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Background: Depression and anxiety are two to four times more prevalent in people with CF (pwCF) than the general population. COVID-19 may exacerbate mental health challenges, increasing demand for psychological services, while decreasing their availability. We assessed the impact of the pandemic on depression and anxiety in pwCF, including how COVID-19 affected the frequency of mental health screening and the types of services provided., Methods: A 38-item internet survey, completed in June 2020, assessed how COVID-19 affected: 1) the mental health clinician's role and screening processes; 2) barriers to screening and resource needs; 3) impact of COVID-19 on depression and anxiety, and 4) positive outcomes and confidence in sustaining mental health screening and treatment, including telehealth services, after the pandemic., Results: Responses were obtained from 131 of the 289 US CF programs. Overall, 60% of programs (n=79) continued mental health screening and treatment, although less frequently; 50% provided individual tele-mental health interventions, and 9% provided telehealth group therapy. Clinically elevated depression symptoms (PHQ-9≥10; moderate to severe), were found in 12% of 785 pwCF, with 3.1% endorsing suicidal ideation. Similarly, elevated anxiety (moderate to severe; GAD-7≥10) was found in 13% of pwCF (n=779)., Conclusions: The COVID-19 pandemic created an opportunity to implement innovative solutions to disruptions in mental health screening and treatment in CF programs. We found that pwCF had increased access to psychological interventions during the pandemic via telehealth, supporting the continued integration of tele-mental health screening and treatment into CF care., (Copyright © 2021. Published by Elsevier B.V.)

Background: The International Depression Epidemiological Study (TIDES) found elevated rates of screen positivity for depression and anxiety among individuals with cystic fibrosis (CF). Depression is associated with worse adherence and health-related quality of life in CF. We investigated the relationship with mortality., Methods: Subjects were untransplanted participants in TIDES 12+ years of age receiving care at one of 45 collaborating US CF care centers who completed the Hospital Anxiety and Depression Scale and/or Center for Epidemiologic Studies Depression Scale during a stable visit between 2006 and 2010. Clinical characteristics and mortality data were obtained from the CF Foundation Patient Registry. The association of a positive screen with 5-year survival was evaluated using Cox Proportional Hazards modeling., Results: Of 1005 eligible patients, 25% screened positive for depression and 34% screened positive for anxiety. Patients who screened positive for depression were more likely to be older, have a residual function mutation, public insurance, and more pulmonary exacerbations in the screening year. There were 96 deaths. The unadjusted 5-year Hazard Ratio (HR) for death among those with depression was 2.0; 95% CI (1.3, 3.0)]. When adjusted for predetermined potential confounders the HR for the entire population was 1.4; 95% CI (0.9, 2.2). The adjusted HR was higher in adults [1.6; 95% CI (1.0, 2.4)] and those screening in the severe range [2.0; 95% CI (1.2, 3.4)]. Anxiety was not associated with mortality., Conclusions: A positive depression screen is associated with increased mortality among adults with CF. Research into the etiology of this relationship is needed., Competing Interests: Declaration of Competing Interest The authors report no conflicts of interest., (Copyright © 2020. Published by Elsevier B.V.)

Objective: The Hospital Anxiety and Depression Scale (HADS) is a widely used screening measure of anxiety and depression symptoms. However, prior analyses of the measure have found heterogeneous factor structures and called into question its ability to differentiate between symptoms of anxiety and depression. As part of efforts to implement mental health screening in cystic fibrosis (CF) care, the European Cystic Fibrosis Society (ECFS) and Cystic Fibrosis Foundation (CFF) conducted an international survey of 1,454 CF professionals. The HADS was the most commonly used measure in Europe and third most across all 48 countries surveyed. However, the HADS has not been validated for CF. Thus, the objective of this study was to examine its factor structure in a sample of adolescents and young adults with CF., Methods: Three theory-based models were tested in 727 individuals with CF (ages 12-25 years, 54% female) using confirmatory factor analyses, with an additional two models tested to improve model fit., Results: Chi-square difference tests and majority of fit indices indicated a three-factor structure based on Clark and Watson's tripartite model best fit the data., Conclusions: The original HADS two-factor structure demonstrated problematic fit in this sample, indicating poor discrimination between symptoms of anxiety and depression. A three-factor structure demonstrated best fit, indicating existing scoring guidelines and cutoffs would be inappropriate for use with this patient population. Use of the HADS to screen for anxiety and depression in CF could lead to an underestimation of clinically relevant symptomatology for depression and potential overestimation of anxiety symptoms.

