Improving implementation and team communication by integrating a cystic fibrosis transition readiness (CF R.I.S.E.) program into electronic health records.

BACKGROUND: The cystic fibrosis (CF) Responsibility. Independence. Self-care. Education. (R.I.S.E.) program was developed to provide assessment and education, supporting transition readiness for people with cystic fibrosis (pwCF). Lack of integration within electronic health records (EHR) was a barrier to implementation of CF R.I.S.E. University of Michigan was able to integrate CF R.I.S.E. into EHR. AIM: To improve implementation and EHR documentation of CF R.I.S.E. module completion by pwCF across two (CF) programs from baseline (10.5%) to 75% per month in 6 months (January through June 2023). METHODS: Two CF programs utilized quality improvement (QI) methods and tools and ad hoc support by a CF Learning Network (QI) specialist. Eligibility included pwCF ≥16 years old seen in CF clinics who accepted CF R.I.S.E. PARTICIPATION: Beginning January 2, 2023, programs met in biweekly, virtual meetings to discuss implementation. Deidentified data were collected monthly tracking modules completed by pwCF and number of team members engaging with CF R.I.S.E. and documenting in EHR. Data timelines were baseline (November-December 2022), project period (January-June 2023), and post-project (July-December 2023). RESULTS: Completion rates increased from baseline (10.5%) to 48% (range 33% to 81%) through December 2023. During the project, an average 7.7 team members completed an average 19.2 modules per month. Post-project, an average 8 team members completed an average 16.5 modules per month. CONCLUSIONS: This collaboration demonstrated how utilization of EHR allowed for successful CF R.I.S.E. improvement at both programs. Shared software utilization and QI initiatives may be a way to facilitate timely dissemination of best practices through learning health systems.

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. METHODS: A survey was emailed to pediatric care teams in the CF Foundation Care Center Network in April 2022. Individuals or care teams could complete the survey. Responses were aggregated for descriptive analysis. RESULTS: Sixty-seven programs responded representing 52% of United States pediatric CF centers. Most centers (88%) indicated social workers primarily address school concerns. Care teams often complete school forms (99%), coach families to communicate with schools (96%), communicate with schools directly (90%), and develop educational plans (76%). Formal education risk assessment and support programs are relatively uncommon (19%). Common student-specific needs include carrying medications (75%) and leaving class for gastrointestinal issues (54%). Needs reported are informational materials for families and schools (94%), staff education about school concerns and how to address them (91%), additional staff for education-related issues (65%), and expertise in education plan development (62%). CONCLUSION: CF care teams often lack comprehensive resources to identify and address education-related concerns. Systematically performing needs assessments, improving training for providers, and evaluating the benefits of education specialists on care teams may better identify and address education-related needs. Supporting educational progression will foster continued independence and well-being in adulthood.

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. METHODS: Retrospective chart reviews were conducted for people 12 years and older with at least one Generalized Anxiety Disorder-7 (GAD-7) or Patient Health Questionnaire-9 (PHQ-9) screening for six years. Descriptive statistics were used to summarize demographic variables and logistic regression and linear mixed models were used to evaluate the relationship between screening scores and clinical variables. RESULTS: Analyses included 150 participants (ages 12-22 years). The percentage of minimal to no symptom scores increased over time for anxiety and depression. Increased mental health visits and CFRD were associated with higher PHQ-9 and GAD-7 scores. Higher FEV1pp was associated with lower GAD-7 and PHQ-9 scores. More effective modulator use was associated with lower PHQ-9 scores. Mean PHQ-9 and GAD-7 scores were not significantly different when comparing pre-pandemic and pandemic scores. CONCLUSION: Disruptions in screening during the pandemic were minimal and symptom scores remained stable. Individuals with higher mental health screening scores were more likely to have CFRD and utilization of mental health services. Consistent mental health monitoring and support is needed so individuals with CF can endure anticipated and unanticipated stressors including changes in physical health, healthcare, and societal stressors such as COVID-19 pandemic.

Associations of actigraphy measures of sleep duration and continuity with executive function, vigilance, and fine motor control in children with snoring and mild sleep-disordered breathing.

