Abstract
Nutritional status and weight are critical to cystic fibrosis (CF) care; however, this emphasis increases risk for body image concerns. Limited research has examined body image and esteem in people with CF (PWCF) after the approval of elexacaftor/tezacaftor/ivacaftor (ETI), a highly effective gene modulator that can cause significant weight gain for those taking it. This study investigated associations among body image, body esteem, and weight status in 375 PWCF, ages 7–74 years (M = 26), taking ETI. Participants completed validated questionnaires assessing body image dissatisfaction and body esteem. Weight status was categorized based on BMI/BMI percentile. Most (65%) PWCF endorsed body image dissatisfaction. Body esteem and dissatisfaction differed between sexes, across age groups and weight status. PWCF who wanted to be thinner had the lowest body esteem. Results suggest that contemporary CF care ideally should assess and address body image concerns in PWCF, particularly those most at risk.
Introduction
Achieving optimal weight and nutritional status has been a foundational component of cystic fibrosis (CF) care, as research consistently documents associations among BMI, life expectancy, and pulmonary outcomes, including lung function (Hanna and Weiner, 2015; McPhail et al., 2008; Stallings et al., 2008; Steinkamp and Wiedemann, 2002; Welter et al., 2022; Zemel et al., 2000). Historically, malnutrition and lack of weight gain was standard for PWCF. Females with CF typically reported a more positive body image than males due to their lower body weight and thinness, whereas males with CF reported dissatisfaction with their perceived weight and wished to be more muscular (Tierney, 2012), aligning with Western cultural ideals. PWCF who perceived themselves as underweight were more likely to be adherent to CF cares that promote weight gain (e.g. enzymes, nutritional supplements), whereas those who were satisfied with their body size (even if they were objectively underweight) were less likely to do so (Walters, 2001)).
The release of elexacaftor/tezacaftor/ivacaftor (ETI), however, shifted the landscape of weight for PWCF. Improved weight gain for PWCF taking ETI is likely multifactorial, resulting from improved nutritional absorption, increased appetite and nutritional intake, and reduced energy expenditure due to improvements in pulmonary disease (Gramegna et al., 2023; Mouzaki et al., 2023; Petersen et al., 2022; Wisniewski et al., 2022). Reports (e.g. Bailey et al., 2022) indicate that after the introduction of ETI, the prevalence of overweight and obesity increased significantly in the CF population—a marked change from the typical underweight/malnourished profile that previously defined CF. However, research has yet to clarify whether the rapid weight gain experienced by many PWCF taking ETI has shifted body image perceptions, as most CF-specific body image research occurred prior to the availability of ETI. The emphasis on nutrition and weight status currently remains a critical component of CF medical care; however, this focus can foster a preoccupation that places some people with CF (PWCF) at risk for body image concerns (Lyons et al., 2024; Simon et al., 2020;). Consequently, it is important for research to explore the association of weight and body image in the context of the ETI era.
In addition, pre-ETI body image research targeted only adolescents and young adults with CF and did not explore how body image presents among children or middle-aged and/or older adults. Given that ETI is available to children as young as age 2 and that ETI has contributed to increased life expectancy (Cystic Fibrosis Foundation, 2023), it is essential to understand how body image may vary across development, and between males and females. Body image encompasses a person’s thoughts, feelings, and perceptions about their body (Grogan, 2016). Positive body image fosters emotional and physical wellbeing, including improved mental health and quality of life, self-acceptance, confidence, and engagement in health-promoting behaviors, whereas negative body image can contribute to low self-esteem, depression, anxiety, and increased risk of disordered eating (Tylka and Subich, 2002). Body dissatisfaction and body esteem are two components of body image, and differ in that body dissatisfaction describes a person’s evaluation of their body shape and size (Smolak and Thompson, 2009), while body esteem refers to an individual’s perception of their physical appearance and how their body is viewed by others (Mendelson et al., 2001), beyond shape and size. No research to date has examined the construct of body esteem in PWCF, and it may differ from body dissatisfaction when considering age and sex.
We aimed to describe how body dissatisfaction and body esteem present for PWCF across age groups, between sexes, and among those in different weight status categories (e.g. underweight, healthy weight, overweight, and obese). It was expected that across age groups, females with CF would have greater body dissatisfaction and lower body esteem than males based on trends seen in research pre-ETI, and that PWCF who are considered overweight and/or obese may also have lower body esteem and body dissatisfaction. As no research currently exists on age group differences in body esteem and body dissatisfaction for PWCF on ETI, we aimed to explore and describe differences that emerged among children, adolescents, young adults, middle adults, and older adults with CF.