Background: Treatment adherence in cystic fibrosis (CF) is often poor, however, less is known about adherence to pancreatic enzymes, a critical component of the CF treatment regimen. Parent caregivers often report elevations in depression, and parental depression may adversely affect children's adherence., Methods: This prospective study evaluated adherence to pancreatic enzymes in 83 patients (1-13 years) . Adherence was measured across 3 months with electronic pill-caps . Weight was measured at baseline and a 3-month follow-up. Parental depressive symptoms were evaluated by using the Center for Epidemiologic Studies Depression Scale (CES-D)., Results: Adherence to pancreatic enzymes was 49.4% ± 3.4%. Adherence was higher at school (94.4% ± 6.1%) than at home (42.3% ± 3.1%), and higher for toddlers (50.6% ± 5.2%) than for school-aged children (37.5% ± 3.7%). Parents reported high rates of depressive symptoms (30% in the clinical range, 18% with moderate symptoms). Children of parents with symptoms of depression versus those without were less adherent (34.8% ± 4.5% vs 48.5% ± 4.1%), and adherence to enzymes was significantly related to 3-month weight outcomes. Average gain in weight z scores across 3 months was 0.5 ± 0.2for children who were >50% adherent and -0.1 ± 6.1for children who were <33% adherent. Parental depression had a signifcant, indirect effect on weight via adherence (-0.005 ± 0.003 gain in weight z score per CES-D unit )., Conclusions: High rates of parental depressive symptoms, coupled with its negative effects on adherence, suggest that measuring and treating parental depression may improve children's adherence to therapy., (Copyright © 2016 by the American Academy of Pediatrics.)

Rationale: Cystic fibrosis is a progressive disease requiring a complex, time-consuming treatment regimen. Nonadherence may contribute to an acceleration of the disease process. Spirituality influences some parental healthcare behaviors and medical decision-making., Objectives: We hypothesized that parents of children with cystic fibrosis, when classified into groups based on adherence rates, would share certain psychosocial and religious and/or spiritual variables distinguishing them from other adherence groups., Methods: We conducted a multisite, prospective, observational study focused on parents of children younger than 13 years old at two cystic fibrosis center sites (Site 1, n= 83; Site 2, n = 59). Religious and/or spiritual constructs, depression, and marital adjustment were measured by using previously validated questionnaires. Determinants of adherence included parental attitude toward treatment, perceived behavioral norms, motivation, and self-efficacy. Adherence patterns were measured with the Daily Phone Diary, a validated instrument used to collect adherence data. Cluster analysis identified discrete adherence patterns, including parents' completion of more treatments than prescribed., Measurements and Main Results: For airway clearance therapy, four adherence groups were identified: median adherence rates of 23%, 52%, 77%, and 120%. These four groups differed significantly for parental depression, sanctification of their child's body, and self-efficacy. Three adherence groups were identified for nebulized medications: median adherence rates of 35%, 82%, and 130%. These three groups differed significantly for sanctification of their child's body and self-efficacy., Conclusions: Our results indicated that parents in each group shared psychosocial and religious and/or spiritual factors that differentiated them. Therefore, conversations about adherence likely should be tailored to baseline adherence patterns. Development of efficacious religious and/or spiritual interventions that promote adherence by caregivers of children with cystic fibrosis may be useful.

