Psychological well-being in adults with cystic fibrosis during pregnancy and parenthood: A systematic review

Abstract

Cystic fibrosis is a genetic condition affecting multiple organ systems, and advances in CFTR modulator therapies have improved both life expectancy and reproductive possibilities. However, the psychological impact of pregnancy and parenthood for PwCF remains underexplored. This review synthesised qualitative research published between 1998 and 2025 to examine how pregnancy and parenthood shape the psychological well-being of PwCF. Searches were conducted across PsychInfo, CINAHL, Embase, Scopus and PubMed and eligible studies were appraised and synthesised using thematic methods. Eighteen studies were identified. The synthesis highlighted four overarching themes illustrating the emotional and psychological complexity of becoming a parent with CF. Participants described navigating hope, fear, guilt and shifting identities while balancing the dual roles of parent and patient. Experiences of stigma, constrained autonomy and inconsistent support were common, yet parenthood also provided meaning, motivation and redefined wellness. The findings emphasise the need for psychologically informed, relationally responsive care.

Introduction

Cystic Fibrosis (CF) is a health condition that affects the lungs, digestive system and other organs and was once viewed as a fatal disease of infants and young children, however life expectancy has increased substantially. CF is the most prevalent life-limiting autosomal recessive condition in the Western world, affecting more than 10,000 people in the UK and around 35,000 across Europe (Sapru et al., 2025). CF is considered to affect at least 100,000 people world-wide (Shteinberg et al., 2021) and is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is responsible for mucus exacerbation particularly in the lungs (Hubert et al., 2017). Based on 2024 CF registry data, the median predictive survival age for people born between 2020 and 2024 is 65 years (Cystic Fibrosis Foundation, 2025). CFTR modulator therapies have transformed the clinical management of CF by directly targeting the underlying protein defect rather than it’s downstream complication. These modulators are small molecules that improve the folding, trafficking and function of the defective CFTR protein and are described as a life changing therapy for the majority of PwCF worldwide. CFTR therapy produces substantial improvements in lung function, gastrointestinal symptoms, nutritional status, sweat chloride concentration and infertility/subfertility (Bacalhau et al., 2023). Sustained benefits have been demonstrated across both short and long term follow up, which may help to reshape personal priorities and enable greater consideration of parenthood (Bacalhau et al., 2023).

There are many multifaceted considerations that play into the decision of when to become a parent, but these may be especially complex for PwCF. One qualitative study has reported that decisions about parenthood may be guided by a sense of moral responsibility, including concerns about passing on the condition, the ability to care for a child long term and the potential impact on partners and future children (Vanhollebeke and Van Steijvoort, 2025). With the expanding availability of CFTR modulator therapy, more people with CF are beginning to consider the potential for parenthood; something that, for many, was previously unimaginable (Jain et al., 2023). Wilson et al. (2019) noted the importance of granting more significance to discussions regarding sexual reproductive health given the increases in CF life expectancy and reported that almost 40% of female patients have difficulty with fertility and around 98% of men with CF have a congenital bilateral absence of the vas deferens (CBAVD), resulting in infertility due to azoospermia (Sapru et al., 2025).

Fertility may increase for women using CFTR modulators, leading to successful pregnancies without treatment, while male fertility remains largely unchanged; however, data on the safety of CFTR modulators during pregnancy is limited, as pregnant women were excluded from clinical trials (Boehm-Cohen and Shteinberg, 2025; Khan and Tran, 2025). As new reproductive possibilities emerge, people with CF face complex emotional and psychological challenges navigating pregnancy and parenthood, though many describe the experience as deeply meaningful and report a strong sense of fulfilment in the parenting role (Jacob et al., 2021a,b; Milo and Tabarini, 2022).

Mental health needs among people with CF are increasingly recognised, yet access to appropriate psychological support, particularly around parenthood, remains limited (Guta et al., 2021; Tickner et al., 2022). While many CF parents report positive well-being, emotional challenges such as guilt, role conflict and feelings of inadequacy, especially around infertility and fatherhood are common and under-supported in clinical care (Gaitzsch et al., 2020; Jacob et al., 2021b; Kazmerski et al., 2022).

