Sickle Cell Disease and Anesthesia Management in Laboring Women

  • Limor Sharoni Golzarpoor
  • Carolyn F. Weiniger


Sickle cell disease (SCD) is a genetic mutation with important implications for pregnancy. In this review we discuss the most common sickle cell hemoglobinopathies and associated morbidities, including vaso-occlusive crisis and acute chest syndrome. We present specific concerns in pregnancy such as increased risk of preeclampsia and the perils of dehydration and hypoxia. Finally we discuss recommendations for care in pregnancy including anesthesia management.


Anemia Crisis Morbidity Pain Pregnancy Sickle cell disease Vaso-occlusive 


  1. 1.
    Herrick JB. Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. JAMA. 2014;312(10):1063.CrossRefGoogle Scholar
  2. 2.
    Ingram VM. A specific chemical difference between the globins of normal human and sickle-cell anaemia haemoglobin. Nature. 1956;178(4537):792–4.CrossRefGoogle Scholar
  3. 3.
    Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364(9442):1343–60.CrossRefGoogle Scholar
  4. 4.
    Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312(10):1033–48.CrossRefGoogle Scholar
  5. 5.
    Meremikwu MM, Okomo U. Sickle cell disease. BMJ Clin Evid. 2016;2016:2402.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Management of sickle cell disease in pregnancy 2011. Royal College of Obstetrics and Gynaecologists, UK. Available from Retrieved 16 August 2016.
  7. 7.
    Oteng-Ntim E, Chase AR, Howard J, Khazaezadeh N, Anionwu EN. Sickle cell disease in pregnancy. Obstet Gynaecol Reprod Med. 2008;18(10):272–8.CrossRefGoogle Scholar
  8. 8.
    Fabry ME, Kaul DK. Sickle cell vaso-occlusion. Hematol Oncol Clin North Am. 1991;5(3):375–98.CrossRefGoogle Scholar
  9. 9.
    Oteng-Ntim E, Meeks D, Seed PT, Webster L, Howard J, Doyle P, et al. Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood. 2015;125(21):3316–25.CrossRefGoogle Scholar
  10. 10.
    Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol. 2008;199(2):125.e1–5.CrossRefGoogle Scholar
  11. 11.
    Boulet SL, Okoroh EM, Azonobi I, Grant A, Craig Hooper W. Sickle cell disease in pregnancy: maternal complications in a Medicaid-enrolled population. Matern Child Health J. 2013;17(2):200–7.CrossRefGoogle Scholar
  12. 12.
    Boafor TK, Olayemi E, Galadanci N, Hayfron-Benjamin C, Dei-Adomakoh Y, Segbefia C, 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(5):691–8.CrossRefGoogle Scholar
  13. 13.
    Lesage N, Deneux Tharaux C, Saucedo M, Habibi A, Galacteros F, Girot R, et al. Maternal mortality among women with sickle-cell disease in France, 1996–2009. Eur J Obstet Gynecol Reprod Biol. 2015;194:183–8.CrossRefGoogle Scholar
  14. 14.
    Pintova S, Cohen HW, Billett HH. Sickle cell trait: is there an increased VTE risk in pregnancy and the postpartum? PLoS One. 2013;8(5):e64141.CrossRefGoogle Scholar
  15. 15.
    Koshy M, Burd L. Management of pregnancy in sickle cell syndromes. Hematol Oncol Clin North Am. 1991;5(3):585–96.CrossRefGoogle Scholar
  16. 16.
    Rajab KE, Issa AA, Mohammed AM, Ajami AA. Sickle cell disease and pregnancy in Bahrain. Int J Gynaecol Obstet. 2006;93(2):171–5.CrossRefGoogle Scholar
  17. 17.
    Powars DR, Sandhu M, Niland-Weiss J, Johnson C, Bruce S, Manning PR. Pregnancy in sickle cell disease. Obstet Gynecol. 1986;67(2):217–28.CrossRefGoogle Scholar
  18. 18.
    Kuczkowski KM. A review of current anesthetic concerns and concepts for cesarean hysterectomy. Curr Opin Obstet Gynecol. 2011;23(6):401–7.CrossRefGoogle Scholar
  19. 19.
    Parrish MR, Morrison JC. Sickle cell crisis and pregnancy. Semin Perinatol. 2013;37(4):274–9.CrossRefGoogle Scholar
  20. 20.
    Danzer BI, Birnbach DJ, Thys DM. Anesthesia for the parturient with sickle cell disease. J Clin Anesth. 1996;8(7):598–602.CrossRefGoogle Scholar
