Criteria for Inclusion

The topic of interest was studies exploring the experiences of women with SCD or SCT during their encounters with antenatal, intrapartum or postnatal care (up to 6 weeks post discharge). To provide further context studies reporting on the views and experiences of health professionals providing maternity care (antenatal, intrapartum or postnatal) to women with SCD or SCT were also included. Qualitative studies and mixed methods studies that utilized a qualitative component, either for design (i.e., ethnography, phenomenology), data collection (i.e., focus groups, interviews, observations, diaries, oral histories), or analysis (i.e., thematic analysis, framework approach, grounded theory) were all included. To qualify for inclusion studies had to report first-hand accounts of women with SCD or SCT or health professionals providing maternity care to women with SCD or SCT in recognized health facilities (primary, secondary or specialist SCD centres). Studies reporting on haemoglobinopathy screening alongside the general population were also included provided there was sufficient data from women diagnosed with SCT. Studies published before 2000 were excluded to ensure that the data reflected the views of the most recent generation of women of childbearing age. Studies reporting on the experiences of women with SCD or SCT outside of maternity care (i.e., experiences of general healthcare) were also excluded as were studies reporting on experiences of non-routine diagnostic tests, e.g., non-invasive prenatal testing (NIPD). A summary of the inclusion and exclusion criteria is shown in Table 1 below.

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Table 1. Summary of inclusion and exclusion criteria.

https://doi.org/10.1371/journal.pone.0352992.t001

Search strategy

A search strategy was developed in conjunction with a university information specialist (CH) utilizing a PEO (Population, Exposure, Outcome) structure with the addition of search terms to identify qualitative studies. Systematic searches were carried out on 18th July 2023 and updated on 31^st October 2024 utilizing the following databases, Ovid MEDLINE, Ovid Embase, Ovid MIDIRS, CINAHL Ultimate (EBSCOhost), PsycINFO (EBSCOhost), Social Sciences Citation Index (via Web of Science), Global Index Medicus (all indexes) and CNKI (China National Knowledge Institute). Searches were limited by date to publications from 2000 onwards. No language restrictions were imposed, although all searches were conducted in English. Search strategies for each database are shown in the S1 Appendix.

Study selection

After removing duplicates in EndNote records were uploaded into Rayyan and, where possible, irrelevant records were removed based on title. Two members of the review team (KF, GM) independently assessed 50% of the abstracts each to determine eligibility against the a priori inclusion/exclusion criteria. Twenty percent of the total number of abstracts were screened in duplicate to ensure consistency between the reviewers and, in the event of a discrepancy, agreement on inclusion was reached by consensus. Following the removal of unrelated articles the full texts of potentially relevant articles were obtained and independently assessed for eligibility by KF and GM. References for books and book chapters identified from the database searches were acquired through the University library and assessed for eligibility, i.e., peer reviewed, reporting relevant, empirical, qualitative data. The reference lists of included articles were examined at this stage to check for further studies not identified in the searches. KF and GM selected relevant articles for inclusion with adjudication by a third author (SD) in the event of any disagreement. For studies published in languages other than English the full texts were translated into English using freely available online software (Google Translate) [25] and the decision to include or exclude was taken by consensus between GM and KF.

Quality appraisal

Included studies were appraised using an instrument developed by Walsh and Downe [22] and modified by Downe et al [26]. Studies were rated against 11 pre-defined criteria, and then allocated a score from A–D, where A represented a study with no, or few flaws, with high credibility, transferability, dependability and confirmability; B, a study with some flaws, unlikely to affect the credibility, transferability, dependability and/or confirmability of the study; C, a study with some flaws that may affect the credibility, transferability, dependability and/or confirmability of the study; and D, a study with significant flaws that are very likely to affect the credibility, transferability, dependability and/or confirmability of the study. Each study was appraised by two authors independently (KF, GM) and then cross-checked by the same authors to ensure consistency. Any studies where there were scoring discrepancies of more than a grade were referred to another author (SD) for moderation. Studies scoring C or higher were included in the final analysis.

Data extraction and analysis

An Excel spreadsheet was used to record pertinent details for each included study. i.e., author(s); date of publication; language; country where study conducted; resource setting; study population (women or health professional); phase of maternity care (antenatal, intrapartum, postnatal); study type and participant details. Details of the quality appraisal score for each study were also recorded on this spreadsheet. The data extraction was carried out by KF and GM between the dates of 14^th and 29^th November 2023.