While there is an increasing number of studies exploring the medical outcomes of pregnancy in CF, less is known about the psychological impact of pregnancy and parenting within this group. Jacob et al. (2021b) provided an important early synthesis of parenthood in CF, however, an updated review was needed because their work focused solely on descriptive accounts from parents in the pre-modulator era, whereas the present review incorporates substantial post-modulator research, includes people considering pregnancy as well as parents, examines the psychological impact of pregnancy and parenthood rather than general experiences and applies a theory-informed meta-synthesis with CERQual to generate a deeper, more contemporary understanding of the emotional, identity-based and relational dimensions of becoming a parent with CF. Building on this, the existing literature continues to reveal a notable gap in qualitative research specifically examining the psychological experience of becoming a parent with CF. Despite the growing number of people with CF pursuing pregnancy and parenthood, little is known about how these transitions shape their psychological well-being or the support they require, further underscoring the need for the present review.

Given these developments and knowledge gaps, this review aims to systematically review and synthesise the available qualitative literature on how pregnancy and parenthood affect the psychological well-being of PwCF, identifying key challenges, coping mechanisms, implications for clinical care and identify priorities for future research. The research question specifically asks, how do experiences of pregnancy and parenthood influence the psychological well-being of adults with CF?

Method

This review was pre-registered with PROSPERO International Prospective Register of Systematic Reviews (ID CRD420250652034) and can be accessed in full on https://www.crd.york.ac.uk/PROSPERO/view/CRD420250652034. The preparation of this review was guided by the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines (Page et al., 2021), and Enhancing Transparency In Reporting the Synthesis of Qualitative Research (ENTREQ; Tong et al., 2012). The PRISMA checklist can be found in Supplemental Table 1 and the ENTREQ checklist can be seen in Supplemental Table 2. This systematic review involved analysis of data reported in published literature only. As no new data were generated and no participants were recruited, ethical approval and informed consent to participate were not required.

Search strategy and study selection

Systematic searches of electronic databases included; EMBASE, Scopus, PubMed, CINAHL Ultimate and PsycINFO from data inception until 26th March 2025. The term “cystic fibrosis” was combined with key terms such as “parenting” and “psychological well-being” to represent the qualitative aspects of well-being of PwCF who have experienced pregnancy or parenthood. This search strategy was reviewed using the Peer Review of Electronic Search Strategies (PRESS) Checklist (McGowan et al., 2016) to ensure accuracy, appropriateness and completeness (Supplemental Table 3). The full search strategy is shown in Supplemental Table 4. Additionally, grey literature databases such as OpenGrey, ProQuest, Ethos, Open Access Theses and Dissertations and OAlster were also searched. CF specific websites were searched to identify relevant reports as were preprint platforms such as BioRxiv, MedRxiv and OSF preprints. No restrictions were set according to publication date. The reference list of included studies was also hand searched. Results of the searches were uploaded to EndNote reference manager (The Endnote Team, 2013), where duplicates were removed. Covidence, a web-based platform for systematic reviews was then used to facilitate collaborative screening (Veritas Health Innovation, 2025). One hundred percent of titles and abstracts were screened by two reviewers (SB & ERM), and 33.3% of full text screening was completed by two reviewers with disagreements resolved through consensus.

Eligibility criteria

In order to capture all potentially relevant research, no year limit was applied to searches and inclusion criteria was broad in nature, capturing the full scope of available evidence and diverse perspectives. The review was structured using the PICO framework: Population: adults with cystic fibrosis (“Cystic Fibrosis” OR “CF”); Interest: pregnancy and parenthood (“Pregnancy” OR “Pregnan*” OR “Parent”); Context: psychological well-being, including mental health, emotional impact and quality of life (“Mental health” OR “Psychological well being/wellbeing/well-being” OR “Quality of life” OR “QOL” OR “Challenge*”). Qualitative peer-reviewed and grey literature studies and reports were included.

Inclusion criteria

Studies written in the English language primarily focusing on the adult population with a diagnosis of CF, that explore the experience of pregnancy, parenthood or those considering parenthood, within the context of psychological well-being were included. For the purpose of this review, we understood psychological well-being to refer to one’s emotional health and overall functioning, consisting of social relationships, autonomy, environmental mastery, self-acceptance, sense of purpose and personal growth (Ryff, 1989, 2014). Primary research studies utilising qualitative methods that were published and peer reviewed as well as grey literature such as theses, dissertations, reports, conference abstracts, unpublished studies, government reports and clinical trial results were also included. Conference abstracts were included with recognition that they often provide early access to emerging evidence and a valuable window into contemporary research properties, methodological developments and topics not yet represented in peer-reviewed literature (Scherer and Saldanha, 2019). In fields where the evidence base is small and evolving, such as reproductive and psychological experiences in CF, conference material can help reduce time-lag bias and ensure the synthesis reflects the most current insights. Every effort was made to access full text relating to included conference abstracts to maximise accuracy and contextual depth.

