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Cystic fibrosis
Assessing the health impacts of parenthood on people with cystic fibrosis: the HOPeCF prospective cohort protocol
  1. Traci M Kazmerski1,2,
  2. Olivia M Stransky1,2,
  3. MinJae Lee3,
  4. Kelly A Prangley1,2,
  5. Raksha Jain4,
  6. Anna M Georgiopoulos5,
  7. Daniel S Shaw6 and
  8. Jennifer Taylor-Cousar7
  1. 1Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  2. 2Center for Innovative Research on Gender Health Equity (CONVERGE), University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  3. 3University of Texas Southwestern Medical Center, Dallas, Texas, USA
  4. 4Department of Medicine, University of Texas Southwestern, Dallas, Texas, USA
  5. 5Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts, USA
  6. 6Department of Psychology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  7. 7Internal Medicine and Pediatrics, National Jewish Health, Denver, Colorado, USA
  1. Correspondence to Dr Traci M Kazmerski; traci.kazmerski{at}chp.edu

Abstract

Introduction People with cystic fibrosis (CF) are living longer and healthier lives with a growing number considering and pursuing parenthood. The decision of whether to become a parent is complex for people with CF, and CF is a major factor in reproductive decision-making. Unfortunately, in people with CF who become parents, there are no prospective studies of disease trajectory, no data on the impact of parenthood on mental health, disease self-management, or quality of life, and no research regarding non-genetic parenthood.

Methods and analysis Health Outcomes of Parents with CF (HOPeCF) is a prospective, multicentre observational cohort study which will enrol 146 new parents with CF of children less than 5 years of age. The primary aim of this 60-month study is to assess the rate of lung function decline as impacted by mental health, parental stress and responsibility, and the use of CF transmembrane conductance regulator modulators. In addition, we will conduct dyadic interviews with a subset of study participants and their key supports (partner/family/friend) to inform future interventions.

Ethics and dissemination This longitudinal, observational multicentre study is a necessary and timely step in understanding parental health outcomes in CF and will provide data essential for care guidance to people with CF, their partners, and healthcare providers. The University of Pittsburgh Institutional Review Board approved this study (STUDY23080161). As people with a variety of paediatric-onset chronic diseases are living longer and considering parenthood, these results may have widespread applicability and will be distributed at international meetings and submitted to peer-reviewed journals.

  • Cystic Fibrosis

Data availability statement

Data sharing not applicable as no datasets generated and/or analysed for this study.

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/bmjresp-2024-002383

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    WHAT IS ALREADY KNOWN ON THIS TOPIC

    • Parenthood adversely impacts short-term cystic fibrosis (CF) health outcomes and CF modulator use may mitigate such effects. However, prior research is limited in its follow-up duration, did not include widespread highly effective CF modulator use, did not measure key mental health or psychosocial outcomes, and did not include all paths to parenthood.

    WHAT THIS STUDY ADDS

    • The Health Outcomes of Parents with CF (HOPeCF) study is the first, prospective, multicentre study in CF to assess the comprehensive health impact of parenthood on people with CF in the era of CF modulators. It is the first, large-scale prospective epidemiological data collection of how becoming a parent intersects with having a chronic disease.

    HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY

    • The results of the HOPeCF study will provide clear evidence of the comprehensive health impacts of parenthood on all people with CF and the potential protective effects of CF modulators to support clinical guidance. Importantly, it responds to the research priorities of the CF community as it will enable people with CF to make informed decisions about their reproductive futures.

    Introduction

    Due to advances in therapy and management, people with cystic fibrosis (CF) are now living into their fifth decade of life and beyond, with a growing number considering and pursuing parenthood.1 2 Approximately four of five people with CF desire children.3 4 Since the widespread use of highly effective CF transmembrane conductance regulator (CFTR) modulators (a class of drugs that addresses the basic defect in CF5), the US CF Foundation (CFF) Patient Registry report demonstrated a near-doubling of pregnancies in people with CF from 310 in 2019 to 634 in 2022 (figure 1).1 However, the decision of whether to become a parent is complex for people with CF, and CF is a major factor in reproductive decision-making.2 6 People with CF express concerns around balancing roles as parent and patient, communicating with children about CF and the impact of their health on children.7 8 As more people with CF live through their reproductive years, a greater understanding of the health impacts of parenthood on those with CF is critical to inform their reproductive decisions and to provide effective interventions for those who decide to become parents.

    Figure 1
    Figure 1

    Pregnancies among people with CF in the US CFFPR, 2018–2022

    In the general population, current literature suggests that parenthood is associated with parental longevity and health.9 10 However, there is little consensus on this association’s direction. Potential mechanisms by which parenthood may influence mortality include evolutionary, biomedical and sociological constructs. Evolutionary perspectives theorise that parity accelerates ageing due to higher investments in fertility than maintenance of one’s own health.11 Biomedical perspectives focus on the indirect physical and mental health impacts of parenthood. Many studies demonstrate that female hormonal changes associated with pregnancy, childbirth and lactation are associated with increased risks of cancer, diabetes, depression, infection and cardiovascular disease.12–14 Children may be a source of stress leading to a variety of health morbidities.15 However, parenthood may also have positive impacts via child-motivated increases in healthy behaviours.16 17 Sociological perspectives often highlight a positive association between parenthood and health via improved support networks,18 strengthening of social control via parents’ heightened sense of responsibility and commitment19 and increased joy and fulfilment.20 However, parenthood may result in longitudinal declines in marital satisfaction21 and shifts in social support and roles that may lead to increased isolation and stress.22 Furthermore, parental depression and anxiety are well-recognised perinatal complications that can become persistent, leading to diminished self-care, physical health and quality of life.11

