Cystic fibrosis foundation position paper: Redefining the cystic fibrosis care team.

Interdisciplinary teams care for people with cystic fibrosis (pwCF) at specialized treatment centers. These teams have laid the foundation for the cystic fibrosis (CF) care model responsible for gains in health outcomes and quality of life within the CF community. However, the landscape of CF care is transforming, invigorated by new technologies, accessibility of cystic fibrosis transmembrane conductance regulator (CFTR) therapies, and increased utilization of telemedicine.

Cystic fibrosis foundation position paper: Redefining the CF care model.

Specialized care is provided to people with cystic fibrosis (pwCF) by interdisciplinary teams nested within the CF Foundation's accredited care center network. This network allows for standardization of the care model, implementation of clinical care guidelines, efficient communication, and outcomes reporting. Recent developments have impacted this care model.

Giving adolescents with cystic fibrosis a voice: Predicting cystic fibrosis nutritional adherence from their decision-making involvement.

Objective: Suboptimal nutritional adherence in adolescents with cystic fibrosis (awCF) has been associated with lower lung function. AwCF often have more independence in dietary decisions than younger children, yet little research has examined how adolescent decision-making relates to nutritional adherence. This study explored whether components of adolescent decision-making involvement facilitate enzyme and caloric adherence in awCF.

Education-related needs for children with cystic fibrosis: Perspectives of US pediatric care teams.

Objective: Patients with cystic fibrosis (CF) often bring education-related concerns to their medical teams. Concerns around the ability for CF care teams to identify and address these concerns exist. We sought to describe CF care team perceptions of (1) patient and family education-related needs, (2) how these needs are identified, documented and addressed, and (3) education-related resource gaps.

Longitudinal mental health trends in cystic fibrosis.

Background: Mental health screening in accordance with consensus guidelines became routine clinical practice in our cystic fibrosis (CF) Center in 2015. We hypothesized improvement in anxiety and depression symptoms over time and associations between elevated screening scores and disease severity. We aimed to observe the impact of the COVID-19 pandemic and modulator use on mental health symptoms.

Management of Mental Health in Cystic Fibrosis.

This article is intended for use among all cystic fibrosis care team members. It covers common mental health concerns and their unique presentations in persons with cystic fibrosis (pwCF) in areas such as depression, anxiety, trauma, behavioral disorders emerging in childhood, sleep, problematic eating patterns, and the impact of substance use. Furthermore, the authors address ways to manage these mental health symptoms through risk assessment, psychological interventions, and/or psychotropic medications.

Longitudinal Assessment of Educational Risk for K-12 Students with Cystic Fibrosis.

Objectives: To assess the use of services provided by a cystic fibrosis (CF) center school specialist and evaluate relationships among student educational risk scores, family concerns for school engagement and performance, and disease severity.

Study Design: This was a retrospective review of medical records for 126 children with CF in grades K-12 who were screened for educational risk or used school intervention services during the 2017- 020 school years. Regression analyses were performed to identify and quantify predictors of educational risk, family concern for school performance and ability to advocate, and use of school specialist services.

Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project.

Background: Treatment regimens for cystic fibrosis (CF) continue to evolve and grow in complexity. Treatment regimen burden, and associated sequelae, are incompletely understood.

Objective: Quality improvement (QI) methods were used to investigate treatment burden of CF care, family and care team partnerships, and potential interventions to reduce burden.

Promoting emotional wellness in children with CF, part II: Mental health assessment and intervention.

This is the second of two companion papers that examine the emotional wellness of children with cystic fibrosis (CF) during the early years of life, defined here as the period between birth and age 12. Both papers promote optimal mental health and well-being, with an emphasis on early identification and intervention. The first paper explores child and family resilience.

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.

A Pilot Study of Mindfulness-Based Cognitive Therapy to Improve Well-Being for Health Professionals Providing Chronic Disease Care.

Objective: To assess the efficacy of mindfulness-based cognitive therapy delivered onsite during work hours in reducing stress and improving well-being in an interdisciplinary chronic care health care team.

Study Design: A longitudinal, mixed methods, observational pilot study using a survey created from validated assessment tools to measure effectiveness of training. Surveys were completed before training, and 1 and 15 months after training.

Introduction to Cystic Fibrosis for Mental Health Care Coordinators and Providers: Collaborating to Promote Wellness.

To maximize health, individuals with cystic fibrosis (CF) follow a complex, burdensome daily care regimen. Managing CF is associated with a range of significant biopsychosocial challenges and places individuals with CF, and their caregivers, at greater risk for developing anxiety and depression. To promote wellness and address the potential barriers that affect management of this complex chronic illness, many individuals would benefit from treatment from a behavioral health provider.

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.

Executive functioning in pediatric cystic fibrosis: A preliminary study and conceptual model.

Background: Research has shown that broad cognitive functioning in individuals with CF is intact. Specific executive functioning (EF) deficits have been identified, however, and adults with CF report more symptoms of ADHD than the general population. EF skills are critical to the management of a complex disease like CF although studies have not adequately examined EF mechanisms in CF.

