A Qualitative Exploration of Women With Cystic Fibrosis and Urinary Incontinence.

IMPORTANCE: Women with cystic fibrosis (CF) historically experience a high prevalence of urinary incontinence (UI). However, this area is understudied, especially in the modern era of highly effective modulator therapy (HEMT). OBJECTIVE: This study aimed to explore the UI experiences, knowledge, care-seeking behavior, and treatment preferences of women with CF. STUDY DESIGN: We recruited women aged ≥18 years through the CF Foundation's Community Voice national registry if they had a diagnosis of CF and reported UI. Participants underwent individual, semistructured interviews exploring their experiences, attitudes, and preferences toward UI that were audiorecorded and transcribed. Two coders performed thematic analysis using deductive and inductive coding approaches. RESULTS: Twenty-six participants completed interviews (average age, 45.1 years; range, 24-61 years). Key themes included the following: (1) most women with CF and UI report low bother from symptoms likely related to stress UI, and HEMT has greatly improved UI symptoms and decreased bother; (2) most women with CF and UI had previously discussed UI symptoms with family and/or peers but had not sought care due to stigma or low priority; (3) women with CF and UI had minimal knowledge about UI in general and how it relates to CF; (4) most desired broad screening for UI from their CF team and improved multidisciplinary care; and (5) highly effective UI treatment options with low-time commitment and easily accessible resources are desired. CONCLUSIONS: Women with CF and UI report low knowledge and care-seeking behavior related to this condition and desire improved care provision. Importantly, HEMT may improve UI symptoms among people with CF.

Prescription patterns relevant to young people with epilepsy of childbearing potential.

RATIONALE: Young people with epilepsy of childbearing potential (YPWECP) are vulnerable to a variety of adverse health outcomes due to teratogenic antiseizure medications (ASMs) and drug-drug interactions between ASMs and contraceptives that can lead to breakthrough seizures and/or contraceptive failure. To better understand reproductive healthcare provision for YPWECP, we conducted a retrospective analysis of relevant prescription patterns. METHODS: We analyzed procedural and medication data for YPWECP ages 13-21 years (n = 1525) from 2011 through 2021 at a single tertiary-care pediatric medical center to investigate rates of (1) prescription of folic acid, (2) prescription of an enzyme-inducing ASM<6 months before or after hormonal contraception initiation (or < 3 years after subdermal implant placement), (3) prescription of lamotrigine < 6 months before or after an estrogen-containing contraceptive that could affect lamotrigine serum concentrations, and (4) documentation of any contraceptive medication or device that overlaps initiation of a patient's first teratogenic ASM. We performed statistical analyses with sample proportion z-tests. We then used logistic regression and generalized estimating equations to evaluate for associations between patient characteristics and prescription patterns. RESULTS: Among 1525 YPWECP, less than half (41 %, n = 629) were prescribed folic acid during the study period (95 % CI 38.8-43.7). Of YPWECP prescribed an enzyme-inducing ASM, 24 % (186/766) were co-prescribed a hormonal contraceptive that adversely interacts with the ASM (95 % CI 21.2-27.3 %). Of those prescribed lamotrigine during the study period, 24 % (111/472) had documentation of an estrogen-containing medication that could affect lamotrigine serum concentrations < 6 months before or after that prescription (95 % CI 19.7-27.3 %). Of those prescribed a teratogenic ASM, only 13 % (82/638) had documentation of contraception prior to (or within the same month as) starting their first teratogenic ASM (95 % CI 10.3-15.5 %). Older age was associated with increased odds of contraceptive coverage prior to initiation of the first teratogenic ASM and was also associated with increased odds of having contraceptives co-prescribed with ASMs that could interact. No significant associations were found between race/ethnicity and any outcomes. CONCLUSIONS: YPWECP experience low rates of folic acid prescription and low rates of contraceptive coverage while prescribed teratogenic ASMs. Many YPWECP, particularly older adolescents, are at increased risk for contraceptive failure and/or breakthrough seizures due to drug-drug interactions. Results demonstrate a need for increased focus on reproductive healthcare for YPWECP. Future studies should evaluate interventions aimed at improving these outcomes.

