Parental perceptions of body weight and appetite in infants and toddlers with cystic fibrosis.

Nutritional status has clinical relevance and is a target of guidance to parents of children with cystic fibrosis (CF). Growth is routinely monitored in CF clinics but there is no standardized way of assessing appetitive behaviors or parents' perceptions of their children's appetite. Greater understanding of these factors could improve clinical guidance regarding parent feeding behaviors. We therefore aimed to assess parent perceptions of child weight, and parent reports of child appetite using the Baby Eating Behavior Questionnaire (BEBQ), in a sample of infants and toddlers with CF, compared with a community sample. We additionally assessed relationships of parent perceptions of child weight with parent feeding behaviors in the sample with CF. Anthropometric and questionnaire data were collected for 32 infants and toddlers with CF, as well as 193 infants and toddlers drawn from RESONANCE, a community cohort study. Parents perceived children with CF to be lower in weight than their actual weight, to a greater extent than was evident in the community sample. Parents who perceived their children with CF to be underweight vs. right weight reported greater slowness in eating on the BEBQ. Parents perceived children with CF to have greater slowness in eating and lower enjoyment of food, compared to parents of children in the community sample, independent of sample differences in child weight, age, and sex. Our results demonstrate the potential utility of the BEBQ in a clinical sample and suggest it may be helpful for clinicians to assess parents' perceptions of their child's weight and appetite to promote a fuller understanding of the child's nutritional status, facilitate appropriate feeding behaviors and alleviate unnecessary concerns.

A comparative study among adolescents with cystic fibrosis and primary ciliary dyskinesia and their caregivers: psychological aspects / Estudio comparativo entre adolescentes con fibrosis quística y discinesia ciliar primaria y sus cuidadores: aspectos psicológicos

This work aimed to conduct a comparative study between cystic fibrosis and primary ciliary dyskinesia among adolescents and their caregivers to understand their emotional adaptation to the disease. Thirty-nine dyads of adolescents aged 9-18 years and their primary caregivers were assessed, 69.20 % of whom had a diagnosis of cystic fibrosis. For the adolescents, anxious-depressive symptomatology was assessed using the HADS, perceived threat of disease (B-IPQ) and quality of life (CRQ-SAS). For the caregivers, emotional symptomatology (HADS) and perceived stress levels (PIP) were assessed. Mean comparisons were made according to diagnosis and severity of anxious-depressive symptomatology, relationships between variables were studied, and qualitative comparative analysis models, QCA, were performed. Higher levels of anxiety and depression were found in the caregiver than in the adolescent. Adolescents with primary ciliary dyskinesia have a more significant emotional impact than adolescents with cystic fibrosis. However, no differences were found in caregivers according to diagnosis. The adolescent's emotional state, quality of life and perceived threat of disease were related to and explained by the caregiver's emotional state, and vice versa. Therefore, there appears to be a certain degree of emotional contagion between the dyad members. This is a preliminary study that has not been carried out before and can help to understand the psychological aspects associated with these diseases to favour the patient's adjustment and thereby ensure more effective management of the disease and adherence to treatment. (AU)

El objetivo fue realizar un estudio comparativo entre la fibrosis quística y la discinesia ciliar primaria entre adolescentes y sus cuidadores para comprender su adaptación a la enfermedad. Evaluando 39 díadas de adolescentes de entre 9 y 18 años y sus cuidadores, el 69,20% de los cuales tenían un diagnóstico de fibrosis quística. Para los adolescentes, se evaluó la sintomatología ansioso-depresiva mediante el HADS, la amenaza percibida de la enfermedad (B-IPQ) y la calidad de vida (CRQ-SAS). Para los cuidadores, se evaluó la sintomatología emocional (HADS) y los niveles de estrés percibido (PIP). Se realizaron comparaciones de medias según el diagnóstico y la sintomatología ansioso-depresiva, se estudiaron las relaciones entre variables y se realizaron modelos de análisis cualitativo comparativo. Se encontraron mayores niveles ansioso-depresivos en el cuidador que en el adolescente. Los adolescentes con discinesia ciliar primaria presentan un impacto emocional mayor que los adolescentes con fibrosis quística. No se encontraron diferencias en los cuidadores según el diagnóstico. El estado emocional, la calidad de vida y la percepción de amenaza de la enfermedad del adolescente estaban relacionados con el estado emocional del cuidador y se explicaban por él, y viceversa. Por lo tanto, parece existir cierto grado de contagio emocional entre los miembros de la díada. Se trata de un estudio preliminar que no se ha realizado previamente y que ayuda a comprender los aspectos psicológicos asociados a estas enfermedades favoreciendo el ajuste del paciente y asegurar así un manejo más eficaz de la enfermedad y la adherencia al tratamiento. (AU)