    In people with CF who become parents, there are no prospective studies of disease trajectory, no data on the impact of parenthood on mental health, disease self-management, or quality of life, and no research regarding non-genetic parenthood (eg, adoption, fostering and step-parenting). The limited prior studies among fathers with CF suggest possible adverse impacts on health outcomes, particularly those with low baseline health.23 24 Historically, long-term survival and respiratory trends may not be negatively impacted by pregnancy among those with CF with high baseline lung function.25 However, the first year postdelivery is a time of extreme stress and hormonal shifts and has been associated with loss in body mass index (BMI) and per cent predicted forced expiratory volume in one second (ppFEV1) below prepregnancy baseline in the CF population.26

    We recently used data from the UK registry to demonstrate that parenthood adversely impacts short-term health outcomes for people with CF.27 Critically, we showed that these impacts could be mitigated by CFTR modulators. Since 2011, two moderately effective and two highly effective CFTR modulators have been approved for use, but their impact on parental health is largely unexplored. Prior retrospective studies do not include the widespread use of CFTR modulators or assess the longitudinal effects of parenthood on health.

    The Health Outcomes of Parents with CF (HOPeCF) study is the first, prospective, multicentre study in CF to assess the comprehensive health impact of parenthood on people with CF in the era of CFTR modulators. It is the first prospective epidemiological data collection of how becoming a parent intersects with having a chronic disease.

    Methods and analysis

    Conceptual model

    The HOPeCF study will prospectively evaluate people with CF after they become new parents to globally assess the impact of parenthood on health outcomes. Figure 2 highlights our conceptual model of the global impact of parenthood on CF health. This model highlights the known associations between quality of life, mental health and pulmonary health among those with CF.28 It also includes the role of adherence on pulmonary health outcomes and the interplay among adherence, quality of life and mental health.29 30 The model includes potential confounders (eg, sex and age) and considerations such as level of parental responsibilities/stress, social support and sleep and the impact of CF modulators. We realise that pulmonary or mental health, adherence or quality of life could all be valid primary outcomes. While several of these outcomes may modify parenthood’s effect on health and serve as targets of future intervention, we have intentionally focused on ppFEV1 because it is firmly established as a surrogate for CF survival.31 We will also examine the role of mental health outcomes and adherence to modulators as modifiers, as well as quality of life as a secondary outcome among participants.

    Figure 2
    Figure 2

    HOPeCF conceptual model

    Study design overview

    We will follow ~146 new parents of children less than 5 years of age to assess the primary outcome of ppFEV1 change. Comprehensive inclusion and exclusion criteria are described in box 1. In the anticipated rare event that both parents have CF and meet all inclusion/exclusion criteria, both will be eligible to participate. To model potential confounding/effect modification on the association between parenthood and outcomes, we will combine objective physical health data and survey assessment of mental health, parental responsibility/stress, quality of life and adherence. As noted above, a prospective approach will capture the immediate and long-term impact of the use of highly effective CFTR modulators by ~90% of US adults with CF. By combining objective health measures and participant surveys, we can comprehensively assess the psychosocial impacts of parenthood and explore the transactional interplay between the parenting role and physical and mental health. We anticipate being able to identify modifiable factors that may ameliorate negative health impacts of parenthood. We will also conduct hypothesis-generating, semistructured interviews with a subset of parents and their key supports (partner/family/friend) to inform future interventions.

    Box 1

    Inclusion and exclusion criteria

    • Written informed consent obtained from the participant.

    • Documentation of CF diagnosis with sweat/genotype analysis.

    • First-time parent to child(ren) <5 years of age within 90 days of enrollment visit.

    • Has not undergone a lung transplant prior to enrolment visit.

    • Can speak/read English or Spanish.

    We will enroll participants within 90 days of when they became a first-time parent to a child(ren) less than 5 years of age via pregnancy, partner pregnancy, adoption, surrogacy, foster care and/or step-parenthood (figure 3). Participants will complete surveys and research staff will enter objective health data at set intervals over the course of the study. In year 1, participants will complete surveys every 3 months. In years 2–5, participants will complete surveys every 6 months. The duration of participation for each participant is expected to be approximately 60 months.

    Figure 3
    Figure 3

    HOPeCF study overview and timeline. Abbreviations: S, participant-facing survey; EMR, electronic medical record

    At enrolment, each participant will complete an initial parenthood survey to assess the circumstances and details surrounding their parenthood. Participants will complete quarterly surveys during the first year of parenthood (S1–S4) and biannual surveys (S5–S12), thereafter, using the computer-based survey system on an iPad protected for infection control or via personal device or computer via emailed survey link. Surveys will include the 8-item Patient Health Questionnaire (PHQ-8) to evaluate for depression,32 the 7-item Generalised Anxiety Disorder 7-Item Scale to evaluate for anxiety,33 the 18-item Parental Stress Scale,34 the 50-item CF Questionnaire-Revised35 to evaluate health-related quality of life, the 40-item Adapted Parental Responsibility Scale,36 the 12-item Interpersonal Support Evaluation List,37 the 19-item Pittsburgh Sleep Quality Index,38 the 43-item Holmes-Rahe Stress Inventory to assess occurrence of life events associated with stress39 and the 18-item Daily Care Check-In Tool to assess barriers to treatment adherence.40 Additionally, surveys will include questions about childcare arrangements, self-reported modulator adherence and parental leave policies. We will collect select demographic data, including gender, sex assigned at birth, race/ethnicity (as proxies of the social determinants of health), marital status, health insurance coverage, education level, income, employment status and US state of residence. We piloted surveys with community partners with CF to assure readability and assess time burden. Each survey includes a combined subset of the above measures and will last ~15–30 min and are available in English and Spanish. For participants with literacy below an eighth-grade reading level, all surveys may be administered verbally by the research team. The research team will repeat the initial parenthood survey with participants for each child less than age 5 years added to their family during the duration of the study.

