://doi.org/10.1353/wic.0.0053
Scharff DP, Mathews KJ, Jackson P, et al. More than Tuskegee: understanding mistrust about research participation. J Health Care Poor Underserved 2010;21:879-97. DOI: https://doi.org/10.1353/hpu.0.0323
Laurencin CT. Addressing Justified Vaccine Hesitancy in the Black Community. J Racial Ethn Health Disparities 2021;8:543-6. DOI: https://doi.org/10.1007/s40615-021-01025-4
Matthie NS, Clayton-Jones DL, Jenerette CM. “Into the Lion’s Den”: COVID-19 experiences of Black adults with sickle cell disease. Qual Health Res 2022;32:1328-41. DOI: https://doi.org/10.1177/10497323221094143
Jain A, Patel A, Oyoyo U, Wiafe S. Impact of COVID-19 pandemic on health care utilization for patients with sickle cell disease-a specialty treatment center experience. Blood 2021;138:4955. DOI: https://doi.org/10.1182/blood-2021-151375
Mucalo L, Brandow AM, Dasgupta M. et al. Comorbidities are risk factors for hospitalization and serious COVID-19 illness in children and adults with sickle cell disease. Blood Adv 2021;5:2717-24. DOI: https://doi.org/10.1182/bloodadvances.2021004288
Hoogenboom WS, Alamuri TT, McMahon DM, et al. Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature. Blood Rev 2022;53:100911. DOI: https://doi.org/10.1016/j.blre.2021.100911
Arlet JB, de Luna G, Khimoud D, et al. Prognosis of patients with sickle cell disease and COVID-19: a French experience. Lancet Haematol 2020;7:e632-e634. DOI: https://doi.org/10.1016/S2352-3026(20)30204-0
Telfer P, De la Fuente J, Sohal M, et al. Real-time national survey of COVID-19 in hemoglobinopathy and rare inherited anemia patients. Haematologica 2020;105:2651-4. DOI: https://doi.org/10.3324/haematol.2020.259440
Minniti CP, et al. Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection. Blood Adv 2021;5:207-15. DOI: https://doi.org/10.1182/bloodadvances.2020003456
Anusim N, Gupta R, Ahaneku H, et al. Presentation, management, and outcomes of COVID-19 in patients with sickle cell disease. EJHaem 2021;2:124-7. DOI: https://doi.org/10.1002/jha2.162
de Sanctis V, Canatan D, Vives Corrons JL, et al. Preliminary data on COVID-19 in patients with hemoglobinopathies: a multicentre ICET-A study. Mediterr J Hematol Infect Dis 2020;12. DOI: https://doi.org/10.4084/mjhid.2020.046
Beerkens F, John M, Puliafito B, et al. COVID-19 pneumonia as a cause of acute chest syndrome in an adult sickle cell patient. Am J Hematol 2020;95:E154-E156. DOI: https://doi.org/10.1002/ajh.25809
Hussain FA, Njoku FU, Saraf SL, et al. COVID-19 infection in patients with sickle cell disease. Br J Haematol 2020;189:851-2. DOI: https://doi.org/10.1111/bjh.16734
Allison D, Campbell-Lee S, Crane J, et al. Red blood cell exchange to avoid intubating a COVID-19 positive patient with sickle cell disease? J Clin Apher 2020;35:378-81. DOI: https://doi.org/10.1002/jca.21809
Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood 2010;115:2354-63. DOI: https://doi.org/10.1182/blood-2009-05-221333
Ware RE. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 2010;115:5300-11. DOI: https://doi.org/10.1182/blood-2009-04-146852
Resurreccion WK, Hulsizer J, Shi Z, et al. Association of sickle cell trait with risk and mortality of COVID-19: results from the United Kingdom biobank. Am J Trop Med Hyg 2021;105:368-71. DOI: https://doi.org/10.4269/ajtmh.20-1657
Singh A, Brandow AM, Panepinto JA. COVID-19 in individuals with sickle cell disease/trait compared with other black individuals. Blood Adv 2021;5:1915-21. DOI: https://doi.org/10.1182/bloodadvances.2020003741
Merz LE, Mistry K, Neuberg D, et al. Impact of sickle cell trait on morbidity and mortality from SARS-CoV-2 infection. Blood Adv 2021;5:3690-3. DOI: https://doi.org/10.1182/bloodadvances.2021004977
Chen-Goodspeed A, Idowu M. COVID-19 Presentation in patients with sickle cell disease: a case series. Am J Case Rep 2021;22:e931758 DOI: https://doi.org/10.12659/AJCR.931758
Teulier M, Elabbadi A, Gerotziafas G, et al. Severe COVID-19 with acute respiratory distress syndrome (ARDS) in a sickle cell disease adult patient: case report. BMC Pulm Med 2021;21:46. DOI: https://doi.org/10.1186/s12890-021-01412-x
Scott S, Edwards CL, Boggan M, et al. Subcutaneous panniculitis in sickle cell disease (SCD): a rare disease complication. Gen Med 2023;1:1-5.
Edwards CL, Scott S, Boggan M, et al. A rare blood malignancy in a genetic hematological disorder: polycythemia vera in sickle cell disease. J Clin Haematol. 2023;4:9-19. DOI: https://doi.org/10.33696/haematology.4.053
Edwards CL, Scott S, Boggan M, et al. Intrahepatic cholestasis in sickle cell disease: a review of diagnostic criteria, treatments, and case reports. J Nat Med Assn 2023;115:26-37. DOI: https://doi.org/10.1016/j.jnma.2022.12.004
Ventocilla M. Ophthalmologic manifestations of sickle cell disease (SCD): Overview. Medscape. 2021.
Gupta S, Yui JC, Xu D, et al. Gout and sickle cell disease: not all pain is sickle cell pain. Br J Haematol 2015;171:872-5. DOI: https://doi.org/10.1111/bjh.13433

How to Cite

Boggan, M., Edwards, C. L., Meek, J., Wood, M., Bryson, W. J., Sollers, J. J., Parker, D. O., Barker, C. S., Miller, J., Downey, B., Lockett, A., Rosales, J., Munroe, Jr., C., Wax, N., & Scott, S. (2024). Adult sickle cell disease and SARS-CoV-2: an increasingly common comorbidity for a rare disease. Italian Journal of Medicine, 18(3). https://doi.org/10.4081/itjm.2024.1701