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.

Treatment Preference Among People With Cystic Fibrosis: The Importance of Reducing Treatment Burden.

BACKGROUND: There is a growing consensus that the perspective of the patient should be considered in the evaluation of novel interventions. RESEARCH QUESTION: What treatment outcomes matter to people with cystic fibrosis (CF), and what trade-offs would they make to realize these outcomes? STUDY DESIGN AND METHODS: Adults attending a specialist CF center were invited to complete an online discrete choice experiment (DCE). The DCE required participants to evaluate hypothetical CF treatment profiles, defined by impact on lung function, pulmonary exacerbations, abdominal symptoms, life expectancy, quality of life, inhaled medicine usage, and physiotherapy requirement. Choice data were analyzed, using multinomial logit and latent class models. RESULTS: One hundred and three people with CF completed the survey (median age, 35 years; range, 18-76 years); 52% were female; mean FEV1 % predicted, 69% [SD, 22%]). On average, an improvement in life expectancy by 10 years or more had the greatest impact on treatment preference, followed by a 15% increase in lung function. However, it was shown that people would trade substantial reductions in these key outcomes to reduce treatment time or burden. Preference profiles were not uniform across the sample: three distinct subgroups were identified, each placing markedly different importance on the relative importance of both life expectancy and lung function compared with other attributes. INTERPRETATION: The relative importance of treatment burden to people with CF, compared with life expectancy and lung function, suggests it should be routinely captured in clinical trials as an important secondary outcome measure. When considering the patient perspective, it is important that decision-makers recognize that the values of people with CF are not homogeneous.

Understanding the sleep-aggression relationship in a forensic mental health sample.

The contribution of cognition to the sleep-aggression relationship is explored via three connected studies, involving adult male forensic patients detained in a high secure hospital. Study 1 included 31 patients, interviewed to examine their experiences of specific sleep problems. In Study 2, 42 patients completed a series of measures examining sleep dysfunction, aggression, and cognition, while Study 3 was designed to impact on sleep via a cognitive approach. In the latter, 48 patients were randomly assigned as part of a feasibility trial to one of three conditions: mindfulness (cognitive approach), sleep education, and treatment as usual. Collectively, the studies demonstrated the multifaceted nature of cognition in the sleep-aggression relationship, with a need to account fully for cognitive factors. A preliminary conceptual model is outlined - the Cognitive Sleep Model for Aggression and Self Harm (CoSMASH), as a direction for future research to consider.

Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis.

BACKGROUND: Being able to function cognitively is imperative for successful achievement in school, working life, and disease self-management. Diabetes is known to cause changes in brain structure and long-term cognitive dysfunction. This work investigated cystic fibrosis-related diabetes (CFRD) as a mechanism for cognitive impairment in people with CF. It was hypothesised that cognition would be poorer in adults with CFRD than in those with CF without diabetes (CFND) or in healthy controls. METHODS: Cognitive performance was assessed using the Cambridge Neuropsychological Test Automated Battery which provides a comprehensive cognitive assessment with tests mapping onto specific brain regions. Demographic, clinical and self-reported health data were documented for all participants. CF specific clinical variables were recorded for the two CF groups. RESULTS: Ninety-eight people with CF (49CFRD,49CFND) and 49 healthy controls were recruited. People with CF demonstrated deficits in aspects of verbal and spatial memory, processing speed and cognitive flexibility compared with healthy controls, with all areas of the brain implicated. Those with CFRD had additional difficulties with higher-level processes known collectively as 'executive function', which demand greater cognitive load and recruit the prefrontal cortex. Compared with healthy controls, those with CFND and CFRD had an estimated 20% and up to 40% reduction in processing speed respectively. CONCLUSION: Managing CF requires higher order executive function. Impairments may be sufficient to interfere with self-care and the ability to perform everyday tasks efficiently. At which point in the CF disease trajectory these difficulties begin, and what may attenuate them, has yet to be determined.

Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis [version 1; peer review: 2 approved].

