A Qualitative Exploration into Experiences and Attitudes Regarding Psychosocial Challenges, Self-compassion, and Mindfulness in a Population of Adults with Cystic Fibrosis.

To investigate the current psychosocial challenges faced by adults with Cystic Fibrosis (CF), while exploring attitudes and experiences of mindfulness and self-compassion. Mindfulness and self-compassion are important resources for supporting psychological and physical well-being, yet there is limited research exploring these positive psychology concepts in CF literature. Twenty UK domiciled adults with a diagnosis of CF, took part in a semi-structured interview. Using a thematic analysis approach, four themes were developed: (a) "I didn't expect to be here": Surpassing the CF life expectancy, (b) "Am I psychologically bringing this upon myself?": Psychological complexities of CF, (c) "I've had to really learn to be kind to myself": The importance of compassion and being self-compassionate, (d) "I think it's a great tool": The benefits of practising mindfulness. This research demonstrates a robust need for increased integration of accessible psychological practices into routine CF-care and self-care for adults with CF. Particularly, practises and interventions that draw on the concepts of mindfulness and self-compassion, which may benefit patient's health and wellbeing profoundly.

Understanding motivation for Australian adolescents and young adults with cystic fibrosis: Modifiable factors to support self-management.

Cystic fibrosis (CF) is Australia's most common life limiting genetic condition, characterised by declining health and quality of life (QoL) over time. Despite improvements in treatment, there remains no cure. Adolescents and young adults (AYAs) with CF experience broad impacts to psychosocial functioning and QoL, as well as major transitions in care, all at a time of significant developmental change. The importance of developmentally tailored approaches to youth health care and self-management for young people with CF are well understood. However, to date, models of youth specific self-management have been lacking and motivation for young people with CF has not been well explored. This qualitative study, based on a social constructionist epistemological framework, addresses this gap. A total of 21 AYAs aged 15-30 years were recruited through one paediatric and one adult Australian CF centre. Demographic, clinical and distress data were captured to describe health complexity. Semi-structured interviews were audio-recorded, transcribed and analysed using thematic analysis. Participants were representative of Australian AYAs with CF by demography and clinical status. Alarmingly, over a third reported clinically significant distress. Two themes emerged. The first Identified impacts to motivation and self-management resulting from the challenges of managing CF, life and care. These included time and competing priorities, changing health statis, mental health, social factors, unmet needs and health system complexity. The second identified factors that support motivation including: achievement, meaning and purpose; consequence avoidance; and accountability. These results illustrate the importance of AYA specific, theoretically founded, holistic self-management models which extend beyond current theoretical approaches that aim to understand behaviour change or address barriers, in isolation from motivation. Improved approaches to care based on these findings are essential to foster positive behavioural change, support self-management and foster the best health outcomes for young people living with CF.

Transition to adult care in cystic fibrosis: The challenges and the structure.

In developed countries, it is projected that there will be a 70% increase in the number of adults living with Cystic Fibrosis (CF) between 2010 and 2025. This shift in demographics highlights the importance of high-quality transition programmes with developmentally appropriate integrated health care services as the individual moves through adolescence to adulthood. Adolescents living with CF face additional and unique challenges that may have long-term impacts on their health, quality of life and life-expectancy. CF specific issues around socially challenging symptoms, body image, reproductive health and treatment burden differentiate people with CF from their peers and require clinicians to identify and address these issues during the transition process. This review provides an overview of the health, developmental and psychosocial challenges faced by individuals with CF, their guardians and health care teams considering the fundamental components and tools that are required to build a transition programme that can be tailored to suit individual CF clinics.

Promoting emotional wellness in children with cystic fibrosis, Part I: Child and family resilience.

Attention should be given to individual and family well-being from a child's first interaction with the medical team and continuing throughout development, especially for families who experience chronic illnesses, such as cystic fibrosis (CF). While much attention has been given to the mental health of people with CF 12 years and older, this paper explores various areas for CF teams to assess and provide additional resources during the first 12 years of a child's life to promote child and family wellness. In this paper, we discuss parental mental health, social determinants of health, adherence/self-care, nutrition, attention to family lifestyle factors, engagement with school and peers, and modulator therapy for this age group of people with CF. This is the first of two companion papers which examines emotional wellness of children during the early years. The second paper examines mental health assessment and intervention for children under 12. Both encourage teams to strive to promote optimal child and family emotional health and wellness, emphasizing holistic health promotion and prevention, early identification, and intervention.

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.

Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia's Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.

The dance of cystic fibrosis: Experiences of living with cystic fibrosis as an adult.

