Skip to main content
SpringerLink
Log in

An integrative review of factors that influence reproductive decisions in women with sickle cell disease

  • Review
  • Published:
Journal of Community Genetics Aims and scope Submit manuscript

Abstract

Sickle cell disease is one of the most prevalent genetic diseases in the world. Improvements in care have enabled those affected to experience an improved quality of life, with many affected persons now reaching reproductive age. However, pregnancy poses significant risks for women with the disease and this may affect their reproductive decisions. We conducted an integrative review of the published relevant scientific evidence to ascertain the factors that may influence these women with their reproductive decisions. The electronic databases MEDLINE, CINAHL, and PsychInfo and relevant journals were searched for peer-reviewed papers published between 2005 and 2015. Of the 440 papers identified, six papers satisfied the inclusion criteria and were assessed for quality. Results were analysed and synthesised using a thematic approach to produce a narrative report of the findings. Two main themes were identified: (1) factors influencing reproductive decision-making and (2) experiences during pregnancy. Education regarding disease knowledge and genetic implications may improve participation into screening programmes, facilitate the effective treatment needed to create trust in healthcare services, and promote better self-management. Educating professionals involved in family planning will facilitate women with this condition to make informed decisions regarding pregnancy. However, further research is required to explore the understanding of the risks involved with sickle cell disease, the best methods to educate people with sickle cell disease, and the influence that partners and families may have on women’s reproductive decisions.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Ahmed K, Ahmed M, Potrata B, Willis TA, Grant HL, Allsop MJ, Hewison J, Downey L, Gale R, McKibbin M (2015) Patient attitudes towards prenatal diagnostic testing for inherited retinal disease. Prenat Diagn 35(9):913–918

    Article  PubMed  Google Scholar 

  • Ahmed S, Ahmed M, Sharif SM, Sheridan E, Taylor GR (2012) Attitudes towards prenatal testing and termination of pregnancy in British Pakistani parents and relatives of children with recessive conditions in the UK. Prenat Diagn 32:954–959

    Article  CAS  PubMed  Google Scholar 

  • Andemariam B, Browning SL (2013) Current management of sickle cell disease in pregnancy. Clin Lab Med 33:293–310

    Article  PubMed  Google Scholar 

  • Anderson M, Asnani M (2015) ‘The white blood cell always eat the red’: how Jamaicans with sickle cell disease understand their illness. Ethn Health 21:103–117. https://doi.org/10.1080/13557858.2015.1028522

    Article  PubMed  Google Scholar 

  • Atkin K, Berghs M, Dyson S (2015) ‘Who’s the guy in the room?’ Involving fathers in antenatal care screening for sickle cell disorders. Soc Sci Med 128:212–219

    Article  PubMed  Google Scholar 

  • Aygun B, Odame I (2012) A global perspective on sickle cell disease. Pediatr Blood Cancer 59:386–390. https://doi.org/10.1002/pbc.24175

    Article  PubMed  Google Scholar 

  • Boafer TK, Olayemi E, Galadanci N, Hayfron-Benjamin C, Dei-Adomakoh Y, Segbefia C, Kassim AA, Aliyu MH, Galadanci H, Tuuli MG, Rodeghier M, DeBaun MR, Oppong SA (2015) Pregnancy outcomes in women with sickle-cell disease in low and high income countries: a systematic review and meta-analysis. Int J Gynaecol Obstet 123(5):691–698. https://doi.org/10.1111/1471-0528.13786

    Article  Google Scholar 

  • Braun V, Clarke V (2006) Using thematic analysis in psychology. Qual Res Psychol 3:77–101

    Article  Google Scholar 

  • Cammidge SL, Duff AJA, Latchford GJ, Etherington, C (2016) When women with cystic fibrosis become mothers: psychosocial impact and adjustments. Pulm Med Article ID: 9458980. doi:https://doi.org/10.1155/2016/9458980.