Exclusion criteria

Studies focusing on children/adolescents, or non CF populations were excluded, as were studies solely focusing on physical health outcomes of pregnancy. Review articles and quantitative studies were excluded, as well as opinion pieces, editorials and blog posts. However, reference lists were screened from relevant reviews for potentially relevant studies.

Data extraction and synthesis

Data extraction was carried out by SB on all studies that met inclusion criteria. ERM, second reviewer, extracted 33.3% of the data on studies that met inclusion criteria. Information such as study details, design, aim, participants, analysis, key themes, quotes, main findings, ethical considerations, recommendations and limitations were extracted. The data was extracted using Covidence and the data extraction template was piloted before official extraction commenced. This synthesis of qualitative studies followed the guidance outlined by Lachal et al. (2017), which adapts Thomas and Harden (2008) thematic synthesis to include phenomenological perspectives. This approach allows for deeper insight into complex, subjective experiences by re-interpreting the findings of primary studies. Extracted data including both quotations (first order data) and previous authors interpretations (second order data) was exported into NVivo 12 (Lumivero, 2023) and analysed using Thematic Synthesis. Key data extraction challenges such as transparency, preserving contextual information, ensuring sufficient data has been extracted, ensuring internal consistency, authenticity and iteration were considered (Boland et al., 2024). Coding was completed using NVivo software. Codes from the first paper (in authorship alphabetical order) were translated into remaining papers along with the development of new codes until all studies were coded. Descriptive codes were then developed and analytical inductive themes were developed in the third stage of the analysis with reference to the principal research question, how do experiences of pregnancy and parenthood influence the psychological well-being of adults with CF?

Quality assessment

The Critical Appraisal Skills Program (CASP) checklist was used to evaluate the quality of included studies. CASP is one of the instruments recommended by the Cochrane Collaboration (Noyes et al., 2018) and addresses key principles and assumptions underpinning the quality of qualitative research studies. The AACODS (Authority, Accuracy, Coverage, Objectivity, Date and Significance) checklist was used to critically appraise grey literature (Jess, 2010). This allowed appropriate quality appraisal across different sources of data.

Reflexivity

The research team comprised Selina Braddish and Eimear Ruane McAteer, both Psychologists in Clinical Training at the University of Limerick; Sharon Houghton, Senior Clinical Psychologist at University Hospital Limerick and Adjunct Senior Clinical Lecturer at the University of Limerick; and Donal G. Fortune, Professor of Clinical Psychology at the University of Limerick. As suggested by Olmos-Vega et al. (2023) it includes members who are both experts in the topic being studied and trained in qualitative synthesis, spanning different clinical specialities and career stages. This brought different perspectives and experiences to the study. This multidisciplinary composition enriched the analytic process and contributed to a more nuanced interpretation of the data.

Results

Study selection and characteristics

The final search yielded 3487 papers. In total 2046 studies remained after removing duplicates, Figure 1 provides an overview of the selection process. The included studies collectively explored the psychosocial and reproductive health experiences of PwCF, focusing on diverse perspectives across gender, geography and age. A total of 18 studies, published between 1989 and 2025 are represented in the synthesis. These studies originate from various countries, including USA, UK, Sweden, Australia and France, involving data from a total of 326 participants, ranging from 19 to 62 years of age, as described in Table 1.

Figure 1.

PRISMA flowchart illustrating the search strategy and paper selection process (Page et al., 2021).

Table 1.

Study characteristics.