AIMS AND OBJECTIVES: This study sought to understand the experience of living with cystic fibrosis (CF) as an adult. BACKGROUND: Cystic fibrosis is one of the most difficult chronic diseases to manage long term because of numerous challenges faced on a day-to-day basis. The majority of studies focus on improving symptom response to new treatment regimens with the hope of prolonging life. Yet few qualitative studies explore the experience of adults with CF. What is missing from the current literature are the voices of people living with CF, especially as they age. DESIGN: This study follows a hermeneutic phenomenology design guided by Martin Heidegger's philosophy. METHODS: Semi-structured interviews were conducted with nine adults living with CF. Hermeneutic phenomenology guided the data interpretation. This manuscript follows COREQ reporting guidelines. RESULTS: Hermeneutic analysis revealed the overarching theme The Dance of Cystic Fibrosis which is supported by five themes: (a) the paradox of control, (b) living deaths, (c) dancing with death, (d) relearning to dance, and (e) role of the dance partner. CONCLUSIONS: The experience of living with CF as an adult is multidimensional and unique to each person. Despite the uniqueness, there is a shared experience that manifested as The Dance of Cystic Fibrosis. As life expectancy continues to increase for persons with CF, it is essential that researchers and healthcare professionals intentionally consider the life-prolonging effects of the treatment regimen alongside the persons' experience with those effects. RELEVANCE TO CLINICAL PRACTICE: Nurses at all levels of practice should be prepared to address the multifaceted experience of living with CF through thoughtful incorporation of open-ended questions. This allows patients to share their experience with nurses, augmenting their practice of delivering holistic care.

TREATMENT ADHERENCE AMONG CHILDREN AND ADOLESCENTS IN A CYSTIC FIBROSIS REFERENCE CENTER.

OBJECTIVE: To evaluate the level of self-referenced treatment adherence (TA) and its association with clinical and sociodemographic variables in patients with cystic fibrosis assisted at a reference center, as well as compare the level of self-referenced TA with that presumed by the multidisciplinary team. METHODS: This is a cross-sectional study that included children and adolescents aged between 0-20 years with cystic fibrosis. Adolescents older than 14 years or their guardians, when younger than 14 years old, were interviewed using a standardized questionnaire. Professionals from the multidisciplinary clinic filled out another form with their impressions of the patients' TA. Clinical and laboratory data were obtained in the medical records. The TA was considered satisfactory if the total adherence index (TAI) was equal or higher than 80%. RESULTS: 53 patients were included with a median age of 112 months. The mean TAI was 83.2%. The mean TAIs for dornase alfa, pancreatic enzymes, continued use of inhaled tobramycin, vitamins supplements, nutritional supplements and dietary orientation was respectively: 86.1; 96.6; 78.6; 88.1; 51.8 and 78%. Children younger than 14 years presented better TA (p=0.021). The correlation between the self-referenced TA and the one presumed by the multidisciplinary team ranged from 0,117 to 0.402, being higher for Psychology and Nutrition professionals. CONCLUSIONS: The TAI was high particularly among children younger than 14 years. There was a positive correlation between the self-referenced TA and the one presumed by the Psychology (p=0.032) and the nutrition (p=0.012) professionals.

Adults with cystic fibrosis: spiritual coping with lifelong disease.

Cystic fibrosis (CF) is a chronic life-shortening disease requiring significant coping. Spiritual belief relates to treatment behaviors. Little is known about spirituality's role in adults diagnosed as children, nor how it compares with adults diagnosed as adults. Adults over 18 years, diagnosed as children completed a questionnaire; some were randomized to also participate in an interview or daily phone diary to measure adherence. Qualitative analyses of 25 adults are presented. Participants reframed their disease as part of a Divine Plan, in which Divine assistance was conditional upon adherence. Linear regression models of spiritual constructs on airway clearance, nebulized medication, and exercise are presented. Adults diagnosed as children related spirituality to CF in ways both consistent and different from adults diagnosed as adults. Spiritual beliefs were related to adherence determinants and intentions. Increased understanding of the relationship between spirituality and health behaviors is important to providing person-centered care.

Assessing and responding to stress related to pulmonary function testing in cystic fibrosis through quality improvement.