Me, myself, and I: A systematic review of cystic fibrosis and self-concept.

Given extensive pertinent disease factors and evolving medical treatments, this systematic review explores qualitative and quantitative cystic fibrosis (CF) research surrounding self-concept, an overarching perception of self. Research methodologies, self-concept dimensions, prominent self-concept findings and clinical recommendations are identified. Preferred Reporting Items for Systematic Review and Meta-analyses guidelines were applied. PubMed, Scopus, Medline, Psycinfo, CINAHL (ebsco), and CENTRAL Cochrane electronic databases were searched from 2012 to 2022. Methodological quality was assessed using the critical appraisal skills program. Data-based convergent synthesis was applied to analyze and report on qualitative and quantitative studies in parallel. Thirty-seven publications met the inclusion criteria, most of which employed a cross-sectional, single-center design within an adolescent and adult population. Self-efficacy, self-esteem, and self-identity studies were dimensions of self-concept identified, with studies relating to self-efficacy surrounding physical health management most prevalent. All three dimensions were positively associated with improved treatment adherence and psychosocial health. Efficacy tested intervention programs to enhance self-concept are limited; however, an extensive range of clinical recommendations are offered, highlighting the importance of clinician self-concept awareness, quality clinician-patient conversations and online CF peer-support. Self-concept is an important mechanism to optimize patient outcomes. Further CF self-concept research is required, particularly multicenter, longitudinal, and interventional studies. Early childhood, post lung transplant and the older adult CF population in particular, lack research attention. Given the potential impact of rapidly evolving CF transmembrane conductance regulator modulator drugs on many aspects of self, future self-concept research beyond the dimension of self-efficacy may be beneficial.

Transcending Limitations: A Phenomenological Exploration of How Hygge Practices Enrich the Lived Experiences of Adults with Cystic Fibrosis.

Hygge practices embody joy, peace, mindfulness, coziness, and conviviality. Cystic fibrosis (CF) is a progressive condition with complex therapies and physical limitations. Little is known about how hygge practice may impact individuals living with CF. A qualitative study explored how adults with CF use hygge practices to promote wellness and cope with their disease. A purposive network sample of 15 adults with CF who utilized hygge practices completed semistructured audio-recorded telephone interviews. Recordings were transcribed and analyzed using Colaizzi's thematic analysis approach. Results reveal that hygge practices influenced individuals' aesthetics, attitudes, and activities, deeply impacting the physical and emotional experience of living with CF. Incorporating hygge into CF care may improve psychological well-being and quality of life for members of this community.

Preferences and perspectives regarding telehealth exercise interventions for adults with cystic fibrosis: A qualitative study.

