World Journal of Anemia

Register      Login

VOLUME 2 , ISSUE 3and4 ( July-December, 2018 ) > List of Articles


Sickle Cell Anemia in Pregnancy

Richa Saxena, Huma Sadaf

Keywords : Anemia, Eclampsia, Folic acid, Hemoglobin, Hypertension, Mortality, Preeclampsia, Sickle cell disease, Ultrasound

Citation Information : Saxena R, Sadaf H. Sickle Cell Anemia in Pregnancy. World J Anemia 2018; 2 (3and4):90-95.

DOI: 10.5005/jp-journals-10065-0038

License: CC BY-NC 4.0

Published Online: 01-04-2020

Copyright Statement:  Copyright © 2018; The Author(s).


Sickle cell disease (SCD) is the most common hereditary disease globally, linked with anemia and recurrent severe pain. Pregnancy in a patient with SCD is related with high levels of maternal and fetal morbidity and mortality. Studies on potential complications during pregnancy have generated mixed data. However, it is generally accepted that women with SCD are at higher risk of adverse pregnancy outcomes and maternal mortality. Patient care has developed much over time. Nevertheless, some of the definite management issues still remain unanswered. Excellent prenatal monitoring and aggressive intervention should be established when difficulties arise for the effective management of the pregnant patient with SCD. This article discusses the etiology and the diagnosis including the clinical presentation, investigations, neonatal screening, peripheral smear, and the available management options.

