Assessing disease disclosure in adults with cystic fibrosis: the Adult Data for Understanding Lifestyle and Transitions (ADULT) survey Disclosure of disease in adults with cystic fibrosis

<p>Abstract</p> <p>Background</p> <p>As more patients with cystic fibrosis (CF) reach adulthood and participate in age-appropriate activities (e.g. employment, dating), disclosure of medical status becomes more important. This study assessed rates of disclosure and its perceived impact on relationships using the Adult Data for Understanding Lifestyle and Transitions (ADULT) online survey.</p> <p>Methods</p> <p>Adults with CF participated in the survey via the United States national network of CF Centers. Descriptive and inferential statistics were utilized.</p> <p>Results</p> <p>Participants (n = 865) were more likely to disclose to relatives (94%) and close friends (81%) than to dating partners (73%), bosses/supervisors/teachers (51%) or co-workers (39%). Respondents generally reported a neutral/positive effect on relationships following disclosure. Negative effects of disclosure were infrequent, but more likely with dating partners or bosses/supervisors/teachers. Results also indicated that disclosure may be influenced by severity of lung disease and gender, with those having normal/mild lung disease less likely to disclose their diagnosis to both co-workers (p < 0.01) and bosses/supervisors/teachers (p < 0.01), and women being more likely to disclose to close friends (p < 0.0001) and dating partners (p < 0.05) than men.</p> <p>Conclusions</p> <p>Most adults with CF disclosed their disease to relatives and close friends. Individuals with severe CF lung disease were more likely to disclose their diagnosis to coworkers and supervisors/teachers. It may be helpful to provide support for disclosure of disease in situations such as employment and dating.</p

Psychological treatments for people with epilepsy.

BackgroundGiven the significant impact epilepsy may have on the health-related quality of life (HRQOL) of individuals with epilepsy and their families, there is increasing clinical interest in evidence-based psychological treatments, aimed at enhancing psychological and seizure-related outcomes for this group. This is an updated version of the original Cochrane Review published in Issue 10, 2017.ObjectivesTo assess the impact of psychological treatments for people with epilepsy on HRQOL outcomes.Search methodsFor this update, we searched the following databases on 12 August 2019, without language restrictions: Cochrane Register of Studies (CRS Web), which includes randomized or quasi-randomized controlled trials from the Specialized Registers of Cochrane Review Groups including Epilepsy, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (Ovid, 1946 to 09 August 2019), and PsycINFO (EBSCOhost, 1887 onwards), and from PubMed, Embase, ClinicalTrials.gov, and the World Health Organization International Clinical Trials Registry Platform (ICTRP). We screened the references from included studies and relevant reviews, and contacted researchers in the field for unpublished studies.Selection criteriaWe considered randomized controlled trials (RCTs) and quasi-RCTs for this review. HRQOL was the main outcome. For the operational definition of 'psychological treatments', we included a broad range of skills-based psychological treatments and education-only interventions designed to improve HRQOL, seizure frequency and severity, as well as psychiatric and behavioral health comorbidities for adults and children with epilepsy. These psychological treatments were compared to treatment as usual (TAU), an active control group (such as social support group), or antidepressant pharmacotherapy.Data collection and analysisWe used standard methodological procedures expected by Cochrane.Main resultsWe included 36 completed RCTs, with a total of 3526 participants. Of these studies, 27 investigated skills-based psychological interventions. The remaining nine studies were education-only interventions. Six studies investigated interventions for children and adolescents, three studies investigated interventions for adolescents and adults, and the remaining studies investigated interventions for adults. Based on satisfactory clinical and methodological homogeneity, we pooled data from 11 studies (643 participants) that used the Quality of Life in Epilepsy-31 (QOLIE-31) or other QOLIE inventories (such as QOLIE-89 or QOLIE-31-P) convertible to QOLIE-31. We found significant mean changes for the QOLIE-31 total score and six subscales (emotional well-being, energy and fatigue, overall QoL, seizure worry, medication effects, and cognitive functioning). The mean changes in the QOLIE-31 total score (mean improvement of 5.23 points, 95% CI 3.02 to 7.44; P Authors' conclusionsImplications for practice: Skills-based psychological interventions improve HRQOL in adults and adolescents with epilepsy. Adjunctive use of skills-based psychological treatments for adults and adolescents with epilepsy may provide additional benefits in HRQOL when these are incorporated into patient-centered management. We judge the evidence to be of moderate certainty.Implications for researchInvestigators should strictly adhere to the CONSORT guidelines to improve the quality of reporting on their interventions. A thorough description of intervention protocols is necessary to ensure reproducibility. When examining the effectiveness of psychological treatments for people with epilepsy, the use of standardized HRQOL inventories, such as the Quality of Life in Epilepsy Inventories (QOLIE-31, QOLIE-31-P, and QOLIE-89) would increase comparability. Unfortunately, there is a critical gap in pediatric RCTs and RCTs including people with epilepsy and intellectual disabilities. Finally, in order to increase the overall quality of RCT study designs, adequate randomization with allocation concealment and blinded outcome assessment should be pursued. As attrition is often high in research that requires active participation, an intention-to-treat analysis should be carried out. Treatment fidelity and treatment competence should also be assessed. These important dimensions, which are related to 'Risk of bias' assessment, should always be reported

Barriers to Treatment Adherence for Children with Cystic Fibrosis and Asthma: What Gets in the Way?