OBJECTIVE: Physical activity and exercise are key components in the management of cystic fibrosis (CF). Completing exercise programs online may minimize the risk of cross-infection and increase access for people with CF. This study aimed to understand the perspectives of people with CF regarding intervention content for a telehealth exercise program. METHODS: Individual semistructured qualitative interviews were conducted in adults with CF purposefully sampled for age, disease severity, and social demographics. Interviews were recorded, transcribed verbatim, and analyzed thematically by two researchers independently. RESULTS: Participants were 23 adults with CF (14 females) aged from 21 to 60 years. Three major themes (subthemes) were generated: "Personalizing components to an exercise program" (customizing an exercise program to the individual person and their unique health and exercise needs, enjoyment and variety of exercise activities, accessibility and exercise fitting around competing demands or commitments), "The importance of maintaining connections" (challenges regarding face-to-face interactions for people with CF, accountability of scheduled exercise sessions with others, shared experiences between people with CF and specialist support from the CF care team), and "Monitoring health and exercise" (perception of health status and monitoring and recording exercise participation and health). CONCLUSION: This study provides important information regarding the preferences of adults with CF for telehealth exercise interventions. Interventions should be tailored to the individual person with CF, include an opportunity to maintain connections with peers and the CF multidisciplinary team, and provide a method to monitor progress over time.

Improvement in cystic fibrosis newborn screening program outcomes with genetic counseling via telemedicine.

INTRODUCTION: The Cystic Fibrosis Foundation (CF Foundation) recommends the provision of genetic counseling (GC) to help educate families and decrease anxiety around the cystic fibrosis (CF) newborn screening process. Unfortunately, access to genetic counselors is limited, especially for CF trained genetic counselors. We hypothesized that the GC process for families could be improved by utilizing telemedicine to leverage the availability of two dedicated, CF trained genetic counselors to provide access to GC for several CF centers. In addition, we hoped to demonstrate that use of trained CF genetic counselors, delivering GC via telemedicine at the time of sweat testing, would provide families with understanding of CF genetics as well as result in high satisfaction with the newborn screening process. METHODS: GC was provided by CF trained genetic counselors via telemedicine at the time of sweat testing. Following the counseling session, families were administered an anonymous written survey to evaluate their impression of the services provided. A subset of 50 families was recruited for an assessment of gained knowledge regarding CF genetics using the Ciske knowledge inventory. Using χ2  analysis, Ciske knowledge inventory data from our telemedicine GC families was compared to counseled and uncounseled Ciske historical controls. Lastly, in-depth interviews about the newborn screening process for CF were performed with 10 families and interviews were coded for emerging themes. RESULTS: During the 4 years of the study, 250 patients received GC. Overall comfort with the counseling rated 4.77 out of 5 using a Likert scale. After counseling by telemedicine, parents demonstrated improved understanding of the genetic implications of an abnormal CF newborn screen for their family, with 100% of families understanding that their child was a carrier for CF as compared to 97.2% of counseled (p = .023) and 78.5% of uncounseled (p = .0007) from Ciske historical controls. The study group also showed improvement in understanding of both parents possibly being carriers, with an 87.7% correct response rate compared to a 37.0% correct response rate in the counseled group (p < .0001) and a 35.4% correct response rate in the non-counseled group (p < .0001) from Ciske historical controls. Subgroup analysis at one site showed a significant increase in the number of infants with completed sweat tests from previous years (49% in 2013 vs. 80% in 2017 during the study, p < .0001). CONCLUSIONS: GC by telemedicine was well received by families and demonstrated improved family knowledge acquisition and understanding of CF as it related to risks for their child as well as identification of risks for other family members. Furthermore, in addition to an increase is those receiving GC, a subgroup analysis demonstrated a significant increase in the number of infants receiving sweat tests. This study demonstrates that GC via telemedicine for CF is feasible and demonstrates improvement in parent understanding of CF genetics. Furthermore, this method can be implemented effectively across a wide geographical area with a limited number of CF trained genetic counselors to improve access to care for patients and families.

Spanish adaptation and validation of the child- and parent-report cystic fibrosis questionnaire-revised (CFQ-R).