Methods
Participants and procedures
The “your ETI” (yETI) study was approved by Boston Children’s Hospital Institutional Review Board (IRB), which served as the sIRB. Participants were recruited from 18 U.S. pediatric and adult CF centers affiliated with the Cystic Fibrosis Foundation’s (CFF) Success with Therapies Research Consortium (STRC, Sawicki et al., 2023) between March and July 2024. Eligible participants were PWCF aged 7 and older prescribed ETI for at least 18 months, not listed for transplantation or self-reported pregnancy. Consent and assent, as applicable, were obtained for all participants.
Demographic information was collected, with caregiver reports used for children. Forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) were extracted from the medical record and FEV1 percent predicted (FEV1pp) calculated using the race-neutral Global Lung Initiative Calculator (Quanjer et al., 2012). The highest FEV1pp in the year leading up to the survey was used. Height and weight were abstracted from medical records at the date closest to enrollment, and BMI/BMI percentile (BMIp) were calculated (CDC, 2024). Number of years on ETI was calculated as the difference between the survey date and the date first taken (obtained via medical record if available, or participant report). All participants were asked to complete a survey (online for adolescents, adults, and caregivers; verbally administered to children ages 7–12 years).
Measures
Weight status (Szentpetery, 2023) was categorized as underweight (BMIp < 10% for participants < 18 years; BMI < 18 kg/m2 for adults), healthy weight (BMIp between 10% and 85%; BMI between 18 and 25 kg/m2 for adults), overweight (BMIp between 85% and 95%; BMI between 25 and 29.9 kg/m2 for adults), or obese (BMIp ⩾ 95%; BMI ⩾ 30 kg/m2 for adults).
Body dissatisfaction was measured using the Kids’ Eating Disorder Survey (KEDS; Childress et al., 1993) for children (ages 7–12) and Figure Rating Scale (FRS; Stunkard et al., 1983) for adolescents and adults (ages 13+). Both measures include schematic female and male silhouettes, ranging from very thin to obese. Participants were asked to rate their current and ideal body image. Discrepancies between current and ideal body image indicated body dissatisfaction. Individuals were categorized as “do not want to change” (no discrepancy), “want to be thinner” (ideal < current), or “want to be larger” (ideal > current).
Body esteem was assessed using the Body-Esteem Scale (BES; Mendelson and White, 1985), a 20-item measure of self-evaluation of body image using a yes/no scale of agreement and a score range of 1–20. Higher scores indicate more positive body image. Statements on the BES include: “I like what I see when I look in the mirror,” and “I often wish I looked like someone else.” The BES has been validated with children as young as 7 years old (Mendelson and White, 1982), and has strong psychometric properties established with school-aged children (α = 0.88; Mendelson et al., 1996; Mendelson and White, 1993; Shriver et al., 2013). Cronbach’s alpha in this sample was high for children (α = 0.90), adolescents (α = 0.91), and adults (α = 0.92).
Statistical analyses
Descriptive statistics were presented for all variables. Welch’s analysis of variance (ANOVA) was used to compare body esteem scores across age groups (children (aged 7–12), adolescents (13–17), emerging adults (18–25), young adults (26–35), and older adults (36+)) to accommodate heterogeneity of variance. Welch’s t-test was used to compare body esteem scores between males and females. Chi-squared tests of independence and Fisher’s Exact tests were used to examine associations between categorical variables, including direction of body dissatisfaction and weight status. Post-hoc pairwise comparisons for both chi-squared and ANOVA tests were conducted using Bonferroni adjustments for multiple comparisons. Pairwise deletion was employed, such that participants with missing BMI/BMI percentile, BES scores, and body dissatisfaction data were excluded from analyses. Comparisons by age group and sex assigned at birth were not conducted to compare weight status or direction of dissatisfaction within underweight, overweight, and obese groups due to small cell sizes. Statistical tests were two-tailed with a significance threshold of p < 0.05. All analyses were performed using Statistical Analysis System (SAS) version 9.4.