    The research team will collect objective study measures (associated with routine CF care during clinic visits or hospitalisations) by electronic medical record review. Study measures will include the results of pulmonary function tests, microbiology screenings, genotype screenings, liver disease status, diabetes status (including haemoglobin A1c (HgbA1c) measurements, glucose tolerance test results and insulin use), medication use, height and weight as well as the number of pulmonary exacerbations (PEx), hospitalisations, oral and intravenous antibiotic courses, and clinic visits. When applicable, we will also capture data about organ transplant and mortality. We will request CFTR modulator pharmacy refill data biannually. We will obtain record release consent from study participants for specialty pharmacies, which dispense all CFTR modulators in the USA.

    We will collect and manage study data using Research Electronic Data Capture tools hosted at the University of Pittsburgh Clinical and Translational Science Institute (UL1-TR-001857).41

    In addition, we will conduct hypothesis-generating, semistructured interviews in study years 1 and 2 with a subset of parents and their key supports (partner/family/friend) to inform future interventions. We will purposively sample a geographically diverse equal number of mothers (n=20) and fathers (n=20) with a range of disease severity and parenting pathways (pregnancy, adoption, surrogacy, fostering and stepparent) to gain a breadth of perspectives. Each participating site will recruit one mother and one father dyad. We will recruit on a ‘first come, first serve’ basis among eligible participants while adhering to the sampling strategy based on parent gender and parenting path.

    Study sites and coordination

    The HOPeCF study is sponsored by the National Institutes of Health and the CFF. It is enrolling across 18 participating sites; the University of Pittsburgh is serving as the coordinating centre. Figure 4 details the locations of the study enrolment sites.

    Figure 4
    Figure 4

    HOPeCF study sites

    Patient and public involvement

    We designed this study with feedback and review by people with CF. We reviewed study survey instruments and data collection with people with CF (see the ‘Acknowledgements’ section). People with CF also reviewed the protocol design and provided feedback and critiques. To minimise participant burden, the study allows remote data collection for survey completion. Furthermore, we intentionally chose objective study measures that aligned with routine clinical CF care in the USA, negating the need for in-person, separate study visits. The study is being conducted according to the International Conference of Harmonisation E6: Good Clinical Practice Consolidated Guidance to protect participant safety and data integrity.

    Objective and endpoints

    We hypothesise that (1) parents with CF and moderate-to-severe depression have more rapid ppFEV1 decline versus those with mild or no depression; (2) parents with CF who have more parental responsibility and/or stress have more rapid ppFEV1 decline than those with less responsibility/stress and (3) parents using CFTR modulators have decreased ppFEV1 decline versus those not using CFTR modulators. Critically, a prospective approach permits us to comprehensively assess the psychosocial impacts of parenthood using survey-based data collected from parent participants using validated measures and to measure hypothesised key components of the interplay between parenthood and physical/mental health, parental responsibilities/stress, adherence and quality of life. Conducting dyadic interviews enables us to better understand the experience of parents with CF and their support systems and can directly inform the development of preventive interventions. The combined results will inform future prenatal and perinatal and early childhood parenthood interventions on modifiable factors that influence parental health during high-risk periods and among high-risk populations for parental health decline. Participation in the study for the first 5 years of parenthood will inform how parenting an infant, toddler and preschool-age child (ages when children need the most daily assistance and experience frequent viral illnesses and when parents may have increased depression and/or poor sleep) impacts parental health.

    Our primary endpoint is a rate of ppFEV1 decline over the study period. We will use a race-neutral Global Lung Function Initiative equation to calculate all pulmonary function values.42 Secondary outcomes include rates of PEx, hospitalisations, clinic visit attendance, BMI, medication use, CF-related diabetes control and microbiologic profile.

    Statistical plan

    We will summarise cohort characteristics using descriptive statistics. We will estimate the annualised rate of change in ppFEV1 by computing the slope over using all available ppFEV1 measures. We hypothesise that the following conditions will be associated with a rate of ppFEV1 decline: parental depression, level of parental responsibility/stress and CFTR modulator use. To assess whether ppFEV1’s longitudinal trajectory differs by these effect modifiers, we will conduct univariable and multivariable longitudinal analysis using mixed effect regression models, which account for correlations of repeated measures within a participant over time. Specifically, we will use a primary longitudinal model with an interaction term between time and the effect modifier to be tested (ie, depression, parental responsibility/stress and modulator use). We will also examine short-term and long-term effects to identify the timing of critical periods of poor parental health for people with CF, including the first and second years of parenthood. We will evaluate and properly adjust for potential confounders, such as demographic characteristics (eg, age, sex, race/ethnicity, socioeconomic status and insurance status) and baseline data (eg, baseline depression severity) that may impact ppFEV1 decline after becoming a parent. We will consider potential time-varying covariates (ie, PEx, clinic visit attendance), which could impact the longitudinal trajectory of outcomes. For example, we will assess the association of cumulative number of events over time for PEx, visits, etc with ppFEV1 change. However, we will also construct models without adjustment for these time-varying measures, as they could be potential mediators, which must be evaluated as part of the causal pathway between parenthood and reduced health.