OBJECTIVE: To evaluate the psychometric properties of the Spanish versions of the child- and parent-report cystic fibrosis questionnaire-revised (CFQ-R). METHODS: A Spanish adaptation of the CFQ-R was performed; 68 children with CF (6-13 years) and their parents completed the child- and parent-report CFQ-R, respectively, and the Revidierter KINDer Lebensqualitätsfragebogen (KINDL) questionnaire. The CFQ-R was completed twice, 7-10 days apart, and its psychometric properties were analyzed. RESULTS: The internal consistency of both CFQ-R versions was adequate (child-report version, Cronbach's α >.60 for all domains except "Treatment Burden" [α = .42] and "Social Functioning" [α = .57]; parent-report version, α > .60 for all domains except "Social Functioning" [α = .58]). For the child-report version, the lowest measurement error was for "Emotional Functioning" (standard error of measurement [SEM]: 8.3%; minimal detectable change [MDC90 ]: 19.3%), and the highest was for "Body Image" (SEM: 15%; MDC90 : 35%). For the parent-report version, the lowest measurement error was for "Physical Functioning" (SEM: 7.1%; MDC90 : 16.5%), and the highest was for "Weight" (SEM: 17.2%; MDC90 ; 40.1%). The correlation between the versions showed higher agreement for the domains related to observable signs ("Physical Functioning") and lower agreement for "Emotional Functioning." There was a significant correlation between the CFQ-R and KINDL. CONCLUSION: Both the child- and parent-report versions of the Spanish CFQ-R have adequate reliability and validity for clinical and research purposes. These versions can be administered before and after starting modulator therapy to assess its effect on daily functioning. The MDC90 can help identify, with a high probability, whether real changes have occurred in the quality-of-life subscales in children with CF.

Alcohol and opiate misuse in adults with cystic fibrosis.

BACKGROUND: International consensus statements on depression and anxiety in adolescents and adults with cystic fibrosis (awCF) recommend assessment for comorbid substance misuse. However, at CF centers, the frequency and impact of substance misuse have not been well characterized, and best practices for prevention, identification, and evidence-based treatment have not been routinely implemented. METHODS: Medical records of 148 awCF over 3 years were reviewed to determine the prevalence of substance misuse (alcohol or opiates) and its relationship with clinical variables and healthcare utilization. Independent sample t test for continuous outcomes and χ2 test for binary outcomes were used to compare groups with and without substance misuse. RESULTS: Substance misuse was documented in 28 (19%) awCF, equally distributed between alcohol (n = 13) and opiates (n = 15). Adults with substance misuse were more likely to be male. The prevalence of diagnosed anxiety and depression did not differ significantly between groups, but those with substance misuse had more severe anxiety (Generalized Anxiety Disorder-7 Item [GAD-7]: 10.0 ± 6.1 vs. 3.3 ± 4.4; p < 0.001) and depressive symptoms (Patient Health Questionnaire-9: 10.4 ± 6.5 vs. 4.0 ± 4.8; p < 0.001). Adults with substance misuse had higher annual rates of missed outpatient CF visits, more frequent "sick" visits, more frequent and longer hospitalizations, and a higher mortality rate. CONCLUSIONS: In awCF, substance misuse is common and associated with adverse indicators of emotional and physical health, including via proxy of service utilization, suggesting that systematic approaches to addressing substance misuse in CF clinics should be considered. Prospective, longitudinal study is warranted to elucidate the complex relationships between depression, anxiety, substance misuse, and health outcomes in individuals with CF.

How personality influences health outcomes and quality of life in adult patients with cystic fibrosis.

