Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Pregnancy in women with sickle cell disease

Elliott P Vichinsky, MD
Section Editors
Stanley L Schrier, MD
Lynn L Simpson, MD
Deputy Editors
Jennifer S Tirnauer, MD
Vanessa A Barss, MD, FACOG


Although most pregnancies complicated by maternal sickle cell disease (SCD) are likely to result in livebirth, these pregnancies are at increased risk of obstetrical and fetal complications, as well as medical complications of SCD [1-4]. These risks are due, at least in part, to the metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy. Access to a multidisciplinary care team knowledgeable about sickle cell disease and high-risk obstetrics can significantly decrease morbidity and mortality.

Pregnancy-related issues in management of patients with SCD will be discussed here. General issues regarding clinical features, pathophysiology, diagnosis, and management of SCD are reviewed separately.

(See "Overview of the clinical manifestations of sickle cell disease".)

(See "Mechanisms of vaso-occlusion in sickle cell disease".)

(See "Diagnosis of sickle cell disorders".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Sep 2017. | This topic last updated: Jul 27, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Smith JA, Espeland M, Bellevue R, et al. Pregnancy in sickle cell disease: experience of the Cooperative Study of Sickle Cell Disease. Obstet Gynecol 1996; 87:199.
  2. Serjeant GR, Loy LL, Crowther M, et al. Outcome of pregnancy in homozygous sickle cell disease. Obstet Gynecol 2004; 103:1278.
  3. Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199:125.e1.
  4. Pregnancy, contraception and fertility. In: Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK, 2008. p.59.
  5. Morris RK, Riley RD, Doug M, et al. Diagnostic accuracy of spot urinary protein and albumin to creatinine ratios for detection of significant proteinuria or adverse pregnancy outcome in patients with suspected pre-eclampsia: systematic review and meta-analysis. BMJ 2012; 345:e4342.
  6. http://www.nhlbi.nih.gov/health-pro/guidelines/current/management-sickle-cell-disease.htm (Accessed on September 30, 2014).
  7. Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA 2014; 312:1033.
  8. NHLBI. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. p.24. http://www.nhlbi.nih.gov/guidelines (Accessed on August 11, 2014).
  9. Kuliev A, Pakhalchuk T, Verlinsky O, Rechitsky S. Preimplantation genetic diagnosis for hemoglobinopathies. Hemoglobin 2011; 35:547.
  10. www.reprotox.org (Accessed on September 20, 2012).
  11. Briggs GG, Freeman RK, Yaffe SJ. Drugs in Pregnancy and Lactation, 8th edition, Lippincott Williams & Wilkins.
  12. Singer ST, Vichinsky EP. Deferoxamine treatment during pregnancy: is it harmful? Am J Hematol 1999; 60:24.
  13. Howard J, Oteng-Ntim E. The obstetric management of sickle cell disease. Best Pract Res Clin Obstet Gynaecol 2012; 26:25.
  14. Oteng-Ntim E, Meeks D, Seed PT, et al. Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood 2015; 125:3316.
  15. de Montalembert M, Deneux-Tharaux C. Pregnancy in sickle cell disease is at very high risk. Blood 2015; 125:3216.
  16. Powars DR, Sandhu M, Niland-Weiss J, et al. Pregnancy in sickle cell disease. Obstet Gynecol 1986; 67:217.
  17. Adams S. Caring for the pregnant woman with sickle cell crisis. Prof Care Mother Child 1996; 6:34.
  18. Rees DC, Olujohungbe AD, Parker NE, et al. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Haematol 2003; 120:744.
  19. Martin JN Jr, Martin RW, Morrison JC. Acute management of sickle cell crisis in pregnancy. Clin Perinatol 1986; 13:853.
  20. Al Jama FE, Gasem T, Burshaid S, et al. Pregnancy outcome in patients with homozygous sickle cell disease in a university hospital, Eastern Saudi Arabia. Arch Gynecol Obstet 2009; 280:793.
  21. Rajab KE, Issa AA, Mohammed AM, Ajami AA. Sickle cell disease and pregnancy in Bahrain. Int J Gynaecol Obstet 2006; 93:171.