Results
Sample sociodemographic and health status characteristics
A total of 415 participants consented to the study, with 356 (86%) having sufficient data for inclusion in the primary analyses (i.e. BMI/BMI percentile, BES scores, and body dissatisfaction). Socio-demographic characteristics and health status are reported in Table 1. Participants were on average 26 years old (range 7–74 years). For each age group, average BMI and BMI percentiles fell within the healthy range. Mean FEV1pp across all age groups was 95.7%; the highest FEV1pp values were seen in younger participants and declined with age. On average, participants had been taking ETI for 3.9 years (SD = 0.84). Demographic and health characteristics were similar for participants included in analyses and those excluded due to missing data.
Participant sociodemographic and health status characteristics.
Age of participants ranged from 7 to 74 years. For participants < 18 years old, BMI percentile was used; for those ⩾18 years, BMI was used. Years on ETI ranged from 1.54 to 6.16 years. Percentages may not add to 100 due to rounding. Missing data: sex (n = 1, <1%), race (n = 6, 2%), ethnicity (n = 1, <1%), annual household income (n = 69, 19%), health insurance (n = 7, 2%), FEV1 percent predicted (n = 2, 1%), years on ETI (n = 10, 3%).
BMI: body mass index; FEV1: forced expiratory volume in the first second.
Relation among weight status, direction of body dissatisfaction, and body esteem
A chi-squared test of independence revealed that direction of body dissatisfaction varied significantly by weight status (Χ2 = 75.31, p < 0.001). Pairwise comparisons using chi-squared and Fisher’s Exact tests showed significant differences in direction of body dissatisfaction between individuals within a healthy weight range and those who were overweight (Χ2 = 41.99, p < 0.001) or obese (Χ2 = 30.51, p < 0.001). None of the participants who were underweight reported they wanted to be thinner, compared to the 60.8% of overweight and 70.3% of obese participants (both p < 0.001; Fisher’s exact test; Figure 1).
Comparison of body dissatisfaction by weight status. The proportion of individuals in each weight status category (underweight, healthy weight, overweight, obese) who reported different directions of body dissatisfaction (do not want to change, want to be thinner, want to be larger). Statistical comparisons were conducted using chi-squared or Fisher’s Exact tests with Bonferroni-adjusted post-hoc comparisons. **p < 0.001 and ns denotes non-significant comparisons.
The association between BES scores and weight status was evaluated using Welch’s ANOVA. BES scores were significantly associated with weight status [F(3, 56.44) = 10.64, p < 0.001, Figure 2]. Post-hoc pairwise comparisons using Bonferroni adjustment for multiple comparisons showed that individuals who were obese had significantly lower BES scores (M = 10.1, SD = 6.30) compared to individuals within the healthy weight range (M = 15.9, SD = 4.50, p < 0.001). Overweight individuals also had significantly higher BES scores (M = 14.2, SD = 5.77) compared to obese individuals (p < 0.001).
Body esteem scores by weight status. Body esteem scale (BES) scores across weight status categories (underweight, healthy weight, overweight, obese). Higher scores indicate more positive body esteem. Statistical comparisons were conducted using Welch’s ANOVA with Bonferroni-adjusted post-hoc tests. **p < 0.001 and ns denotes non-significant comparisons.
A Welch’s ANOVA was conducted to examine differences in BES scores across direction of body dissatisfaction. Results showed significant association between body dissatisfaction direction and BES scores [F(2,194.1) = 100.66, p < 0.001]. Post-hoc pairwise comparisons using Bonferroni correction showed that participants who did not want to change (M = 18.5, SD = 2.32) had significantly higher BES scores compared to those who wanted to be larger or thinner (M = 15.0, SD = 4.51 and M = 11.4, SD = 5.58, respectively; both p < 0.001). Additionally, those who wanted to be larger had significantly higher BES scores compared to those who wanted to be thinner (p < 0.001; Figure 3).
Body esteem scores by body dissatisfaction. Body esteem scale (BES) scores by direction of body dissatisfaction. Higher scores indicate greater body esteem. Statistical comparisons were conducted using Welch’s ANOVA with Bonferroni-adjusted post-hoc tests. **p < 0.001.