BACKGROUND: The present study evaluates personality traits in adult patients with cystic fibrosis (CF) and correlates these results with health-related quality of life (HRQoL) and other clinical parameters indicative of disease severity. METHODS: Seventy adults completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R 14+), a CF-specific measure of HRQoL, and a self-administered questionnaire about personality traits and disorders. Mean subscale scores and the prevalence of extreme personality traits on the `Persönlichkeits-Stil- und Störungs-Inventar (PSSI)´ were compared to the norming sample. Moreover, a cluster analysis was conducted to identify personality styles among people with cystic fibrosis (pwCF). The relationship between mean PSSI subscale scores and personality clusters with HRQoL and clinical outcomes, e.g., percent predicted forced expiratory volume in one second (ppFEV1), and body mass index (BMI), was studied by regression analysis considering important confounders. RESULTS: On several of the subscales of the personality questionnaire, people with cystic fibrosis (pwCF) showed either significantly higher or lower scores than the norm sample. In further analyses, two personality clusters could be identified. PwCF from the cluster with predominantly low scores on the subscales 'negativistic', 'schizoid', 'borderline', 'depressed', and 'paranoid' showed better HRQoL than pwCF from the other cluster with mainly high normal or elevated scores. The studied health outcomes proved to be independent of the respective personality clusters. CONCLUSIONS: In pwCF, HRQoL is mainly determined by psychological factors, including personality. Since more recent personality theories assume that personality is modifiable, our findings imply that patients with accentuated personality traits may benefit from psychosocial support.

Integrating Primary Care into the Management of Cystic Fibrosis.

Over the last 50 years, cystic fibrosis has radically transformed from a fatal disease of infancy to a chronic disease of adulthood. By 2025 it is estimated that 70% of individuals with cystic fibrosis (iwCF) will be cared for in adult clinics. We believe the role of a dedicated primary care provider (PCP) for preventative care will be crucial for the longevity of iwCF. There are various models for incorporating primary care medicine into CF management, but no universally accepted standard exists. Ideally, the PCP and pulmonologist practice in a patientcentered medical home, given the growing evidence that these care models are associated with improved quality-of-life measures, mental health, and disease-specific outcomes. To improve engagement with primary care in CF, there needs to be a shift in education at the undergraduate medical education and provider training levels. Increasing the knowledge of CF-related illness is vital in fostering a close relationship between the PCP and their patient. To meet this need, primary care doctors will need tools and practical experiences in managing this rare condition. This can start being addressed by providing ample opportunities for the inclusion of PCPs into subspecialty clinics and through engagement with community providers through readily available didactics, seminars, and open lines of communication. As PCPs and CF clinicians, we feel that shifting the domain of preventative care to the expertise of a primary care physician will allow for a more CF-specific focus in subspecialty clinics and help prevent these vital health maintenance tasks from being overlooked, altogether advancing the health and well-being of iwCF.

National integration of mental health screening and treatment into specialized care for cystic fibrosis: What predicts success?

OBJECTIVES: The CF Foundation sponsored competitive awards for Mental Health Coordinators (MHCs) from 2016 to 2018 to implement the international guidelines for mental health screening and treatment in US CF centers. Longitudinal surveys evaluated success in implementing these guidelines using the Consolidated Framework for Implementation Research (CFIR). METHODS: MHCs completed annual surveys assessing implementation from preparation/basic implementation (e.g., using recommended screeners) to full implementation/sustainability (e.g., providing evidence-based treatments). Points were assigned to questions through consensus, with higher scores assigned to more complex tasks. Linear regression and mixed effects models were used to: (1) examine differences in centers and MHC characteristics, (2) identify predictors of success, (3) model the longitudinal trajectory of implementation scores. RESULTS: A total of 122 MHCs (88.4% responded): Cohort 1, N = 80; Cohort 2, N = 30; and Cohort 3, N = 12. No differences in center characteristics were found. Significant improvements in implementation were observed across centers over time. Years of experience on a CF team was the only significant predictor of success; those with 1-5 years or longer reported the highest implementation scores. Change over time was predicted by >5 years of experience. CONCLUSIONS: Implementation of the mental health guidelines was highly successful over time. Funding for MHCs with dedicated time was critical. Longitudinal modeling indicated that CF centers with diverse characteristics could implement them, supported by evidence from the CF Patient Registry showing nearly universal uptake of mental health screening in the United States. Years of experience predicted better implementation, suggesting that education and training of MHCs and retention of experienced providers are critical to success.

Psychometric properties of the Cystic Fibrosis Eating Attitudes and Behaviours scale (CFEAB) in an adult population.

