Fetomaternal outcome in sickle cell hemoglobinopathy in a tertiary care centre of North Kerala, India
Keywords:Sickle cell disease (SCD), Maternal morbidity, Perinatal outcome
Background: Pregnancy is a serious burden to women with sickle cell disease (SCD). Our centre is the only tertiary care referral centre in the public sector which caters to the districts of Wayanad and the Nilgiris which has the maximum prevalence for SCD in South India. Hence this study was conducted to assess complications in pregnancy and maternal and perinatal outcome among women with SCD.
Methods: A retrospective observational study was conducted by reviewing the medical records of all the pregnant women with SCD who had delivered in the Department of Obstetrics and Gynecology, Government Medical College, Kozhikode from January 2014 to December 2016.
Results: There were 72 antenatal women with SCD during the study period with a prevalence of 0.15%. 54.17% (n = 39) patients were HbSS (sickle cell anemia), 44.44% (n = 32) were HbAS (sickle cell trait) and 1.39% (n = 1) were HbS-β thalassemia trait. There was increased risk of obstetric complications like gestational hypertension (16%), preeclampsia (11.11%), eclampsia (5.56%), HELLP syndrome (4.16%), intrauterine growth retardation (38.89%), and oligohydramnios (18.06%). Medical complications observed were mainly anaemia (76.38%), vasoocclusive crisis (18.05%), acute chest syndrome (5.56%) and infections like urinary tract infection (8.33%) and pneumonia (5.56%). The incidence of low birth weight babies (56.94%), low Apgar score (14.49%) and neonatal ICU admissions (31.88%) were high. There was no maternal mortality, but perinatal mortality was high (6.94%).
Conclusion: Pregnancy in SCD is associated with an increased maternal morbidity and high perinatal mortality due to obstetric and medical complications.
Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364:1343-60.
Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-31.
Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Williams TN, et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010;1:104.
Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med. 2013;10(7):e1001484.
Colah RB, Mukherjee MB, Martin S, Ghosh K. Sickle cell disease in tribal populations in India. Indian J Med Res. 2015;141:509-15.
Odièvre MH, Verger E, Silva-Pinto AC, Elion J. Pathophysiological insights in sickle cell disease. Indian J Med Res. 2011;134:532-7.
Hematological Disorders. In: Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL, et al, eds. Williams Obstetrics 24th ed. Mc Graw Hill; 2014:1101-8
Sparkenbaugh E, Pawlinski R. Interplay between coagulation and vascular inflammation in sickle cell disease. Br J Haematol. 2013;162(1):3-14.
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;125:3316-25.
Elenga N, Adeline A, Balcaen J, Vaz T, Calvez M, Terraz A, et al. Pregnancy in sickle cell disease is a very high-risk situation: an observational study Obstet Gynecol Int. 2016;2016:9069054.
Anju SS, Pralhadrao ZS. Pregnancy outcome in women with sickle cell disease/trait. J Obstet Gynecol India. 2005;55(5):415-8.
Ugboma HAA, George IO. sickle cell disease in pregnancy: maternal and fetal outcome in Port Harcourt. Nigeria Br J Med Med Res. 2015;7(1):40-4.
Balgir R. Epidemiology, population health genetics and phenotypic diversity of sickle cell disease in India. Internet J Biol Anthropol. 2007;1(2):1-26.
Feroze M, Aravindan KP. Sickle cell disease in Wayanad, Kerala: gene frequencies and disease characteristics. Nat Med J India. 2001;14(5):267-70.
Silva-Pinto AC, Ladeira SD, Brunetta DM, Santis GC, Angulo ID, Covas DT. Sickle cell disease and pregnancy: analysis of 34 patients followed at the Regional Blood Center of Ribeirão Preto, Brazil. Brazilian J Hematol Hemotherapy. 2014;36(5):329-33.
Ngô C, Kayem G, Habibi A, Benachi A, Goffinet F, Galactéros F, et al. Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions. Obstet Gynecol Survey. 2011;66(2):73-4.
Wilson NO, Ceesay FK, Hibbert JM, Driss A, Obed SA, Adjei AA, et al. stiles pregnancy outcomes among patients with sickle cell disease at Korle-Bu Teaching Hospital, Accra, Ghana: Retrospective cohort study. Am J Trop Med Hyg. 2012;86(6):936-42.
Al Jama FE, Gasem T, Burshaid S, Rahman J, Al Suleiman SA, Rahman MS. Pregnancy outcome in patients with homozygous sickle cell disease in a university hospital, Eastern Saudi Arabia. Arch Gynecol Obstet. 2009;280(5):793-7.
John CO, Orazulike N, Ijeoma C. Feto-maternal outcome in patients with sickle cell anaemia at the University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria: a ten-year retrospective review. Int J Obstet Gynecol. 2015;3(4):95-9.
Desai G, Anand A, Sha P, Shah S, Dave K, Bhatt H, et al. Sickle cell disease and pregnancy outcomes: a study of the community-based hospital in a tribal block of Gujarat. India J Health Population Nutrition. 2017;36:3.
Sun PM, Wilburn W, Raynor BD, Jamieson D. Sickle cell disease in pregnancy: twenty years of experience at Grady Memorial Hospital, Atlanta, Georgia. Am J Obstet Gynecol. 2001;184(6):1127-30.
Ocheni S, Onah HE, Ibegbulam OG, Eze MI. Pregnancy outcomes in patients with sickle cell disease in Enugu, Nigeria. Niger J Med. 2007;16(3):227-30.
Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol. 2008;199:125.
Muganyizi PS, Kidanto H. Sickle Cell disease in pregnancy: trend and pregnancy outcomes at a tertiary hospital in Tanzania. PLoS One. 2013;8(2):e56541.