BACKGROUND: Pulmonary function tests (PFTs) are performed routinely to evaluate lung function in patients with cystic fibrosis (CF). Staff at the Cincinnati Children's Hospital Medical Center CF Center observed stress in patients before PFTs. An interdisciplinary quality improvement (QI) team was assembled to address this clinical issue. METHODS: The Plan-Do-Study-Act method of QI was used to investigate feasibility of assessing stress and offering brief interventions to reduce stress before PFTs. Interventions included listening to music, covering the PFT screen, or doing breathing meditation before PFTs. Patients rated stress levels on a 1 to 5 Likert scale before and after testing. RESULTS: Of 75 patient encounters, interventions were trialed in 20. Fifteen patients who tried an intervention reported wanting to use the intervention again (five encounters had missing data); patients reported that the intervention benefited performance on PFTs in eight encounters (40%). The average pre-PFT stress rating for encounters that trialed an intervention was 2.1 and post-PFT rating was 2.0. Average stress pre-PFT and post-PFT ratings were 1.7 and 1.6 respectively, for encounters that did not trial an intervention. Median length of PFT encounter was 15 minutes regardless of whether intervention was trialed. CONCLUSION: Some patients with CF utilized interventions, while many opted out. This QI effort identified feasible outpatient clinic interventions that did not negatively impact clinic flow. Finding ways to reduce stress associated with PFTs could have a meaningful impact on patient performance and emotional well-being for a subset of patients.

Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials.

In a recent James Lind Alliance Priority Setting Partnership in cystic fibrosis (CF) the top priority clinical research question was: "What are effective ways of simplifying the treatment burden of people with CF?" We aimed to summarise the lived experience of treatment burden and suggest research themes aimed at reducing it. An online questionnaire was co-produced and responses subjected to quantitative and thematic analysis. 941 survey responses were received (641 from lay community). People with CF reported a median of 10 (interquartile range: 6-15) current treatments. Seven main themes relating to simplifying treatment burden were identified. Treatment burden is high, extending beyond time taken to perform routine daily treatments, with impact varying according to person-specific factors. Approaches to communication, support, evaluation of current treatments, service set-up, and treatment logistics (obtaining/administration) contribute to burden, offering scope for evaluation in clinical trials or service improvement.

Treatment adherence among children and adolescents in a cystic fibrosis reference center / Adesão ao tratamento da fibrose cística entre crianças e adolescentes de um centro de referência

ABSTRACT Objective: To evaluate the level of self-referenced treatment adherence (TA) and its association with clinical and sociodemographic variables in patients with cystic fibrosis assisted at a reference center, as well as compare the level of self-referenced TA with that presumed by the multidisciplinary team. Methods: This is a cross-sectional study that included children and adolescents aged between 0-20 years with cystic fibrosis. Adolescents older than 14 years or their guardians, when younger than 14 years old, were interviewed using a standardized questionnaire. Professionals from the multidisciplinary clinic filled out another form with their impressions of the patients' TA. Clinical and laboratory data were obtained in the medical records. The TA was considered satisfactory if the total adherence index (TAI) was equal or higher than 80%. Results: 53 patients were included with a median age of 112 months. The mean TAI was 83.2%. The mean TAIs for dornase alfa, pancreatic enzymes, continued use of inhaled tobramycin, vitamins supplements, nutritional supplements and dietary orientation was respectively: 86.1; 96.6; 78.6; 88.1; 51.8 and 78%. Children younger than 14 years presented better TA (p=0.021). The correlation between the self-referenced TA and the one presumed by the multidisciplinary team ranged from 0,117 to 0.402, being higher for Psychology and Nutrition professionals. Conclusions: The TAI was high particularly among children younger than 14 years. There was a positive correlation between the self-referenced TA and the one presumed by the Psychology (p=0.032) and the nutrition (p=0.012) professionals.

RESUMO Objetivo: Avaliar o grau de adesão ao tratamento (AT) autorreferida e pesquisar sua associação com variáveis clínicas e sociodemográficas em pacientes com fibrose cística (FC) de um centro de referência e comparar o grau de AT autorreferida com o presumido pela equipe multidisciplinar. Métodos: Estudo transversal, incluindo crianças e adolescentes com FC entre zero e 20 anos. Foram realizadas entrevistas com os adolescentes maiores de 14 anos ou com os responsáveis dos mais jovens, utilizando-se formulário padronizado. Profissionais do centro preencheram outro formulário com suas impressões da AT dos pacientes. Registraram-se dados clínicos por meio da revisão de prontuários. A AT foi considerada satisfatória se o índice de adesão global (IAG) fosse igual ou superior a 80%. Resultados: Foram incluídos 53 pacientes, com mediana de idade de 112 meses. O IAG médio foi de 83,2%. Os índices de adesão à terapêutica (IAT) médios para alfadornase, enzimas pancreáticas, tobramicina inalatória de uso contínuo, suplementos vitamínicos, suplementos nutricionais e orientações dietéticas foram, respectivamente, 86,1; 96,6; 78,6; 88,1; 51,8 e 78%. Crianças com idade inferior a 14 anos apresentaram melhor AT (p=0,021). As correlações entre a AT autorreferida e a presumida pelos profissionais variaram de 0,117 a 0,402, sendo maiores para a psicologia e a nutrição. Conclusões: A taxa global de AT foi elevada, principalmente para menores de 14 anos. Houve correlação positiva entre a AT autorreferida e as avaliações da psicologia (p=0,032) e da nutrição (p=0,012).

Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis.

BACKGROUND: Despite the significant impact of chronic symptoms on quality of life with cystic fibrosis (CF), the role of palliative care in management of this disease is not well defined. The coping, goal assessment, and relief from evolving CF symptoms (CF-CARES) model is a primary palliative care intervention designed to provide chronic symptom management at all stages of the disease. The goal of this pilot study was to estimate the effectiveness of the CF-CARES intervention on improving chronic symptoms and quality of life for people living with CF. METHODS: A structured assessment was used to guide referral to supportive services intended to address burdensome symptoms. Follow-up assessments were performed approximately 3 and 6 months later. Longitudinal regression analyses of changes in symptoms and quality of life were performed for all participants regardless of utilization of supportive services. Subgroup analyses were performed for subjects participating in mental health and alternative health services. RESULTS: Forty-one subjects completed assessment and referral processes. The mean number of CF-associated symptoms decreased over time, as did respiratory symptom-related distress and depressive symptoms. Subjects utilizing alternative health services reported less psychological distress at follow-up. Among subjects with severe disease, mental health, and quality of life improved, especially for those using mental health services. CONCLUSIONS: The CF-CARES model resulted in significant mental health and quality-of-life benefits, suggesting the value of integrating symptom management interventions into routine CF care. Moreover, mental health services can play a key role in CF-specific primary palliative care, especially for those with advanced disease.

Bio-psycho-social-spiritual needs of adolescents and young adults with life-threatening illnesses: Implications for social work practice.

This paper explores the biopsychosocial and spiritual needs of adolescents and young adults (AYA) with life-threatening or terminal illnesses. AYA are situated between childhood and adulthood (ages 15-25) and have distinct biopsychosocial and spiritual needs unique to their developmental stage. Having a life-threatening or terminal illness directly challenges normal AYA developmental tasks and identity formation. AYA experience more troubling physical symptoms during the dying process compared to other age groups, which leads to significant psychological distress and an increased need for pharmacological treatments. In general, AYA desire to be fully informed and involved in the health care decision-making process, leading to ethical dilemmas when the AYA is a minor and their wishes differ from the wishes of their legal guardian(s). Social workers are especially well-equipped to serve this population due to aligning professional standards and ability to advocate for holistic care within interdisciplinary teams. Additional research is needed to tailor holistic interventions to meet the needs of this population.

Integrated Behavioral Health Care in Pediatric Subspecialty Clinics.

Comorbid behavioral and physical health conditions are accompanied by troubling symptom burden, functional impairment, and treatment complexity. Pediatric subspecialty care clinics offer an opportunity for the implementation of integrated behavioral health (BH) care models that promote resiliency. This article reviews integrated BH care in oncology, palliative care, pain, neuropsychiatry, cystic fibrosis, and transplantation. Examples include integrated care mandates, standards of care, research, and quality improvement by child and adolescent psychiatrists (CAPs) and allied BH clinicians. The role of CAPs in integrated BH care in subspecialty care is explored, focusing on cost, resource use, financial support, and patient and provider satisfaction.

Don't Push Your Luck! Educational Family Board (Not Bored) Game for School-Age Children Living with Chronic Conditions.

