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Dipty Jain
Pooja Lodha
Roshan Colah
Prachi Atmapoojya
Prachi Atmapoojya


Sickle Cell Disease, Anemia, Pregnancy, Developing Countries




Sickle Cell Disease (SCD) is a group of inherited single-gene autosomal recessive disorders caused by the ‘sickle’ gene, which affects haemoglobin structure. Sickle cell anemia is the most common hemoglobinopathy worldwide. The burden of sickle cell disease in pregnancy has been exponentially increasing with more number of women reaching the reproductive age, and having successful pregnancies. It has been proven beyond doubt that SCD in pregnancy poses the pregnant woman and fetus to significantly higher risks than a lady without SCD. SCD is associated with both maternal and fetal complications and is associated with an increased incidence of perinatal mortality, premature labour,  fetal growth restriction and acute painful crises during pregnancy.  Some studies also describe an increase in spontaneous miscarriage, antenatal hospitalisation, maternal mortality, delivery by caesarean section, infection, thromboembolic events and antepartum haemorrhage.


This review aims to discuss the risks of SCD in pregnancy - to the mother and fetus . It also reviews the difference between complications in SCD and sickle cell trait.


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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.
PMid:23874164 PMCid:PMC3712914
2. Koshy M. Sickle cell disease and pregnancy. Blood Rev. 1995;9(3):157-
3. Rogers DT, Molokie R. Sickle cell disease in pregnancy. Obstetrics and
Gynecology Clinics of North America. 2010; 37(2):223-237. [pubmed:
4. Luban NL, Leikin SL, August GA. Growth and development in sickle
cell anemia. Preliminary report. Am J pediatrhematol Oncol. 1982;4:61-
5. Jesus AC, Konstantyner T, Lôbo IK, Braga JA. Socioeconomic and
nutritional characteristics of children and adolescents with sickle cell
anemia: a systematic review. Revistapaulista de Pediatria. 2018
PMid:30540112 PMCid:PMC6322809
6. Zemel BS, Kawchak DA, Ohene-Frempong K, et al. Effects of delayed
pubertal development, nutritional status, and disease severity on
longitudinal patterns of growth failure in children with sickle cell
disease. Pediatr Res. 2007;61:607-613
7. Carvalho FA, Souza AI, Ferreira ALCG, et al. Profile of reproductive
issues associated with different sickle cell disease genotypes. Rev Bras
Ginecol Obstet. 2017;39(8):397-402
8. Hagag AA, El-Farargy MS, Elrefaey S, et al. Study of gonadal
hormones in Egyptian female children with sickle cell anemia in
correlation with iron overload: single center study. Hematoloncolstem
Cell Ther
9. Chase AR, Howard J, Oteng-Ntim E. Ovarian sickling as a proposed
mechanism for premature ovarian failure necessitating ovum donation.
Menopause Int. 2009;15:70-71.
10. Gaytan M, Morales C, Bellido C, et al. Non-steroidal anti-inflammatory
drugs (nsaids) and ovulation: lessons from morphology.
Histolhistopathol. 2006;21:541-556
11. Villers MS, Jamison MG, De Castro LM, James AH. Morbidity
associated with sickle cell disease in pregnancy. American journal of
obstetrics and gynecology. 2008 Aug 1;199(2):125-e1.
12. Hassell K. Pregnancy and sickle cell disease. Hematology/Oncology
Clinics. 2005 Oct 1;19(5):903-16.
13. Rahimy MC, Gangbo A, Adjou R, Dequenon C, Goussanou S, et
al.(2000) Effect of active prenatal management on pregnancy outcome
in sickle cell disease in an African setting. Blood 96:1685-1689
14. Boafor TK, Olayemi E, galadancin,Hayfron-Benjamin C, DeiAdomakoh Y, Segbefia C, Kassim AA, Aliyu MH, Galadanci H, Tuuli
MG, Rodeghier M, debaun MR. Pregnancy outcomes in women with
sickle-cell disease in low and high income countries: a systematic
review and meta-analysis, Systematic review, BJOG, ??
15. Barfield WD, Barradas DT, Manning SE, Kotelchuck M, ShapiroMendoza CK. Sickle cell disease and pregnancy outcomes: women of
African descent. Am J Prev Med. 2010;38(4):S542-9.
16. Powars D R, Sanhu M, Niland-Weiss J, Johnson C, Bruce S, Maming P
R. Pregnancy in sickle cell disease. Obstetgynecol 1986; 67: 217-228
17. Daigavane MM, Jena RK, Kar TJ. Perinatal outcome in sickle cell
anemia: a prospective study from India. Hemoglobin. 2013 Dec
18. Natu N, Khandelwal S, Kumar R, Dave A. Maternal and perinatal
outcome of women with sickle cell disease of a tribal population in
Central India. Hemoglobin. 2014 Apr 1;38(2):91-4.
19. Nagar P. Pregnancy outcome in women with sickle cell disease/trait.
20. Desai G, Anand A, Shah P, Shah S, Dave K, Bhatt H, Desai S, Modi D.