OBJECTIVES: Individuals with Cystic Fibrosis (CF) may be at an increased risk of developing a range of eating difficulties. Scales designed to measure disordered eating in the general population do not cover CF-specific behaviours resulting in a knowledge gap. The CFEAB was developed as a CF-specific measure assessing eating behaviours and attitudes however little evidence exists regarding its psychometric quality. The aim of this cross-sectional study was to provide a robust assessment of its internal consistency, structural validity, and criterion validity. METHODS: One-hundred and thirty-two people with CF completed self-report scales pertaining to mental health, eating disorders, and the Cystic Fibrosis Eating Attitudes and Behaviours (CFEAB). RESULTS: Results of exploratory structural equation modelling indicated that a three-factor structure produced good fit with the 24-item CFEAB but a purified 12-item CFEAB displayed superior fit and internal consistency. Also, the 12-item scale predicted significant amounts of variance for anxiety, depression, and eating disorders showing enhanced relevance for clinical use. Conclusions These findings add emphasis to the importance of the validation and development of CF-specific measures and the possible inclusion at clinics to help improve CF patient care.

Clearing up the smoke: Physical and mental health considerations regarding cannabis use in adolescents with cystic fibrosis.

The cannabis plant is the most used federally illegal drug in the United States and is widely used by adolescents. Cannabis has complex effects on the body and mind. All health professionals who take care of adolescents with cystic fibrosis (CF) should be aware of the factors impacting cannabis use in CF. Given limited evidence regarding the benefits of cannabis and the significant risks, clinicians have the responsibility to identify risk of cannabis use early, counsel patients about the risks, provide a safe space for ongoing conversations about cannabis use in the context of CF care, and deliver evidence-based interventions.

Clinician perspectives on assessing for disordered eating and body image disturbance in adolescents and young adults with cystic fibrosis.

BACKGROUND: Maintaining a healthy weight is a focus of Cystic Fibrosis (CF) care. With the increased use of highly effective CFTR modulators, many people with CF are gaining weight more easily, which may affect eating habits and body image. This study investigates providers' understanding and current practices surrounding body image disturbance and disordered eating in people with CF. METHODS: We distributed a one-time web-based survey to United States (U.S.)-based CF healthcare providers via CF Foundation list servs. The survey investigated providers' understanding and perceived importance of issues surrounding disordered eating and body image disturbance in adolescent and young adults (AYA) with CF as well as current screening practices. We used descriptive statistics to analyze participants' characteristics and practices. RESULTS: A total of 232 healthcare providers completed the survey. While most participants felt that screening for both body image disturbance and disordered eating should be standardized in CF care (79% and 82%, respectively), fewer than one third felt comfortable screening, and only one quarter actually screened for various eating disordered behaviors in daily practice. Only 2.7% reported using a formal screening tool. Participants reported provider assessment tools (86%), standardized partnerships with eating disorder specialists (80%), and CFF or national guidelines (79%) would be helpful to improve screening and counseling. CONCLUSION: While most CF providers believe that body image disturbance and disordered eating are important topics in AYA with CF, few address these topics with their patients. The development of educational sessions and national guidelines may improve screening and counseling practices.

German newborn screening for Cystic fibrosis: Parental perspectives and suggestions for improvements.