Variations in body dissatisfaction and body esteem by weight status, age, and sex
The average BES score for the total sample was 14.9 (SD = 5.29; Table 2). Body esteem varied significantly across age groups (F(4, 168.3) = 12.75, p < 0.001), with the highest mean score among child participants (M = 17.9, SD = 2.90) and the lowest mean score among adults aged 36+ (M = 13.4, SD = 5.56). Male participants had a significantly higher average score (M = 16.0, SD = 4.48; p < 0.001) than females (M = 13.9, SD = 5.82; t(350.41) = 3.59, p ⩽ 0.001). Most participants expressed body dissatisfaction (65%); however, this did not differ significantly across age groups or when comparing male and female participants (Χ2 = 7.48, p = 0.112 and Χ2 = 0.0002, p = 0.989 respectively). Across age groups, the percentage of participants who expressed body dissatisfaction ranged from 58% amongst adolescents to 73% of adults aged 26–35 years. Additionally, 65% of both male and female participants expressed body dissatisfaction.
Participant body esteem, body dissatisfaction, and weight status by age and sex.
Percentages may not add to 100 due to rounding or missing data. One participant classified as obese was missing data for sex and was excluded from sex-specific comparisons. p-Values calculated using Welch’s ANOVA and chi-square tests. Comparisons by age and sex were not analyzed for the underweight, overweight, and obese categories due to insufficient sample size. For participants <18 years old, BMI percentile was used; for those ⩾18 years, BMI was used.
BMI percentile < 10% or BMI < 18.5 kg/m2.
BMI percentile between 10% and 84.9% or BMI between 18.5 and 24.9 kg/m2.
BMI percentile between 85% and 95% or BMI between 25 and 29.9 kg/m2.
BMI percentile > 95% or BMI ⩾ 30 kg/m2.
Patterns of direction of body dissatisfaction within different categories of weight status across age groups and comparing male and female participants are described in Table 2. Due to small sample sizes in some groups, we did not formally test these differences for statistical significance within the underweight, overweight, and obese weight categories. Most participants in the overall sample were within the healthy weight range (64%), followed by overweight (21%), obese (10%), and underweight (5%). Among underweight participants, the majority (61%) wanted to be larger, while most individuals categorized as overweight or obese wanted to be thinner (61% and 70% respectively). The healthy weight group was more evenly distributed, with 37% not wanting to change, 37% wanting to be larger, and 26% wanting to be thinner.
Across age groups, participants within the healthy weight range did not differ significantly in the direction of their body dissatisfaction (Χ2 = 7.93, p = 0.441). However, when comparing male and female participants in the healthy weight range, a significant difference was observed (Χ2 = 19.62, p < 0.001). Males were significantly more likely to express a desire to be larger (52%), whereas females were more likely to report that they did not want to change (42%) or wanted to be thinner (34%).
Discussion
This study explored body dissatisfaction, body esteem, and weight status among PWCF across age and between sexes in the post-ETI era. Most participants were within the healthy weight range; however, a notable proportion, particularly among older adults, were classified as overweight and only a small minority were underweight. Despite most PWCF falling within the healthy weight range, approximately two-thirds expressed body dissatisfaction. Underweight participants more frequently desired a larger body, while overweight and obese participants more often wanted to be thinner.
PWCF classified as obese overwhelmingly reported the lowest body esteem across weight status groups. These findings are critical when considering the emerging prevalence of overweight and obesity in PWCF post-ETI and its potential impact on health. Although increased weight is understood to produce better pulmonary outcomes (Stallings et al., 2008) there is no evidence that being overweight or obese confers additional benefit to lung health; instead, obesity-related adverse health outcomes may arise (Bailey et al., 2022).
While a desire to lose weight in individuals with obesity may support physical health, it may also be psychologically distressing, particularly given our finding that body esteem was lowest among this group. Wanting to be thinner may also reflect internalized body dissatisfaction and negatively impact mental well-being. CF care that includes conversations about weight and weight loss must therefore be collaborative, personalized, and non-stigmatizing to prioritize both physical and psychological wellbeing. Clinicians should prioritize health-promoting behaviors and/or markers of health other than BMI (Bailey et al., 2022; e.g. cardiovascular health; lean body mass and/or body composition changes) that can encourage comprehensive health improvements. Interdisciplinary collaboration is also necessary to provide interventions that can target the psychological components of weight, body dissatisfaction, and body esteem as they relate to CF.