    We will check each model to assure that underlying assumptions (eg, normality and linearity) are met. If the normality assumption is not met, we will consider data transformations. If the linearity assumption is not met, we would categorise the continuous variables. Specifically, we anticipate that the association between parental depression score and ppFEV1 will be non-linear and thus will consider it as categorical or binary (eg, comparing those with moderate/severe PHQ-8 scores (10+) to those with mild/no depression (0–9)). We would also categorise factors on the Parental Responsibility Scale and Parenting Stress Scale. CFTR modulator use can be categorised as <25% use as prescribed, 25%–75% use and >75% use based on self-report/pharmacy refill data.

    We will also examine the data from participants who undergo transplant or die during the study period, using data censoring/truncation at the time of transplant. If there are few cases of transplant or death, we will remove them from the analysis or adjust the model. We will conduct similar analyses for other outcome variables including annual rate of PEx/hospitalisations, clinic visit attendance, BMI, quality of life, medication use, diabetes control (HgbA1c) and microbiological profile.

    We anticipate high availability of our primary outcome measure ppFEV1, as it is part of routine CF care. Nevertheless, we will examine missingness patterns to select the best statistical approach. We will also assess the characteristics of participants with missing values and perform a sensitivity analysis when appropriate.

    We will conduct all statistical analyses by using SAS V.9.4 (SAS Institute) at a 0.05 significance level.

    For dyadic interviews, we will analyse transcripts through deductive and inductive thematic coding.43 We will develop an initial codebook and two coders will independently apply this approach to each transcript and identify additional codes using Dedoose qualitative software. Using a consensus coding approach, after approximately five transcripts are independently coded, the coders will meet to review their coding, discuss any discrepancies and define any new codes; this strategy will be repeated using an iterative process until no new codes emerge. A senior investigator will be available to adjudicate any differences in interpretation and review the codebooks. We will apply the final coding scheme to transcripts and identify central themes/quotations on parenting with CF and potential future interventions. We will also reexamine interview content and themes after completion of the 5-year cohort analysis period to attempt to better contextualise our findings.44

    Sample size

    We will enrol 146 new parents in the study. We will examine whether presumed binary variables (eg, depression) are effect modifiers and assess whether the parents in group A (eg, parents with moderate/severe depression) have increased ppFEV1 decline compared with group B (eg, parents without mild/no depression), assuming that a difference in ppFEV1 between two groups is linearly increasing over time, table 1 shows the required samples sizes based on various scenarios of intrasubject correlation coefficient (ICC) and SD. With 146 parents, we will have 80% power at a 0.05 two-sided significance level to detect depression as an effect modifier of ppFEV1 decline over 5 years when a difference in rate of ppFEV1 decline between parents with and without moderate/severe depression is 1.32, assuming 12% SD in ppFEV1 and ICC of 0.05 over 5 years (ie, 6 time points).

    View this table:
    Table 1

    Required sample size with 80% statistical power

    For dyadic interviews, determining sample size in qualitative research is a matter of judgement and experience in evaluating data collected guided by content saturation. For this project, we estimate the need for ~20 mothers and ~20 fathers with their key supports based on previous qualitative work of a similar nature.45

    Study status

    The first participant was enrolled on 1 July 2024. Our goal date for the last new parent enrolment into the study is 31 January 2026. We hope to have our last participant complete the 5-year follow-up by 31 January 2031.

    Impact and Dissemination

    Most previous studies assessing the implications of parenthood for CF health are limited to the child’s first years of life.2 23–25 Thus, the impact of having a toddler, school-age child or adolescent on parents with CF remains unknown. Also, prior work is restricted to those who became parents via pregnancy,25 26 and the effects of adoption, surrogacy, step-parenthood or foster care have not been investigated. Our recent UK registry analysis demonstrates that parenthood adversely impacts short-term CF health outcomes and CF modulator use mitigates such effects.27 However, this study was limited in its follow-up duration, did not include a study population with widespread highly effective CF modulator use or mental health/psychosocial outcomes and because of healthcare system differences may not be applicable to the US CF population. Qualitative studies provide an understanding of the experience of parenthood in CF but primarily focus on individual sociological perspectives, limiting generalisability.6–8 In total, existing research remains limited as it lacks longitudinal data and key psychosocial factors and has not examined widespread CF modulator use or the outcomes of US males with CF and those pursuing other paths to parenthood.

    Furthermore, the current approach when counselling people with CF who are considering parenthood is to provide emotional support and assistance with contingency planning for health decline. People with CF desire evidence-based guidance related to the impact of becoming a parent on their health.46 The HOPeCF study is significant because it will be the first to comprehensively investigate the health impacts of parenthood in the modern era of CF and thus will facilitate a substantive departure from this status quo by providing clear evidence of the comprehensive health impacts of parenthood on all people with CF and the potential protective effects of CFTR modulators to support clinical guidance. This longitudinal, observational multicentre study, a necessary and timely step in understanding the risk factors for poor parental health outcomes in CF, will provide data essential for care guidance to people with CF, their partners and providers. It is important to ensure that people with CF have the resources and information they need to make critical, time-sensitive decisions about their reproductive futures. A 2023 CFF research prioritisation survey found that the impact of parenthood and family planning ranked highly for CF community members.47 The planned study, which includes the critical input of community stakeholders, is a comprehensive examination of the interplay of parenthood with physical and mental health, adherence and quality of life.