  22. Ngô C, Kayem G, Habibi A, et al. Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions. Eur J Obstet Gynecol Reprod Biol 2010; 152:138.
  23. Al Kahtani MA, AlQahtani M, Alshebaily MM, et al. Morbidity and pregnancy outcomes associated with sickle cell anemia among Saudi women. Int J Gynaecol Obstet 2012; 119:224.
  24. Boafor TK, Olayemi E, Galadanci N, et al. Pregnancy outcomes in women with sickle-cell disease in low and high income countries: a systematic review and meta-analysis. BJOG 2016; 123:691.
  25. Sun PM, Wilburn W, Raynor BD, Jamieson D. Sickle cell disease in pregnancy: twenty years of experience at Grady Memorial Hospital, Atlanta, Georgia. Am J Obstet Gynecol 2001; 184:1127.
  26. Koshy M, Chisum D, Burd L, et al. Management of sickle cell anemia and pregnancy. J Clin Apher 1991; 6:230.
  27. Brown AK, Sleeper LA, Pegelow CH, et al. The influence of infant and maternal sickle cell disease on birth outcome and neonatal course. Arch Pediatr Adolesc Med 1994; 148:1156.
  28. Hassell K. Pregnancy and sickle cell disease. Hematol Oncol Clin North Am 2005; 19:903.
  29. Vichinsky E, Hurst D, Earles A, et al. Newborn screening for sickle cell disease: effect on mortality. Pediatrics 1988; 81:749.
  30. Milner PF, Jones BR, Döbler J. Outcome of pregnancy in sickle cell anemia and sickle cell-hemoglobin C disease. An analysis of 181 pregnancies in 98 patients, and a review of the literature. Am J Obstet Gynecol 1980; 138:239.
  31. Serjeant GR, Hambleton I, Thame M. Fecundity and pregnancy outcome in a cohort with sickle cell-haemoglobin C disease followed from birth. BJOG 2005; 112:1308.
  32. Oteng-Ntim E, Ayensah B, Knight M, Howard J. Pregnancy outcome in patients with sickle cell disease in the UK--a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. Br J Haematol 2015; 169:129.
  33. Goldsmith JC, Bonham VL, Joiner CH, et al. Framing the research agenda for sickle cell trait: building on the current understanding of clinical events and their potential implications. Am J Hematol 2012; 87:340.
  34. Jans SM, de Jonge A, Lagro-Janssen AL. Maternal and perinatal outcomes amongst haemoglobinopathy carriers: a systematic review. Int J Clin Pract 2010; 64:1688.
  35. Pintova S, Cohen HW, Billett HH. Sickle cell trait: is there an increased VTE risk in pregnancy and the postpartum? PLoS One 2013; 8:e64141.
  36. Austin H, Key NS, Benson JM, et al. Sickle cell trait and the risk of venous thromboembolism among blacks. Blood 2007; 110:908.
  37. Buckner TW, Key NS. Venous thrombosis in blacks. Circulation 2012; 125:837.
  38. Rahimy MC, Gangbo A, Adjou R, et al. Effect of active prenatal management on pregnancy outcome in sickle cell disease in an African setting. Blood 2000; 96:1685.
  39. Abudu OO, Macaulay K, Oluboyede OA. Serial evaluation of iron stores in pregnant Nigerians with hemoglobin SS or SC. J Natl Med Assoc 1990; 82:41.
  40. Askie LM, Duley L, Henderson-Smart DJ, et al. Antiplatelet agents for prevention of pre-eclampsia: a meta-analysis of individual patient data. Lancet 2007; 369:1791.
  41. Benirschke K, Kaufmann P, Baergen R. Pathology of the Human Placenta, 5th edition, Springer, 2006. p.592.
  42. Rathod KB, Jaiswal KN, Shrivastava AC, Shrikhande AV. Study of placenta in sickle cell disorders. Indian J Pathol Microbiol 2007; 50:698.
  43. Pham BN, Peyrard T, Juszczak G, et al. Analysis of RhCE variants among 806 individuals in France: considerations for transfusion safety, with emphasis on patients with sickle cell disease. Transfusion 2011; 51:1249.
  44. Chou ST, Jackson T, Vege S, et al. High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors. Blood 2013; 122:1062.
  45. Chou ST. Transfusion therapy for sickle cell disease: a balancing act. Hematology Am Soc Hematol Educ Program 2013; 2013:439.
  46. Reid ME, Halter Hipsky C, Hue-Roye K, Hoppe C. Genomic analyses of RH alleles to improve transfusion therapy in patients with sickle cell disease. Blood Cells Mol Dis 2014; 52:195.