Background: Individuals with chronic diseases and parent caregivers are at increased risk for symptoms of depression and anxiety. Prevalence of psychological symptoms was evaluated in adolescents and adults with cystic fibrosis (CF) and parent caregivers across nine countries., Methods: Patients with CF, ages 12 years and older, and caregivers of children with CF, birth to18 years of age, completed measures of depression and anxiety across 154 CF centres in Europe and the USA. Psychological symptoms were compared across countries using χ(2). Logistic regression examined extent of comorbid symptoms, predictors of depression and anxiety, and concordance between parent and adolescent symptomatology., Results: Psychological symptoms were reported by 6088 patients with CF and 4102 parents. Elevated symptoms of depression were found in 10% of adolescents, 19% of adults, 37% of mothers and 31% of fathers. Elevations in anxiety were found in 22% of adolescents, 32% of adults, 48% of mothers and 36% of fathers. Overall, elevations were 2-3 times those of community samples. Participants reporting elevated anxiety were more likely to report depression (ORs: adolescents=14.97, adults=13.64, mothers=15.52, fathers=9.20). Significant differences in reports of depression and anxiety were found by patient age and parent respondent. Concordance between 1122 parent-teen dyads indicated that adolescents whose parents reported depression were more likely to be elevated on depression (OR=2.32). Similarly, adolescents whose parents reported anxiety were more likely to score in the elevated range on the anxiety measure (OR=2.22)., Conclusions: Symptoms of depression and anxiety were elevated in both patients with CF and parents across several European countries and the USA. Annual screening of psychological symptoms is recommended for both patients and parents., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Background and Purpose: Bronchiectasis causes pulmonary infections and loss of lung function, resulting in chronic respiratory symptoms and worsening health-related quality of life. The aims of this study were to measure symptoms of depression and anxiety in a sample of patients with bronchiectasis and evaluate their relationship to health outcomes and health-related quality of life., Methods: This cross-sectional study included adolescents and adults with bronchiectasis. Patients completed the hospital anxiety and depression scale and the St. George respiratory questionnaire. Health outcome data, including clinical, radiological and spirometric values, were recorded from medical charts., Results: Ninety-three participants with bronchiectasis of any aetiology were recruited: 20 % had elevated depression-related scores and 38 % had elevated anxiety-related scores. Increased symptoms of depression and anxiety were significantly associated with age; anxiety was associated with more frequent exacerbations. Regression analyses indicated that after controlling for demographic (gender and age) and clinical variables (exacerbations frequency, daily sputum, aetiology and spirometry), both depression and anxiety symptoms predicted significantly worse health-related quality of life. In comparison with other predictors, psychological symptoms explained the largest amount of variance in health-related quality of life., Conclusions: Symptoms of depression and anxiety were significant predictors of health-related quality of life in patients with bronchiectasis, independently of respiratory involvement, gender, age or other variables.

Background: Chronic illness is a significant risk factor for the development of internalizing psychopathology; however, evidence for the prevalence of these symptoms in patients with cystic fibrosis (CF) is limited. We investigated the prevalence of symptoms of anxiety and depression in German-speaking patients with CF and the association of these symptoms to physical health status., Methods: A representative sample of German patients with CF (N = 670; age range, 12-64 years; 52.7% men) completed the Hospital Anxiety and Depression Scale. Their medical data were taken from the German CF registry. Data on the study sample were compared with data on a control group from the German general population., Results: Elevated anxiety scores were found in 20.6% of the patients with CF, and 9.6% reported high levels of symptoms of depression. Adult patients with CF reported more elevated symptoms of anxiety than healthy control subjects, whereas no age group of patients was more or less depressed than the general population. Younger patients reported fewer symptoms of anxiety and depression than older patients, and women reported more symptoms of anxiety than men. Recent hemoptysis/pneumothorax and recent diagnosis of diabetes were associated with anxiety, whereas impaired lung function and transplant listing status were associated with depression., Conclusions: Anxiety in particular is an important issue for a large proportion of patients with CF. The risk of depression increased with greater impairment in pulmonary function. Annual screening of symptoms of anxiety and depression as well as appropriate referrals for those in the clinically elevated range are recommended.

Objective: Evaluate depressive symptoms in caregivers of children with type 1 diabetes (T1D) or cystic fibrosis (CF) and identify associated risk factors., Methods: A total of 195 caregivers completed demographic, stress, and depressive symptoms questionnaires. Children's health status was obtained from medical records., Results: Approximately 33% of caregivers reported elevated symptoms of depression (i.e., exceeded clinical cutoff of 16 on the Center for Epidemiological Studies-Depression Scale). For caregivers of children with T1D, elevations were associated with less caregiver education, more family stress, older child age, and worse glycemic control. For caregivers of children with CF, more family stress and lack of employment outside of the home were associated with elevated depressive symptoms., Conclusions: Many caregivers of children with T1D or CF experience depressive symptoms, although risk factors may differ in these two populations. Screening of caregiver depressive symptoms as part of routine clinic visits may provide opportunities for needed intervention.

