Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study

Abstract

Aims:

Explore the knowledge, experiences, preferences, and concerns related to fertility preservation as an option for building a biological family among women with cystic fibrosis.

Design:

Convergent mixed methods study design.

Methods:

We recruited women with cystic fibrosis of childbearing age in the United States through cystic fibrosis centres, snowball sampling, and social media. Participants completed an anonymous survey about fertility and fertility preservation (n = 50). We also conducted audio-recorded, semi-structured interviews with a subset of women to gain a better understanding of their perspectives (n = 20). We transcribed the interviews verbatim and analysed them using thematic analysis.

Results:

For the quantitative arm, 78% of women indicated that they would like to have a child in the future; however, 74% reported never having had conversations about fertility preservation with their providers. For the qualitative arm, four major themes emerged: (1) Women with cystic fibrosis have inadequate knowledge about fertility and fertility preservation; (2) fertility is a low priority area for the cystic fibrosis care team; (3) women with cystic fibrosis recommend that the cystic fibrosis care team provide specific fertility resources; and (4) providers and literature lack information on fertility and cystic fibrosis. Integrated findings identified that while the majority of women with cystic fibrosis want to become mothers in the future, including post-lung transplantation, they have not received education on fertility preservation, and there is a general lack of knowledge on the topic of fertility in cystic fibrosis.

Conclusion:

Women with cystic fibrosis desire to have children but have little knowledge about fertility preservation, and cystic fibrosis providers do not initiate family planning discussions.

Impact:

Findings from the study support that additional education is needed for women with cystic fibrosis who are considering parenthood. Clinical care models should include early, regular, and thoughtful discussions about reproductive health issues, including fertility preservation.

1 |. INTRODUCTION

Cystic fibrosis (CF) is a multi-system disease that not only affects the lungs but also the reproductive tract (Cystic Fibrosis Foundation [CFF], 2021a). Although accurate data are neither currently collected nor available, the literature estimates that approximately 35% of women with CF have subfertility or altered fertility (Shteinberg et al., 2019). Discussions about sexual and reproductive health between CF care providers and women with CF are generally lacking (Kazmerski et al., 2018). For women with CF with advanced lung disease requiring lung transplantation to prolong life and improve quality of life, discussions do not occur about fertility preservation (FP) to ensure an opportunity for creating biological families post-transplantation (Ladores et al., 2018). Thus, current clinical practice in CF must be updated to accommodate the patient population’s emerging needs to ensure delivery of holistic, comprehensive care.

1.1 |. Background

Due to advances in early diagnosis, clinical care, and drug breakthroughs, CF is no longer considered to be a ‘disease of childhood.’ Over half of the CF patient population are now adults (CFF, 2021b) and achieving developmental milestones previously not possible, including parenthood. CF does not currently have a cure, thus, most, if not all, individuals with CF will require lung transplantation to save or prolong their lives (Clausen & Hadjiliadis, 2021). With lung transplantation techniques and clinical outcomes continually improving, adults with CF may want to explore opportunities to create families post-transplantation, given their ‘second lease on life.’ However, this group does not receive information about FP as a reproductive option before undergoing transplantation (Ladores et al., 2018). Immunosuppressive therapy post-transplantation may be gonadotoxic and teratogenic, subsequently, rendering the use of fresh sperm or eggs post-transplantation inadvisable (DeFilippis et al., 2020; DePinho & Sauer, 2014; Hirschfield-Cytron et al., 2011). A recent case report highlighted one couple’s devastation on learning that they missed an opportunity to have biological children because of a lack of FP counselling provided by their CF care team pre-lung transplantation (Ladores et al., 2018). In contrast, oncology patients of childbearing age receive FP counselling as part of comprehensive care before undergoing treatments that may impair their reproductive capacity (Loren et al., 2013). Oncofertility programme practice guidelines clearly delineate the timeline for FP planning, and research indicates that FP should be introduced as early as possible to facilitate the greatest potential for reproductive capacity (Oktay et al., 2018). This FP care delivery model is not currently established in the transplant patient population. Therefore, the purpose of this study was to investigate the current state of FP counselling for women with CF and gain a better understanding of their knowledge, experiences, concerns, and preferences.

