A longitudinal study of disease progression in facioscapulohumeral muscular dystrophy (FSHD).

INTRODUCTION/AIMS: In preparation for clinical trials, it is important to better understand how disease burden changes over time in facioscapulohumeral muscular dystrophy (FSHD) and to assess the capability of select metrics to detect these changes. This study aims to evaluate FSHD disease progression over 1 year and to examine the sensitivity of several outcome measures in detecting changes during this interval. METHODS: We conducted a 12-month prospective observational study of 41 participants with FSHD. Participants were evaluated at baseline, 6 months, and 12 months with serial strength testing (manual muscle testing or MMT and maximum voluntary isometric contraction testing or MVICT), functional testing (FSHD-Composite Outcome Measure or FSHD-COM, FSHD Clinical Severity Score or CSS, and FSHD Evaluation Score or FES), sleep and fatigue assessments, lean body mass measurements, respiratory testing, and the FSHD-Health Index patient-reported outcome. Changes in these outcome measures were assessed over the 12-month period. Associations between changes in outcome measures and both age and sex were also examined. RESULTS: In a 12-month period, FSHD participant function remained largely stable with a mild worsening of strength, measured by MMT and standardized MVICT scores, and a mild loss in lean body mass. DISCUSSION: The abilities and disease burden of adults with FSHD are largely static over a 12-month period with participants demonstrating a mild average reduction in some measures of strength. Selection of patients, outcome measures, and trial duration should be carefully considered during the design and implementation of future clinical studies involving FSHD patients.

Professional Identity and Motivation for Medical School in First-Year Medical Students: A Cross-sectional Study.

Background: Professional identity formation (PIF) is a life-long process, starting even before professional education. High levels of motivation for medical school are essential for effective learning and academic success. Both are key factors in future physicians' professional and personal development, and according to self-determination theory, professional identity (PI) and students' levels of motivation could be closely linked. Therefore, we sought to investigate whether PI and strength of motivation for medical school are associated in new medical students. Methods: In a cross-sectional survey, all new medical students in Munich, Germany, were asked to complete the Macleod Clark Professional Identity Scale (MCPIS-9) and the Strength of Motivation for Medical School-Revised questionnaire (SMMS-R) as well as to provide information about age, gender, and waiting time before starting medical school. Results: Eight hundred eleven out of 918 new medical students participated in the survey. A positive correlation between the MCPIS-9 and the SMMS-R (p < 0.001) was found. Female students showed higher scores in the SMMS-R (p < 0.05) and the SMMS-R-subscale Readiness to Start (p < 0.001). The amount of waiting semesters showed a positive correlation with the total SMMS-R score (p < 0.01) as well as with the subscales Readiness to Start and Persistence (both p < 0.001). Discussion: We found an association between PI and strength of motivation for medical school in a large cohort of new medical students. Female gender and more waiting semesters were associated with higher levels of self-perceived motivation and higher scores on the SMMS-R-subscale Readiness to Start. More research is needed to better understand this topic to further improve medical education.

Calling for Help-Peer-Based Psychosocial Support for Medical Staff by Telephone-A Best Practice Example from Germany.

BACKGROUND: A telephone support hotline (PSU-HELPLINE) was established at the beginning of the pandemic due to the burden on health professionals and the lack of support at the workplace. The aim of this study was to evaluate the telephone support service for health professionals in terms of its burden, benefits, and mechanisms of action. METHODS: Data collection was conducted during and after calls by PSU-HELPLINE counsellors. In addition to the socio-demographic data evaluation, burdens of the callers and the benefits of the calls were collected. The content-analytical evaluation of the stresses as well as the effect factors were based on Mayring's (2022). RESULTS: Most of the callers were highly to very highly stressed. The usefulness of the conversation was rated as strong to very strong by both callers and counsellors. The PSU-HELPLINE was used primarily for processing serious events and in phases of overload. The support work was carried out through the following aspects of so-called effect factors, among others: psychoeducation, change of perspective, resource activation, problem actualization, connectedness, information, problem solving, self-efficacy, and preservation of resources. CONCLUSIONS: The expansion of local peer support structures and the possibility of a telephone helpline are recommended. Further research is needed.

First Results of Peer Training for Medical Staff-Psychosocial Support through Peer Support in Health Care.