In a process aligned with thematic synthesis [23] two review authors (KF & GM) worked together to develop codes from the author findings and quote material in the included papers. In studies where the author findings included data from participants not relevant to the research question, i.e., participants who did not have SCD or SCT or had thalassemia rather than SCD, efforts were made to code and extract data from pertinent sections of the text. Using an iterative process the same two authors (KF, GM) then synthesized these codes into descriptive themes (review findings) and reached agreement on the analytical themes following input and discussion from a third author (SD). KF conducted GRADE-CERQual [24] assessments of the descriptive themes (review findings) and the final grade was reached by consensus following a cross-check of each assessment by GM. Any grade disagreements were referred to SD for moderation. GRADE-CERQual assesses the methodological limitations and relevance of the studies contributing to a review finding, the coherence of the review finding, and the adequacy of data supporting a review finding. Based on these criteria, review findings were graded for confidence using a classification system ranging from ‘high’ to ‘moderate’ to ‘low’ to ‘very low’ [24]. Following GRADE-CERQual assessment the review findings were grouped into higher order, analytical themes and agreed by consensus amongst the authors. The data extraction, analysis, synthesis and CERQual grading stages are illustrated in the S2 Appendix.

Findings

Fourteen descriptive themes (review findings) were generated, most of which were graded as moderate or low using GRADE-CERQual. From these four analytical themes were developed: Beyond the routine of antenatal screening; Myths, misunderstandings and submissiveness; Fear and uncertainty in the face of adversity; The importance of familial and organizational support. The descriptive themes, analytical themes and associated GRADE-CERQual assessments are shown in Table 3 below.

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Table 3. Descriptive and analytical themes.

https://doi.org/10.1371/journal.pone.0352992.t003

Beyond the Routine of Antenatal Screening

This analytical theme incorporates three of the descriptive themes relating to ‘the importance of screening women early’; ‘screening as a moral imperative’ and ‘coping with the unexpected consequences of routine antenatal screening’.

The importance of screening for SCD before reproductive decision making was highlighted by a number of women in HICs [29,31,34,35]. Some women wanted to be screened for SCD status before the development of relationships to avoid awkward conversations with prospective partners [31,35]. This was particularly important for women with SCT who felt that earlier screening (before antenatal care) might have prevented them from engaging in relationships with partners who were also carriers [31,35]. For others already in relationships, there was a belief that pre-pregnancy screening would have given them (and their partners) time to make important decisions about reproductive healthcare [35]. In HIC contexts some health professionals felt that universal screening, rather than targeted screening for haemoglobinopathies (including SCD and Thalassemia), should be available for all women during the early antenatal phase [29].

Women sometimes couched their responses to screening in terms of ‘being a good mother’ [28,34,35,37]. For some this meant accepting all screening opportunities, including foetal screening, in the understanding that they were making the safest choices to try and ensure the best possible outcomes for themselves and their baby [34,35]. For others, the safest choice sometimes meant declining a screening test, particularly a foetal screening test, because of the potential risks to their baby [37]. In both situations the women were expressing their perception of what a good mother should be, but they arrived at different conclusions as a result.

Some women also felt unprepared for antenatal SCD screening tests and expressed shock and surprise when they received a positive result [28–31,34,35,37]. Usually, this was because they did not consider themselves to be at risk of SCD [28–31,34,37]. However, some women did not really understand what they were being tested for or the test had been presented to them as ‘routine’ and therefore nothing to worry about [30,31,35]. A positive antenatal screening test was particularly challenging for women identifying as ‘white’ because they were under the impression that SCD was not a disease they could inherit [29,37]. Health professionals also reported difficulties in explaining a positive result to women who, for whatever reason, didn’t expect to receive one [29,34]. This was particularly apparent when health professionals had to discuss the implications of a positive result and the various choices available, including the possibility of terminating a pregnancy [34].

Myths, Misunderstandings and Submissiveness

This analytical theme is made up of five descriptive themes, namely, ‘doubts about ability to become pregnant based on myth and misinformation’; ‘lack of knowledge and information about SCD and screening’; ‘fatalistic beliefs lead to passivity and conflict’; ‘deferential attitudes towards health professionals leads to directive care’ and ‘language barriers hinder communication’.