1.2 |. Conceptual framework

This study was guided by the principles of design thinking which is defined as an analytic and creative process that engages a person in opportunities to experiment, create and prototype models, gather feedback, and redesign (Kelley & Kelley, 2013). Design Thinking originated from product development and the business community; however, its utility in health care has increased in popularity (Bernstein, 2015; Roberts et al., 2016; Seidel & Fixson, 2013). Rapid changes and advancements in health discoveries and health care have led to complexities wherein the patient experience gets ‘lost in the maze’. Design Thinking facilitates a return to keeping the patient at the front and centre of health care (Wingo et al., 2020). For biomedical research purposes, design thinking is a 5-step (empathize, define, ideate, prototype, and test), creative problem-solving approach that uses the scientist’s sensibility and an established practical methodology to meet patient needs through innovations in clinical approaches (Kelley & Kelley, 2013). For this study, we used the first three design thinking steps (empathize, define and ideate), wherein the successful creation and translation of innovative ideas rely on the ability to empathize with others to understand problems from another’s point of view, followed by defining the problem and ideating for potential solutions. The primary focus of this paper is to describe the operationalization of empathize through data collection, followed by define through sharing of study results, and finally, a preliminary ideation of solutions through the discussion of next steps.

2 |. THE STUDY

2.1 |. Aims

The purpose of this study was to investigate the current state of FP counselling for women with CF using four main constructs: knowledge, experiences, preferences, and concerns.

2.2 |. Design

We used a convergent mixed methods research design to explore the current state of FP counselling for women with CF. The quantitative strand examined patient knowledge while the qualitative strand explored patient experiences, preferences, and concerns. Reporting of quantitative and qualitative results follow the STROBE and COREQ checklists, respectively, to ensure quality and comprehensiveness.

2.3 |. Sample/Participants

For this mixed methods study, we recruited 50 women with CF ages of 18–35 who had not had a lung transplant and can understand and speak English, from across the United States. Participants were recruited from July 2019 to March 2020 by posting IRB-approved flyers in several CF Foundation-accredited CF Centres, sharing study information via the North American CF Conference’s mobile app, and using a snowball sampling strategy. To address potential bias, all CF Foundation-accredited clinics were invited to participate in the study. Additionally, in-clinic recruitment visits at a single CF centre were done by research staff rather than clinic staff to avoid the potential for coercion.

2.4 |. Data collection

The first step of design thinking, empathize, was operationalized by eliciting the participants’ perspectives through both quantitative (via cross-sectional survey) and qualitative (via one-on-one interviews) arms of this mixed methods study.

2.4.1 |. Quantitative arm–survey

Participants were asked to complete an anonymous online survey. The survey took approximately 30 min to complete and included demographic items, a self-assessed knowledge scale, and exploratory questions related to FP experiences, preferences, and concerns to gain an understanding of the current state of FP counselling for women with CF. The knowledge scale is titled, ‘Knowledge of Fertility Preservation in Women with CF Instrument’. The instrument was developed by the research team based on a thorough literature review, previous qualitative work, and modification of an existing instrument that assessed FP knowledge in women with cancer (Jukkala et al., 2012). Pre-testing was completed with 12 diverse stakeholders, including women with CF, partners of women with CF, CF providers, PhD-prepared nurses, PhD students, and undergraduate Honours students. Full description of the FP knowledge instrument development and psychometric testing procedures are described elsewhere (Corcoran et al., 2020, 2021). The instrument is a 10-item scale that measures self-assessed fertility and FP knowledge (Corcoran et al., 2020). The instrument is scored from 10 to 40 with higher scores indicating greater knowledge. Additionally, participants completed a one-item satisfaction measure asking them to rank how satisfied they were with the FP discussions with their CF healthcare team. Women who participated in the survey were compensated $20 for their time.