BACKGROUND: In view of the increasing strain on health workers, psychosocial support measures are becoming more important. The core of a sustainable concept is the establishment of peer support teams. Two aspects are central: first, target group-specific training content, and second, suitable staff members who are trained as peers. The goal of the study was to obtain a first look at what content can be taught in peer training for medical staff, how the training is evaluated by the target group, and which people are interested in training from peers. METHODS: During the period 2017-2022, Peer Training for medical staff was developed by a non-profit institution in Germany with state funding and the support of a medical professional association and evaluated during the project. Participants (N = 190) in the Peer Training course were interviewed in advance about their experiences and stresses at work using an anonymous questionnaire. After completing the training modules, the participants filled out an evaluation form. RESULTS: The participants of the Peer Training were predominantly female (70.5%) and middle-aged (between 31 and 50 years old). Most (80.3%) experienced stressful events themselves, mostly without any preparation (93.5%) or follow-up (86.8%) by the employer. The participants estimate their workload in the medium range. The proportion of stressed individuals among the participants was below that of various comparison groups as available reference values. The training module itself was evaluated very positively. CONCLUSIONS: The content and framework parameters of the training were rated very well. There is a high degree of fit with the requirements in the health sector. The participants in the Peer Training seem to represent a good cross-section of the target group medical staff, also regarding their own experiences, seem to have a good psychological constitution and are therefore very suitable to work as peers after the training.

Is it time to rethink education and training? Learning how to perform under pressure: An observational study.

Emergency medicine workers are exposed daily to various stressors, especially work-related stress, which have been aggravated by the current SARS-CoV 2 pandemic and impact their physical and mental wellbeing. Nonetheless, although the efficacy of programs and strategies to improving the health of medical staff and patient care has been demonstrated, such programs and strategies are scarce. To assess the prevalence, types and consequences of stress in emergency medical workers in healthcare institutions and explore tools to cope with stressful situations at workplace. Two surveys were conducted. Survey 1 assessed the subjective stress levels and stressors of 21 emergency medicine professionals. Survey 2 was conducted amongst 103 healthcare workers at 3 hospitals in Germany. It comprised selected aspects of the German Mental Risk Assessment and a validated workload scale. None. The answer frequencies on Likert scales were descriptively evaluated. Survey 1: Emergency medical professionals experienced and reported the following high stress levels in acute situations: multitasking during a complex situation; factors associated with the work environment; fear of not appropriately controlling the situation; and lack of sleep. Survey 2: The highest stress levels were experienced in the areas "work environment" and "work organization." The highest scores on the workload scale were obtained for statements on work division, exhaustion, insufficient patient care due to time constraints, regulations, and lack of information. Approximately 80% of healthcare workers had experienced emotionally stressful situations at the workplace, and > 30% had lost a colleague to suicide. There are effective and proven methods to learn how to deal with stress that can easily be established in everyday clinical practice. Healthcare workers are subjected to numerous stressors in their work environment and observe the consequences of these stressors on their own and their colleagues' wellbeing. Coping strategies for high-pressure reduces and resists the job- immanent pressure and stress in healthcare workers.

Measuring hygiene competence: the picture-based situational judgement test HygiKo.

BACKGROUND: With the onset of the COVID-19 pandemic at the beginning of 2020, the crucial role of hygiene in healthcare settings has once again become very clear. For diagnostic and for didactic purposes, standardized and reliable tests suitable to assess the competencies involved in "working hygienically" are required. However, existing tests usually use self-report questionnaires, which are suboptimal for this purpose. In the present study, we introduce the newly developed, competence-oriented HygiKo test instrument focusing health-care professionals' hygiene competence and report empirical evidence regarding its psychometric properties. METHODS: HygiKo is a Situational Judgement Test (SJT) to assess hygiene competence. The HygiKo-test consists of twenty pictures (items), each item presents only one unambiguous hygiene lapse. For each item, test respondents are asked (1) whether they recognize a problem in the picture with respect to hygiene guidelines and, (2) if yes, to describe the problem in a short verbal response. Our sample comprised n = 149 health care professionals (79.1 % female; age: M = 26.7 years, SD = 7.3 years) working as clinicians or nurses. The written responses were rated by two independent raters with high agreement (α > 0.80), indicating high reliability of the measurement. We used Item Response Theory (IRT) for further data analysis. RESULTS: We report IRT analyses that show that the HygiKo-test is suitable to assess hygiene competence and that it allows to distinguish between persons demonstrating different levels of ability for seventeen of the twenty items), especially for the range of low to medium person abilities. Hence, the HygiKo-SJT is suitable to get a reliable and competence-oriented measure for hygiene-competence. CONCLUSIONS: In its present form, the HygiKo-test can be used to assess the hygiene competence of medical students, medical doctors, nurses and trainee nurses in cross-sectional measurements. In order to broaden the difficulty spectrum of the current test, additional test items with higher difficulty should be developed. The Situational Judgement Test designed to assess hygiene competence can be helpful in testing and teaching the ability of working hygienically. Further research for validity is needed.

Facioscapulohumeral muscular dystrophy functional composite outcome measure.