Impact of Cystic Fibrosis Transmembrane Conductance Regulator Modulators on Maternal Outcomes During and After Pregnancy.

BACKGROUND: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are available to the majority of people with CF in the United States (US); little is known about pregnancy outcomes with modulator use. This retrospective study aims to determine the impact of CFTR modulators on maternal outcomes. RESEARCH QUESTION: Does pregnancy differentially impact outcomes in females with CF with and without CFTR modulators? STUDY DESIGN AND METHODS: We collected data on pregnancies from 2010-2021 from 11 US adult CF centers. We conducted multivariable longitudinal regression analysis to assess whether changes in percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), pulmonary exacerbations (PEx), and Pseudomonas aeruginosa prevalence differed from before, during, and after pregnancy by CFTR modulator use, while adjusting for confounders. We also describe infant outcomes based on maternal modulator use. RESULTS: Among 307 pregnancies, mean age at conception was 28.5 years (range: 17-42), pre-pregnancy ppFEV1 was 74.2 and BMI was 22.3 kg/m2. One hundred and fourteen pregnancies (37.1%) had CFTR modulator exposure during pregnancy (77 with highly effective modulator therapy [HEMT] and 37 with other modulators). The adjusted mean change in ppFEV1 from pre- to during pregnancy was -2.36 (95%CI: -3.56, -1.16) in the unexposed group and +2.60(95%CI: 0.23, 4.97) in the HEMT group, with no significant change from during to one-year post-pregnancy. There was an overall decline in ppFEV1 from pre- to post-pregnancy in the no modulator group (-2.56; 95%CI:-3.62, -1.49) that was not observed in the HEMT group (1.10; 95%CI: -1.13, 3.34). PEx decreased from pre- to post-pregnancy in the HEMT group and BMI increased from pre- to during pregnancy in all groups but without a significant change post-pregnancy. Missing infant outcome data precluded firm conclusions. INTERPRETATION: We observed superior pregnancy and post-pregnancy pulmonary outcomes in individuals who used HEMT, including a preservation of ppFEV1, compared with those unexposed to HEMT.

Assessing the health impacts of parenthood on people with cystic fibrosis: the HOPeCF prospective cohort protocol.

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.

Communication and Counseling Preferences of Women Who Chose Abortion During Adolescence: A Qualitative Study.

STUDY OBJECTIVE: As part of a larger study about pregnancy options counseling with adolescents, we interviewed women in the United States who chose abortion during adolescence about preferences and experiences regarding communication from healthcare professionals during abortion care. DESIGN, SETTING, AND PARTICIPANTS: We conducted individual semi-structured interviews with women ages 18-35 years old who were pregnant before age 20 years old and chose abortion. We recruited participants through social media, a research registry, and flyers in healthcare facilities. We recorded and transcribed interviews. Two investigators coded interview transcripts using thematic analysis. RESULTS: We conducted interviews with 17 US women (median age 32 years old, range 20-35 years old) from 11/2020-4/2021. The median age at time of abortion was 18 years old (range 14-19 years). The sample was 58% (n = 10) Caucasian and 65% (n = 11) heterosexual. Themes included: 1) Participants perceived options counseling before abortion as important and necessary but did not always feel they personally needed it. 2) Participants reported that clinicians including nurses, physicians, and other staff sometimes had poor bedside manner, which was not aligned with their perceived need for gentleness due to their adolescence. 3) Participants valued nonjudgmental communication including normalization of abortion care. 4) Participants desired privacy and confidentiality throughout their clinic appointment, but clinic logistics led to concerns about limited privacy. 5) Participants appreciated medically accurate information about abortion in plain language balancing safety and risk information for reducing fear before the procedure. CONCLUSION: Responses suggested specific best practices that healthcare providers can adopt to improve care for youth considering abortion.

Exploring the Experiences and Expectations of Adolescent Females Undergoing Periacetabular Osteotomy.

