sickle cell disease during pregnancy
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Monitor
Laboratory
- urinalysis
- 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
Diagnostic procedures
- 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
- 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]
More general terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 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 - ↑ Rogers DT, Molokie R. Sickle cell disease in pregnancy. Obstet Gynecol Clin North Am. 2010 Jun;37(2):223-37 PMID: https://pubmed.ncbi.nlm.nih.gov/2068555
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/19160301
- ↑ 4.0 4.1 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: https://pubmed.ncbi.nlm.nih.gov/24297507
- ↑ Andemariam B, Browning SL. Current management of sickle cell disease in pregnancy. Clin Lab Med. 2013 Jun;33(2):293-310 PMID: https://pubmed.ncbi.nlm.nih.gov/23702119
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/24338132
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/26672916
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/37866731 PMCID: PMC10961101