  • Creary M, Williamson D, Kulkarni R (2007) Sickle cell disease: current activities, public health implications, and future directions. J Women's Health 16(5):575–582

    Article  Google Scholar 

  • Da Cruz Santos AC, Cândida Cordeiro R, Silva Gomes Xavier A, Ferreira SL (2012) Feelings of women with sickle cell anemia with regard to reproductive experiences. J Nurs UFPE On line 6(12):2934–2941. https://doi.org/10.5205/reuol.2265-25464-1-LE.0612201209

    Article  Google Scholar 

  • Dalibalta S, Ellory JC, Browning JA, Wilkins RJ, Rees DC, Gibson JS (2010) Novel permeability characteristics of red blood cells from sickle cell patients heterozygous for HbS and HbC (HbSC genetype). Blood Cell Mol Dis 45:46–52

    Article  CAS  Google Scholar 

  • Gerrish K, Lacey A (2010) The research process in nursing, 6th edn. Wiley-Blackwell, Oxford

    Google Scholar 

  • Godlewski CA, Castellanos PF (2017) Pre-emptive awake airway management under dexmedetomidine sedation in a parturient with spinal muscular atrophy type-2. Int J Obstet Anesth 33:81–84

    Article  PubMed  Google Scholar 

  • Gough D, Oliver S, Thomas J (2012) An introduction to systematic reviews. SAGE Publications Ltd, London

    Google Scholar 

  • Harper PS (2010) Practical genetic counselling, 7th edn. Hodder Arnold, London

    Google Scholar 

  • Hewison J, Green JM, Ahmed S, Cuckle HS, Hirst J, Hucknall C, Thornton JG (2007) Attitudes to prenatal testing and termination of pregnancy for fetal abnormality: a comparison of white and Pakistani women in the UK. Prenat Diagn 27(5):419–430

    Article  PubMed  Google Scholar 

  • James AH (2014) Sickle cell disease in pregnancy. Contemporary OB/GYN November, pp. 24–30.ho. Available at: http://contemporaryobgyn.modernmedicine.com/contemporary-obgyn/news/sickle-cell-disease-pregnancy?page=full. Accessed 13 April 2016

  • Jenerette CM, Lauderdale G (2008) Successful aging with sickle cell disease: using qualitative methods to inform theory. J Theory Constr Test 12(1):16–24

    PubMed  PubMed Central  Google Scholar 

  • Jenerette CM, Leak AN, Sandelowski M (2011) Life stories of older adults with sickle cell disease. ABNF J 22(3):58–63

    PubMed  Google Scholar 

  • Kmet LM, Lee RC, Cook LS (2004) Standard quality assessment criteria for evaluating primary research papers from a variety of fields. Alberta Heritage Foundation for Medical Research HTA Initiative #3

  • Mann-Jiles V, Morris DL (2009) Quality of life of adult patients with sickle cell disease. J Am Acad Nurse Pract 21(6):340–349

    Article  PubMed  Google Scholar 

  • Memish ZA, Saeedi MY (2011) Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. Ann Saudi Med 31(3):229–235

    Article  PubMed  PubMed Central  Google Scholar 

  • Moher D, Liberati A, Tetzlaff J, Altman DG (2009) The PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Public Libr Sci: Med 6(6):e1000097. https://doi.org/10.1371/journal.pmed.1000097

    Article  Google Scholar 

  • Mütze U, Thiele AG, Baerwald C, Ceglarek U, Kiess W, Beblo S (2016) Ten years of specialized adult care for phenylketonuria—a single-centre experience. Orphanet J Rare Dis 11 (27). doi:https://doi.org/10.1186/s13023-016-0410-6.

  • Naik RP, Lanzkron S (2012) Baby on board: what you need to know about pregnancy in the hemoglobinopathies. Am Soc Hematol. pp. 208–214

  • Offredy M, Vickers P (2010) Developing a healthcare research proposal. Wiley-Blackwell, Oxford

    Google Scholar 

  • Oteng-Ntim E, Meeks D, Seed PT, Webster L, Howard J, Doyle P, Chappell LC (2015) Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood 125(21):3316–3325

    Article  CAS  PubMed  Google Scholar 

  • Panepinto JA, Bonner M (2012) Health-related quality of life in sickle cell disease: past, present, and future. Pediatr Blood Cancer 59(2):377–385

    Article  PubMed  Google Scholar 

  • Pereira SA, Brener S, Cardoso CS, Proietti ABC (2013) Sickle cell disease: quality of life in patients with hemoglobin SS and SC disorders. Rev Bras Hematol Hemoter 35(5):325–331