Background: Despite the importance of reducing treatment burden for people with cystic fibrosis (CF), it has not been fully understood as a concept. This study aims to quantify the treatment burden perceived by CF adults and explore the association between different validated treatment burden measures. Methods: This is a cross-sectional observational study of CF adults attending a single large UK adult center. Participants completed an online survey that contained three different treatment burden scales; CF Questionnaire-Revised (CFQ-R) subscale, CF Quality of Life (CFQoL) subscale, and the generic multimorbidity treatment burden questionnaire (MTBQ). Results: Among 101 participants, the median reported treatment burden by the CFQ-R subscale was 55.5 (IQR 33.3 - 66.6), the CFQoL subscale was 66.6 (IQR 46.6 - 86.6), and the MTBQ reversed global score was 84.6 (IQR 73.1 - 92.3). No correlation was found between respondents' demographic or clinical variables and treatment burden measured via any of the three measures. All treatment burden measures showed correlations against each other. More treatments were associated with high treatment burden as measured by the CFQ-R, CFQoL subscales, and the MTBQ. However, longer treatment time and more complex treatment plans were correlated with high treatment burden as measured by the CFQ-R and CFQoL subscales, but not with the MTBQ. Conclusions: Treatment burden is a substantial issue in CF. Currently, the only available way to evaluate it is with the CF-specific quality of life measure treatment burden subscales (CFQ-R and CFQoL); both indicated that treatment burden increases with more treatments, longer treatment time, and more complex treatments.

Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis.

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.

Flares in patients with systemic lupus erythematosus.

OBJECTIVE: SLE is characterized by relapses and remissions. We aimed to describe the frequency, type and time to flare in a cohort of SLE patients. METHODS: SLE patients with one or more 'A' or 'B' BILAG-2004 systems meeting flare criteria ('new' or 'worse' items) and requiring an increase in immunosuppression were recruited from nine UK centres and assessed at baseline and monthly for 9 months. Subsequent flares were defined as: severe (any 'A' irrespective of number of 'B' flares), moderate (two or more 'B' without any 'A' flares) and mild (one 'B'). RESULTS: Of the 100 patients, 94% were female, 61% White Caucasians, mean age (s.d.) was 40.7 years (12.7) and mean disease duration (s.d.) was 9.3 years (8.1). A total of 195 flares re-occurred in 76 patients over 781 monthly assessments (flare rate of 0.25/patient-month). There were 37 severe flares, 32 moderate flares and 126 mild flares. By 1 month, 22% had a mild/moderate/severe flare and 22% had a severe flare by 7 months. The median time to any 'A' or 'B' flare was 4 months. Severe/moderate flares tended to be in the system(s) affected at baseline, whereas mild flares could affect any system. CONCLUSION: . In a population with active SLE we observed an ongoing rate of flares from early in the follow-up period with moderate-severe flares being due to an inability to fully control the disease. This real-world population study demonstrates the limitations of current treatments and provides a useful reference population from which to inform future clinical trial design.

Integration of mental health screening and treatment into cystic fibrosis clinics: Evaluation of initial implementation in 84 programs across the United States.

BACKGROUND: A large-scale epidemiological study of 6088 individuals with cystic fibrosis (CF) and 4102 caregivers in nine countries documented elevated symptoms of depression and anxiety, leading to international guidelines for annual screening and follow-up. To facilitate national implementation, 84 CF programs funded a mental health coordinators (MHC). Implementation was evaluated after 1 year using the consolidated framework for implementation research (CFIR) to identify facilitators and barriers. METHODS: A 45-item internet survey was developed to assess relevant CFIR implementation steps. Surveys were completed in 2016. It assessed five domains tailored to study aims: (a) Intervention characteristics, (b) outer setting, (c) inner setting, (d) characteristics of individuals, and (e) process of implementation. RESULTS: Response rate was 88%, with pediatric and adult programs equally represented. A majority of MHCs were social workers (54.1%) and psychologists (41.9%); 41% had joined the team in the past year. Facilitators across the five domains included universal uptake of screening tools, greater awareness and detection of psychological symptoms, reduced stigma, and positive feedback from patients and families. Barriers included limited staff time, space, and logistics. DISCUSSION: This is the largest systematic effort to integrate mental health screening and treatment into the care of individuals with a serious, chronic illness and their caregivers. MHCs implementing screening, interpretation and follow-up reported positive results, and significant barriers. This national implementation effort demonstrated that depression and anxiety can be efficiently evaluated and treated in a complex, chronic disease. Future efforts include recommending the addition of screening scores to national CF Registries and examining their effects on health outcomes.