A total of 56 women who were screened and met the eligibility criteria accessed the online survey. Six of these women did not complete the survey, leaving a total of 50 women who completed the survey. We hypothesize that the individuals who did not complete the survey may have stopped when they arrived at items that required multiple drop-down choices and ranking. Recruitment stopped once 50 women completed the survey as this sample size was pre-determined based on the pilot nature of the study. Construct validity of the FP knowledge instrument was assessed using confirmatory factor analysis, wherein the model fit was evaluated using standardized root mean square residual, root mean square error of approximation, and comparative fit index. Cronbach’s alpha was used to examine internal consistency reliability. The criterion validity was assessed using inferential statistics.

2.4.2 |. Qualitative arm-interviews

At the end of the survey, participants were asked if they would be interested in completing a separate telephone or in-person interview as part of the study. We contacted those who indicated they were interested, and if they agreed, they completed an interview with the first author who is a PhD-prepared nurse scientist with extensive experience in qualitative research.

The interview guide included questions such as: (1) Do you feel you have enough knowledge or information about fertility and fertility preservation? (2) Has anyone on the CF care team discussed fertility and fertility preservation with you? (3) Have you talked with anyone about options for having a baby after transplant? The interview guide was modified several times throughout the data collection phase to better elicit rich descriptive narratives from one participant to the next and to promote continuous quality improvement. Interview questions were assessed for clarity, readability and flow by members of the research team before the first interview. The interview guide was subsequently reviewed, edited and approved by two women with CF to ensure that all questions were relevant and appropriate.

Twenty participants completed the interviews, which ranged from 25 to 90 min. No participants elected to withdraw once the interviews began. The interviews were audio recorded and transcribed verbatim by a professional transcriptionist service. The interview participants were compensated an additional $20 for their time.

2.5 |. Ethical considerations

The study received institutional review board approval prior to study initiation.

2.6 |. Data analysis

2.6.1 |. Quantitative arm

From the survey data, descriptive statistics were reported as mean (standard deviation) or proportion to describe the patient demographic data as well as describe the participants’ experiences, preferences, and concerns about FP discussions. Spearman’s correlation coefficient (ρ) was used to assess the strength of the correlation between the FP knowledge scores and scores on FP discussion satisfaction with the CF care team. Independent Welch’s t tests were used to evaluate group differences in FP knowledge scores. The normality assumption was evaluated with Q-Q plots. All statistical analyses were conducted using R software (R Core Team, 2019). There was no missing data in this dataset.

2.6.2 |. Qualitative arm

Notes were taken during the interview process to capture initial ideas expressed by the participants. Transcriptions were uploaded into NVivo and coded via thematic analysis (Nowell et al., 2017). After reading through the transcripts for familiarization, initial codes were generated using a line-by-line coding procedure. These codes were then collapsed into broader themes. To establish rigour, two coders coded the data separately and then met to review the coding schema, with differences discussed until a consensus was reached (Nowell et al., 2017). Data saturation was reached at Participant #18 (out of 20); however, we continued to 20 to ensure that no other new data emerged from the interviews.

3 |. RESULTS/FINDINGS

3.1 |. Quantitative

Psychometric testing of the FP knowledge instrument demonstrated that it was a valid and reliable instrument. The confirmatory factor analysis with two subscales ‘General Fertility Knowledge’ and ‘Transplant-related Fertility Knowledge’ demonstrated a good fit with standardized root mean residual of 0.07, root mean square error of approximation of 0.06, and comparative fit index of 0.97, indicating good construct validity of the instrument. This instrument demonstrated good internal consistency reliability with Cronbach alpha’s of 0.91 for the ‘General Fertility Knowledge’ subscale, and 0.64 for the ‘Transplant-related Fertility Knowledge’ subscale. Women who reported a pregnancy scored 24.27 (6.69), which was significantly higher than the score of 19.4 (5.57) for women who did not report a pregnancy (t₂₃ = 2.47; p = .02) suggesting criterion validity.