BACKGROUND: Adolescents presenting with symptomatic acetabular dysplasia (AD) complain of pain and reduced participation in activities of daily living (ADLs). Periacetabular osteotomy (PAO) is widely accepted as the preferred treatment for AD. Understanding the patient experience can lead to improvements in psychosocial and physical burden in adolescents. We sought to explore the experiences and expectations of adolescent females with AD who underwent a PAO. METHODS: We conducted semistructured interviews with adolescent females who underwent a PAO >6 months ago. Questions focused on exploring their experiences with AD and their PAO expectations and decision-making. Participants also completed a 7-item Likert-scale questionnaire related to factors they considered in their decision-making, which was followed by a ranking of those considerations. We utilized an inductive and deductive coding approach to identify key themes from interviews and descriptively analyzed questionnaire responses. RESULTS: Eighteen adolescent females between 13 and 19 years (17.2±1.9 y) at the time of PAO participated in the study. Time from surgery to interview ranged from 203 to 1534 days (927.7±320.8 d). Key themes included (1) prolonged time from symptom onset to PAO, with many seeing several providers; (2) major preoperative apprehensions of surgical outcome and setbacks in school and recreational activities; (3) discussion with the physician and people who underwent PAO were the most beneficial sources of information; (4) Postoperative worries include surgical outcome and return to daily living. Eighty-nine percent of participants reported that return to daily activities and sustaining long-term hip health were very important factors in their PAO decision-making, and 61% ranked their return to daily activities as their top priority. CONCLUSIONS: Adolescent females with AD report frustrating delays in diagnosis and appropriate intervention and value their return to daily living in their decision to undergo PAO. The development of future patient-centered interventions may improve the PAO decision-making process and should include information related to surgical recovery and anecdotes of others who underwent this procedure. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Wellness in the modulator era: An observational study of the impact of CFTR modulator therapy on the well-being of people with cystic fibrosis.

BACKGROUND: People with cystic fibrosis (PwCF) have experienced substantial improvements in health following use of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies. However, less is known about how modulator therapies impact well-being. METHODS: We used a cross-sectional observational study to identify relationships between CFTR modulator therapies, health-related quality of life (HRQoL), and well-being. Adult PwCF and caregivers of children with CF completed the Wellness in the Modulator Era (Well-ME) survey between June 22 and July 31, 2022. HRQoL was measured with PROMIS Global 10/Global 7 + 2 Parent Proxy. We used a mixed methods analysis to compare experiences and concerns of PwCF who currently (n = 665), no longer (n = 51), or never (n = 184) took modulator therapy. RESULTS: Adult PwCF taking a modulator (n = 416) reported better PROMIS global physical health than those who no longer (n = 37) or never took a modulator (n = 94) and better PROMIS global mental health than those who never took a modulator. Caregiver-reported HRQoL was similar across children with CF who currently, no longer, or never took a modulator. PwCF taking a modulator reported larger improvements in physical health, quality of life, social well-being, and treatment burden than those who no longer or never took a modulator. Nearly one-quarter (23 %) of PwCF taking modulator therapy reported worsening of mental well-being. CONCLUSIONS: This study expands our knowledge of well-being among PwCF in the CFTR modulator era as reported by patients and parents. Findings lay the groundwork for establishing future research priorities, policy efforts, and communications in areas that improve well-being for PwCF.

Implementation and evaluation of a fertility preservation telehealth counseling intervention for males with cystic fibrosis.

BACKGROUND: Most males with cystic fibrosis (MwCF) have congenital bilateral absence of the vas deferens and require assisted reproductive technology to conceive, yet many have limited knowledge about how CF affects sexual and reproductive health (SRH). This study evaluates the feasibility, acceptability, and potential effectiveness of telehealth fertility preservation (FP) counseling for MwCF. METHODS: Pre-lung transplant MwCF ≥18 years, recruited from U.S. CF centers, social media, and via snowball sampling, received individualized telehealth counseling. Participants completed intervention feasibility/acceptability one week post-counseling and FP knowledge, care satisfaction, and self-efficacy assessments at baseline and two months post-counseling. We completed acceptability interviews one-week post-counseling and audio-recorded, transcribed, and thematically analyzed results. We descriptively analyzed survey results and conducted pre/post comparisons using paired t-tests. RESULTS: Thirty MwCF (ages 22-49 years) completed counseling. Most were in a relationship (70 %) and White (86.7 %). Telehealth FP counseling was acceptable (M = 4.38/5 ± 0.60), appropriate (M = 4.37/5 ± 0.60), and feasible (M = 4.60/5 ± 0.45) to MwCF. FP knowledge (9.53 vs. 10.40/12; p = .010), care satisfaction (20.23 vs 26.67/32; p<.001), and self-efficacy (22.87 vs 25.20/30; p = .016) improved at two months post-counseling. Despite desiring provider-initiated SRH, wanting children (81 %), and perceiving the CF team as their primary care provider (97 %), 44 % report not receiving information about infertility by the CF team. CONCLUSIONS: Integrating FP counseling into CF care is feasible and acceptable to MwCF and can improve FP knowledge, self-efficacy, and care satisfaction. MwCF desire early and regular provider-initiated SRH education.