    Article  PubMed  PubMed Central  Google Scholar 

  • Polit DF, Beck CT (2010) Essentials of nursing research: appraising evidence for nursing practice, 7th edn. Lippincott Williams & Wilkins, Philadelphia

    Google Scholar 

  • Rees DC, Willliams TN, Gladwin MT (2010) Sickle-cell disease. Lancet 376:2018–2031

    Article  CAS  PubMed  Google Scholar 

  • Robinson GE (2014) Pregnancy loss. Best Pract Res Clin Obstet Gynaecol 28:169–178

    Article  PubMed  Google Scholar 

  • Silva Gomes Xavier A, de Medeiros Lopes D, Ferreira SL (2014) Use of contraceptive methods by women with sickle cell anemia. Ciênc Cuid Saúde 13(1):27–34. https://doi.org/10.4025/cienccuidsaude.v13i1.19459

    Article  Google Scholar 

  • Silva Gomes Xavier A, Ferreira SL, Souza de Santana Carvalho E, de Araújo EM, Cândida Cordeiro R (2013) Perception of women suffering from sickle cell anemia regarding pregnancy: an exploratory study. Online Braz J Nurs 12(4):834–843. https://doi.org/10.5935/1676-4285.20134289

    Article  Google Scholar 

  • Sives A (2016) Mourning involuntary childlessness. Therapy Today 27(4):18–21

    Google Scholar 

  • Skirton H, Cordier C, Ingvoldstad C, Taris N, Benjamin C (2014) The role of the genetic counsellor: a systematic review of research evidence. Eur J Hum Genet 23:452–458

    Article  PubMed  PubMed Central  Google Scholar 

  • Smith-Whitley K (2014) Reproductive issues in sickle cell disease. Blood 124(24):3538–3543

    Article  CAS  PubMed  Google Scholar 

  • Thomas JA, Lipps GA (2011) Subjective well-being of adults with homozygous sickle cell disease in Jamaica. West Indian Med J 60(2):181–187

    CAS  PubMed  Google Scholar 

  • Thompson Ross T (2015) Motivations of women with sickle cell disease for asking their partners to undergo genetic testing. Soc Sci Med 139:36–43

    Article  Google Scholar 

  • United States. Centers for Disease Control and Prevention (2015) Facts about sickle cell disease. Available at: http://www.cdc.gov/ncbddd/sicklecell/facts.html. Accessed 22 March 2016

  • University of York: Centre for Reviews and Dissemination (2009) Our research. Available at: http://www.york.ac.uk/crd/research/. Accessed 02 June 2016

  • Van Tuijn CFJ, Van Beers EJ, Schnog JB, Biemond BJ (2010) Pain rate and social circumstances rather than cumulative organ damage determine the quality of life in adults with sickle cell disease. Am J Hematol 94(48):532–535

    Article  Google Scholar 

  • Wonkam A, de Vries J, Royal CD, Ramesar R, Angwafo ΙΙΙ FF (2014) Would you terminate a pregnancy affected by sickle cell disease? Analysis of views of patients in Cameroon. J Med Ethics 40:615–620

    Article  PubMed  Google Scholar 

  • World Health Organization (2017) Sexual and reproductive health. Available at: http://www.who.int/reproductivehealth/topics/infertility/definitions/en/. Accessed 26 Nov 2017

Download references

Funding

This systematic review was produced as part of self-funded MSc studies.

Author information

Authors and Affiliations

  1. Faculty of Health and Human Sciences, University of Plymouth, Plymouth, PL4 8AA, UK

    Josephine Claire Rance & Heather Skirton

Authors
  1. Josephine Claire Rance
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Heather Skirton
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Josephine Claire Rance.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethics approval and consent to participate

This article does not contain any studies with human or animal subjects performed by any of the authors.

Electronic supplementary material

ESM 1

(DOCX 48 kb)

Supplemental Fig. 1

(DOCX 74 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Rance, J.C., Skirton, H. An integrative review of factors that influence reproductive decisions in women with sickle cell disease. J Community Genet 10, 161–169 (2019). https://doi.org/10.1007/s12687-018-0386-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12687-018-0386-0

Keywords

Search

Navigation