Of the 50 women who completed the survey, 70% were between the ages of 26 and 35, with an average age of 28. Most women reported being in a relationship (86%) and the majority were not mothers (78%). Additional descriptive information is included in Table 1. Overall, the mean score of 20.86 (6.27) on the FP knowledge instrument indicated that women had little knowledge about FP options, with 74% reported never having had conversations about FP with their healthcare providers. Furthermore, 34% reported that they had never discussed options for parenthood with anyone, although 78% indicated that they would like to have a child in the future. There was a moderate correlation between FP knowledge score and FP discussion satisfaction score (ρ = 0.44, t₄₈ = 3.27; p < .01). Women with CF who reported that they had discussed options for FP with someone scored 24.31 (7.22) on the FP knowledge instrument, which was significantly higher than the score of 19.65 (5.52) for women who had not discussed FP options with anyone (t₁₇ = 2.12; p < .05). Additionally, women who were married scored 24.22 (6.76) on the FP knowledge instrument, which was significantly higher than the score of 17.94 (4.14) for women who were not married (t₃₅ = 3.84; p < .01).

TABLE 1.

Characteristics of study sample

Characteristics	Participants (N = 50)
	n	%
Age
18-21	3	6
22–25	12	24
26-30	19	38
31-35	16	32
Race
White	49	98
American Indian or Alaskan native	1	2
Education
Some high school or less	0	0
High school graduate or GED	4	8
Some college	16	32
Vocational school	2	4
College degree	22	44
Professional or graduate degree	6	12
Relationship status
Single/never married	17	34
Living with partner	7	14
Married	23	46
Divorced	3	6
Region
Northeast	12	24
Mid-Atlantic	2	4
Southeast	25	50
Northwest	4	8
Midwest	3	6
Southwest	4	8
Self-assessed health status
Excellent	7	14
Very good	17	34
Good	13	26
Fair	13	26
Poor	0	0
Have an OB/GYN
Yes	38	76
No	12	24
Parent through pregnancy
Yes	11	22
No	39	78
Ever experienced a pregnancy
Yes	15	30
Naturally	9	60
Required medical help	6	40
No	35	70

3.2 |. Qualitative

Four themes emerged from the interviews with women: (1) Women with CF have inadequate knowledge about fertility and FP; (2) fertility is a low priority area for the CF care team; (3) women with CF recommend that the CF care team provide specific fertility resources; and (4) providers and literature lack information pertinent to fertility and CF.

3.2.1 |. Theme 1: Women with CF have inadequate knowledge about fertility and FP

Eighteen out of 20 women reported a lack of knowledge about FP as well as limited knowledge about the intersection of CF and fertility. Patient 9 shared, ‘I don’t have very much knowledge about it [fertility and FP] especially as it relates to CF…’ Many women felt they were somewhat knowledgeable about general female fertility; however, they reported that there was a difference between that general knowledge and CF-specific knowledge.

Many times, the lack of knowledge resulted in women looking for information themselves: ‘I did not feel there was a lot of information about fertility and CF. I was doing a lot of Googling and a lot of asking other women with CF about [their] experiences’ (Patient 5). Likewise, Patient 10 felt the need to look for information herself, as she had no one to guide her: ‘I had to do a lot of my own research and figuring out stuff myself. It wasn’t really given to us [by CF care team].’

Additionally, the concept of FP was poorly understood by most women. Patient 6 shared: ‘I would say my pretty limited knowledge, I would describe that as taking some sort of extraordinary measure to preserve either an egg or eggs…’ Oftentimes, participants had heard of FP but were unsure of the specifics of the process and why it could be pertinent to them: ‘I know I’ve heard it mentioned, but—I don’t think I know totally what it means’ (Patient 8).

Subtheme: Learning from others (social media).

A subtheme that emerged from this first major theme was related to where the women obtained their information about fertility and FP. Eighteen out of 20 women shared that in the absence of information from CF providers and the scientific literature, they sought out information from other women with CF, primarily through social media. Patient 1 explained: ‘I had already joined other women’s [social media] groups that involved pregnancy…so I asked them a lot of questions …I read through previous posts that other women may have had.’ Patient 5 reported that she first talked to her CF care team about fertility, and their response indicated she would not have any difficulty getting pregnant. However, on joining social media groups, she realized that what the CF care team told her may not be true: ‘Then as I started talking to other women about their experiences…I began to realize that there are women out there who have CF and have fertility struggles.’