For some women with SCD the very notion of becoming pregnant was obscured in a cloud of myth and misinformation [28–32,34–38]. In some contexts, socio-cultural understandings of SCD fostered the belief that women with the condition could not become pregnant, leading to the assumption that contraception was not important [28,32,36]. Such beliefs occasionally resulted in shock and surprise when an unintended and/or unwanted pregnancy came to light [28,32]. In other settings the prevailing socio-cultural belief was that women with SCD should not become pregnant, largely based on the view that it would be amoral or cruel to pass on the condition to a child [36]. This latter view was sometimes supported by health professionals who expressed their concerns about the health and welfare of mother and baby by blaming and shaming the women in their care for becoming pregnant [36].

The myths associated with SCD and conception appeared to be born out of a lack of knowledge and information [29–31,34–37]. Women in a variety of settings felt they did not know enough about SCD and lacked understanding about basic genetics, screening practices and the implications of being a carrier [31,35,37]. These issues were compounded for those who were not fluent in the local native language, especially where translation or interpretation services were inadequate or unavailable [29,31,34,35]. Some women with SCD also found that health professionals lacked knowledge about the condition and were not able to offer advice and guidance about how to manage their pregnancies [29,35,36]. This was confirmed by health professionals in several settings who felt they needed more training to better understand the condition and how it affected women during pregnancy [29,30,36]. Other health professionals involved in antenatal screening in high-income settings (HICs) also felt awkward and uncomfortable asking questions about race and ethnicity during SCD screening and wanted more training in cultural awareness [29,30]. Arguably, because of a lack of knowledge about their condition or their carrier status, women in several contexts exhibited a deferential attitude to the views and opinions of health professionals [31,34–36]. In some instances this view was maintained or encouraged by health professionals leading to directive care and poor engagement in shared decision-making [34–36]. Some women also adopted a passive or fatalistic attitude to decision-making by deferring their choices around screening or potential termination to ‘the will of God’ [31,34,36,38]. Views influenced by religious beliefs also had an impact on acceptance or opposition to interventions like foetal diagnostic testing and created mental and emotional turmoil in some women who decided to terminate a pregnancy [38].

Fear and Uncertainty in the Face of Adversity

This theme includes four descriptive themes, namely, ‘stigmatization, discrimination and insensitivity’, ‘pregnancy is marked by uncomfortable and painful eruption of SCD symptoms’; ‘Intense feelings of fear, guilt and anxiety throughout the maternity journey’ and ‘the joy of achievement and despair of loss’.

Women with SCD recounted an exhausting array of physical, practical, emotional and psychological challenges that impacted on their experiences of maternity care [28–34,36–38]. In navigating these challenges women displayed remarkable resilience and determination in order to fulfil their dream of becoming a mother. For some, the confirmation of pregnancy generated powerful emotions, eliciting feelings of guilt, fear and anxiety [28,32,33,38]. Women described feeling particularly anxious about the wellbeing of their baby and, where the foetal SCD status was unknown, whether the baby would be born with the condition [28,33,38]. In some instances these concerns persisted throughout pregnancy and prompted more apprehension about their ability to cope and, in some extreme examples, the prospect of dying during childbirth [32,38]. Women also worried about their future and the potential burden of having a child with SCD as well as their long-term ability to look after a child as a mother with a serious health condition [28,32]. In some contexts, women with SCD felt compelled to keep their pregnancy secret to avoid feeling stigmatized or judged by family members, the wider community and, occasionally, health professionals [36–38]. Health professionals, in turn, struggled with their own preconceptions and poorly informed notions of SCD prevalence and ethnicity and, in screening situations, made assumptions (sometimes incorrectly) about susceptibility to SCD based on appearance [29,30,37]. In some HIC settings these practices were perceived as discriminatory by the women being screened [33,37]. Pregnancy also prompted women to reflect on their reproductive choices and some expressed guilt about becoming pregnant and/or experienced regret about their choice of partner [31,32].

In addition to these emotional struggles, pregnancy often precipitated a flare-up of SCD related symptoms and women described experiencing acute pain, fevers and infections [28,36,38]. Sometimes these crises required hospital treatment and occasionally admission to hospital for blood transfusions [28,36,38]. Despite these practical, emotional and physical challenges women with SCD expressed an overwhelming desire to become a mother [31,32]. A few felt that pregnancy ‘normalized’ their condition, enabling them to be perceived as ‘normal women’, capable of carrying a pregnancy, rather than ‘sick women’ who couldn’t or shouldn’t conceive [38]. For some, the successful birth of a healthy child elicited feelings of incredible relief and, ultimately, a sense of triumph or victory [32,38]. For others, the profound sense of loss following a miscarriage or a pregnancy termination, generated feelings of despair, trauma and grief sometimes leading to prolonged periods of emotional turmoil or depression [33,36,38].

