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.

BACKGROUND: To address sexual and reproductive health (SRH) concerns among people with cystic fibrosis(PwCF), the CF Foundation created the Sexual Health, Reproduction, and Gender Research (SHARING) Working Group. This report summarizes CF community SRH research priorities and workshop discussions/future study planning. METHODS: Pre-workshop, we distributed a community prioritization survey on CF SRH research/care. During the workshop, we used results and reviewed existing research to establish research priorities and design studies to address identified knowledge gaps. RESULTS: A total of 303 respondents (85 % PwCF, 15 % caregivers) completed the survey. Highly-rated SRH topics were: 1) effects of CF modulator therapy on sex hormones; 2) effects of sex hormones on CF; 3) fertility; 4) pregnancy; and 5) SRH/mental health. Twenty-four workshop participants established the need for further research on sex hormones and CF, optimizing SRH care provision, and fertility/ART. CONCLUSION: SRH is an important and emerging area in CF and thoughtful consideration of community perspectives can ensure that future research is relevant and responsive.

Clinician perspectives and practices related to sexual and reproductive care provision for males with cystic fibrosis.

BACKGROUND: Males with cystic fibrosis (MwCF) have unique sexual and reproductive health (SRH) concerns. This study investigates multidisciplinary CF clinician perspectives related to SRH for MwCF in the current era of CF care. METHODS: We surveyed multidisciplinary clinicians exploring attitudes, practices, and preferences toward male CF SRH care. We compared responses across groups by population served (pediatric vs. adult vs. both pediatric and adult MwCF) using chi square/Fisher's exact tests. RESULTS: A total of 297 clinicians completed the survey (41 % pediatric, 36 % adult, 23 % both; 27 % physicians, 24 % social workers, 11 % nurses, 41 % other). Nearly all (98 %) believed the CF team had a role in SRH care with 75 % believing they should be primarily responsible. Pediatric clinicians were less likely to deem SRH topics important and less likely to report annual discussions compared to adult colleagues (all p<0.05). Pediatric clinicians reported less comfort in their SRH knowledge than adult colleagues (p<0.001) and in their ability to provide SRH care (p<0.05). Common barriers endorsed by respondents included lack of SRH knowledge (75 %) and presence of family/partners in exam room (64 %). A majority rated SRH screening tools (91 %), partnerships with SRH specialists (90 %), clinician training (83 %), and management algorithms (83 %) as potential facilitators. CONCLUSION: Multidisciplinary CF clinicians perceive SRH for MwCF as important but report suboptimal SRH discussions. Pediatric clinicians report significantly less comfort and skill in discussing and managing male SRH. Identified barriers and facilitators should be used to improve SRH care for MwCF.

What Is Most Important to Family Caregivers When Helping Patients Make Treatment-Related Decisions: Findings from a National Survey.

Prioritizing patient values-who/what matters most-is central to palliative care and critical to treatment decision making. Yet which factors are most important to family caregivers in these decisions remains understudied. Using data from a U.S. national survey of cancer caregivers (N = 1661), we examined differences in factors considered very important by caregivers when partnering with patients in cancer treatment decision making by cancer stage and caregiver sociodemographics. Fifteen factors were rated on a 4-point Likert-scale from 'very unimportant' to 'very important.' Descriptive statistics were used to characterize caregiver factors and tabulate proportions of importance for each. Generalized linear mixed effect modeling was used to examine the importance of factors by cancer stage, and chi-square analyses were performed to determine associations between caregiver sociodemographics and the five most commonly endorsed factors: quality of life (69%), physical well-being (68%), length of life (66%), emotional well-being (63%), and opinions/feelings of oncology team (59%). Significant associations (all p's < 0.05) of small magnitude were found between the most endorsed factors and caregiver age, race, gender, and ethnicity, most especially 'opinions/feelings of the oncology team'. Future work is needed to determine the best timing and approach for eliciting and effectively incorporating caregiver values and preferences into shared treatment decision making.

Sexual and reproductive health experiences and care of adult women with cystic fibrosis.

BACKGROUND: As survival and health improve in people with cystic fibrosis (CF), more women with CF (wwCF) are considering their sexual and reproductive health (SRH). This study compared SRH experiences, behaviors, and care utilization of wwCF to the general population and defined CF-impacted considerations and care preferences. METHODS: We surveyed wwCF aged ≥25 years regarding SRH and compared results to the US National Survey of Family Growth (NSFG;n = 4357) and friend controls(n = 123). We used descriptive statistics and chi-squared/Fisher's exact testing and linear regression for comparisons. RESULTS: A total of 460 wwCF (mean age 36.1 years) completed the survey. WwCF were less likely to report current contraceptive use (43%vs76% NSFG, p<0.001;60% friends, p = 0.005). Nearly 25% of wwCF reported worsened CF symptoms during their menstrual cycles, 50% experienced urinary incontinence, and 80% vulvovaginal candidiasis. WwCF were significantly less likely to be parents (46%vs62% friends, p = 0.015) and to have experienced pregnancy (37%vs78% NSFG, p<0.001;58% friends, p = 0.002). More wwCF required medical assistance to conceive (29%vs12% NSFG, p<0.001 and 5% friends, p<0.001). Eighty-four percent of wwCF view their CF doctor as their main physician and 41% report no primary care provider (vs19% friends; p<0.001). WwCF report suboptimal rates of contraceptive and preconception counseling/care and are less likely to have received HPV vaccination (42%vs55%friends, p = 0.02). Despite desiring SRH conversations with their CF team, <50% report discussing SRH topics. CONCLUSION: WwCF have significantly different SRH experiences than non-CF peers. They report suboptimal SRH care compared to their preferences highlighting an urgent need to encourage SRH counseling/care in the CF model.