  21. 21.
    Smith JA, Espeland M, Bellevue R, Bonds D, Brown AK, Koshy M. Pregnancy in sickle cell disease: experience of the cooperative study of sickle cell disease. Obstet Gynecol. 1996;87(2):199–204.CrossRefGoogle Scholar
  22. 22.
    Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F, et al. Acute chest syndrome in adults with sickle cell disease. Chest. 2000;117(5):1386–92.CrossRefGoogle Scholar
  23. 23.
    Kato GJ, McGowan V, Machado RF, Little JA, Taylor J, Morris CR, et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006;107(6):2279–85.CrossRefGoogle Scholar
  24. 24.
    Camous J, N’da A, Etienne-Julan M, Stephan F. Anesthetic management of pregnant women with sickle cell disease—effect on postnatal sickling complications. Can J Anaesth. 2008;55(5):276–83.CrossRefGoogle Scholar
  25. 25.
    Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855–65.CrossRefGoogle Scholar
  26. 26.
    Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Vasile M, Kasbi F, et al. Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia. Blood. 2015;125(10):1653–61.CrossRefGoogle Scholar
  27. 27.
    Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647–56.CrossRefGoogle Scholar
  28. 28.
    Asma S, Kozanoglu I, Tarim E, Sariturk C, Gereklioglu C, Akdeniz A, et al. Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy. Transfusion (Paris). 2015;55(1):36–44.CrossRefGoogle Scholar
  29. 29.
    Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med. 1995;332(20):1317–22.CrossRefGoogle Scholar
  30. 30.
    Diav-Citrin O, Hunnisett L, Sher GD, Koren G. Hydroxyurea use during pregnancy: a case report in sickle cell disease and review of the literature. Am J Hematol. 1999;60(2):148–50.CrossRefGoogle Scholar
  31. 31.
    Foucan L, Bourhis V, Bangou J, Merault L, Etienne-Julan M, Salmi RL. A randomized trial of captopril for microalbuminuria in normotensive adults with sickle cell anemia. Am J Med. 1998;104(4):339–42.CrossRefGoogle Scholar
  32. 32.
    Costa VM, Viana MB, Aguiar RA. Pregnancy in patients with sickle cell disease: maternal and perinatal outcomes. J Matern Fetal Neonatal Med. 2015;28(6):685–9.CrossRefGoogle Scholar
  33. 33.
    Yu CK, Stasiowska E, Stephens A, Awogbade M, Davies A. Outcome of pregnancy in sickle cell disease patients attending a combined obstetric and haematology clinic. J Obstet Gynaecol. 2009;29(6):512–6.CrossRefGoogle Scholar
  34. 34.
    Rees DC, Olujohungbe AD, Parker NE, Stephens AD, Telfer P, Wright J. Guidelines for the management of the acute painful crisis in sickle cell disease. Br J Haematol. 2003;120(5):744–52.CrossRefGoogle Scholar
  35. 35.
    American Society of Anesthesiologists Task Force on Obstetric Anesthesia. Practice guidelines for obstetric anesthesia: an updated report by the American Society of Anesthesiologists Task Force on Obstetric Anesthesia. Anesthesiology. 2007;106(4):843–63.CrossRefGoogle Scholar
  36. 36.
    Litos M, Sarris I, Bewley S, Seed P, Okpala I, Oteng-Ntim E. White blood cell count as a predictor of the severity of sickle cell disease during pregnancy. Eur J Obstet Gynecol Reprod Biol. 2007;133(2):169–72.CrossRefGoogle Scholar
  37. 37.
    Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med. 1991;325(1):11–6.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Limor Sharoni Golzarpoor
    • 1
    • 2
  • Carolyn F. Weiniger
    • 1
    • 3
  1. 1.Department of Anesthesiology and Critical Care MedicineHadassah Hebrew University Medical CenterJerusalemIsrael
  2. 2.Department of AnesthesiologySchneider Children’s Medical Center of IsraelPetah TikvaIsrael
  3. 3.Division of Anesthesia and Critical Care and Pain, Tel Aviv Medical CenterTel AvivIsrael

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