The Importance of Familial and Organizational Support

This analytical theme is comprised of two descriptive themes, ‘influence of family, professional and social support’ and ‘infrastructure and organizational issues impact on quality of care’.

In various contexts women with SCD recalled the importance of support during their maternity journeys [29–38]. In most cases this related to support from families and, in particular, the support of their partner [32,33,36–38]. Despite the challenges of carrying a pregnancy women remarked on the strength they received from having a supportive partner and highlighted the importance of feeling loved and cared for in these circumstances [33,36,37]. In other contexts, however, partners were less supportive and women recalled feeling rejected or abandoned by a partner when the foetus was confirmed to have SCD [32,37,38]. In addition, some pregnant women (with the condition) felt isolated by their families and/or communities because of the associated stigma [37,38]. This sometimes generated feelings of loneliness and depression and, in these circumstances, women began to doubt their ability to cope and considered pregnancy termination [38]. In other contexts, women kept their carrier status secret in order to avoid feelings of rejection or stigmatization and to maintain family support networks [37,38].

Pregnant women with SCD also valued the support of health professionals and highlighted the importance of empathy, understanding and respect during their maternity journey [36]. Women also appreciated the support, advice and expertise of health professionals working in specialist SCD clinics or in hospitals with SCD support services (where available) [36]. However, some women highlighted the lack of beds in specialist centres and delays in receiving support and care leading to extended periods of pain and discomfort while waiting to receive treatment [38]. In one setting access to specialist services appeared to be facilitated by informal payments resulting in an inequitable service where women without the required finance were unable to receive support and treatment [36]. Women also remarked on the lack of time spent with health professionals during consultations or antenatal appointments [35]. Women wanted to receive more information and advice about SCD screening or the opportunity to discuss their pregnancy or birthing options in more detail [31,35,37]. Health professionals also highlighted the limited amount of time they were able to spend with women during screening sessions or at antenatal appointments and the difficulties of discussing quite complex issues within a 15–20 minute time slot [29,30,34].

Limitations of the Review

Although the impact of SCD during pregnancy and childbirth is more common in Sub-Saharan Africa, the Middle East and parts of South-East Asia this review only found one relevant study, conducted in Uganda [35]. This is a limitation as there are likely to be additional and/or more nuanced socio-cultural and socio-economic considerations in other contexts, particularly in LICs where resource constraints and competing healthcare priorities may impact on SCD service delivery and engagement.

Given that the other ten studies in the review were conducted in HICs or Upper-Middle Income countries where SCD services are relatively well established, findings largely reflect the views of a particular population of women with access to SCD screening programmes, health surveillance systems, treatments and specialist services that may not be available in resource-limited settings. In addition, nine of the eleven included studies were conducted in just two countries, six in the UK and three in Brazil, and only one [33] was conducted within the last five years. This rather jeopardizes the transferability and generalizability of the findings and also highlights the paucity of research in this area. Further research exploring the experiences of pregnant women with SCD, especially in high burden countries may provide valuable insights into how they can be better supported during their maternity journey.

The review also identified only three studies reporting on the views of healthcare providers (all from the UK), and all were focused on their experiences of providing antenatal screening for SCD. This is another limitation of the review and further undermines the transferability and generalizability of the findings to other contexts. Health system structures, approaches to screening and treatment options for pregnant women are likely to be different in other countries, particularly in LMICs, where a lack of resources, infrastructure and understanding of the condition may impede the provision of quality maternity care for women with SCD or SCT.

Given the paucity of studies in this area and the limitations outlined above it is important that further qualitative research is conducted in areas where SCD is prevalent and where understanding of the condition is poor. More up to date studies focused on women’s experiences of SCD or SCT during their engagement with maternity services are likely to highlight current needs and concerns for this population of women. In addition, qualitative studies with maternity providers working in areas where SCD is prevalent may be able to offer further insights into their educational needs, resource requirements and attitudes towards SCD as well as their perspective on supporting a group of women with specific care requirements.