The Indian Practitioner

Open Access Open Access  Restricted Access Subscription Access
Open Access Open Access Open Access  Restricted Access Restricted Access Subscription Access

Sickle Cell Disease and Pregnancy


Affiliations
1 Professor and Head, Department of Obstetrics and Gynaecology, H. B. T. Medical College and Dr. R. N. Cooper Municipal General Hospital, Mumbai., India
2 Research Associate, H. B. T. Medical College, and Dr. R. N. Cooper Municipal General Hospital, Mumbai., India
     

   Subscribe/Renew Journal


Sickle cell disease is caused by a mutation in the beta-globin gene, leading to the formation of abnormal haemoglobin and various complications. Managing this condition during pregnancy requires a collaborative approach, involving close follow-up, patient education, and active patient involvement. The process begins with pre-conception genetic counselling and assessing the patient’s baseline health. If the disease is uncontrolled or if the patient has kidney disease, hypertension, or frequent vaso-occlusive crises, pregnancy may need to be delayed or alternative reproductive options considered. The use of hydroxyurea during pregnancy is a subject of debate and is determined on a case-by-case basis. High-dose folic acid supplementation is recommended before and during pregnancy. Medications should be carefully reviewed, and any potentially harmful drugs to the fetus should be discontinued. Low-dose aspirin and adequate hydration are used to prevent hypertensive and vaso-occlusive crises, respectively. Severe anaemia may require a blood transfusion, but haemoglobin levels are maintained below 10g/dl to prevent increased blood viscosity. Vaginal delivery can be safely performed with appropriate measures such as hydration, warmth, and nasal oxygen. Cesarean sections are reserved for obstetric indications.

Keywords

Sickle Cell Disease, Pregnancy, Vaso-occlusive crises, Anemia, Hydroxyurea, Delivery.
Subscription Login to verify subscription
User
Notifications
Font Size


  • Jain D, Atmapoojya P, Colah R, Lodha P. Sickle Cell Disease and Pregnancy. Mediterr J Hematol Infect Dis. 2019;11(1): e2019040. doi: 10.4084/MJHID.2019.040.

  • Esoh K, Wonkam A. Evolutionary history of sickle-cell mutation: implications for global genetic medi-cine. Hum Mol Genet. 2021Sep 1;30(R1): R119-R128. doi: 10.1093/ hmg/ddab004.

  • Royal College of Obstetrics and Gynaecology. Management of Sickle Cell Disease in Pregnancy, Green-top GuidelineNo. 61, July 2011.

  • Oppong SA, Asare EV, Olayemi E, et al. Multidisciplinary care results in similar maternal and perinatal mortality rates for women with and without SCD in a low-resource setting. Am J Hematol. 2019Feb;94(2): 223-223. doi: 10.1002/ajh.25356.

  • Asare EV, Olayemi E, Boafor T, et al.Implementation of multidisciplinary care reduces maternal mor-tality in women with sickle cell disease living in low-resource setting. Am J Hematol. 2017Sep;92(9): 872-878. doi: 10.1002/ajh.24790.

  • Asare EV, Olayemi E, Boafor T, et al. Third trimester and early postpartum period of pregnancy have the greatest risk for ACS in women with SCD. Am J Hematol. 2019 Sep;94(9): E328-E331. doi: 10.1002/ajh.25643.

  • Early ML, Eke AC, Gemmill A, et al. Severe Maternal Morbidity and Mortality in Sickle Cell Disease in the National Inpatient Sample, 2012-2018. JAMA Netw Open. 2023Feb 1;6(2): e2254552. doi: 10.1001/ jamanetworkopen.2022.54552.

  • Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. Pregnancy, contraception, and fertility. 2008. p.59.

  • Buckner TW, Key NS. Venous thrombosis in blacks. Circulation. 2012Feb 14;125(6): 837-9.doi: 10.1161/ CIRCULATIONAHA.111.073098.

  • Bates SM, Rajasekhar A, Middeldorp S, et al. American Society of Hematology 2018 guidelines for management of venous thromboembolism: venous thromboembolism in the context of pregnancy. Blood Adv. 2018Nov 27; 2(22): 3317–3359.

  • Savitt TL, Goldberg MF. Herrick’s 1910 case report of sickle cell anemia. The rest of the story. JAMA. 1989Jan 13;261(2):266-271. doi: 10.1001/jama.261.2.266. PMID: 2642320.

  • Drasar E, Igbineweka N, Vasavda N, Free M, Awogbade M, Allman M, et al. (March 2011). “Blood transfusion usage among adults with sickle cell disease – a single institution experience over ten years”. British Journal of Haematology. 152 (6): 766–770. doi:10.1111/j.1365-2141.2010.08451.x. PMID 21275951. S2CID 44562296.

  • Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. (May 1995). “Effect of hy-droxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia”. The New England Journal of Medicine. 332 (20): 1317–1322. doi:10.1056/NEJM199505183322001. PMID 7715639.

  • Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. (April 2003). “Effect of hy-droxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment”. JAMA. 289 (13): 1645–1651. doi:10.1001/jama.289.13.1645. PMID 12672732.

  • Schmalzer, E. A., Lee, J. O., Brown, A. K., Usami, S., & Chien, S. (1987). Viscosity of mixtures of sickle and normal red cells at varying hematocrit levels. Implications for transfusion. Transfusion, 27(3), 228–233. https://doi.org/1 0.1046/j.1537-2995.1987.27387235626.


Abstract Views: 56

PDF Views: 0




  • Sickle Cell Disease and Pregnancy

Abstract Views: 56  |  PDF Views: 0

Authors

Reena J. Wani
Professor and Head, Department of Obstetrics and Gynaecology, H. B. T. Medical College and Dr. R. N. Cooper Municipal General Hospital, Mumbai., India
Mahin Bhatt
Research Associate, H. B. T. Medical College, and Dr. R. N. Cooper Municipal General Hospital, Mumbai., India

Abstract


Sickle cell disease is caused by a mutation in the beta-globin gene, leading to the formation of abnormal haemoglobin and various complications. Managing this condition during pregnancy requires a collaborative approach, involving close follow-up, patient education, and active patient involvement. The process begins with pre-conception genetic counselling and assessing the patient’s baseline health. If the disease is uncontrolled or if the patient has kidney disease, hypertension, or frequent vaso-occlusive crises, pregnancy may need to be delayed or alternative reproductive options considered. The use of hydroxyurea during pregnancy is a subject of debate and is determined on a case-by-case basis. High-dose folic acid supplementation is recommended before and during pregnancy. Medications should be carefully reviewed, and any potentially harmful drugs to the fetus should be discontinued. Low-dose aspirin and adequate hydration are used to prevent hypertensive and vaso-occlusive crises, respectively. Severe anaemia may require a blood transfusion, but haemoglobin levels are maintained below 10g/dl to prevent increased blood viscosity. Vaginal delivery can be safely performed with appropriate measures such as hydration, warmth, and nasal oxygen. Cesarean sections are reserved for obstetric indications.

Keywords


Sickle Cell Disease, Pregnancy, Vaso-occlusive crises, Anemia, Hydroxyurea, Delivery.

References