    Results can serve as the basis for future mechanistic studies exploring the causes of parental health decline (such as via increased viral infections/changes in the lung microbiome, inadequate sleep or poor adherence). It will also inform future randomised trials optimising the clinical management of parents with CF during high-risk periods of health decline through the development and testing of patient-centred interventions.48–50 We plan to communicate results at international meetings and via submission to peer-reviewed journals. Additionally, we will partner with the CFF to communicate study findings via social media and online.

    Similar research has not been conducted among parents with other chronic conditions. As people with paediatric-onset chronic diseases (ie, congenital heart disease, sickle cell disease and epilepsy) are living longer and considering parenthood, these results may have widespread applicability.51–53

    Data availability statement

    Data sharing not applicable as no datasets generated and/or analysed for this study.

    Ethics statements

    Patient consent for publication

    Ethics approval

    This study involves human participants and was approved by University of Pittsburgh (STUDY23080161). Participants gave informed consent to participate in the study before taking part.

    Acknowledgments

    We would like to express our gratitude to Alexandra Wilson, Alexander Riley, and Chelsea Krug for assistance with database set-up and survey content. We would also like to thank our community partners who piloted the surveys for this project. Importantly, we would like to thank the participating parents with CF and their families/support systems for devoting time and energy to the study at a busy time in their lives.