A unique contribution of this study is the connection between body dissatisfaction and body esteem. To our knowledge, no previous studies have explored this connection in the CF population. PWCF who were satisfied with their body shape and size had significantly higher body esteem than those who wanted to be thinner or larger. Importantly, those who expressed a desire to be thinner had the lowest body esteem of all groups. This is consistent with research showing that the “thin ideal” is pervasive among people in Westernized cultures and has detrimental impacts on psychological wellbeing and self-esteem (Frederick et al., 2022). Providers should be attuned to comments regarding body size, as they may reflect more complex feelings about body esteem in general. Body esteem may be a potential target for intervention; providers can focus on a person’s overall sense of value and respect for their body, thereby fostering a more positive (or neutral) body image. More research is needed though, to fully understand how wanting to be larger versus thinner may differently impact body esteem among PWCF.
Age group and sex differences in body dissatisfaction and body esteem
Body esteem uniformly worsened with age, with the highest body esteem reported among children aged 7–12 years and the lowest among adults aged 36+. Body dissatisfaction followed a similar trend, except for adults aged 26–35 years, who reported the highest levels of dissatisfaction across all groups. This pattern may reflect the cumulative impact of chronic illness, societal pressures on body image, and the increased likelihood of weight gain that accompanies aging. Notably, adults in this sample were approximately 50% more likely to be overweight than younger age groups.
These trends may also relate to when individuals began ETI treatment. Given that ETI has been approved for approximately 6 years, adults included in this study may have been more cognitively aware of changes in their experience with CF pre- and post-ETI initiation. In contrast, children and adolescents may have been somewhat protected from these changes solely due to their developmental stage and level of awareness. However, because this study did not collect pre- and post-ETI weight data, we cannot determine whether post-ETI weight gain contributed to declining body image. Retrospectively examining change in BMI and/or BMI percentile pre- and post-ETI initiation and impacts on body image is an important area for further investigation.
Adolescents in this sample had the next highest body esteem after children, which is somewhat surprising given adolescence is often considered the peak developmental period for experiencing body image concerns (Bucchianeri et al., 2013). Prior to ETI, adolescents with CF were no exception to this trend (Tierney, 2012). The relatively high body esteem seen in adolescents included in this study could be reflective of several factors that warrant further investigation, including cultural and/or societal changes toward body ideals for younger people, greater awareness and discussion of mental health in general, or even factors related to overall ETI-induced health improvements and the changing landscape of CF care.
Consistent with pre-ETI research (Tierney, 2012), we found sex differences in both body esteem and body dissatisfaction. Among PWCF in the healthy weight range, males were more likely to desire to be larger and females were more likely to desire thinness or remain the same size. Despite falling objectively into the healthy weight range, men were still more likely to want to be larger and women thinner or the same. This is clinically meaningful in that PWCF in the healthy weight range also are at risk for body image concerns despite objective markers of positive health, such as BMI. Clinicians should tailor conversations about body image to include all PWCF regardless of objective weight class and consider how cultural messages about men’s versus women’s bodies may impact approaches to CF care.
Importantly, this study did not include gender-diverse PWCF, and only utilized sex assigned at birth. The unique needs of transgender and gender-diverse individuals with CF is understudied and highly important (Shaffer et al., 2022), especially considering that body image concerns are nuanced among this population (Nowakowski, 2019). Future research should prioritize this vulnerable group, to fully explore the complexities facing gender-diverse individuals.
Clinical implications
Our findings can guide clinicians in conversations about body image, weight, and esteem for PWCF in the post-ETI era. Body image, although highly important to PWCF, is not regularly addressed as a routine part of clinical care (Helms et al., 2017) and can be somewhat uncomfortable for providers to bring up (Kass et al., 2023). Given the large number of PWCF in our sample who endorsed body dissatisfaction, providers should be aware of the potential for body image concerns in PWCF on ETI. Interventions for body image concerns should first be approached by simply asking how PWCF feel about their bodies and/or body changes experienced after ETI initiation. Validating and actively listening to concerns are also recommended, as discussing weight and body size can be challenging and create vulnerability.
Routine screening for body image issues should be integrated into CF care, with targeted interventions to support those identified as at risk. For instance, interventions that focus on body functionality (Alleva et al., 2018; e.g. shifting focus from how the body looks to what it can do), incorporate independent exercise (Annesi, 2005), include self-compassion (Rodgers et al., 2018), and are delivered in a group format (Alleva et al., 2015) have been shown to be effective in adolescents and adult women with and without chronic illnesses (Guest et al., 2019). Children and adolescents tend to benefit from interventions that include cognitive dissonance, peer support, and psychoeducation (Guest et al., 2022). Of note, research on effectiveness of body image interventions for men is limited; therefore, specific strategies may differ when addressing male body image. Some evidence supports cognitive dissonance-based approaches as effective tools for improving body image in men (Hendricks et al., 2023), but more research is needed. In general, promoting a holistic approach to CF care for all PWCF, including psychological support and interventions, can help mitigate the negative impact of body dissatisfaction and improve well-being.