3.2.2 |. Theme 2: Fertility is a low priority area for the CF care team

Seventeen women observed that fertility was not the primary concern of the CF care team. Patient 2 explained: ‘They [CF care team] just look at me as, ‘Okay, your lung function’s this, if you’re sick right now, I want to give you an antibiotic,’ they don’t see me… as somebody who wants to be a mother, somebody who wants to have a family…’ Additionally, Patient 6 reported that the expertise of the CF care team limits discussions of fertility or sexual and reproductive health: ‘They [CF care team] are specialized so much in maintaining lung function and studying the bacteria that grows in our lungs… I think with the fertility portion, while it should be part of the conversation, that’s just another piece that isn’t talked about enough…’ Patient 7 echoed the same sentiments about her CF care team: ‘They’re more passionate about figuring out the whole realm of just the lung and digestive issues… and not reproductive issues.’

The recognition that fertility is a low priority was so evident to women that some felt uncomfortable bringing the topic up to their CF care team: ‘You [the patient] feel like an inconvenience, ‘Oh, whoops, I don’t want to step on your toes, but I’d like to have babies. I know my lungs are okay, but can I have a kid?’ (Patient 15). Women perceived a significant disconnect between the care provided by the CF care team and anything related to reproductive health. This disconnect resulted in Patient 19’s decision to have minimal interaction with the CF care team when she decided to pursue motherhood: ‘If and when I get pregnant, they [CF care team] will work on my lungs and I will go see my OB-GYN for everything else.’

Subtheme: Normalize desire to have a family or discuss sexual and reproductive health issues.

Nine women expressed their desire for the CF care team to recognize that prognosis and management of CF have changed over time; therefore, they wished the CF care team would normalize discussions of reproductive health or desire to have a family like similarly aged peers without CF. Patient 2 shared her negative experience of being seen by a doctor who did not understand or acknowledge her wishes: ‘At first, my doctor was dumbfounded as to why I would want children…’ Part of this misunderstanding may stem from the perspective of providers who are primarily concerned with the pulmonary system rather than the whole body and person. Patient 6 expressed her desire for the CF care team to view patients more holistically: ‘Looking at the whole person with CF as—we are normal people with… with hopes and dreams, and…fertility.’ This desire to feel ‘normal’ was echoed by several women. Patient 7 stated, ‘As CF patients, we also try to live the most normal life…We don’t constantly want CF to take over our life.’

3.2.3 |. Theme 3: Women with CF recommend that the CF care team provide specific fertility resources

Nineteen women wanted the CF care team to provide specific fertility resources to empower patients to make well-informed decisions about family planning. They presented two main recommendations: (1) Adding a women’s health provider to the CF care team, and (2) providing topic-specific resources and information during clinic appointments. Additionally, a subtheme emerged from this category about the preference that the CF care team initiate fertility discussions.

Women’s health care provider on team.

Women overwhelmingly recommended having a women’s health specialist be part of the CF care team. The lack of structured collaborative practice prevented several women from receiving ‘holistic’ seamless care. Patient 9 shared her experience related to this issue: ‘I had to go to the gynecologist for—because I’m taking a lot of antibiotics, I have a lot more yeast infections. They [gynecologists] just treat me like, ‘Oh, you’ve got chronic yeast infections’, or they say, ‘You have a lot of them.’ If there was a connection between the CF team and an OB/GYN, then they could share the information and work together with that.’ Participants also emphasized that they may have different reproductive needs than other non-CF women and that ‘average’ women’s health providers lack specific knowledge and training. Patient 18 shared an embarrassing experience of visiting an OB/GYN who was unfamiliar with CF disease processes: ‘I distinctly remember starting to see OB-GYN as a teenager and they just are not familiar with CF. One of the whole things with CF is like the thickened cervical mucus. I would go to the OB and they would just be like, “What is going on?” It was very embarrassing…I just remember them saying like, “There’s just so much fluid here and it’s not normal. I’m not really sure what’s goin’ on.”’.