Maternal and fetal outcomes in multiparous women with Cystic Fibrosis.

BACKGROUND: Quality of life and survival in Cystic Fibrosis (CF) have improved dramatically, making family planning a feasible option. Maternal and perinatal outcomes in women with CF (wwCF) are similar to those seen in the general population. However, the effect of undergoing multiple pregnancies is unknown. METHODS: A multinational-multicenter retrospective cohort study. Data was obtained from 18 centers worldwide, anonymously, on wwCF 18-45 years old, including disease severity and outcome, as well as obstetric and newborn complications. Data were analyzed, within each individual patient to compare the outcomes of an initial pregnancy (1st or 2nd) with a multigravid pregnancy (≥3) as well as secondary analysis of grouped data to identify risk factors for disease progression or adverse neonatal outcomes. Three time periods were assessed - before, during, and after pregnancy. RESULTS: The study population included 141 wwCF of whom 41 (29%) had ≥3 pregnancies, "multiparous". Data were collected on 246 pregnancies, between 1973 and 2020, 69 (28%) were multiparous. A greater decline in ppFEV1 was seen in multiparous women, primarily in pancreatic insufficient (PI) wwCF and those with two severe (class I-III) mutations. Multigravid pregnancies were shorter, especially in wwCF over 30 years old, who had high rates of prematurity and newborn complications. There was no effect on pulmonary exacerbations or disease-related complications. CONCLUSIONS: Multiple pregnancies in wwCF are associated with accelerated respiratory deterioration and higher rates of preterm births. Therefore, strict follow-up by a multidisciplinary CF and obstetric team is needed in women who desire to carry multiple pregnancies.

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.

Virtual interviews and equity: The pediatric pulmonary fellow perspective.

BACKGROUND AND OBJECTIVES: The SARS-CoV-2 pandemic shifted medical training programs to utilize virtual interviews (VIs) starting with the 2020 interview cycle. Fellowship interviews continue in the virtual format. It is unknown how this shift has affected equity for applicants as compared to in-person interviews. Equity in this study includes consideration of the opportunity for an applicant to accept, access, and conduct a VI. This study assessed pediatric pulmonary fellows' perception of equity associated with VIs and preferences for future cycles. METHODS: An anonymous survey link was emailed to Pediatric Pulmonology Program Directors to disseminate to incoming and first-year pediatric pulmonary fellows who participated in the 2022-2023 and 2021-2022 VI seasons. Responses were summarized by frequency and percentages. Inductive coding was used to thematically analyze free-text responses. RESULTS: Nearly 30% of eligible incoming and first-year pulmonary fellows (n = 35/119, 29.4%) completed the survey. Seventy-four percent felt that VIs reduce inequities as compared to in-person interviews. Sixty percent felt that VIs were the most equitable format, and 51% chose a VI as their preferred future format. Important practice considerations to promote equity for future VIs included providing applicants with instruction for the expected dress code, followed by providing applicants with virtual technology (91% and 89% of respondents ranked as at least "somewhat important," respectively). CONCLUSION: VIs were perceived as a more equitable interview format by pediatric pulmonology fellows compared to in-person interviews in our study. To increase equity for VIs, program directors can consider additional adaptations such as providing standardized instruction for dress code and providing the required technology.

Preferences and experiences of parents/guardians of youth with epilepsy and intellectual disability on reproductive health counseling.