  47. Barrett AN, McDonnell TC, Chan KC, Chitty LS. Digital PCR analysis of maternal plasma for noninvasive detection of sickle cell anemia. Clin Chem 2012; 58:1026.
  48. Phylipsen M, Yamsri S, Treffers EE, et al. Non-invasive prenatal diagnosis of beta-thalassemia and sickle-cell disease using pyrophosphorolysis-activated polymerization and melting curve analysis. Prenat Diagn 2012; 32:578.
  49. Wang X, Seaman C, Paik M, et al. Experience with 500 prenatal diagnoses of sickle cell diseases: the effect of gestational age on affected pregnancy outcome. Prenat Diagn 1994; 14:851.
  50. Santana DS, Cecatti JG, Parpinelli MA, et al. Severe maternal morbidity due to abortion prospectively identified in a surveillance network in Brazil. Int J Gynaecol Obstet 2012; 119:44.
  51. Martí-Carvajal AJ, Peña-Martí GE, Comunián-Carrasco G, Martí-Peña AJ. Interventions for treating painful sickle cell crisis during pregnancy. Cochrane Database Syst Rev 2009; :CD006786.
  52. Winder AD, Johnson S, Murphy J, Ehsanipoor RM. Epidural analgesia for treatment of a sickle cell crisis during pregnancy. Obstet Gynecol 2011; 118:495.
  53. Asnani MR, McCaw-Binns AM, Reid ME. Excess risk of maternal death from sickle cell disease in Jamaica: 1998-2007. PLoS One 2011; 6:e26281.
  54. Rogers DT, Molokie R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am 2010; 37:223.
  55. Howard RJ, Tuck SM, Pearson TC. Pregnancy in sickle cell disease in the UK: results of a multicentre survey of the effect of prophylactic blood transfusion on maternal and fetal outcome. Br J Obstet Gynaecol 1995; 102:947.
  56. Koshy M. Sickle cell disease and pregnancy. Blood Rev 1995; 9:157.
  57. Grossetti E, Carles G, El Guindi W, et al. Selective prophylactic transfusion in sickle cell disease. Acta Obstet Gynecol Scand 2009; 88:1090.
  58. Koshy M, Burd L, Wallace D, et al. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study. N Engl J Med 1988; 319:1447.
  59. Okusanya BO, Oladapo OT. Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. Cochrane Database Syst Rev 2013; :CD010378.
  60. 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; 126:2424.
  61. Asma S, Kozanoglu I, Tarım E, et al. Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy. Transfusion 2015; 55:36.
  62. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 1995; 333:206.
  63. Vichinsky E. Management and therapy of sickle cell disease. In: Publication No. 95-2117, 4th ed, Reid C, Charles S, Lubin B (Eds), U.S. Department of Health and Human Services, National Institutes of Health, 2002. p.153.
  64. The management of sickle cell disease. National Institutes of Health; National Heart, Lung, and Blood Institute, Division of Blood Diseases and Resources. NIH publication 04-2117, revised 2004. This reference is also available for downloading or purchase at: www.nhlbi.nih.gov/health/prof/blood/sickle/ (Accessed on June 25, 2009).
  65. Lottenberg R, Hassell KL. An evidence-based approach to the treatment of adults with sickle cell disease. Hematology Am Soc Hematol Educ Program 2005; :58.
  66. Voskaridou E, Christoulas D, Terpos E. Sickle-cell disease and the heart: review of the current literature. Br J Haematol 2012; 157:664.
  67. Bates SM, Greer IA, Middeldorp S, et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012; 141:e691S.
  68. Management of Sickle Cell Disease in Pregnancy. Green-top guideline No. 61. July 2011. https://www.rcog.org.uk/globalassets/documents/guidelines/gtg_61.pdf (Accessed on June 20, 2017).
  69. Haddad LB, Curtis KM, Legardy-Williams JK, et al. Contraception for individuals with sickle cell disease: a systematic review of the literature. Contraception 2012; 85:527.
  70. Manchikanti A, Grimes DA, Lopez LM, Schulz KF. Steroid hormones for contraception in women with sickle cell disease. Cochrane Database Syst Rev 2007; :CD006261.