An educational intervention to prevent overweight in pre-school years: a cluster randomised trial with a focus on disadvantaged families.

BACKGROUND: Early prevention is a promising strategy for reducing obesity in childhood, and Early Years settings are ideal venues for interventions. This work evaluated an educational intervention with the primary aim of preventing overweight and obesity in pre-school children. METHODS: A pragmatic, cluster randomised trial with a parallel, matched-pair design was undertaken. Interventions were targeted at both the cluster (Early Years' Centres, matched by geographical area) and individual participant level (families: mother and 2-year old child). At the cluster level, a staff training intervention used the educational resource Be Active, Eat Healthy. Policies and provision for healthy eating and physical activity were evaluated at baseline and 12-months. The intervention at participant level was the Healthy Heroes Activity Pack: delivered over 6 months by Centre staff to promote healthy eating and physical activity in a fun, interactive way. Child and parent height and weight were measured at four time-points over 2 years. The trial primary outcome was the change in BMI z-score of the child between ages 2 and 4 years. Secondary outcomes consisted of parent-reported measures administered at baseline and two-year follow-up. RESULTS: Five pairs of Early Years' Centres were recruited. Four pairs were analysed as one Centre withdrew (47 intervention families; 34 control families). At the cluster level, improvement in Centre policies and practices was similar for both groups (p = 0.830). At the participant level, the intervention group reduced their mean BMI z-score between age 2 and 4 years (p = 0.002; change difference 0.49; 95% CI 0.17 to 0.80) whereas the control group showed increasing BMI z-score throughout. Changes in parent-reported outcomes and parent BMI (p = 0.582) were similar in both groups. CONCLUSIONS: The Healthy Heroes educational resource deterred excess weight gain in pre-school children from poor socioeconomic areas. With training, Early Years' staff can implement the Healthy Heroes programme. TRIAL REGISTRATION: ISRCTN22620137 Registered 21st December 2016.

Reciprocal Prospective Relationships Between Loneliness and Weight Status in Late Childhood and Early Adolescence.

Adolescents who do not conform to weight ideals are vulnerable to disapproval and victimization from peers in school. But, missing from the literature is a prospective examination of weight status and feelings of loneliness that might come from those experiences. Using data from the Québec Longitudinal Study of Child Development, we filled that gap by examining the prospective associations between loneliness and weight status when the sample was aged 10-13 years. At ages 10, 12, and 13 years, 1042 youth (572 females; 92% from French speaking homes) reported on their loneliness and were weighed and measured. Family income sufficiency was included in our analyses given its relationship with weight status, but also its possible link with loneliness during early adolescence. The findings showed that (1) weight status and loneliness were not associated concurrently; (2) weight status predicted increases in loneliness from ages 12 to 13 years; and (3) loneliness predicted increases in weight from ages 12 to 13 years among female adolescents, but weight loss among male adolescents. The fact that loneliness was involved in weight gain for females suggests that interventions focused on reducing loneliness and increasing connection with peers during early adolescence could help in reducing obesity.

Implementing the International Committee on Mental Health in Cystic Fibrosis (ICMH) guidelines: Screening accuracy and referral-treatment pathways.