OBJECTIVE: To explore the experiences and preferences of parents/guardians of adolescents and young adults (AYA) of childbearing potential with co-occurring epilepsy and intellectual disability (ID) regarding counseling by neurologists on sexual and reproductive health (SRH) topics such as pregnancy, contraception, menstruation, and folic acid supplementation. METHODS: We conducted semi-structured interviews with parents/guardians of AYAs (12-28 years old) of childbearing potential with co-occurring epilepsy and ID, recruited from a tertiary-care children's hospital. We confirmed the diagnoses of epilepsy and ID with the patient's neurologist and parent/guardian. All degrees of ID (e.g. mild/moderate/severe) were eligible. We audio-recorded and transcribed interviews. Two coders performed qualitative thematic analysis. RESULTS: Twenty-five parents/guardians completed interviews. Themes included: (1) Parents/guardians believe their child to be immune from sexual abuse due to their supervision, yet desire counseling about abuse recognition and prevention, which they also report not occurring (2) A common opinion was that counseling on menstruation was more relevant to their child's life than counseling about pregnancy-related topics (3) Parents/guardians reported a lack of counseling on pregnancy-related topics such as folic acid supplementation and teratogenesis and generally also reported some degree of interest in hearing about these topics from neurologists (4) Parents/guardians also reported a lack of counseling on drug interactions between contraception and ASMs, and were highly interested in learning more about this topic (5) Parents/guardians want neurologists to initiate annual comprehensive SRH counseling at puberty about most topics, but report that they often initiate SRH discussions themselves. CONCLUSION: Parents/guardians of AYAs with epilepsy and ID prefer more frequent, neurologist-initiated, comprehensive conversations surrounding SRH particularly emphasizing menstruation and sexual abuse recognition/prevention. Findings may inform professional and patient education and health systems interventions including development of discussion guides and/or decision aides to improve SRH care for AYAs with epilepsy and ID.

Work-Life Integration for Women in Pediatric Emergency Medicine: Themes Identified Through Group Level Assessment.

OBJECTIVE: Women in medicine generally have higher burnout and lower career satisfaction and work-life integration compared with men. This study identifies factors that contribute to burnout, career satisfaction, and work-life integration in women pediatric emergency medicine (PEM) physicians. METHODS: Self-identified women PEM physicians in the United States participated in a virtual focus group using Group Level Assessment methodology. Participants completed Group Level Assessment process steps of climate setting, generating, appreciating, reflecting, understanding, selecting, and action to (1) identify themes that contribute to burnout, career satisfaction, and work-life integration and (2) determine actionable factors based on these themes. Data were collected and thematically analyzed in real time through iterative processing. The group prioritized identified themes through rounds of distillation. RESULTS: Seventeen women participated, representing 10 institutions (ages 30s-70s, 69% employed full-time). Participants identified 3 main themes contributing to burnout, career satisfaction, and work-life integration: (1) gender inequities, (2) supportive leadership, and (3) balance with family life. Actionable items identified were as follows: (1) development of initiatives to equalize pay, opportunity, and career advancement among genders; (2) implementation of an institutional focus on supportive and collaborative leadership; and (3) improvement of resources and supports for physicians with family responsibilities. CONCLUSIONS: Women PEM physicians identified gender inequities, leadership, and balance with family life as major themes affecting their burnout, career satisfaction, and work-life integration. Several action steps were identified and can be used by individuals and institutions to improve work-life integration for women PEM physicians.

Use of Natural Language Processing to Identify Sexual and Reproductive Health Information in Clinical Text.