  1. Chakravarti A, Li CC, Buetow KH. Estimation of the marker gene frequency and linkage disequilibrium from conditional marker data. Am J Hum Genet 1984;36(1):177–186.
  2. Davies SC, Brozovi M. The presentation, management and prophylaxis of sickle cell disease. Blood Rev 1989;3(1):29–44. DOI: 10.1016/0268-960X(89)90023-4.
  3. Serjeant GR. The emerging understanding of sickle cell disease. Br J Haematol 2001;112(1):3–18. DOI: 10.1046/j.1365-2141.2001.02557.x.
  4. Streetley A, Latinovic R, Hall K, et al. Implementation of universal newborn bloodspot screening for sickle cell disease and other clinically significant haemoglobinopathies in England: screening results for 2005–2007. J Clin Pathol 2009;62(1):26–30. DOI: 10.1136/jcp.2008.058859.
  5. Nwabuko OC, Okoh DA, Iyalla C, et al. Prevalence of sickle cell disease in pregnancy in Nigeria. Sub-Saharan Afr J Med 2016;3(3):132–136. DOI: 10.4103/2384-5147.190843.
  6. Steinberg MH. Pathophysiology of sickle cell disease. Baillieres Clin Haematol 1998;11(1):163–184. DOI: 10.1016/S0950-3536(98)80074-7.
  7. Rodgers GP. Overview of pathophysiology and rationale for treatment of sickle cell anemia. Semin Hematol 1997;34(3 Suppl 3):2–7.
  8. Chakravorty S, Williams TN. Sickle cell disease: a neglected chronic disease of increasing global health importance. Arch Dis Child 2015;100(1):48–53. DOI: 10.1136/archdischild-2013-303773.
  9. Kato GJ, Gladwin MT, Steinberg MH. Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007;21(1):37–47. DOI: 10.1016/j.blre.2006.07.001.
  10. Serjeant GR. The clinical features of sickle cell disease. Baillieres Clin Haematol 1993;6(1):93–115. DOI: 10.1016/S0950-3536(05)80067-8.
  11. Resende Cardoso PS, Lopes Pessoa de Aguiar RA, Viana MB. Clinical complications in pregnant women with sickle cell disease: prospective study of factors predicting maternal death or near miss. Rev Bras Hematol Hemoter 2014;36(4):256–263. DOI: 10.1016/j.bjhh.2014.05.007.
  12. Simonneau G, Parent F. Pulmonary hypertension in patients with sickle cell disease: not so frequent but so different. Eur Resp J 2012;39(1):3–4. DOI: 10.1183/09031936.00119811.
  13. Willen SM, Gladwin MT. What is the role of screening for pulmonary hypertension in adults and children with sickle cell disease? Hematol Am Soc Hematol Educ Program 2017;2017(1):431–434. DOI: 10.1182/asheducation-2017.1.431.
  14. Uzan J, Carbonnel M, Piconne O, et al. Pre-eclampsia: pathophysiology, diagnosis, and management. Vasc Health Risk Manag 2011;7:467–474. DOI: 10.2147/VHRM.S20181.
  15. El-Haj N, Hoppe CC. Newborn screening for SCD in the USA and Canada. Int J Neonatal Screen 2018;4(4):36. DOI: 10.3390/ijns4040036.
  16. Piety NZ, George A, Serrano S, et al. A paper-based test for screening newborns for sickle cell disease. Sci Rep 2017;7:45488. DOI: 10.1038/srep45488.
  17. Ilesanmi OO. Pathological basis of symptoms and crises in sickle cell disorder: implications for counseling and psychotherapy. Hematol Rep 2010;2(1):e2. DOI: 10.4081/hr.2010.e2.
  18. Rogers DT, Molokie R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am 2010;37(2):223–237. DOI: 10.1016/j.ogc.2010.02.015.
  19. RCOG. (2011). Green top Guidelines no. 61. Management of Sickle Cell Disease in Pregnancy. [online] Available from, last accessed December, 2019.
  20. Brousse V, Makani J, Rees DC. Management of sickle cell disease in the community. BMJ 2014;348:g1765. DOI: 10.1136/bmj.g1765.
  21. Ghosh K, Colah R, Manglani M, et al. Guidelines for screening, diagnosis and management of hemoglobinopathies. Indian J Hum Genet 2014;20(2):101–119. DOI: 10.4103/0971-6866.142841.
  22. Blencowe H, Cousens S, Modell B, et al. Folic acid to reduce neonatal mortality from neural tube disorders. Int J Epidemiol 2010;39(1):i 110–i121. DOI: 10.1093/ije/dyq028.
  23. Russell EW. Hydroxycarbamide: clinical aspects. C R Biol 2013;336(3):177–182. DOI: 10.1016/j.crvi.2012.09.006.
  24. Ballas SK, McCarthy WF, Guo N, et al. Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia. J Natl Med Assoc 2009;101(10):1046–1051. DOI: 10.1016/S0027-9684(15)31072-5.
  25. Sasongko TH, Nagalla S, Ballas SK. Angiotensin-converting enzyme (ACE) inhibitors for proteinuria and microalbuminuria in people with sickle cell disease. Cochrane Database Syst Rev 2015;6:CD009191. DOI: 10.1002/14651858.CD009191.pub3.
  26. Ajmal A, Gessert CE, Johnson BP, et al. Effect of angiotensin converting enzyme inhibitors and angiotensin receptor blockers on hemoglobin levels. BMC Res Notes 2013;6:443. DOI: 10.1186/1756-0500-6-443.
  27. Asare EV, Olayemi E, Boafor T, et al. Implementation of multidisciplinary care reduces maternal mortality in women with sickle cell disease living in low-resource setting. Am J Hematol 2017;92(9):872–878. DOI: 10.1002/ajh.24790.
  28. Di Nuzzo DV, Fonseca SF. Sickle cell disease and infection. J Pediatr (Rio J) 2004;80(5):347–354. DOI: 10.2223/1218.
  29. Mehta SR, Afenyi-Annan A, Byrns PJ, et al. Opportunities to improve outcomes in sickle cell disease. Am Fam Physician 2006;74(2):303–310.
  30. Ryan K. NHS Foundation Trust. 2019. Sickle cell disease and pregnancy. [online] Available from, last accessed December, 2019.
  31. NHS. Sickle Cell and Thallassaemia Centre. Pregnancy advice for women with Sickle Cell Disease. [online] Available from, last accessed December, 2019.
  32. Malinowski AK, Shehata N, D'Souza R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood 2015;126(21):2424–2435. DOI: 10.1182/blood-2015-06-649319.
  33. O'Driscoll BR, Howard LS, Davison AG, et al. BTS guideline for emergency oxygen use in adult patients. Thorax 2008;63(Suppl 6):vi1–vi68. DOI: 10.1136/thx.2008.102947.
  34. Wright D, Kenny-Scherber C, Montgomery A, et al. Acute coronary syndrome in pregnancy. Clin Med Cardiol 2009;3:125–131. DOI: 10.4137/CMC.S3284.
  35. Nguyen QT, Sifer C, Schneider V, et al. Novel human erythrovirus associated with transient aplastic anemia. J Clin Microbiol 1999;37(8):2483–2487. DOI: 10.1128/JCM.37.8.2483-2487.1999.
  36. Amel EA, Mangaiarkkarasi A. Evaluation of anaemia in booked antenatal mothers during the last trimester. J Clin Diagn Res 2013;7(11):2487–2490. DOI: 10.7860/JCDR/2013/6370.3586.
  37. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anaemia. A randomized trial. N Engl J Med 1986;314(25):1593–1599. DOI: 10.1056/NEJM198606193142501.
  38. Boulet SL, Okoroh EM, Azonobi I, et al. Sickle cell disease in pregnancy: maternal complications in a medicaid-enrolled population. Matern Child Health J 2013;17(2):200–207. DOI: 10.1007/s10995-012-1216-3.
  39. Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force. Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. BMJ 1996;312(7028):430–434. DOI: 10.1136/bmj.312.7028.430.
  40. Milman N. Postpartum anemia II: prevention and treatment. Ann Hematol 2012;91(2):143–154. DOI: 10.1007/s00277-011-1381-2.
  41. Olugbenga AO. Managing sickle cell disease in pregnancy, the success and the challenges: our experience in a semi-urban tertiary health-care facility, Southwest, Nigeria. Trop J Obstetr Gynaecol 2018;35(3):342–347. DOI: 10.4103/TJOG.TJOG_37_18.
  42. Cardosa D, Ridout A, Nanda S, et al. Maternal sickle cell disease and twin pregnancy: a case series and review of the literature. Hematology 2019;24(1):148–158. DOI: 10.1080/10245332.2018.1535534.
  43. Elenga N, Adeline A, Balcaen J, et al. Pregnancy in sickle cell disease is a very high-risk situation: an observational study. Obstet Gynecol Int 2016;2016:9069054. DOI: 10.1155/2016/9069054.
  44. Jain D, Atmapoojya P, Colah R, et al. Sickle cell disease and pregnancy. Mediterr J Hematol Infect Dis 2019;11(1):e2019040. DOI: 10.4084/mjhid.2019.040.
  45. Sifakis S, Pharmakides G. Anemia in pregnancy. Ann N Y Acad Sci 2000;900:125–136. DOI: 10.1111/j.1749-6632.2000.tb06223.x.
  46. Akhter S, Momen MA, Rahman MM, et al. Effect of maternal anemia on fetal outcome. Mymensingh Med J 2010;19(3):391–398.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.