More information and resources.

Women also strongly advocated for having information and resources available during clinic appointments to enhance their ability in making informed decisions. Patient 5 stated, ‘What’s most important to me is just having more information available to women with CF …as they’re trying to conceive.’ Lack of knowledge on the part of patients and lack of available information in general related to fertility and FP contributed to a major source of stress for the women: ‘I wish that they [CF care team] were more prepared to provide me information that I would need to know or maybe even ask me some questions, get me thinking more about it and be comfortable with it’ (Patient 15).

Subtheme: Bringing up the topic of fertility and FP.

Thirteen women desired for the topic of fertility and FP to be discussed regularly with their CF care team and to be initiated by the team members. Several advocated for the topic to be brought up regularly, even if it had been addressed previously: ‘Bring it [topic of fertility and FP] up every now and again. Just because I have a decision this year doesn’t mean in two years, I’m gonna have the same decision…Just keeping that conversation open and [have the CF care team] bring it up again’ (Patient 14). Additionally, having a standardized procedure in place to guide discussions was deemed helpful: ‘I feel like just a baseline assessment…would be really helpful for any woman with CF…The question of, “Are you [the patient with CF] interested in having children?” If the answer is yes, there is a protocol’ (Patient 16). The request for a standardized procedure to follow in CF clinic was echoed by several participants, who posited that having one would enable both providers and patients to know what and when to expect the conversations, subsequently making the process feel less awkward and more comfortable.

3.2.4 |. Theme 4: Providers and literature lack information on fertility and CF

Sixteen participants expressed concerns that neither the CF care team nor the literature have current information about CF fertility. One shared: ‘The information isn’t generally known by CF team or even literature’ (Patient 3). Several reported negative experiences when they attempted to gather more information from their providers. Patient 6 shared her discouraging experience: ‘If you [the patient] talk to your doctor, they say, ‘Well, there’s no data.’ That was extremely difficult.’

Subtheme: Lack of knowledge surrounding CF modulator therapy and pregnancy/breastfeeding.

Eleven participants reported concerns about the lack of empirical evidence on the safety of using a group of new CF precision medications called ‘modulators’ during pregnancy or breastfeeding. Patient 1 described her CF care team’s hesitancy to start her on modulators because of the unknown fetal effects: ‘My doctors consider me not a candidate for the modulators because …[the CF care team is] not sure what’s going to happen to the baby if [I] take them…They don’t want me to have to be that science experiment.’ Currently, women have to balance their concerns about the child’s well-being in utero -or when breastfeeding-with their current health to decide whether or not to remain on the modulators. Patient 6 expressed the anxiety of this decision: ‘That was probably my most difficult parenting experience with trying to weigh out what’s best for me. What do I want to do as a mom? What do I want for my son?’.

3.3 |. Integrated findings (define step of design thinking)

Using the convergent mixed methods approach, the quantitative and qualitative results were merged to provide both general trends in knowledge (quantitative strand) and personal perspectives (qualitative strand) surrounding FP counselling in women with CF (Creswell, 2015). The two types of data were merged and compared with identify areas of complimentarity, convergence, and divergence between the two data types (Curry & Nunez-Smith, 2015). Both the quantitative and qualitative arms of this study revealed that women with CF have a profound lack of knowledge, negative experiences with their CF care team, and a list of preferences and concerns related to fertility and FP. The qualitative arm demonstrated that this lack of knowledge expanded to concern over the lack of information and knowledge from both providers and literature related to fertility and FP for women with CF. Both arms of the study found that many women with CF are currently mothers or are interested in becoming mothers even post-transplantation; therefore, education and counselling on FP is relevant and beneficial for women with CF to create biological families in the future. Lastly, while the majority of women who participated in the survey reported having an OB-GYN, the qualitative results showed that women recommended having a women’s health provider embedded as part of the CF care team to provide more specialized care and resources to women with CF.