    References

    1. ↵
      Patient Registry Annual Data Report. Bethesda(MD): Cystic Fibrosis Foundation, Available: https://www.cff.org/medical-professionals/patient-registry
    2. ↵
      1. Kazmerski TM,
      2. West NE,
      3. Jain R, et al
      . Family-building and parenting considerations for people with cystic fibrosis. Pediatr Pulmonol 2022;57 Suppl 1:S75–88:34407321. doi:10.1002/ppul.25620
      OpenUrl
    3. ↵
      1. Sawyer SM,
      2. Farrant B,
      3. Cerritelli B, et al
      . A survey of sexual and reproductive health in men with cystic fibrosis: new challenges for adolescent and adult services. Thorax 2005;60:326–30. doi:10.1136/thx.2004.027599
      OpenUrlAbstract/FREE Full Text
    4. ↵
      1. Kazmerski TM,
      2. Sawicki GS,
      3. Miller E, et al
      . Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J Cyst Fibros 2018;17:57–63. doi:10.1016/j.jcf.2017.07.017
      OpenUrl
    5. ↵
      1. Hisert KB,
      2. Birket SE,
      3. Clancy JP, et al
      . Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy. Lancet Respir Med 2023;11:916–31. doi:10.1016/S2213-2600(23)00324-7
      OpenUrl
    6. ↵
      1. Kazmerski TM,
      2. Gmelin T,
      3. Slocum B, et al
      . Attitudes and decision making related to pregnancy among young women with cystic fibrosis. Matern Child Health J 2017;21:818–24. doi:10.1007/s10995-016-2181-z
      OpenUrlPubMed
    7. ↵
      1. Jacob A,
      2. Journiac J,
      3. Fischer L, et al
      . How do cystic fibrosis patients experience parenthood? A systematic review. J Health Psychol 2021;26:60–81. doi:10.1177/1359105320916539
      OpenUrl
    8. ↵
      1. Stransky OM,
      2. Benipal S,
      3. Pam M, et al
      . “Find ways to work parenting into cystic fibrosis”: a photovoice exploration of being a parent and having CF. Pediatr Pulmonol 2023;58:1527–34. doi:10.1002/ppul.26355
      OpenUrl
    9. ↵
      1. Högnäs RS,
      2. Roelfs DJ,
      3. Shor E, et al
      . J-Curve? A meta-analysis and meta-regression of parity and parental mortality. Popul Res Policy Rev 2017;36:273–308. doi:10.1007/s11113-016-9421-1
      OpenUrlCrossRef
    10. ↵
      1. Zeng Y,
      2. Ni Z,
      3. Liu S, et al
      . Parity and all-cause mortality in women and men: a dose-response meta-analysis of cohort studies. Sci Rep 2016;6:19351. doi:10.1038/srep19351
      OpenUrlCrossRefPubMed
    11. ↵
      1. Howard LM,
      2. Khalifeh H
      . Perinatal mental health: a review of progress and challenges. World Psychiatry 2020;19:313–27. doi:10.1002/wps.20769
      OpenUrlCrossRefPubMed
    12. ↵
      1. Shadyab AH,
      2. Gass MLS,
      3. Stefanick ML, et al
      . Maternal age at childbirth and parity as predictors of longevity among women in the united states: the women’s health initiative. Am J Public Health 2017;107:113–9. doi:10.2105/AJPH.2016.303503
      OpenUrl
    13. ↵
      1. Jaffe DH,
      2. Neumark YD,
      3. Eisenbach Z, et al
      . Parity-related mortality: shape of association among middle-aged and elderly men and women. Eur J Epidemiol 2009;24:9–16. doi:10.1007/s10654-008-9310-y
      OpenUrlCrossRefPubMedWeb of Science
    14. ↵
      1. Henretta JC
      . Early childbearing, marital status, and women’s health and mortality after age 50. J Health Soc Behav 2007;48:254–66. doi:10.1177/002214650704800304
      OpenUrlCrossRefPubMedWeb of Science
    15. ↵
      1. Thoits PA
      . Stress and health: major findings and policy implications. J Health Soc Behav 2010;51 Suppl:S41–53. doi:10.1177/0022146510383499
      OpenUrlCrossRefPubMed
    16. ↵
      1. Ringbäck Weitoft G,
      2. Burström B,
      3. Rosén M
      . Premature mortality among lone fathers and childless men. Social Science & Medicine 2004;59:1449–59. doi:10.1016/j.socscimed.2004.01.026
      OpenUrl
    17. ↵
      1. Read S,
      2. Grundy E,
      3. Wolf DA
      . Fertility history, health, and health changes in later life: a panel study of british women and men born 1923–49. Popul Stud (Camb) 2011;65:201–15. doi:10.1080/00324728.2011.572654
      OpenUrlCrossRefPubMed
    18. ↵
      1. Munch A,
      2. McPherson JM,
      3. Smith-Lovin L
      . Gender, children, and social contact: the effects of childrearing for men and women. Am Sociol Rev 1997;62:509. doi:10.2307/2657423
      OpenUrlCrossRefWeb of Science
    19. ↵
      1. Umberson D
      . Family status and health behaviors: social control as a dimension of social integration. J Health Soc Behav 1987;28:306–19:3680922.
      OpenUrlCrossRefPubMedWeb of Science
    20. ↵
      1. Nelson SK,
      2. Kushlev K,
      3. English T, et al
      . In defense of parenthood: children are associated with more joy than misery. Psychol Sci 2013;24:3–10. doi:10.1177/0956797612447798
      OpenUrlCrossRefPubMed
    21. ↵
      1. Twenge JM,
      2. Campbell WK,
      3. Foster CA
      . Parenthood and marital satisfaction: a meta‐analytic review. J of Marriage and Family 2003;65:574–83. doi:10.1111/j.1741-3737.2003.00574.x
      OpenUrl
    22. ↵
      1. Widarsson M,
      2. Engström G,
      3. Rosenblad A, et al
      . Parental stress in early parenthood among mothers and fathers in Sweden. Scand J Caring Sci 2013;27:839–47. doi:10.1111/j.1471-6712.2012.01088.x
      OpenUrlCrossRefPubMed
    23. ↵
      1. Duguépéroux I,
      2. Hubert D,
      3. Dominique S, et al
      . Paternity in men with cystic fibrosis: a retrospective survey in France. J Cyst Fibros 2006;5:215–21. doi:10.1016/j.jcf.2006.03.004
      OpenUrlPubMed
    24. ↵
      1. Bianco B,
      2. Horsley A,
      3. Brennan A
      . Implications of fatherhood in cystic fibrosis. Paediatr Respir Rev 2019;31:18–20. doi:10.1016/j.prrv.2019.02.008
      OpenUrl
    25. ↵
      1. Cohen-Cymberknoh M,
      2. Gindi Reiss B,
      3. Reiter J, et al
      . Baseline cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression. J Cyst Fibros 2021;20:388–94. doi:10.1016/j.jcf.2020.09.002
      OpenUrlPubMed
    26. ↵
      1. Schechter MS,
      2. Quittner AL,
      3. Konstan MW, et al
      . Long-term effects of pregnancy and motherhood on disease outcomes of women with cystic fibrosis. Ann Am Thorac Soc 2013;10:213–9. doi:10.1513/AnnalsATS.201211-108OC
      OpenUrlCrossRefPubMed
    27. ↵
      1. Kazmerski TM,
      2. Jain R,
      3. Lee M, et al
      . Parenthood impacts short-term health outcomes in people with cystic fibrosis. J Cyst Fibros 2022;21:662–8. doi:10.1016/j.jcf.2022.02.006
      OpenUrl
    28. ↵
      1. Riekert KA,
      2. Bartlett SJ,
      3. Boyle MP, et al
      . The association between depression, lung function, and health-related quality of life among adults with cystic fibrosis. Chest 2007;132:231–7. doi:10.1378/chest.06-2474