Background: Little is currently known about the co-morbidity of depression and cystic fibrosis (CF) and there is currently no empirical research on the effects of depressive symptoms on adherence in children and adolescents with CF. The primary aim of this study was to evaluate the extent of depressive symptoms in children and adolescents with CF and their parents, and determine whether depressive symptoms in the child and/or parent was associated with adherence to airway clearance. We also evaluated whether children's perceptions of relational security with a parent were associated with adherence to airway clearance., Methods: Participants included 39 children with CF ages 7-17 years and their primary caregivers. Depressive symptoms were measured with the Children's Depression Inventory (CDI) and the Center for Epidemiological Studies-Depression Scale (CES-D). The Relatedness Questionnaire assessed the quality of parent-child relational security. Adherence to airway clearance was measured using the daily phone diary (DPD), an empirically validated adherence measure for youth with CF., Results: Rates of depressive symptoms were elevated in children with CF and their parents (29% for children; 35% for mothers; 23% for fathers). Child depressive symptoms were significantly associated with lower rates of adherence to airway clearance, after controlling for demographic variables (r = -0.34, P = 0.02). Child depressive symptoms were associated with worse perceptions of parental relationships (t(35) = 3.2; P = 0.002) and the quality of this relationship was also related to worse adherence (r = 0.42, P = 0.005)., Conclusions: A large percentage of youth with CF and their parents reported elevated symptoms of depression. Children scoring in the depressed range on a standardized screening measure and those with less secure parent-child relationships were at greatest risk for poor adherence. Thus, depressive symptoms and family relationships are appropriate targets for adherence promotion interventions, which may ultimately improve health outcomes., (Copyright 2010 Wiley-Liss, Inc.)

Symptoms of anxiety and depression have important consequences for patients with chronic disease, including lower rates of adherence and increased morbidity. This review discusses the prevalence of anxiety and depression in patients with cystic fibrosis (CF) across the life span and highlights current understanding of how these symptoms affect adherence, health outcomes, and health-related quality of life (HRQOL). Anxiety and depression appear to be more common in patients with CF than in the general population. Recent studies also indicate that symptoms of anxiety occur more frequently than depression in this population. Both anxiety and depression have been shown to be associated with poorer disease outcomes, including HRQOL. Depression, specifically, leads to lower adherence to recommended therapies. Given the prevalence and impact of anxiety and depression in CF, it seems appropriate to recommend annual screening using a validated approach, with referral for either pharmacotherapy or cognitive behavioral therapy of those with apparent symptoms. A large-scale epidemiological study of anxiety and depression in CF is under way and should provide better prevalence estimates and more information on the effects of these psychological symptoms., (Copyright Thieme Medical Publishers.)

Purpose of Review: Numerous studies have demonstrated that patients with chronic illnesses are at an increased risk for depression, with serious direct and indirect consequences for health outcomes. The few studies examining rates of depression in patients with cystic fibrosis (CF) or parent caregivers have found clinically significant elevations. Given the importance of identifying and treating symptoms of depression, the purpose of this review was to highlight recent data on the prevalence and impact of depression in CF patients and to propose recommendations for screening and treatment., Recent Findings: Recent studies in CF have revealed higher rates of depression in children, adolescents, adults, and parent caregivers than in healthy populations. Evidence also suggests that depression has negative effects on treatment adherence, family functioning, and health-related quality of life. Briefly, well validated screening tools for depression are available but are not currently utilized in routine CF care. Effective psychological and pharmacological interventions are available to treat depression, but have not yet been evaluated in patients with CF., Summary: Depression is a significant risk factor for the effective management of CF. Screening and treatment of depression in CF centers is recommended and holds promise for improving social, emotional, and physical health outcomes.

Applied Goldfriend and D'Zurilla's (1969) Behavior-Analytic Model to the development of a context-specific measure of stressful situations for adolescents with a serious, chronic illness. In completing the situational analysis phase of the model, 45 adolescents with cystic fibrosis (CF), 20 parents of adolescents with CF, and 8 health care professionals completed structured interviews or daily diaries to obtain the widest range of problematic situations. The adolescent sample was recruited from two different medical centers, and stratified by sex and illness severity. A total of 1,174 problem situations were elicited across all participants and then content-analyzed into 164 nonredundant items in 10 domains (e.g., Medications and Treatment, and School). Few relations were found between demographic variables (e.g., age and illness severity) and the number or difficulty of problematic situations. Both adolescents and parents mentioned the greatest number of problematic situations in the domains of School, Medications and Treatment, and Parent-Teen Relationship. In terms of difficulty, all three respondents (i.e., teens, parents, and health care professionals) rated problems with Clinic and Hospital Visits as very difficult. For the adolescent sample, problems in the Parent-Teen Relationship and Health Concerns were also highly difficult. Significant associations were found between the problematic situations we identified and standardized measures of social and emotional functioning. Adolescents who rated their problems as more difficult also endorsed more symptoms of depression and lower perceptions of social competence. In a future study, the most salient items will be selected to create a role-play measure to elicit adolescents' coping strategies.