INTRODUCTION: We developed an evaluator-administered functional facioscapulohumeral muscular dystrophy composite outcome measure (FSHD-COM) comprising patient-identified areas of functional burden for future clinical trials. METHODS: We performed a prospective observational study of 41 patients with FSHD at 2 sites. The FSHD-COM includes functional assessment of the legs, shoulders and arms, trunk, hands, and balance/mobility. We determined the test-retest reliability and convergent validity compared to established FSHD disease metrics. RESULTS: The FSHD-COM demonstrated excellent test-retest reliability (intraclass correlation coefficient [ICC] 0.96; subscale ICC range, 0.90-0.94). Cross-sectional associations between the FSHD-COM and disease duration, clinical severity, and strength were moderate to strong (Pearson correlation coefficient range |0.51-0.92|). DISCUSSION: The FSHD-COM is a disease-relevant, functional composite outcome measure suitable for future FSHD clinical trials that shows excellent test-retest reliability and cross-sectional associations to disease measures. Future directions include determining multisite reliability, sensitivity to change, and the minimal clinically important change in the FSHD-COM. Muscle Nerve, 2018.

Validity of the 6 minute walk test in facioscapulohumeral muscular dystrophy.

INTRODUCTION: In preparation for future clinical trials, we determined the reliability, relationship to measures of disease severity, and consistency across sites of the 6 Minute Walk Test (6MWT) in patients with facioscapulohumeral muscular dystrophy (FSHD). METHODS: Genetically defined and clinically affected FSHD participants at 2 sites performed the 6MWT, the Timed Up and Go, and the 30 foot Go/Timed 10 meter test as measures of mobility using standard procedures. RESULTS: Eight-six participants representing the full range of severity performed the 6MWT. The mean 6MWT distance was 404.3 meters (SD 123.9), with no difference between sites. The 6MWT was reliable (n = 25; intraclass correlation coefficient = 0.99) and demonstrated moderate to strong correlations with lower extremity strength, functional outcomes, and FSHD Clinical Score. CONCLUSIONS: The 6MWT is reliable and is associated with other measures of FSHD disease severity. Future directions include assessing its sensitivity to disease progression. Muscle Nerve 55: 333-337, 2017.

The pen and the scalpel: effect of diffusion of information on nonclinical variations in surgical treatment.

BACKGROUND: As information is disseminated about best practices, variations in patterns of care should diminish over time. OBJECTIVE: To test the hypotheses that differences in rates of a surgical procedure are associated with type of insurance in an era of evolving practice guidelines and that insurance and site differences diminish with time as consensus guidelines disseminate among the medical community. METHODS: We use lymph node dissection among women with ductal carcinoma in situ (DCIS) as an example of a procedure with uncertain benefit. Using a sample of 1051 women diagnosed from 1985 through 2000 at 2 geographic sites, we collected detailed demographic, clinical, pathologic, and treatment information through abstraction of multiple medical records. We specified multivariate logistic models with flexible functions of time and time interactions with insurance and treatment site to test hypotheses. RESULTS: Lymph node dissection rates varied significantly according to site of treatment and insurance status after controlling for clinical, pathologic, treatment, and demographic characteristics. Rates of lymph node dissection decreased over time, and differences in lymph node dissection rates according to site and generosity of insurance were no longer significant by the end of the study period. CONCLUSIONS: We have demonstrated that rates of a discretionary surgical procedure differ according to nonclinical factors, such as treatment site and type of insurance, and that such unwarranted variation decreases over time with diminishing uncertainty and in an era of diffusion of clinical guidelines.

Racial disparity in the dose and dose intensity of breast cancer adjuvant chemotherapy.

PURPOSE: The purpose of this study was to investigate the impact of race and obesity on dose and dose intensity of adjuvant chemotherapy. METHODS: We abstracted data on patient/tumor characteristics, treatment course, physicians' intention to give a first cycle dose reduction, and reasons for dose reductions/delays from oncology records of 489 women treated from 1985 to 1997 in 10 treatment sites in two geographical regions. Administered doses and dose intensity were compared to standard regimens. Multivariate regression models determined the impact of race and body mass index (BMI) on dose proportion (actual:expected doses) and relative dose intensity (RDI) controlling for patient characteristics, comorbidity, chemotherapy regimen, site, and year of treatment. Logistic regressions explored race and BMI versus use of first cycle dose reductions. RESULTS: African-Americans received lower chemotherapy dose proportion and RDI than whites (0.80 vs. 0.85, p = 0.03 and 0.76 vs. 0.80, p = 0.01). In multivariate analyses, dose proportion was 0.09 lower (p = 0.002), and RDI was 0.10 (p < 0.001) lower in non-overweight African-Americans than whites. Obesity was associated with lower dose proportion (p < 0.01) and RDI (p < 0.03). Race and BMI were independently associated with first cycle dose reductions. Non-overweight African-Americans (p < 0.05) and overweight and obese African-American and white women (p < 0.001) were more likely to have first cycle dose reductions than non-overweight whites. CONCLUSION: We identified systematic differences in the administration of chemotherapy given to African-Americans and to overweight and obese women. These differences may contribute to documented disparities in outcome.