STUDY OBJECTIVES: Children with snoring and mild sleep-disordered breathing may be at increased risk for neurocognitive deficits despite few obstructive events. We hypothesized that actigraphy-based sleep duration and continuity associate with neurobehavioral functioning and explored whether these associations vary by demographic and socioeconomic factors. METHODS: 298 children enrolled in the Pediatric Adenotonsillectomy Trial, ages 3 to 12.9 years, 47.3% from racial or ethnic minority groups, with habitual snoring and an apnea-hypopnea index < 3 were studied with actigraphy (mean 7.5 ± 1.4 days) and completed a computerized vigilance task (Go-No-Go) and a test of fine motor control (9-Hole Pegboard). Caregivers completed the Behavior Rating Inventory of Executive Function. Regression analyses evaluated associations between sleep exposures (24-hour and nocturnal sleep duration, sleep fragmentation index, sleep efficiency) with the Behavior Rating Inventory of Executive Function Global Executive Composite index, pegboard completion time (fine motor control), and vigilance (d prime on the Go-No-Go), adjusting for demographic factors and study design measures. RESULTS: Longer sleep duration, higher sleep efficiency, and lower sleep fragmentation were associated with better executive function; each additional hour of sleep over 24 hours associated with more than a 3-point improvement in executive function (P = .002). Longer nocturnal sleep (P = .02) and less sleep fragmentation (P = .001) were associated with better fine motor control. Stronger associations were observed for boys and children less than 6 years old. CONCLUSIONS: Sleep quantity and continuity are associated with neurocognitive functioning in children with mild sleep-disordered breathing, supporting efforts to target these sleep health parameters as part of interventions for reducing neurobehavioral morbidity. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Name: Pediatric Adenotonsillectomy for Snoring (PATS); URL: https://clinicaltrials.gov/ct2/show/NCT02562040; Identifier: NCT02562040. CITATION: Robinson KA, Wei Z, Radcliffe J, et al. Associations of actigraphy measures of sleep duration and continuity with executive function, vigilance, and fine motor control in children with snoring and mild sleep-disordered breathing. J Clin Sleep Med. 2023;19(9):1595-1603.

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. RESULTS: Most children with CF (62%-82%) were at moderate-to-high educational risk. Sixteen or more school absences, family concerns for their child's school performance or inability to advocate for their school needs, child mental health visits, and greater frequency of hospitalization predicted greater educational risk scores and more encounters with the school specialist. Better lung function and lower grade level were associated with lower educational risk. Number of encounters with the School Specialist remained high across three pre-coronavirus disease 2019 school years. CONCLUSIONS: Our experience illustrates a need to identify educational risk and support school experiences of children and youth with CF as a component of the care model. Tailored support, based on identification of risk predictors, has potential to improve educational outcomes.

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. Quick reference tables are provided for evidence-based psychological interventions and medications often used for mental health conditions in pwCF.

To treat or not to treat: CFTR modulators after lung transplantation.

Evidence for the extrapulmonary benefits of (CFTR) modulators is rapidly expanding. The use of CFTR modulators in CF patients who have undergone lung transplantation is not clear without guidance published in the medical literature to assist clinicians in the care of these patients. We discuss the potential benefits of CFTR modulators and provide insight into their use based on our experience in a small cohort of CF LTx recipients. We present pros and cons of CFTR modulator therapy for LTx recipients with CF. CFTR modulators should be considered in CF patients after lung transplantation for the time being until further research defines how to best use these therapies in transplant recipients.

Correction to: Influence of Cystic Fibrosis­Related Diabetes on Mental Health in Adults: A Single­Center Study.

The original version of this article unfortunately contained a mistake in one of the co-author name Prof. Don Hayes Jr. During production process, "Jr." was missed to add after the author name. The author name is corrected with this correction. The original article has been corrected.

Influence of Cystic Fibrosis-Related Diabetes on Mental Health in Adults: A Single-Center Study.

PURPOSE: People with cystic fibrosis (CF) are predisposed to chronic conditions, such as CF-related diabetes (CFRD). Recent attention has been focused on the addition of screening for anxiety and depression in the CF population. Independently, CFRD and mental health conditions are associated with worse clinical outcomes; however, research assessing the impact of both conditions together is limited. We aimed to characterize the association between CFRD and selected diagnoses of anxiety or depressive disorders on clinical outcomes in adults with CF. METHODS: A single-center, retrospective, cross-sectional study in adult patients with CF was performed. Group comparisons included selected diagnoses of depression, anxiety, and CFRD using two-sample t-tests or rank-sum tests for continuous variables, and Chi-square or Fisher's exact tests for categorical variables. RESULTS: A total of 209 adults were enrolled (mean age of 31.4 ± 11.4 years). Those with a selected diagnoses of depression had a significantly higher proportion of CFRD than those without depression (48% vs. 28%, respectively, p = 0.005), and CFRD was associated with increased odds of depression [OR (CI) = 2.33 (1.28, 4.26), p = 0.006]. We did not see a higher proportion of adults with CFRD and selected diagnoses of anxiety than those without anxiety (41% vs. 31% respectively, p = 0.12), nor an increased odds of anxiety in those with CFRD [OR (CI) = 1.58 (0.88, 2.84), p = 0.12]. CONCLUSION: We show a significant association between CFRD and selected diagnoses of depression in a cohort of adult patients.

Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor: A Case Series.

Cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators have revolutionized care for individuals with cystic fibrosis (CF) with positive effects on the gastrointestinal (GI) tract. There is emerging evidence linking CFTR dysfunction to celiac disease (CD). We present 3 cases of patients with CF, genotype F508del/G551D, treated with CFTR modulator, ivacaftor, and diagnosed with CD. These patients tested for CD because they had persistent GI symptoms that had partially improved with ivacaftor. This case series highlights the importance of a better understanding of how CFTR modulators impact the GI tract, their possible link to CD, and the importance of considering CD when evaluating GI symptoms in individuals with CF.