Limitations and future research
To date, this was the first large-scale study to explore body image in PWCF across age ranges. Despite the large number of participants, the most significant limitation was that only 18 PWCF were classified as underweight. This restricted the ability to understand body image and body esteem in this group, and the ability to compare weight status across age and sex. Although the small number of underweight individuals likely reflects the vast weight-related improvements typically seen for PWCF on ETI, it is critical to understand body image in this group as they are now among the minority.
This study required participants to be prescribed ETI for 18 months, and did not include PWCF who discontinued ETI use for any reason, including aversive weight gain. Body image concerns may look entirely different among PWCF who found ETI-related weight gain to be distressing enough to discontinue its use. Future research would benefit from examining body image dissatisfaction and esteem in PWCF irrespective of their modulator use, to observe if similar trends across age and weight status groups emerge. Another key limitation was lack of retrospective data on BMI changes before and after ETI initiation. Weight status was assessed only at the time of enrollment, limiting our ability to understand how shifts in weight over time may influence body image dissatisfaction and body esteem. The small sample sizes when examining age-group differences and the convenience sampling method may limit generalizability of our findings to the broader CF population. As our participant cohort was predominantly White, with low representation from those who identified as Hispanic and African American, this also limits the generalizability of our results to the more racially and ethnically diverse CF population.
Finally, future research should employ longitudinal designs and include qualitative follow-up to track changes in body image over time and explore underlying mechanisms driving these changes. Expanding the sample to include more diverse populations and examining the role of cultural factors in body image perceptions will enhance the utility and generalizability of our findings.
Conclusion
This study highlights the complex interplay between weight status, body image, and body esteem for PWCF who have been prescribed ETI. While ETI has brought significant health benefits, it also introduces new challenges for body image, particularly among older adults and females. These findings underscore the need for comprehensive, age-appropriate, and sex-specific interventions for optimizing the overall health and well-being of PWCF.
Footnotes
Acknowledgements
We would like to thank our community partners for participating, and teams at STRC sites for assistance with recruitment and data collection: Boston Children’s Hospital: PI Carolyn Snell, PhD and RC Sara Carvalho; Children’s Colorado: RC Ally Keck; Cincinnati Children’s Hospital: PIs Christopher Siracusa, MD and Stephanie Filigno, PhD and RC Kira Rehn; Johns Hopkins University: RC Austin Pucci; Kansas University Medical Center: PI Amanda Bruce, PhD and RC Megan Behrman; Michigan University: PI Jennifer Butcher, PhD and RCs Dawn Kruse and Jessica Carey; National Jewish Health: PI Milene Saavedra, MD and RC Katie Poch; Northwestern University: PI Michelle Prickett, MD and RCs Rachel Nelson and Alvaro Donayre; Stanford University: PI Diana Naranjo, PhD and RC Jackie Spano; SUNY Buffalo: PI Carla Frederick, MD and RCs Julianne Hergenroder, Nadine Caci, and Christine Roach; University of Alabama Birmingham: PI Gabriela Oates, PhD and RC Jonathan Bergeron; University of Texas Southwestern: PI Preeti Sharma, MD and RCs Aruna Ayalasomayajula, Keianna Brown, and Lindsay Allen; Virginia Commonwealth University: PI Michael Schechter, MD MPH and RC Akilah Pierre-Louis; West Virginia University: PI Casandra Arevalo-Marcano, MD and RC Tammy Clark.
Ethical considerations
This study received ethics approval from the Boston Children’s Hospital Institutional Review Board (IRB-P00046466).
Consent to participate
All participants provided informed consent and assent, when applicable, to participate in the study and for their data to be used.
Consent to publication
All participants provided informed consent and assent, when applicable, to participate in the study and for their data to be used.
Funding
The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was funded by the Cystic Fibrosis Foundation grants YETI23PE0, RIEKER23PE0, and SAWICK14PE1. All authors received grants and/or salary support from the CFF.
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Data availability statement
The datasets generated and/or analyzed during the current study are available from the corresponding author on reasonable request.