PURPOSE: Cystic fibrosis (CF) was added to the German newborn bloodspot screening (NBS) panel in 2016. This study assesses parental perceptions of CF-NBS and confirmatory testing. METHODS: Prospective questionnaire-based survey administered to parents of children with positive CF-NBS over 40 months after initiation of CF-NBS in Southwest Germany. Parental perceptions were compared to results from Bavaria and Switzerland. RESULTS: Questionnaires with 29 standardized questions were sent to 343 families with children born between October 2016 and January 2020. A total of 178 (51.9%) replied. Although required by law, only a minority were informed about CF-NBS by a physician. The information provided about NBS was sufficient for 78% of parents. Regarding the information about positive CF-NBS, 52.9% were satisfied but the majority expressed negative emotions (89.5%). While most of these were resolved after confirmatory diagnostics, 17% of parents of children with false-positive CF-NBS and 66.7% of children confirmed with CF remained anxious. Waiting time for sweat testing was >3 days in 56.1%, considerably longer than in more centralized screening systems. Parents who waited for a maximum of 3 days were significantly more satisfied. 70.7% of parents were satisfied with the information given during confirmatory diagnostics and 91.4% were satisfied with participating in CF-NBS. CONCLUSIONS: CF-NBS stands in high regard with parents. Smooth organization, timely initiation of confirmatory testing, and professional communication are most important to limit parental anxiety. A more centralized system of confirmatory diagnostics appears advantageous in several regards as it reduces time from positive NBS to final diagnosis and increases parental satisfaction.

Perceptions of barriers to and facilitators of physical activity in adults with cystic fibrosis.

BACKGROUND: Physical activity (PA) is a proven therapeutic tool to increase the quality of life and life expectancy in people with cystic fibrosis (pwCF). Despite this, the PA level of pwCF is lower than recommended. OBJECTIVES: This study was conducted to identify the barriers to and facilitators of PA in adults with CF with heterogeneous severity. METHODS: Twenty adults with CF (mean age = 33.3±11.7 years, mean FEV1% = 50.55±20.4%) were recruited from two specialized centers and interviewed about the factors that limit and facilitate their PA. The collected data were transcribed, coded and analyzed using deductive and inductive methods. RESULTS: Barriers and facilitators were classified into physical, psychological and environmental dimensions. The main barriers were fatigue, breathing difficulties, lack of available facilities, negative perceptions of PA and perceived health risks. The most important facilitators were respiratory benefits, well-being, and social support. CONCLUSION: Although some barriers and facilitators were similar to those found in children with CF or adults from other vulnerable populations, others were specific to adults with CF, such as the risk of cross-contamination and transplant preparation. The comprehensive study of the barriers and facilitators in adults will enhance PA counseling for pwCF and help improve their compliance with PA recommendations.

The physiotherapy consultation: A qualitative study of the experience of parents of infants with cystic fibrosis in Australia.

BACKGROUND: Physiotherapy is an essential component in the management of cystic fibrosis (CF). OBJECTIVE: To explore how parents of infants with CF experience physiotherapy clinic consultations. METHODS: A qualitative study, informed by hermeneutic phenomenology, utilizing in-depth semi-structured interviews and daily diaries was conducted with 13 parents of infants (aged 0-2 yrs.) receiving physiotherapy care for CF in Australia. RESULTS: Three themes arose from the text. The first was that parents' physiotherapy clinic experience is influenced by the manner in which health professionals communicate to parents about physiotherapy in CF, as well as their own prior experience and knowledge. Secondly, parents receive conflicting messages from the physiotherapy consultation, but perceive the key message to be to prioritize adherence to physiotherapy. The final theme was that parents' expectations of the physiotherapy interaction were often not met, in particular a lack of practical physiotherapy education and training was reported. CONCLUSION: Parents of infants with CF seek an optimistic, practical and hands on approach during physiotherapy consultations. Exploring options for providing physiotherapy input outside of traditional clinic environments may help accommodate individual needs. Investigation into the manner in which physiotherapy education is communicated to parents is warranted in light of the influence on parents' expectations of physiotherapy.

Ways of coping and survival in Cystic Fibrosis: a 20-year longitudinal study.