Study ID	Country	Age range	Participants		Participants who were parents	Data collection	Data analysis method
Johannesson et al. (1998)	Sweden	Adults	Female	n = 14	Two parents	Interview	Not Reported
Jessup et al. (2018)	Australia	31–42	Male (4) Female (3)	n = 7	All parents	Interview	Selective Reading Approach
Hailey et al. (2019)	USA	22–46	Male (10) Female (10)	n = 20	Two parents	Interview	Thematic Content Analysis
Clarke et al. (2022)	USA	19–60	Male	n = 24	Twelve parents	Interview	Inductive Coding Process
Williams et al. (2023)	UK & USA	26–45	Female	n = 21	Ten parents	Interview	Thematic Analysis
Stransky et al. (2023)	USA	29–53	Male (7) Female (11)	n = 18	All parents	PhotoVoice	Thematic Analysis
Simcox et al. (2009)	UK	21–28	Female	n = 12	Three parents	Interview	Grounded Theory Analysis
Stransky et al. (2024)	USA	22–46	Male (16) Female (21)	n = 37	All parents	Interview	Thematic Analysis
Jacob et al. (2025)	France	28–65	Male (6) Female (12)	n = 18	All parents	Interview	Thematic Analysis
Barker et al. (2017)	UK	21–50	Male (4) Female (5)	n = 9	Seven parents	Interview	Interpretive Phenomenological Analysis
McKenzie Smith et al. (2019)	UK	26–52	Male (15) Female (10)	n = 25	Three parents	Interview	Thematic Analysis
Ladores et al. (2016)	USA	25–34	Female	n = 10	Not reported	Interview	Thematic Analysis
Ladores et al. (2017)	USA	25–34	Female	n = 10	Not reported	Interview	Thematic Analysis
Jacob et al. (2021a)	France	29–62	Male (27) Female (38)	n = 65	All parents	Interview & Focus Groups	Thematic Analysis
Cammidge et al. (2013)	UK	22–41	Female	n = 11	All parents	Interview	Grounded Theory Analysis
Bray and Ladores (2017)	USA	25–34	Female	n = 10	Not reported	Interview	Thematic Analysis
Birney (2024)	UK	23–30	Female	n = 3	Two parents	Interview	Interpretive Phenomenological Analysis
Backström Eriksson (2019)	Sweden	32–55	Male (5) Female (7)	n = 12	Not reported	Interview	Inductive Content Analysis

Note. n refers to the total number of participants in each study. The “participants who were parents” column indicates how many participants within each sample had children at the time of the study.

Quality assessment and risk of bias

Considering that there are no widely accepted, or empirically tested methods for excluding qualitative studies from syntheses on the basis of their quality, we included all studies in analysis regardless of their quality (Daly et al., 2007; Dixon-Woods et al., 2006; Thomas and Harden, 2008). The full results of this assessment are published within this text to enhance the rigour of the synthesis (Walsh and Downe, 2005) see Table 2. The quality of studies varied, with the majority deemed to be of high quality. See Supplemental Table 5 for full quality assessment.

Table 2.

Quality appraisal of included studies using CASP and AACODS tools.

Study	Study type	Appraisal tool	Overall quality
Johannesson et al. (1998)	Peer-reviewed	CASP	Moderate
Jessup et al. (2018)	Peer-reviewed	CASP	High
Hailey et al. (2019)	Peer-reviewed	CASP	High
Clarke et al. (2022)	Peer-reviewed	CASP	High
Williams et al. (2023)	Peer-reviewed	CASP	Moderate
Stransky et al. (2023)	Peer-reviewed	CASP	High
Simcox et al. (2009)	Peer-reviewed	CASP	High
Stransky et al. (2024)	Peer-reviewed	CASP	High
Jacob et al. (2025)	Peer-reviewed	CASP	High
Barker et al. (2017)	Peer-reviewed	CASP	High
McKenzie Smith et al. (2019)	Grey Literature	AACODS	High
Ladores et al. (2016)	Grey Literature	AACODS	Low
Ladores et al. (2017)	Grey Literature	AACODS	Moderate
Jacob et al. (2021a)	Grey Literature	AACODS	Moderate
Cammidge et al. (2013)	Grey Literature	AACODS	High
Bray and Ladores (2017)	Grey Literature	AACODS	Low
Birney (2024)	Grey Literature	AACODS	High
Backström Eriksson (2019)	Grey Literature	AACODS	High

Meta-synthesis findings

A thematic meta-synthesis was conducted yielding nine descriptive themes which were subsequently synthesised into four higher-order analytical themes (Figure 2). See Supplemental Tables 6 and 7 for how each paper contributed to the development of each theme. Confidence in the review findings was assessed using GRADE-CERQual (Lewin et al., 2018), with judgements detailed in Supplemental Table 8.

Figure 2.

Example of the development of descriptive and analytical themes. not all primary codes were presented for simplicity.

Analytical Theme 1: Living with emotional ambivalence – The psychological labour of becoming a parent

This theme explored the emotional complexity experienced by PwCF as they considered or pursued parenthood, revealing a constant negotiation between hope, fear and uncertainty. The psychological labour of becoming a parent was shaped by the weight of morality, delayed or conflicting information around fertility and the emotional strain of navigating unclear futures amidst advancing treatments.