BACKGROUND: The International Committee on Mental Health (ICMH) published screening guidelines in Cystic Fibrosis (CF). This work 1) evaluated the sensitivity of the recommended screening tools against the 'gold standard' clinical psychological assessment and 2) investigated referral and treatment pathways. METHODS: Ninety-six participants (79 caregivers; 17 adolescents with CF) completed the screening tools prior to formal assessment. Agreement between screening data and psychological assessment was evaluated, sensitivity analyses performed and referral pathways tracked. RESULTS: All participants with an elevated screen (moderate/severe range) were subsequently assessed as requiring treatment for major depression/anxiety disorders. However, many were referred for treatment without elevated scores. Hence, sensitivity was poor with the recommended threshold score of 10, but with a threshold of 5 the sensitivity was 76% for adults and 46% for adolescents. The area under the ROC curve (diagnostic test ability) was 0.89 for caregivers but lower at 0.68 for adolescents. CONCLUSION: Mental health screening is complex, particularly in adolescents. Nonetheless, it is a first valuable step to improve mental health care in CF. The need for psychological support is greater than anticipated by the TIDES study.

Initial development of the Psychopathic Processing and Personality Assessment (PAPA) across populations.

Three studies describe development of the Psychopathic Processing and Personality Assessment (PAPA). Study one outlines a literature review and Expert Delphi (n=32) to develop the initial PAPA. Study two validates the PAPA with 431 participants (121 male prisoners and 310 university students: 154 men, 156 women), also using the Levenson Self Report Psychopathy scale and a measure of cognitive schema and affect. Study three refined the PAPA, employing it with 50 male students and 40 male forensic psychiatric patients using clinical (interview) assessments of psychopathy: the Psychopathy Checklist - Screening Version and the Affect, Cognitive and Lifestyle assessment. The PAPA comprised four factors; dissocial tendencies; emotional detachment; disregard for others; and lack of sensitivity to emotion. It positively correlated with existing psychopathy measures. Variations across PAPA subscales were noted across samples when associated with clinical measures of psychopathy. Support for the validity of the PAPA was indicated across samples. Directions for research and application are outlined.

Pain experience, expression and coping in boys and young men with Duchenne Muscular Dystrophy - A pilot study using mixed methods.

UNLABELLED: There is limited research exploring the pain experience of boys and young men with Duchenne Muscular Dystrophy. METHODS: We conducted a mixed-methods pilot study to assess the feasibility of using particular measures of pain, pain coping and quality of life within semi-structured interviews with boys and young men with Duchenne Muscular Dystrophy and a postal survey of their parents. Non-probability, convenience sampling was used. RESULTS: Twelve young men aged 11-21 years (median 15 years), three of whom were still ambulant, and their parents/guardians were recruited. The measures used were acceptable to the young men and demonstrated potential to provide useful data. Two-thirds of young men suffered from significant daily pain which was associated with reduced quality of life. Pain complaints were largely kept within the family. Young men's pain-coping strategies were limited by their restricted physical abilities. Statistical power based on these preliminary results suggests a study of approximately 50 boys/young men which appears feasible. CONCLUSIONS: Further study is needed to explore acceptable and effective methods of pain management in this population and ways of enhancing pain-coping strategies. In clinical practice, assessment of pains and discomfort should form part of all routine consultations.

Sensitivity to Change and Minimal Important Differences of the LupusQoL in Patients With Systemic Lupus Erythematosus.

OBJECTIVE: As a health-related quality of life (HRQOL) measure, the LupusQoL is a reliable and valid measure for adults with systemic lupus erythematosus (SLE). This study evaluates the responsiveness and minimal important differences (MIDs) for the 8 LupusQoL domains. METHODS: Patients experiencing a flare were recruited from 9 UK centers. At each of the 10 monthly visits, HRQOL (LupusQoL, Short Form 36 health survey [SF-36]), global rating of change (GRC), and disease activity using the British Isles Lupus Assessment Group 2004 index were assessed. The responsiveness of the LupusQoL and the SF-36 was evaluated primarily when patients reported an improvement or deterioration on the GRC scale and additionally with changes in physician-reported disease activity. MIDs were estimated as mean changes when minimal change was reported on the GRC scale. RESULTS: A total of 101 patients were recruited. For all LupusQoL domains, mean HRQOL worsened when patients reported deterioration and improved when patients reported an improvement in GRC; SF-36 domains showed comparable responsiveness. Improvement in some domains of the LupusQoL/SF-36 was observed with a decrease in disease activity, but when disease activity worsened, there was no significant change. LupusQoL MID estimates for deterioration ranged from -2.4 to -8.7, and for improvement from 3.5 to 7.3; for the SF-36, the same MID estimates were -2.0 to -11.1 and 2.8 to 10.9, respectively. CONCLUSION: All LupusQoL domains are sensitive to change with patient-reported deterioration or improvement in health status. For disease activity, some LupusQoL domains showed responsiveness when there was improvement but none for deterioration. LupusQoL items were derived from SLE patients and provide the advantage of disease-specific domains, important to the patients, not captured by the SF-36.