Sickle cell disease and pregnancy outcomes: a study of the communitybased hospital in a tribal block of Gujarat, India. Journal of Health,
Population and Nutrition. 2017 Dec;36(1):3.
PMid:28109314 PMCid:PMC5251338
21. Gaddikeri A, Pajai SP, Rathod AD, Pregnancy and its outcomes in
sickle cell hemoglobinopathies: A study of central India. J South Asian
Feder Obst Gynae 2017; 9(4):399-403
22. Minerva Thame DM, dma HT, Graham Serjeant MD. The mechanisms
of low birth weight in infants of mothers with homozygous sickle cell
disease. Pediatrics. 2007;120:e686.
23. Acharya N, Kriplani A, Hariharan C. Study of perinatal outcome in
pregnancy with sickle cell disease.
24. Muganyizi PS, Kidanto H. Sickle cell disease in pregnancy: trend and
pregnancy outcomes at a tertiary hospital in Tanzania. Plos one. 2013
Feb 13;8(2):e56541.
PMid:23418582 PMCid:PMC3572068
25. Elenga N, Adeline A, Balcaen J, Vaz T, Calvez M, Terraz A,
Accrombessi L, Carles G. Pregnancy in sickle cell disease is a very
high-risk situation: an observational study. Obstetrics and gynecology
international. 2016;2016.
PMid:27403164 PMCid:PMC4926018
26. Serjeant GR, Loy LL, Crowther M, Hambleton IR, Thame M. Outcome
of pregnancy in homozygous sickle cell disease. Obstetrics &
Gynecology. 2004 Jun 1;103(6):1278-85.
27. Ashish K, Raseswari P, Pruthviraj S. Perinatal outcome in pregnancy
with sickle cell anemia. The Journal of Obstetrics and Gynecology of
India. 2008;58:500-3.
28. Acharya N, Kriplani A, Hariharan C. Study of perinatal outcome in
pregnancy with sickle cell disease.
29. Boga C, Ozdogu H. Pregnancy and sickle cell disease: a review of the
current literature. Critical reviews in oncology/hematology. 2016 Feb
30. Vermaic.Hemoglobinopathies in India - An overview. Proc. Indo-French
Symposium on Recent Trends in Clinical, Diagnostic and Reserch
Aspects of hemoglobinopathies. Kochi Nov 21-24, 2004; p 2-4
31. Colah RB, Gorakshakar AC, Nadkarni AH. Invasive & non-invasive
approaches for prenatal diagnosis of haemoglobinopathies: experiences
from India.Indian J Med Res. 2011;134:552-60
32. Giambona A Embryo-fetal erythroid cell selection from celomic fluid
allows earlier prenatal diagnosis of hemoglobinopathies. Prenat Diagn
33. Traeger-Synodinos J, Vrettou C, Kanavakis E. Prenatal, noninvasive and
preimplantation genetic diagnosis of inherited disorders:
hemoglobinopathies.Expert Rev moldiagn. 2011 ;11:299-312
34. Andemariam B, Browning SL. Current management of sickle cell
disease in pregnancy. Clinics in laboratory medicine. 2013 Jun
35. Hassel K. Pregnancy and sickle cell disease. Hematol Oncol Clin North
Am 2005;1:803-16.
36. No GT. Management of sickle cell disease in pregnancy. London: Royal
college of Obstetricians and Gynaecologists. 2011 Jul.
37. Cunningham FG, Pritchard JA, Mason R. Pregnancy and sickle cell
hemoglobinopathies: results with and without prophylactic transfusions.
38. Koshy M, Burd L, Wallace D, Moawad A, Baron J. Prophylactic redcell transfusions in pregnant patients with sickle cell disease. A
randomized cooperative study. N Engl J Med 1988;319:1447-5
39. Howard RJ, Tuck SM, Pearson TC. Pregnancy in sickle cell disease in
the UK: results of a multicentre survey of the effect of prophylactic
blood transfusion on maternal and fetal outcome. Br J obstetgynaecol
40. National Toxicology Program. NTP-CERHR monograph on the
potential human reproductive and developmental effects of hydroxyurea.
NTP CERHR MON. 2008;vii-viii, v, ix-III1.
41. Cannas G, Poutrel S, Thomas X. Hydroxycarbamine: from an Old Drug
Used in Malignant Hemopathies to a Current Standard in Sickle Cell
Disease. Mediterr J Hematol Infect Dis. 2017; 9(1):e2017015. doi:
10.4084/MJHID.2017.015. eCollection 2017. Review.
PMid:28293403 PMCid:PMC5333733
42. Brawley OW, Cornelius LJ, Edwards LR, et al. National Institutes of
Health Consensus Development Conference statement: hydroxyurea
treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.
43. Asano Y, Okaniwa A. In utero morphological effects of hydroxyurea on
the fetal development in Sprague-Dawley rats. Jikkendobutsu. Exp
Animals. 1987;36:143-149.
44. Khera KS. A teratogenicity study on hydroxyurea and
diphenylhydantoin in cats. Teratology. 1979;20:447-452.
45. Colah R, Surve R, Nadkarni A, Gorakshakar A, Phanasgaonkar S,
Satoskar P, Mohanty D. Prenatal diagnosis of sickle syndromes in India:
dilemmas in counselling. Prenatdiagn. 2005;25:345-9.
46. Kumar R, Panigrahi I, Dalal A, Agarwal S. Sickle cell anemia-molecular
diagnosis and prenatal counseling: SGPGI experience. Indian J
Pediatr.2012 ;79:68-74.

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