4 |. DISCUSSION

Women with CF have minimal knowledge of fertility and FP concepts, due in part to the CF care team’s lack of prioritization in discussing these topics in clinic (Ladores et al., 2020). Despite most women with CF wanting to have a child in the future, over 70% had never discussed FP with their healthcare providers. Due to a lack of formal education or educational materials to answer questions about fertility and FP in CF, many women turned to other informal options, such as other women with CF and social media. Acquiring this information from these informal sources could be beneficial as a support mechanism but could also lead to sharing inaccurate or misleading information. Additionally, these women desired for their providers to bring up and discuss fertility and FP with them but with the current care model, there is no women’s healthcare provider embedded as part of the CF care team.

These findings extend what is already known about the lack of attention spent on the reproductive health needs of individuals with CF (Kazmerski et al., 2018). Fertility preservation counselling is a focused topic that should be integrated into patient-provider discussions especially for patients whose lung function are steadily declining and lung transplantation is imminent. Findings from this CF group also support the need to expand FP counselling beyond the oncology patient population. For example, in patients with rheumatoid arthritis wherein the disease process and treatment modalities affect fertility and reproductive health, there is an emerging awareness of FP as an option to create biological families (Brouwer et al., 2017; Hunt & Talabi, 2019).

While there are no current FP clinical practice guidelines or resources for women with CF, several medical organizations including the American Society of Clinical Oncology have issued guidelines for care providers and caregivers of adults, adolescents, and children with cancer about FP options for people anticipating cancer treatments (Levine et al., 2015). These guidelines address several concerns identified by women with CF in this study including having care providers initiate discussions on the possibility of infertility as early as possible, as well as discussions of FP options and associated referrals, and documentation of these discussions for regular updates throughout the year (Coccia et al., 2014; Oktay et al., 2018). To date, these resources are not available for those with non-malignant conditions (Ladores et al., 2018).

The third step of design thinking, ideate, is exemplified by future development and dissemination of CF-specific educational materials for women with CF, as well as fertility and FP-specific training for CF providers. The goal would be to develop and implement FP clinical guidelines to ensure standardized assessment and care are provided to all women with CF. Long-term implications related to creating and using FP clinical guidelines include improvement in overall patient-centred care and outcomes among patients requiring transplantation and whose family planning options are affected by non-malignant conditions.

Findings from this study also highlight the need for investigating the unique reproductive health needs of men with CF which has largely been understudied (Clarke et al., 2021; Khan et al., 2020), especially considering that 98% of men with CF are infertile due to congenital absence of the vas deferens (Sawyer et al., 2005). While there has been a recent surge in interest and priority for funding reproductive health studies in women with CF as evidenced by the establishment of the CF Foundation’s Women’s Health Research Working Group (CF Foundation, n.d.), CF men’s health research has not received the same attention. Sawyer (1996; 2005) has done exploratory and descriptive work in CF men’s health, yet her contributions are now outdated given the current era of modulator therapy and included neither intervention development nor specific FP content. Findings from this study on women with CF are translatable and can serve to inform research and clinical care for men with CF.

In summary, we used design thinking as a framework to guide this study. We empathized (connected with participants through surveys and interviews), defined (identified problems through results of our data analyses), and ideated (recommended possible solutions through discussions of next steps) using this conceptual framework. Using the findings from this study, future research should be guided by the last two steps of design thinking (prototype and test) to create, implement and evaluate interventions for women with CF to improve their understanding and use of FP services in planning their families.

4.1 |. Limitations

Limitations of the study included: (1) recruitment of women in the United States only, and (2) use of snowball sampling of those willing to participate, both of which can limit generalizability of the study findings. Despite these limitations, this study illustrates that future research should explore interventional strategies to adequately prepare women with CF for family planning.

5 |. CONCLUSION

Standards of care for women with CF should reflect current patient demographics because women with CF are now expected to reach adulthood and achieve developmental milestones similar to their peers without CF. Additional research on fertility and reproductive health is warranted to develop clinical practice guidelines that comprehensively meet the needs of this patient population.

Summary Statement.

This mixed methods study is the first to explore the current state of fertility preservation in women with cystic fibrosis. Findings can be used to identify gaps in clinical care and inform the development of interventions that meet the unique needs of this patient population.

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.