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.

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