      OpenUrlCrossRefPubMedWeb of Science
    29. ↵
      1. Knudsen KB,
      2. Pressler T,
      3. Mortensen LH, et al
      . Associations between adherence, depressive symptoms and health-related quality of life in young adults with cystic fibrosis. Springerplus 2016;5:1216. doi:10.1186/s40064-016-2862-5
    30. ↵
      1. Schechter MS,
      2. Ostrenga JS,
      3. Fink AK, et al
      . Decreased survival in cystic fibrosis patients with a positive screen for depression. J Cyst Fibros 2021;20:120–6. doi:10.1016/j.jcf.2020.07.020
      OpenUrl
    31. ↵
      1. Szczesniak R,
      2. Heltshe SL,
      3. Stanojevic S, et al
      . Use of FEV1 in cystic fibrosis epidemiologic studies and clinical trials: a statistical perspective for the clinical researcher. J Cyst Fibros 2017;16:318–26. doi:10.1016/j.jcf.2017.01.002
      OpenUrlPubMed
    32. ↵
      1. Kroenke K,
      2. Strine TW,
      3. Spitzer RL, et al
      . The PHQ-8 as a measure of current depression in the general population. J Affect Disord 2009;114:163–73. doi:10.1016/j.jad.2008.06.026
      OpenUrlCrossRefPubMedWeb of Science
    33. ↵
      1. Spitzer RL,
      2. Kroenke K,
      3. Williams JBW, et al
      . A brief measure for assessing generalized anxiety disorder: the GAD-7. Arch Intern Med 2006;166:1092–7. doi:10.1001/archinte.166.10.1092
      OpenUrlCrossRefPubMedWeb of Science
    34. ↵
      1. Berry JO,
      2. Jones WH
      . The parental stress scale: initial psychometric evidence. J Soc Pers Relat 1995;12:463–72. doi:10.1177/0265407595123009
      OpenUrlCrossRefWeb of Science
    35. ↵
      1. Quittner AL,
      2. Sawicki GS,
      3. McMullen A, et al
      . Psychometric evaluation of the cystic fibrosis questionnaire-revised in a national sample. Qual Life Res 2012;21:1267–78. doi:10.1007/s11136-011-0036-z
      OpenUrlCrossRefPubMed
    36. ↵
      1. Ladage JE
      . Motivation, Marital Quality, Maternal Gatekeeping, Breadwinning, and Father Identity: Models of Biological Fathers’ and Stepfathers. Involvement In Childcare. Doctor of Philosophy (PhD), dissertation, Psychology, Old Dominion University, 2015.
    37. ↵
      1. Aftyka A,
      2. Rozalska I,
      3. Pawlak A, et al
      . Internal consistency and accuracy of Interpersonal SupportEvaluation List (ISEL-40) in mothers of healthy children and those with a medical history. Ann Agric Environ Med 2019;26:85–91. doi:10.26444/aaem/91783
      OpenUrl
    38. ↵
      1. Buysse DJ,
      2. Reynolds CF,
      3. Monk TH, et al
      . The Pittsburgh sleep quality index: a new instrument for psychiatric practice and research. Psychiatry Res 1989;28:193–213. doi:10.1016/0165-1781(89)90047-4
      OpenUrlCrossRefPubMedWeb of Science
    39. ↵
      1. Holmes TH,
      2. Rahe RH
      . The Social Readjustment Rating Scale. J Psychosom Res 1967;11:213–8. doi:10.1016/0022-3999(67)90010-4
      OpenUrlCrossRefPubMedWeb of Science
    40. ↵
      1. Riekert K,
      2. Eckmann T,
      3. Psoter K, et al
      . Adherence barriers identified by adolescents & adults with CF: the barriers assessment screener. Pediatr Pulmonol 2018;53.
    41. ↵
      1. Harris PA,
      2. Taylor R,
      3. Minor BL, et al
      . The REDCap consortium: building an international community of software platform partners. J Biomed Inform 2019;95:103208. doi:10.1016/j.jbi.2019.103208
      OpenUrlCrossRefPubMed
    42. ↵
      1. Bowerman C,
      2. Bhakta NR,
      3. Brazzale D, et al
      . A race-neutral approach to the interpretation of lung function measurements. Am J Respir Crit Care Med 2023;207:768–74. doi:10.1164/rccm.202205-0963OC
      OpenUrl
    43. ↵
      1. Morgan DL,
      2. Ataie J,
      3. Carder P, et al
      . Introducing dyadic interviews as a method for collecting qualitative data. Qual Health Res 2013;23:1276–84. doi:10.1177/1049732313501889
      OpenUrlCrossRefPubMedWeb of Science
    44. ↵
      1. Creswell JW,
      2. Plano Clark VL,
      3. Gutmann ML, et al
      . Advanced mixed methods research design. In: Tashakkori & A, ed. Handbook of Mixed Methods in Social & Behavioral Research. Thousand Oaks, CA: Sage, 2003: 209–40.
    45. ↵
      1. Om S,
      2. M P,
      3. M B, et al
      . A qualitative exploration of parenthood experiences and needs among people with cystic fibrosis. J Cyst Fibros 2024;23:424–8. doi:10.1016/j.jcf.2023.08.005
      OpenUrl
    46. ↵
      1. Leech MM,
      2. Stransky OM,
      3. Talabi MB, et al
      . Exploring the reproductive decision support needs and preferences of women with cystic fibrosis. Contraception 2021;103:32–7. doi:10.1016/j.contraception.2020.10.004
      OpenUrl
    47. ↵
      1. Kazmerski TM,
      2. Moy C,
      3. Aliaj E, et al
      . Prioritizing sexual and reproductive health research and care for people with cystic fibrosis: a 2023 workshop report from the cystic fibrosis foundation sexual health, reproduction, and gender (SHARING) research working group. J Cyst Fibros 2024;13:00035–3. doi:10.1016/j.jcf.2024.03.007
      OpenUrl
    48. ↵
      1. Lim AS,
      2. Stewart K,
      3. Abramson MJ, et al
      . Multidisciplinary Approach to Management of Maternal Asthma (MAMMA): a randomized controlled trial. Chest 2014;145:1046–54. doi:10.1378/chest.13-2276
      OpenUrlCrossRefPubMed
    49. ↵
      1. Powell H,
      2. Murphy VE,
      3. Taylor DR, et al
      . Management of asthma in pregnancy guided by measurement of fraction of exhaled nitric oxide: a double-blind, randomised controlled trial. Lancet 2011;378:983–90. doi:10.1016/S0140-6736(11)60971-9
      OpenUrlCrossRefPubMed
    50. ↵
      1. Park E
      . The Fathers and Mothers Invested in Lives of Their Youth (FAMILY) Study: A Pilot Intervention for Families Affected by Parental Cancer. National Cancer Institute, Available: https://clinicaltrials.gov/ct2/show/NCT04092816
    51. ↵
      1. Kirkpatrick L,
      2. Harrison E,
      3. Khalil S, et al
      . A survey of child neurologists about reproductive healthcare for adolescent women with epilepsy. Epilepsy & Behavior 2021;120:108001. doi:10.1016/j.yebeh.2021.108001
      OpenUrl
    52. ↵
      1. Leroy-Melamed M,
      2. Jacob S,
      3. Shew ML, et al
      . Provider attitudes, preferences, and practices regarding sexual and reproductive health for adolescents and young adults with sickle cell disease. J Adolesc Health 2021;69:970–5. doi:10.1016/j.jadohealth.2021.06.008
      OpenUrl
    53. ↵
      1. Stokes N,
      2. Stransky OM,
      3. West SC, et al
      . Sexual and reproductive health care experiences and perceptions of women with congenital heart disease. Pediatr Cardiol 2023;44:564–71. doi:10.1007/s00246-022-02951-8
      OpenUrl