Confronting mortality and guarding against hope

Participants described parenthood as a deeply meaningful but emotionally fraught goal, marked by a constant negotiation between hope and fear. The strong desire to have children was often tempered by the realities of living with a life-limiting condition. One participant reflected “discussing the fact that I might die, that has definitely been the hardest part” (Hailey et al., 2019). This emotional conflict was compounded by efforts to protect themselves from potential disappointment. One participant noted that she would “think about having a child, then I’d go ‘no, don’t think about it in case it doesn’t happen.’” For some, this was tied to a deeper sense of feeling diminished around accessing the experiences afforded to “healthy” others, underpinned by the belief that they should be “happy with what I’ve got” (Birney, 2024). This reflects the underlying self-limiting mindset in which hope or expectation is avoided to guard against potential loss or disappointment. Feelings of being undeserving of a “normal” life also surfaced, with many participants questioning whether they should pursue parenthood at all (Barker et al., 2017; Jessup et al., 2018). The psychological weight of pregnancy itself appeared intense, even when medically supported.

For some, the added stress of fertility treatment and pregnancy heightened anxiety and depression in an already vulnerable population (Bray and Ladores, 2017). Some participants found the emotional burden of pursuing parenthood more challenging than managing CF itself, “it was a very hard process . . . physically . . . morally . . . way more than the disease itself” (Jacob et al., 2025). Overall, the journey to parenthood for PwCF was characterised by a complex mix of hope and emotional strain, reflecting the psychological depth of navigating parenthood within the context of CF.

Delayed truths and disrupted dreams: The emotional impact of infertility and assisted reproduction

Fertility and family planning were emotionally complex and deeply personal experiences for PwCF. Many described learning about CF-related infertility unexpectedly and too late, leading to distress and confusion, “I don’t think even mum knew . . . I read it in a book when I was in hospital . . . it was something that no one ever discussed” (Jessup et al., 2018). Others expressed frustration over inconsistent messages, “I felt cheated, the gynaecologist said there was no problem . . . but my CF doctor said no. It was like a knockout” (Johannesson et al., 1998). Men in particular reported that infertility affected their identity and mental health, “there’s some guy pride in it . . . you’re in the prime of your life and you can’t even have kids. It’s rough on your mental health” (Clarke et al., 2022). Where pursued, ART was experienced as invasive, exhausting and expensive, with one participant reflecting “it was harrowing . . . emotional . . . and involved clinicians poking around” (Jessup et al., 2018). The emergence of CFTR modulators brought new possibilities, but also new pressures, “I can look forward to having a family now” (Williams et al., 2023). Yet others felt conflicted, questioning what might have been if the drug arrived sooner, or worrying about its unknown effects, with one participant stating that the “long term side effects were unknown and this compounded anxieties” (Birney, 2024).

Psychological toll of anticipating and managing uncertainty

Uncertainty was a persistent emotional burden for participants navigating family planning and parenting. Decisions about conception and parenting were shaped by fear of health decline, premature death and unclear treatment outcomes. Anticipating the future brought psychological distress and some avoided planning altogether. The introduction of CFRT modulators added further ambiguity, as participants noted “there is not enough evidence” (Birney, 2024), reflecting frustration over changing guidance, describing these dynamics as “all the goal posts are moving” (Simcox et al., 2009). Uncertainty created conflict between the desire for normalcy and the need to prioritise health with participants described as being “riddled with anxiety” and “emotionally overwhelmed” by decisions made without clear answers (Birney, 2024). Despite medical progress, emotional strain from ongoing uncertainty remained a defining experience.

Analytical Theme 2: Reclaiming identity in the face of chronic illness: From patienthood to parenthood

This theme examined how the transition to parenthood shaped the lived experiences of identity for PwCF, as they negotiated the dual roles of parent and patient. PwCF described both emotional burden and motivational potential of parenthood, highlighting the complex interplay between responsibility, guilt and the desire to maintain health in order to sustain the parenting role.

Guilt, responsibility and the drive to stay well

The transition to parenthood for PwCF was marked by a heightened sense of responsibility. For PwCF, the burden of responsibility extended beyond their own intensive health management and added the pressure of safeguarding a child’s well-being. This led to difficult choices about prioritising treatment to ensure they remain present, “what way would allow me to live as long as possible . . . so that I can see my son grow up?” (Stransky et al., 2024). Alongside this responsibility came persistent guilt, particularly during periods of ill health. Several participants experienced distress about missing out on parenting moments, “there’s sort of a daily guilt . . . that I’m not providing as full a life for him that I would do if I didn’t have CF” (Cammidge et al., 2013) and “not being the primary caregiver constantly” (Stransky et al., 2023). Others expressed guilt and self-blame, fearing that if it went wrong, it would be their fault, “You can’t live your life thinking ‘what if something goes wrong’. . . but it could go wrong at any point” (Birney, 2024). Despite emotional challenges, many found renewed purpose in parenthood, often strengthening their motivation to maintain health and describing the future as “redefined” (Jessup et al., 2018).