OBJECTIVES: This study aimed to enable clinical researchers without expertise in natural language processing (NLP) to extract and analyze information about sexual and reproductive health (SRH), or other sensitive health topics, from large sets of clinical notes. METHODS: (1) We retrieved text from the electronic health record as individual notes. (2) We segmented notes into sentences using one of scispaCy's NLP toolkits. (3) We exported sentences to the labeling application Watchful and annotated subsets of these as relevant or irrelevant to various SRH categories by applying a combination of regular expressions and manual annotation. (4) The labeled sentences served as training data to create machine learning models for classifying text; specifically, we used spaCy's default text classification ensemble, comprising a bag-of-words model and a neural network with attention. (5) We applied each model to unlabeled sentences to identify additional references to SRH with novel relevant vocabulary. We used this information and repeated steps 3 to 5 iteratively until the models identified no new relevant sentences for each topic. Finally, we aggregated the labeled data for analysis. RESULTS: This methodology was applied to 3,663 Child Neurology notes for 971 female patients. Our search focused on six SRH categories. We validated the approach using two subject matter experts, who independently labeled a sample of 400 sentences. Cohen's kappa values were calculated for each category between the reviewers (menstruation: 1, sexual activity: 0.9499, contraception: 0.9887, folic acid: 1, teratogens: 0.8864, pregnancy: 0.9499). After removing the sentences on which reviewers did not agree, we compared the reviewers' labels to those produced via our methodology, again using Cohen's kappa (menstruation: 1, sexual activity: 1, contraception: 0.9885, folic acid: 1, teratogens: 0.9841, pregnancy: 0.9871). CONCLUSION: Our methodology is reproducible, enables analysis of large amounts of text, and has produced results that are highly comparable to subject matter expert manual review.

Factors associated with pubertal growth outcomes in cystic fibrosis: Early Growth and Puberty in CF.

BACKGROUND: Pubertal delays in children with cystic fibrosis (CF) have historically been common. It is unclear to what degree puberty is affected in the new era of CF care or the role of early nutritional status. We hypothesized that more favorable early growth trajectories are associated with improved pubertal growth outcomes. METHODS: We used data from the United States CF Foundation Patient Registry to analyze associations between early weight-for-length/body mass index (WFL-BMI) growth trajectories and pubertal outcomes, using peak height velocity (PHV) and age at PHV (APHV) as proxy measures for puberty in addition to adult height (defined as height at age 18 years). Our analysis consisted of shape invariant mixed modeling and multivariable linear regression. RESULTS: Our sample consisted of 9,186 people with CF aged 18 to 21 years between 2010-2019. APHV was earliest and PHV/adult height were highest in those with WFL-BMI always >50th percentile from 0-6 years. However, there was no difference after adjusting for key covariates. Receiving CF transmembrane conductance regulator (CFTR) modulator therapy in childhood was associated with being taller at 18 years, by 0.92 cm in males (p=0.048) and 1.02 cm in females (p=0.010) in adjusted models. Higher height z-score at 2 years was associated with improved APHV and PHV for males and improved adult height for both males and females (p<0.001) in adjusted models. CONCLUSIONS: Early height, but not early WFL-BMI trajectories, may be associated with pubertal growth outcomes. CFTR modulator therapy shows the potential to improve pubertal growth outcomes, but further research is necessary.

School-based diabetes care: A national survey of U.S. pediatric diabetes providers.

Objectives: To understand the practices, attitudes, and beliefs of type 1 diabetes (T1D) providers towards school-based diabetes care (SBDC), including counseling families and communicating with schools, and explore the barriers and facilitators which affect their support of SBDC. Research Design and Methods: We conducted a national survey of pediatric T1D providers about their perceived support of SBDC, including family counseling and school communication. We used descriptive statistics to analyze results and explored differences by practice size (<500, 500-999, and ≥1000 patients) and environment (academic vs non-academic). Results: A total of 149 providers completed the survey. Nearly all (95%) indicated SBDC was very important. Though most (63%) reported counseling families about SBDC multiple times per year, few (19%) spoke with school staff routinely, reporting that was a shared responsibility among different providers. Close to 90% agreed school feedback on T1D management plans would be helpful, yet only 31% routinely requested this input. Moderate to extremely significant barriers to SBDC communication included internal factors, such as staff resources (67%) and time (82%), and external factors, such as school nurse education needs (62%) and differing school district policies (70%). Individuals from large or academic practices reported more barriers in their knowledge of SBDC, including federal/state laws. Desired facilitators for SBDC included a designated school liaison (84%), electronic transmission for school forms (90%), and accessible school staff education (95%). Conclusions: Though providers universally agree that SBDC is important, there are multilevel internal (practice) and external (policy) barriers to facilitating a bidirectional relationship between schools and health teams.