BACKGROUND: The relationship between ways of coping and health outcomes has been a focus of interest for decades. There is increasing recognition that positive psychological functioning can influence health outcomes beneficially. This work investigated the role of coping in predicting survival in CF. METHODS: A longitudinal observational cohort study with a 20-year follow-up period was undertaken. At entry to the study, demographic and clinical variables were recorded, and ways of coping were assessed using the Cystic Fibrosis Coping Scale which measures four distinct ways of coping: optimism, hopefulness, distraction and avoidance. Survival outcome was measured as time in days from the date of recruitment to exit from the study, where exit was either death, loss to follow-up or the end of the follow-up period. RESULTS: Survival time was modelled using Cox's proportional hazards model. At baseline, 116 people with CF were recruited. By the census date, 54 people had died (14 men had died during 248,565 person-days of observation and 40 women had died during 358,372 person-days of observation). Optimism was the only way of coping that showed any beneficial effect on survival (RR=0.984, p=0.040) after adjustments for age, gender, ppFEV1 and the three other coping variables measured at baseline. CONCLUSION: This work suggests that optimistic coping serves as a prognostic measure of survival in CF beyond key clinical and demographic variables. Ways of coping are modifiable, providing a target for clinical intervention; to improve quality of life and clinical outcomes and potentially increase longevity.

Partner perspectives on women's sexual and reproductive healthcare in cystic fibrosis.

BACKGROUND: Women with cystic fibrosis (CF) face many sexual and reproductive health (SRH) concerns. Studies suggest that educating and involving partners in SRH care can improve outcomes. This study investigated partners' perceptions of and preferences for women's SRH care in CF. METHODS: We surveyed partners of women with CF from ten United States (U.S.) CF centers regarding their attitudes and preferences related to CF SRH care. Items assessed experiences with SRH care, sexual relationships, family planning, pregnancy, fertility, and parenthood. We used descriptive statistics to assess results related to the timing, content, setting and delivery of CF SRH care. RESULTS: A total of 94 partners completed the survey (94% male; average age 36±1 years; 70% married; 36% parents). Among those who/whose partners experienced a pregnancy, 48% received preconception counseling and 29% fertility testing/treatment. One-third of all respondents (32%) worried their children would have CF and 86% would undergo CF genetic testing if their CF partner became pregnant. One-third (34%) indicated that they did not have any SRH conversations with their partner's CF team, while 70% would like to have such discussions. The topics that respondents would most like to discuss were pregnancy (50%), fertility (43%), sexual functioning (36%), sexual activity (31%) and parenthood (29%). CONCLUSIONS: Partners report gaps in SRH care and counseling despite the majority wanting to discuss SRH concerns with their partner's CF team. CF partners serve as key supports for women with CF and results can be used to design patient-centered interventions to optimize CF SRH care.

Parent's report on oral health-related quality of life of children with cystic fibrosis.

BACKGROUND: Health-related quality of life (HRQoL) scales are now widely used in children with cystic fibrosis (cwCF) which reflects the course of the disease. In this cross-sectional study, our primary aim was to compare the Pediatric Oral Health-Related Quality of Life (POQL) and Oral Health Score (OHS) between cwCF and healthy group. Our secondary aim was to evaluate the association between Pseudomonas aeruginosa (PA) colonization, pulmonary function test, OHS and POQL in cwCF. METHODS: The study population (age ranging 6-14) included 55 cwCF followed at the Marmara University Division of Pediatric Pulmonology compared with 50 healthy peers. A survey consisted of general questions (age, sex, etc.) and the POQL instrument were filled by parents. The decayed, missing, and filled teeth for both primary (dft) and permanent dentition (DMFT) was detected according to WHO criteria. Data like current body mass index (BMI z score), colonization status with PA, predicted value for forced expiratory volume in 1 second (FEV1pp), and any hospitalizations during the previous year were obtained from their medical. Differences between the groups were evaluated using Chi-square and Mann-Whitney U test with a significance level set at 0.05. RESULTS: There was no significant difference between PA-colonized cwCF and healthy controls in DMFT (p = 0.916). For all domains of POQL (emotional function, social function, role function), scores of cwCF were significantly better than healthy controls (p < 0.05). There were no statistically significant differences between all domains of POQL scores in PA colonized and non-PA colonized cwCF' POQL scores (p > 0.05). CONCLUSION: Although POQL scores of cwCF were encouraging, dental caries prevention and regular follow-ups should be taken into consideration.