Reconciling dual identities amid anticipatory grief

Balancing the dual identities of parent and patient placed significant emotional strain on PwCF. Participants described the daily challenge of managing self-care while fulfilling parenting duties, often feeling pulled in two directions. One parent shared “I’ve got to prioritise . . . its either taking care of myself or my child” (Barker et al., 2017). Others spoke of the emotional cost, “I am constantly on the go . . . it doesn’t help my mental health because I am exhausted” (Barker et al., 2017). Anticipatory grief was a recurrent theme, over missing time with children, relying on others and fearing future absence. One parent spoke about how “it might just all end at some point” (Jacob et al., 2025), capturing the weight of anticipatory grief. Some avoided hospital admissions to remain at home, while others sacrificed family time for long term health. Participants also grappled with identity shifts, balancing their role as parents with the ongoing reality of being a patient, describing the emotional toll of “figuring out how to be a mom when you can’t be with your kid all the time” (Stransky et al., 2023) and “learning as you go” (Cammidge et al., 2013).

Analytical Theme 3: What matters now: Parenthood as a catalyst for connection, meaning and redefined wellness

Parenthood prompted a shift in what PwCF considered central to well-being. Moving from clinical measures of health to emotional connection, validation and meaningful living. This theme describes how the experience of becoming a parent reshaped their understanding of wellness and highlighted the importance of being seen, supported and connected.

Need for connection and validation

Participants expressed a strong need for emotional connection, peer support and recognition from healthcare providers during the transition to parenthood. Many felt isolated, with limited opportunities to share experiences with others facing similar challenges. One parent shared “if they were to start something that was a support group . . . it would make a big difference” (Stransky et al., 2024). Hearing from other parents with CF was particularly valued, offering emotional resonance that clinical interactions often lacked, “parenting and mental health, you really need some anecdotal real-life experience guidance along the way” (Stransky et al., 2024). Validation from CF teams was equally important, with several participants feeling dismissed or unsupported in their parenting goals, resulting in a disconnect, “I felt they couldn’t offer me a lot of experience stuff, which is what I wanted to hear” (Cammidge et al., 2013). Others desired care that addressed their identity as parents, not just patients. Supportive relationships, particularly partners and extended family were described as essential to coping, “I’ve got a lot of support . . . otherwise I’d not have had any chance of doing it” (Cammidge et al., 2013).

Redefining wellness and quality of life

Parenthood reshaped how PwCF understood wellness, shifting focus from clinical stability to emotional fulfilment and meaningful living. Becoming a parent was described as “a dream come true” (Jessup et al., 2018) and “even more wonderful than they imagined” (Cammidge et al., 2013), with one parent sharing “I always think I’m in a dream” (Jessup et al., 2018). Rather than just hoping to survive, many spoke of wanting to be “more intentional” (Hailey et al., 2019), finding joy, creating memories and being present with their children, “you can’t imagine the happiness I felt holding my baby, after dreaming of it for years” (Jacob et al., 2025). Parenthood offered motivation for selfcare, “the joy that children bring is a further reason they adhere to treatment” (Cammidge et al., 2013). This perspective did not negate hardship, with participants acknowledging the mix of emotions, “its worried, its guilt, its happiness to be with your child” (Stransky et al., 2023).

Analytical Theme 4: Navigating constraints – The psychological struggle for autonomy and recognition

This theme relates to the constant need to assert autonomy and be recognised as a capable decision maker. In many cases, choices around pregnancy and parenthood were scrutinised and challenged by medical professionals and family members. These encounters often led to feelings of disempowerment, stigma and emotional distress.