Engaging Stakeholders in the Development of a Reproductive Goals Decision AID for Women with Cystic Fibrosis.

Objective: More people with cystic fibrosis (pwCF) are reaching adulthood and considering their reproductive futures. Unfortunately, many pwCF report gaps in their reproductive healthcare. We describe measures of stakeholder engagement in developing a reproductive goals decision aid for women with CF called MyVoice:CF. Methods: Stakeholders reviewed the content, design, and usability of the tool, which was informed by prior research related to CF family planning experiences and preferences as well as a conceptual understanding of reproductive decision making. We evaluated stakeholder engagement via process measures and outcomes of stakeholder involvement. We collected data via recorded stakeholder recommendations and surveys. Results: Fourteen stakeholders participated and the majority described their role on the project as "collaborator", "advisor", or "expert." Most felt their expectations for the project were met or exceeded, that they had contributed significantly, and that they received sufficient and frequent information about the process. All stakeholders provided recommen-dations and clarified aims for a CF-specific family planning tool, including its content and focus on facilitating shared decision making. Discussion: Utilizing meaningful stakeholder contributions, we developed MyVoice:CF, a novel web-based decision aid to help women with CF engage in shared decision-making regarding their reproductive goals. Practical Value: Our findings from working with stakeholders for MyVoice:CF indicate that disease-specific reproductive health resources can and should be designed with input from individuals in the relevant communities.

Measuring Knowledge of Fertility Preservation in Women With Cystic Fibrosis: Instrument Development and Psychometric Analysis.

OBJECTIVE: To describe the development, evaluation, and psychometric properties of a new instrument that measures fertility preservation (FP) knowledge in women with cystic fibrosis (CF) titled the Knowledge of FP in Women With CF Instrument (KFP-WCFI). DESIGN: The 10-item KFP-WCFI was developed and evaluated through a cross-sectional survey. SETTING: Participants were recruited nationally from CF Foundation-accredited CF clinics and via snowball sampling. PARTICIPANTS: Fifty women with CF ages 18 through 35 years completed the instrument. MEASUREMENTS: Construct validity was assessed using confirmatory factor analysis (CFA). In the CFA, 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. RESULTS: The CFA with two subscales, General Fertility Knowledge and Transplant-Related Fertility Knowledge, demonstrated good fit, with a standardized root mean square 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 internal consistency reliability with a Cronbach's alpha of .91 for the General Fertility Knowledge subscale and .64 for the Transplant-Related Fertility Knowledge subscale. Women who reported a pregnancy scored higher than women who did not report a pregnancy (p = .02), suggesting criterion validity. CONCLUSION: The newly developed KFP-WCFI appears to be a valid and reliable instrument that can be used to measure self-assessed FP knowledge in women with CF.

Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.

Infertility and subfertility are commonly faced by females with cystic fibrosis (FwCF) and resulting in decreased contraceptive use and increased utilization of reproductive technologies. Elexacaftor-tezacaftor-ivacaftor (ETI) is a CFTR modulator that affects common causes of subfertility. Two CF centers conducted a retrospective chart review on females with CF who were receiving ETI and became pregnant. We analyzed obstetrical-gynecological history, genotype, and clinical response to ETI therapy. Fourteen FwCF on ETI became pregnant. Half (7) of the FwCFs were previously attempting to conceive, but only three were using contraceptives. Four FwCF had a history of infertility; two were reconsidering use of reproductive technologies (IUI). Patients achieved conception at mean 8 weeks after initiating ETI. ETI may lessen CF-associated factors that affect fertility; however, its exact mechanism is unknown. This warrants counseling on contraceptive use and family planning prior to initiation of therapy and at routine intervals while utilizing ETI.

Adolescent Chlamydia Rates by Region, Race, and Sex: Trends From 2013 to 2017.

INTRODUCTION: Chlamydia is the most common bacterial sexually transmitted infection in the United States. Adolescents (ages 15-19 years) have the highest infection rate, second only to young adults (ages 20-24 years). The purpose of this secondary data analysis is to examine trends from 2013 to 2017 in adolescent chlamydia rates by region, race, and sex. METHOD: Adolescent chlamydia rate data were obtained from the Center for Disease Control's National Center for HIV/AIDS, Viral Hepatitis, STD, and TB Prevention database. Descriptive statistics were used to analyze the trends. RESULTS: The overall trend in male and female adolescent chlamydia rates increased from 2013 to 2017. Specifically, the trend increased faster in males, White adolescents, and those in the Northeast and Midwest regions of the country. DISCUSSION: Health care providers are uniquely positioned to positively affect adolescent chlamydia rates through targeted sexual health discussions and increased screening for sexually transmitted infections.

Adolescents' perceptions of sexual health education programs: An integrative review.

INTRODUCTION: Adolescents are key stakeholders in sexual health education, yet they are rarely consulted when developing sexual health programs. Their voices are integral to improving the delivery of relevant and appropriate school-based sexual health education to promote safer adolescent sexual behaviors. METHODS: An integrative review was conducted utilizing three databases: Cumulative Index of Nursing and Allied Health Literature (CINAHL) Complete, PubMed, and Education Resources Information Center (ERIC). The PRISMA and matrix method were used to search the literature and synthesize the findings from 16 articles regarding adolescent perceptions of school-based sexual health education. RESULTS: The main themes that emerged from this review included: (a) factors influencing adolescent perceptions of sexual health education programs, (b) characteristics of good sexual health education programs, and (c) areas of improvement in sexual health education programs. CONCLUSION: Adolescents overwhelmingly requested honest, comprehensive content delivered by nonjudgmental, well-educated health professionals in a comfortable environment.