School Nurse Confidence with Diabetes Devices in Relation to Diabetes Knowledge and Prior Training: A Study of Convergent Validity.

Objective: The Diabetes Device Confidence Scale (DDCS) is a new scale designed to evaluate school nurse confidence with diabetes devices. We hypothesized that DDCS score would be associated with related constructs of school nurse diabetes knowledge, experience, and training. Research Design and Methods: In a cross-sectional study, we co-administered the DDCS and Diabetes Knowledge Test 2 (DKT2) questionnaires to school nurses in Pennsylvania. We summarized DDCS scores (range 1-5) descriptively. We evaluated the relationship between DKT2 percent score and DDCS mean score with the Spearman correlation coefficient. Simple linear regression examined school nurse characteristics as predictors of DDCS score. Results: A total of 271 completed surveys were received. Mean DDCS score was 3.16±0.94, indicating moderate confidence with devices overall. School nurses frequently reported low confidence in items representing specific skills, including suspending insulin delivery (40%), giving a manual bolus (42%), knowing when to calibrate a continuous glucose monitor (48%), changing an insulin pump site (54%), and setting a temporary basal rate (58%). Mean DKT2 score was 89.5±0.1%, which was weakly but not significantly correlated with DDCS score (r=0.12, p=0.06). Formal device training (p<0.001), assisting ≥5 students with diabetes devices in the past 5 years (p<0.01), and a student caseload between 1000-1500 students (p<0.001) were associated with higher mean DDCS score. Conclusions: DDCS score is related to prior training and experience, providing evidence for the scale's convergent validity. The DDCS may be a useful tool for assessing school nurse readiness to use devices and identify areas to enhance knowledge and practical skills.

A provider survey assessing fetal impact of CFTR modulator use in males with CF during assisted and unassisted reproduction and partner pregnancy.

BACKGROUND: Most males with cystic fibrosis (mwCF) are infertile but with CF transmembrane conductance regulator (CFTR) modulator-conferred benefits, more are utilizing assisted reproductive technologies (ART). Administration of normal human doses of modulators in animal reproductive models caused no genotoxicity; no human data exists. Potential health decline following modulator discontinuation makes the decision to withhold therapy during reproduction challenging. METHODS: From August-October 2021, international CF clinicians completed an anonymous questionnaire regarding mwCF who used modulators during reproduction. RESULTS: We received 42 surveys for mwCF with partner pregnancies. Forty of 42 mwCF utilized ART; 35 continued modulators during sperm retrieval and 40/42 during partner pregnancy. One of four males who discontinued modulators experienced clinical deterioration. First trimester miscarriages occurred in 11.9 % of partner pregnancies. No congenital anomalies were reported. CONCLUSIONS: Use of CFTR modulators during reproduction and partner pregnancy in mwCF did not result in a higher-than-expected miscarriage rate nor congenital anomalies.

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.

The modern landscape of fertility, pregnancy, and parenthood in people with cystic fibrosis.

PURPOSE OF REVIEW: With improved long-term survival and the expanding availability of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies that treat the underlying genetic defect in cystic fibrosis, more people are interested in parenthood. Cystic fibrosis care centers and people with cystic fibrosis need more information to guide decisions related to parenting. RECENT FINDINGS: Here we present currently available data on fertility, pregnancy, and parenthood in the modern era of cystic fibrosis care. Fertility may be improving in female individuals with cystic fibrosis with the use of CFTR modulator therapies, and there is an associated increase in annual pregnancies. Infertility in male individuals with cystic fibrosis remains approximately 97-98% and is unchanged with CFTR modulators in those already born with cystic fibrosis. As more female individuals with cystic fibrosis experience pregnancy, questions remain about the impact of pregnancy on their health and that of their child. Fortunately, there are multiple routes to becoming a parent; however, more work is needed to understand the impact of pregnancy and parenthood in the context of CF as some previous data suggests potential challenges to the health of parents with cystic fibrosis. SUMMARY: We encourage cystic fibrosis care teams to have knowledge and resources available to support the reproductive goals of all individuals with cystic fibrosis.