Navigating support and stigma

Participants’ experiences of seeking support around parenting and reproductive choices were deeply shaped by feelings of stigma, judgement and emotional vulnerability. While some described CF teams as supportive, “they basically just said we’re very behind you” (Cammidge et al., 2013), others felt dismissed or invalidated, “they just go ‘no, we’re medically against it’” (Simcox et al., 2009). Support often varied depending on the clinician’s personal stance, with participants noting a lack of psychological insight in reproductive advice. One participant described a particularly distressing encounter in which a member of the clinic team openly expressed disapproval of women with CF having children, leaving her feeling judged and deeply upset (Cammidge et al., 2013). Stigma extends beyond the clinic, with reports of unfair treatment from adoption services and judgement from maternity teams, “the doors closed before they even have a medical on you” (Cammidge et al., 2013). Some participants felt their teams lacked understanding of the lived experience of becoming a parent; “had we wanted to talk about it to the doctors, they would not have understood” (Jacob et al., 2025). Even when clinicians were well-meaning, concerns often centred not around pregnancy itself but the demands of parenthood, “it’s really not the pregnancy that I’m worried about with you. It’s what are you gonna do when you become a parent?” (Stransky et al., 2024). One participant highlighted how their decision to become a mother was “very much dependent on the support of their partners and wider family units” (Birney, 2024), reflecting the protective role of informal networks. Despite these challenges, participants described moments of collaborative, person-centred care. “A shared positive aspect of the experience of navigating pregnancy with CF was the effort made between participants’ CF and maternity clinical teams to adapt, communicate and collaborate with each other when providing care” (Birney, 2024).

Struggles for autonomy and control

Participants described tension between medical guidance and personal autonomy. Some felt disempowered by risk-focused advice, “if you do decide to have children . . . you might not make it to term” (Williams et al., 2023). Others were frustrated that decisions extended beyond the couple, “it included their extended family and healthcare team” (Jessup et al., 2018). Emotional needs were often overlooked in favour of clinical concerns, “medical teams based their advice on clinical strategies neglecting emotional ones” (Simcox et al., 2009). To reclaim agency, participants focused on how to become parents, “I wasn’t talking to him in terms of should I do it . . . just how to do it” (Stransky et al., 2024).

Discussion

This systematic review of 18 studies involving 326 participants thematically synthesised the influence of pregnancy and parenthood on the psychological well-being of PwCF. Across four analytical themes, the findings reveal a powerful interplay between emotional ambivalence, identity reconstruction, and the ongoing struggle for autonomy in reproductive and parenting decisions.

This review highlights the complex emotional terrain navigated by PwCF as they pursue parenthood, offering a deeper understanding of the psychological realities that clinical care may often overlook. Although CFTR modulators have improved outcomes (Boehm-Cohen and Shteinberg, 2025), their introduction has not resolved the psychological uncertainty surrounding pregnancy, longevity and treatment outcomes. Notably, several included studies were conducted before widespread access to CFTR modulators, likely shaping some participants perceptions of risk and possibility. Despite these significant advances in treatment, current care models remain primarily guided by biomedical indicators of readiness (Hailey et al., 2019). While these indicators are important, they risk overshadowing the emotional, relational, and moral considerations that are central to the reproductive journey in this population.

By centring on the lived experiences of PwCF, this review brings into focus the psychological labour involved in negotiating parenthood, labour that includes managing ambivalence, grief and shifting identity. The dominant clinical framing of risk does not fully capture the moral responsibility, emotional toll and hope invested in these decisions. These findings align with previous research highlighting that the decision to have a child often originates from a common desire to become a parent and is further guided by a sense of moral responsibility and the need for a solid partnership and collaborative network (Vanhollebeke and Van Steijvoort, 2025).

Importantly, the review exposes the adverse emotional experiences caused by delayed or inconsistent fertility information. Participants’ accounts suggest that discovering infertility late, or receiving conflicting messages from different providers, can have lasting psychological consequences, reinforcing the importance of early, honest and psychologically sensitive fertility discussions within chronic illness care (Igerc and Schrems, 2023). Another key insight is how participants redefine wellness, not in terms of clinical outcomes alone, but through emotional fulfilment, meaningful connection and the desire to be present. This challenges prevailing notions of what it means to be “well” in CF and suggests a broader, more person-defined concept of wellness that care teams may benefit from adopting.

Participants frequently described feeling judged, discouraged, or denied agency in their decisions, often encountering gatekeeping by clinicians, underscoring persistent barriers to reproductive autonomy. Viewing these findings through a reproductive justice lens emphasises the need for care that upholds autonomy, dignity and equitable access to non-judgemental support, irrespective of an individual’s health status (Hassan et al., 2023).

In response to these insights, this review advocates for a shift towards emotionally attuned, person-centred models of reproductive care. This includes integrating psychological support into routine care, validating emotional ambivalence, facilitating peer connection and improving coordination across CF and maternity services. As more people with CF consider or become parents, supporting their journey calls for care that recognises not just what is medically possible, but what is emotionally meaningful and ethically grounded.