Psychological predictors of nutritional adherence in adolescents with cystic fibrosis.

Background & Aims: The CF medical regimen is notoriously burdensome, comprised of respiratory treatments, oral medications, and nutritional demands. Adequate caloric intake has been identified as a challenge over the lifespan; however, we lack detailed information about nutritional adherence in teens, and the contextual drivers of these behaviors. Adolescence is a time of increased responsibility, reduced parental monitoring, and growing peer connections.

Assessing psychosocial risk in pediatric cystic fibrosis.

Background: Psychosocial risk factors are known to impact quality of life, treatment adherence, and health outcomes. No standardized comprehensive psychosocial risk screener is routinely utilized in cystic fibrosis (CF) care. The objectives of the study were to describe the range and severity of psychosocial risk within this CF population, investigate the reliability of a comprehensive psychosocial screener in pediatric CF clinical care, and explore relationships between psychosocial risk and key factors affecting health outcomes.

Maintenance Following a Randomized Trial of a Clinic and Home-based Behavioral Intervention of Obesity in Preschoolers.

Objective: To assess maintenance of improved weight outcomes in preschoolers with obesity 6 and 12 months following a randomized clinical trial comparing a home- and clinic-based behavioral intervention (Learning about Activity and Understanding Nutrition for Child Health [LAUNCH]) to motivational interviewing and standard care.

Study Design: Randomized controlled trial with children between the ages of 2 and 5 years above the 95th percentile for body mass index for age and sex recruited from 27 pediatrician offices across 10 recruitment cycles between March 12, 2012, and June 8, 2015, were followed 6 and 12 months post-treatment. Child and caregiver weight, height, and caloric intake, child physical activity, and home environment were assessed.

Macronutrient intake in preschoolers with cystic fibrosis and the relationship between macronutrients and growth.

Background: Adequate nutrition is essential for growth in children with cystic fibrosis (CF). The new CF Foundation Clinical Practice Guidelines bring attention to monitoring macronutrient intake as well as total energy.

Methods: Dietary intake of 75 preschool children with CF and pancreatic insufficiency was examined and compared to the Clinical Practice Guidelines.

Learning about Activity and Understanding Nutrition for Child Health (LAUNCH): Rationale, design, and implementation of a randomized clinical trial of a family-based pediatric weight management program for preschoolers.

Obesity affects nearly 2 million preschool age children in the United States and is not abating. However, research on interventions for already obese preschoolers is limited. To address this significant gap in the literature, we developed an intervention targeting obesity reduction in 2 to 5year olds, Learning about Activity and Understanding Nutrition for Child Health (LAUNCH).

Web-Based Intervention for Nutritional Management in Cystic Fibrosis: Development, Usability, and Pilot Trial.

Objectives: Usability and pilot testing of a web intervention (BeInCharge.org [BIC]) of behavior plus nutrition intervention for children with cystic fibrosis (CF) ages 4-9 years.

Methods: Think Aloud methodology was used with five mothers to assess usability and refine the intervention.

Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial.

Importance: Evidence-based treatments that achieve optimal energy intake and improve growth in preschool-aged children with cystic fibrosis (CF) are a critical need.

Objective: To test whether behavioral and nutritional treatment (intervention) was superior to an education and attention control treatment in increasing energy intake, weight z (WAZ) score, and height z (HAZ) score.

Design, Setting, And Participants: This randomized clinical trial included 78 children aged 2 to 6 years (mean age, 3.

Quality of life in children with CF: Psychometrics and relations with stress and mealtime behaviors.

Background: The purpose of this study was to evaluate the utility of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) with toddlers and preschool-aged children. Clinically relevant relations between health-related quality of life (HRQOL), stress, and mealtime behaviors have not been examined. It was hypothesized that problematic mealtime behaviors and increased stress would be negatively associated with HRQOL.

A pilot randomized controlled trial of a behavioral family-based intervention with and without home visits to decrease obesity in preschoolers.

Objective: Tested two family-based behavioral treatments for obesity in preschool children, one meeting the Expert Committee guidelines for Stage 3 obesity intervention criteria (LAUNCH-clinic) and one exceeding Stage 3 (LAUNCH with home visit [LAUNCH-HV]), compared with a Stage 1 intervention, pediatrician counseling (PC).

Methods: In all, 42 children aged 2-5 years with a body mass index (BMI) percentile of ≥95th were randomized. A total of 33 met intent-to-treat criteria.

Changes in parent motivation predicts changes in body mass index z-score (zBMI) and dietary intake among preschoolers enrolled in a family-based obesity intervention.

Objectives: To examine whether changes in parent motivation over the course of a pediatric obesity intervention are significantly associated with long-term changes in treatment outcomes. 

Methods:  Study hypotheses were tested with a secondary data analysis of a randomized controlled trial (N = 42). Study analyses tested whether baseline to posttreatment change in total score for a self-report parent motivation measure (Parent Motivation Inventory [PMI]) was significantly associated with baseline to 6-month follow-up changes in body mass index z-score (zBMI), dietary variables, and physical activity.