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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 - stop at least 3 months prior to conception [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.
  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