    Footnotes

    • X @JenTaylorCousar

    • Contributors TMK was responsible for conceptualisation; funding acquisition; methodology; resources; visualisation; roles/writing–original draft; writing–review and editing. TMK is the guarantor. OMS was responsible for methodology; writing–review and editing. KAP was responsible for methodology; writing–review and editing. ML was responsible for methodology; statistical planning; writing–review and editing. AMG, RJ and DSS were responsible for methodology; writing–review and editing. JTC was responsible for conceptualisation; methodology; supervision; writing–review and editing.

    • Funding This work was supported by the National Institutes of Health grant number 5R01HL161164-02 and the Cystic Fibrosis Foundation grant numbers KAZMER22A0.

    • Map disclaimer The inclusion of any map (including the depiction of any boundaries therein), or of any geographic or locational reference, does not imply the expression of any opinion whatsoever on the part of BMJ concerning the legal status of any country, territory, jurisdiction or area or of its authorities. Any such expression remains solely that of the relevant source and is not endorsed by BMJ. Maps are provided without any warranty of any kind, either express or implied.

    • Competing interests TK receives grant support from the Cystic Fibrosis Foundation (CFF) and the National Institutes of Health (NIH). She receives consulting fees from the CFF as a facilitator in the Partnership Enhancement Program. She is the Vice Chair of the CFF Sexual Health, Reproduction and Gender Research Working Group. She has received honoraria from the CFF, Johns Hopkins University and Spark Healthcare. ML receives grant support from the CFF and the NIH. RJ receives grant support from the CFF and the NIH. She also receives grant funding to her institution from Vertex, Sound Pharma, 4D molecular therapeutics and Armata. She receives consulting fees from Vertex, Boehringer Ingelheim, Recode and Insmed. She receives travel support from the CFF. She is the Chair of the CFF Sexual Health, Reproduction and Gender Research Working Group and serves on the CFF’s Clinical Research Executive Committee. AMG reports personal fees from the Belgian Cystic Fibrosis Foundation/King Baudouin Foundation; grants, personal fees and travel reimbursement from the Cystic Fibrosis Foundation, grants from the Dutch Cystic Fibrosis Foundation; travel reimbursement from the European Cystic Fibrosis Society; travel reimbursement from the French Cystic Fibrosis Society; personal fees from the Italian Cystic Fibrosis Research Foundation; grant funding from the NIH, personal fees from Saudi Pediatric Pulmonology Association; grants and personal fees from Vertex Pharmaceuticals and personal fees from Virginia Commonwealth University. DSS receives grant funding from the NIH. JT-C receives grant support from the CFF and the NIH. She also receives grant funding to her institution from Vertex, Eloxx and 4D: molecular therapeutics. She has received consulting fees from Vertex, Insmed and 4D: molecular therapeutics. She has received speaking fees from Vertex for non-branded speaking engagements. She has received funding from Abbvie for her role as Data Monitoring Committee Chair. She serves as the adult patient care representative to the CFF Board of Trustees, and on the CF Foundation’s Clinical Research Executive Committee, Clinical Research Advisory Board, as immediate past chair of the CF TDN’s Sexual Health, Reproduction and Gender Research Working Group, and as Co-Chair of the Heath Equity Team Science Awards study section and on the Racial Justice Working Group. She also serves on the scientific advisory board for Emily’s Entourage, and on the ATS Respiratory Health Awards Committee and as Chair-Elect of the International Conference Committee (with previous service on the Scientific Grant Review and Clinical Problems Programming Committee). She is an Associate Editor for the Journal of Cystic Fibrosis and a member of the International Advisory Board for the Lancet Respiratory Medicine Journal. She serves on the Clinical Trials Review (CTLR) Study section for the NIH/National Heart. Blood, Lung Institute.

    • Patient and public involvement Patients and/or the public were involved in the design, or conduct, or reporting, or dissemination plans of this research. Refer to the Methods section for further details.

    • Provenance and peer review Not commissioned; externally peer reviewed.