Methodological issues

This review has several key strengths. It was conducted in line with best practice for qualitative evidence synthesis, enhancing its credibility, transparency and comprehensiveness. The review was pre-registered with PROSPERO, ensuring a systematic approach to the process (van der Braak et al., 2023) and adheres to the ENTREQ guidelines (Tong et al., 2012). It draws on data from 326 participants, offering broader insights than individual studies. Methodological rigour was supported by source and analytical triangulation (Walsh and Downe, 2005), a robust search strategy guided by the PRESS checklist (McGowan et al., 2016) and clear focus with the PICo framework (Hosseini et al., 2024). Broad inclusion criteria captured a range of perspectives, while quality appraisal tools like CASP and AACODS ensured systematic evaluation of study quality (Shaheen et al., 2023).

However, some limitations must be acknowledged. Excluding quantitative studies may limit the scope of the review, and although thematic synthesis is an appropriate approach, it inevitably involves a degree of subjectivity (Braun and Clarke, 2023). We can also not exclude the possibility of potential bias in interpretations from original study authors. The included studies were non-homogenous as they had somewhat different research questions and aims. Limiting the review to English language publications introduced potential language bias and every effort was made to contact authors of conference abstracts, however, there were still a small number of included studies with missing information. A further limitation relates to the analytic focus. Although gender may shape experiences of pregnancy and parenthood, the review did not undertake a sex/gender-based comparative analysis. Recent clinical developments suggest increasing rates of pregnancy among younger PwCF in the context of HEMP. However, despite a broad age range (19–62), the included studies did not capture teenage pregnancies and may not fully represent the experiences of adolescents navigating pregnancy in CF.

Future research

This review reveals the emotional complexity and psychological labour involved in becoming a parent with CF, highlighting areas where further inquiry is needed. Future research would benefit PwCF by exploring how PwCF experience and manage emotional ambivalence, particularly the interplay of hope, fear and anticipatory grief, as they navigate the journey to parenthood. There is scope for deeper inquiry into the psychological impact of delayed or conflicting fertility information, especially around identity, loss and medical trust. It is important to examine how individuals balance the dual roles of patient and parent, including the emotional toll of treatment prioritisation and guilt around caregiving. Qualitative research that captures how people redefine psychological well-being through connection, meaning and parenting goals would deepen our understanding of how CF care could better support these transitions. Future researchers may consider how stigma, disempowerment, and constrained autonomy are experienced in clinical encounters and how person-centred, emotionally attuned communication could be improved across CF and maternity services. Finally, given the vastly different experiences of fertility difficulties based on gender, especially when considering HEMT, it would be interesting to explore the psychological impact pre and post ETI, and also how the different genders have been impacted pre and post ETI.

Implications for clinical practice

Several implications for practice in supporting the psychological well-being of PwCF may be derived from the findings of this systematic review. It may be helpful for care teams to initiate regular, proactive conversations about reproductive goals from adolescence, ensuring patients are informed and emotionally supported. Fertility education needs to be clear, consistent and delivered sensitively to both men and women, acknowledging the emotional impact of infertility and assisted reproduction. Integrated psychological support can play a valuable role in helping PwCF navigate identity shifts, parenting stress and anticipatory grief. Peer support groups may provide meaningful shared experiences and insights that compliment clinical care and clinicians can help foster patient autonomy in reproductive decisions. As treatments evolve, particularly with the introduction of CFTR modulators, care pathways could be enhanced by adapting to reflect new possibilities and emerging hopes.

Conclusion

This meta-synthesis offers a nuanced understanding of the psychological experiences of PwCF as they navigate pregnancy and parenthood. It reveals that despite medical advances, current care models often overlook the emotional labour, identity challenges and autonomy struggles that shape reproductive journeys. By centring the voices of PwCF, this synthesis highlights the value of care that moves beyond biomedical readiness to embrace person-centred, emotionally responsive support. A reproductive justice lens further underscores the importance of dignity, autonomy and equitable access to informed, non-judgemental care. As reproductive possibilities expand, CF care has an opportunity to evolve in ways that reflect not only what is clinically possible, but what is emotionally meaningful, ethically grounded, and defined by those living it.

Supplemental Material

sj-docx-1-hpq-10.1177_13591053261446886 – Supplemental material for Psychological well-being in adults with cystic fibrosis during pregnancy and parenthood: A systematic review

Supplemental material, sj-docx-1-hpq-10.1177_13591053261446886 for Psychological well-being in adults with cystic fibrosis during pregnancy and parenthood: A systematic review by Selina Braddish, Sharon Houghton, Eimear Ruane-McAteer and Donal G. Fortune in Journal of Health Psychology