PURPOSE: Children who are living with chronic conditions may be supported in self-care through enjoyable active learning and family social processes. This research focused on development and evaluation of "Don't Push Your Luck!", an educational board game designed to inspire family discussion about chronic conditions, and help affected children learn about self-care choices and consequences. DESIGN AND METHODS: Mixed-method research was conducted with families from one outpatient Cystic Fibrosis Clinic and four Hemophilia Treatment Centres in Canada and United States (N=72). In phase I, board game prototype and questionnaires were refined with affected boys, siblings, and parents living with hemophilia (n=11), compared with families living with cystic fibrosis (n=11). In phase II, final board game was evaluated with families living with hemophilia (n=50). Data collection included pre-post-game questionnaires on decision-making and Haemo-QoL Index©, and post-game enjoyment. Analysis included descriptive statistics, inferential statistics (non-parametric), and qualitative themes. RESULTS: Findings revealed this game was an enjoyable and effective resource to engage families in self-care discussions. Key themes included communication, being involved, knowing, decisions and consequences, and being connected. Qualitative and quantitative findings aligned. Statistical significance suggests the game enhanced family engagement to support decision-making skills, as parents identified that the game helped them talk about important topics, and children gained insight regarding family supports and self-care responsibility. CONCLUSIONS: This board game was an effective, developmentally appropriate family resource to facilitate engagement and conversation about everyday life experiences in preparation for self-care. PRACTICE IMPLICATIONS: There is promising potential to extend this educational family board game intervention with a greater range of school-age children and families living with chronic conditions.

Body Sanctification and Sleep in Adolescents with Cystic Fibrosis: A Pilot Study.

Imbuing one's body with divine significance is associated with health-protective behaviors. The purpose of this study was to determine whether adolescents with a life-shortening, chronic disease (cystic fibrosis) who sanctified their bodies also received adequate sleep. Data from Daily Phone Diaries and questionnaire replies from 45 adolescents with cystic fibrosis (ages 11-19 years) were analyzed. A significant relationship between body sanctification and sleep was found, with between-gender differences. Body sanctification is an understudied construct which is associated with healthy behaviors.

How holistic nursing can enhance the quality of life of children with cystic fibrosis.

Cystic fibrosis (CF) is one of the most common life-limiting genetic conditions. This article presents a case study of a teenager with one of the rarer presentations of CF. This case study explores the experiences of her and her family. It also discusses the effects of CF on the patient and her family, and how it affects their quality of life and well-being.

Emotional health in children and adolescents with cystic fibrosis.

Although modern therapeutic procedures have considerably improved the survival and the quality of life of children with cystic fibrosis, the relevant psychological aspects have been still insufficiently considered similarly to the other chronic diseases. The aim of this research was to evaluate the emotional health: psychological characteristics and adjustment of CF children and their family coping. The study comprises 25 CF children, mean age 13.13 ± 2.29 years (23 boys and only 2 girls), selected from total 60 actually treated children for CF. Children were examined in the period of improved health conditions (without superinfection, wheezing or gastrointestinal problems). Obtained results are compared with a control group of 25 healthy children of the same age, selected by random from primary schools. The psychometric instruments used were: Kohs Design Test, Child Behavior Checklist, Eysenck Personality Questionnaire, General Anxiety Scale, Emotional Profile Index, MMPI-201 and Human Values Test, together with two projective tests of drawing (Machover and Corman). The unexpected good psychological results obtained from psychometric instruments could be explained by the fact that CF children accept the real situation and express vivacity. However, their deep feelings of fear impose on them high level of self-control and resistance. The results obtained for CBCL presented CF children as immature, with accentuated aggressiveness in interpersonal relations. The most important problem is related to the delay of puberty changes, leading to low self-esteem. Generally, family members cope relatively well with the disease in children, in spite to discrepancies in mother/child reports for child psychopathology. Divorces also occurred in some families. Psychological support for both, children and family members are necessary. The need for a holistic approach in the assessment and treatment, including biofeedback techniques was pointed out.

Adolescents' Spirituality and Cystic Fibrosis Airway Clearance Treatment Adherence: Examining Mediators.

OBJECTIVE: Adolescent cystic fibrosis (CF) treatment adherence is a significant multidimensional issue. Using the Theory of Reasoned Action (TRA), this study examined the role of spiritual factors in adherence. METHODS: Forty-five 11-19-year-olds diagnosed with CF completed questionnaires concerning psychosocial, spiritual, and adherence-related constructs and Daily Phone Diaries to calculate treatment adherence. Exploratory Factor Analysis identified two spiritual factors used in subsequent analyses. The mediating roles of attitude toward the treatment's value (utility), subjective behavioral norms (the product of perceived behavioral norms and one's motivation to comply with them), self-efficacy for completing the treatments and treatment intentions in the relationship between spiritual factors and treatment adherence were tested with path analysis. RESULTS: Lower 'spiritual struggle' and greater 'engaged spirituality' predicted treatment attitude (utility) and subjective behavioral norms, which, together with self-efficacy, predicted treatment intentions. Finally, treatment intentions predicted airway clearance adherence. CONCLUSIONS: Findings were consistent with the TRA. Engaged spirituality supports pro-adherence determinants and behavior. Spiritual struggle's negative associations with outcomes warrant screening and intervention.