International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety.

Studies measuring psychological distress in individuals with cystic fibrosis (CF) have found high rates of both depression and anxiety. Psychological symptoms in both individuals with CF and parent caregivers have been associated with decreased lung function, lower body mass index, worse adherence, worse health-related quality of life, more frequent hospitalisations and increased healthcare costs. To identify and treat depression and anxiety in CF, the CF Foundation and the European CF Society invited a panel of experts, including physicians, psychologists, psychiatrists, nurses, social workers, a pharmacist, parents and an individual with CF, to develop consensus recommendations for clinical care. Over 18 months, this 22-member committee was divided into four workgroups: Screening; Psychological Interventions; Pharmacological Treatments and Implementation and Future Research, and used the Population, Intervention, Comparison, Outcome methodology to develop questions for literature search and review. Searches were conducted in PubMed, PsychINFO, ScienceDirect, Google Scholar, Psychiatry online and ABDATA by a methodologist at Dartmouth. The committee reviewed 344 articles, drafted statements and set an 80% acceptance for each recommendation statement as a consensus threshold prior to an anonymous voting process. Fifteen guideline recommendation statements for screening and treatment of depression and anxiety in individuals with CF and parent caregivers were finalised by vote. As these recommendations are implemented in CF centres internationally, the process of dissemination, implementation and resource provision should be closely monitored to assess barriers and concerns, validity and use.

Longitudinal impact of demographic and clinical variables on health-related quality of life in cystic fibrosis.

OBJECTIVES: The insights that people with cystic fibrosis have concerning their health are important given that aspects of health-related quality of life (HRQoL) are independent predictors of survival and a decrease in lung function is associated with a decrease in HRQoL over time. Cross-sectional data suggest that key variables, other than lung function, are also associated with HRQoL--although study results are equivocal. This work evaluates the relationship between these key demographic and clinical variables and HRQoL longitudinally. DESIGN: Longitudinal observational study. Observations were obtained at seven time points: approximately every 2 years over a 12-year period. SETTING: Large adult cystic fibrosis centre in the UK. PARTICIPANTS: 234 participants aged 14-48 years at recruitment. OUTCOME MEASURE: Nine domains of HRQoL (Cystic Fibrosis Quality of Life Questionnaire) in relation to demographic (age, gender) and clinical measures (forced expiratory volume in 1 s, (FEV1)% predicted, body mass index (BMI), cystic fibrosis-related diabetes, Burkholderia cepacia complex, totally implantable vascular access device, nutritional and transplant status). RESULTS: A total of 770 patient assessments were obtained for 234 patients. The results of random coefficients modelling indicated that demographic and clinical variables were identified as being significant for HRQoL over time. In addition to lung function, transplant status, age, having a totally implantable vascular access device, cystic fibrosis-related diabetes, BMI and B. cepacia complex impacted on many HRQoL domains longitudinally. Gender was important for the domain of body image. CONCLUSIONS: Demographic and changes in clinical variables were independently associated with a change in HRQoL over time. Compared with these longitudinal data, cross-sectional data are inadequate when evaluating the relationships between HRQoL domains and key demographic and clinical variables, as they fail to recognise the full impact of the CF disease trajectory and its treatments on quality of life.