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sickle cell disease during pregnancy

Monitor: - blood pressure for hypertension Laboratory: - urinalysis - urine protein - renal function tests - liver function tests - iron studies for iron overload (from transfusions) - type & screen - screening for red cell antibodies - red cell antibodies may indicate an increased risk of haemolytic disease of the newborn Special laboratory: - screening for pulmonary hypertension with echocardiography - funduscopy for retinal screening Complications: - mortality is 0.5-2% Management: - management by a team that includes an obstetrician, an internist & a hematologist - hydroxyurea is contraindicated during pregnancy (maybe not MKSAP20) [1] - stop at least 3 months prior to conception (maybe not MKSAP20) [1] - don't increase (MKSAP20) [1] - increase folic acid [1] - painful crisis - hydration - oxygen if exidence of hypoxia - morphine - routine RBC transfusion not recommended [1,4] - does not reduce pregnancy-related morbidity or mortality [1] - may reduce painful crisis - increases risk of alloantibody - transfusion may be appropriate for severely anemia patients with heart failure, acute chest syndrome or stroke [1] - no data supports cesarean section vs vavinal delivery [1]

General

sickle cell (hemoglobin SS) disease pregnancy disorder; obstetric disorder; pregnancy complication

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18. American College of Physicians, Philadelphia 2012, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  2. Rogers DT, Molokie R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am. 2010 Jun;37(2):223-37 PMID: 2068555
  3. Marti-Carvajal AJ, Pena-Marti GE, Comunian-Carrasco G et al Interventions for treating painful sickle cell crisis during pregnancy. Cochrane Database Syst Rev. 2009 Jan 21;(1):CD006786 PMID: 19160301
  4. Okusanya BO, Oladapo OT Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. Cochrane Database Syst Rev. 2013 Dec 3;12:CD010378. PMID: 24297507
  5. Andemariam B, Browning SL. Current management of sickle cell disease in pregnancy. Clin Lab Med. 2013 Jun;33(2):293-310 PMID: 23702119
  6. Porter B, Key NS, Jauk VC et al Impact of sickle hemoglobinopathies on pregnancy-related venous thromboembolism. Am J Perinatol. 2014 Oct;31(9):805-9. PMID: 24338132
  7. Boga C, Ozdogu H. Pregnancy and sickle cell disease: A review of the current literature. Crit Rev Oncol Hematol. 2016 Feb;98:364-74 Review. PMID: 26672916
  8. Sinkey RG, Ogunsile FJ, Kanter J, et al; Society for Maternal-Fetal Medicine. Society for maternal-fetal medicine consult series #68: sickle cell disease in pregnancy. Am J Obstet Gynecol. 2024;230:B17-B40. PMID: 37866731 PMCID: PMC10961101