The purpose of this study was to systematically identify barriers to treatment adherence for children with cystic fibrosis (CF) and asthma, as well as to examine the relationship between the number of barriers and adherence. Participants included 73 children with CF or asthma and their parents. The mean age of the sample was 9.9 years, and 58% were males. Results indicated that barriers were quite similar by illness and informant (e.g., parent and child) for the same treatments, but unique barriers were identified for disease-specific treatments. Frequently mentioned barriers across diseases included forgetting, oppositional behaviors, and difficulties with time management. Trends were identified between adherence and barriers, suggesting that a greater number of barriers were related to poorer adherence. Overall, this study provided evidence that patients and their parents experience specific barriers within the context of their own illness and highlights the need for disease-specific measures and interventions

Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis

The purpose of the current study was to evaluate the psychometric properties of the Cystic Fibrosis Questionnaire (CFQ)-Child version, a disease-specific health-related quality of life (HRQOL) measure for children with cystic fibrosis (CF). The CFQ was administered to 84 children with CF, ranging in age from 7 to 13 years, and their parents. Multitrait analyses indicated that a majority of items on the CFQ-Child correlated more highly with their hypothesized scale than a competing scale. Internal consistency coefficients were acceptable for all scales (Cronbach's alpha =.60-.76), with the exception of treatment burden (Cronbach's alpha =.44). Results also suggested strong convergence between child and parent-proxy reports on several scales of the CFQ. Results demonstrated that the CFQ-Child is a reliable and valid measure of HRQOL for children with CF

Utilizing Computerized Phone Diary Procedures to Assess Health Behaviors in Family and Social Contexts

The purpose of this study was to illustrate how the daily phone diary (DPD) can be used to measure adherence behaviors in 2 pediatric pulmonary populations, cystic fibrosis (CF; n ??31) and asthma (n ??30). Computerized phone interview data was used to conduct activity pattern analyses, which revealed that parents of children with CF spent significantly more time in medical care and less time in recreation. Reasonable agreement was found for adherence rates between the DPD and electronic monitors. The DPD was also able to identify barriers to adherence, which included oppositional behaviors, forgetting, and competing activities. Overall, these data suggested that the DPD holds promise for measuring adherence behaviors within the family context

Feasibility of a musculoskeletal ultrasound intervention to improve adherence in juvenile idiopathic arthritis: a proof-of concept trial

Abstract Background Non-adherence is a prevalent and modifiable issue in juvenile idiopathic arthritis (JIA) that currently lacks provider-based intervention. Education surrounding disease status is one way in which families remain engaged in their care. Musculoskeletal ultrasound is one such form of demonstrative, real-time education that may impact the way patients and caregivers self-manage their disease. The aims of this study are to 1) assess the feasibility, acceptability and perceived usefulness of musculoskeletal ultrasound as a non-adherence intervention tool and 2) to examine changes in methotrexate adherence in adolescents with JIA following the ultrasound. Methods Eight adolescents with polyarticular or extended oligoarticular JIA and their caregivers completed this 12 week study. A within subject design was used to compare baseline and post-intervention adherence, quality of life and disease activity indices. Adherence measures included electronic measurement of methotrexate in addition to self-reported adherence questionnaires. The ultrasound intervention included a one-time, rheumatologist provided, educational examination of three or more currently or historically active joints. Results The ultrasound intervention was found to be both feasible and acceptable. One hundred percent of eligible participants completed the ultrasound intervention. The ultrasound was well received by patients and caregivers, with most believing this to be a helpful tool. Baseline adherence was 75.3% among participants, with half of the participants being classified as non-adherent. Electronically measured and self-reported adherence measures did not show significant changes during the post-intervention period. Two participants improved, four participants maintained, and two participants decreased adherence. On ultrasound, 18/27 (66.7%) of the examined joints displayed abnormalities, with 63% being discrepant and additive to the rheumatologist’s physical examination. Conclusions While our intervention did not show any changes in adherence, quality of life or disease activity indices in this proof-of-concept trial, the intervention does show promise in acceptability measures and merits future study in a more robust trial design. An additional study benefit was that the musculoskeletal ultrasound intervention was able to demonstrate subclinical disease, leading to clinically impactful therapeutic changes in several participants