Patient Reported Outcome Measures in Adult Spinal Muscular Atrophy: A Scoping Review and Graphical Visualization of the Evidence.

BACKGROUND: Spinal Muscular Atrophy (SMA) is a hereditary neuromuscular disease with an estimated prevalence of 1/10 000 births. SMA is increasingly recognized as a multi-system disease with a need to study additional under-recognized health domains such as quality of life, fatigue, bulbar function, respiratory function, and independence. OBJECTIVE: Identify and assess reported evidence from the literature investigating Patient Reported Outcome Measures (PROMs) in adults with SMA. Develop a novel method drawing from network theory to graphically depict the literature, PROMs, and supporting psychometric evidence. METHODS: A scoping review was completed following PRISM-ScR, COSMIN and JBI scoping review guidelines. Literature investigating PROMs in adult SMA or neuromuscular disease was identified from peer-reviewed and grey databases. A network graph was derived from extracted data. RESULTS: 5292 articles were retrieved, 81 articles met inclusion criteria; corresponding to 31 unique PROMs. Only two PROMs were developed specifically for SMA. Few PROMs covered multiple domains of health. Most PROMs were incompletely validated, focusing on concurrent validity, and few assessed responsiveness or internal consistency. CONCLUSIONS: PROMs are emerging tools for monitoring and assessing adults with SMA. Despite their potential benefits, additional validation studies should be completed prior to their use for clinical decision-making. Network graphics may represent a technique to aid in the visualization of evidence supporting a scoping review.

A Canadian Adult Spinal Muscular Atrophy Outcome Measures Toolkit: Results of a National Consensus using a Modified Delphi Method.

BACKGROUND: Spinal Muscular Atrophy (SMA) is a rare disease that affects 1 in 11 000 live births. Recent developments in SMA treatments have included new disease-modifying therapies that require high quality data to inform decisions around initiation and continuation of therapy. In Canada, there are no nationally agreed upon outcome measures (OM) used in adult SMA. Standardization of OM is essential to obtain high quality data that is comparable among neuromuscular clinics. OBJECTIVE: To develop a recommended toolkit and timing of OM for assessment of adults with SMA. METHODS: A modified delphi method consisting of 2 virtual voting rounds followed by a virtual conference was utilized with a panel of expert clinicians treating adult SMA across Canada. RESULTS: A consensus-derived toolkit of 8 OM was developed across three domains of function, with an additional 3 optional measures. Optimal assessment frequency is 12 months for most patients regardless of therapeutic access, while patients in their first year of receiving disease-modifying therapy should be assessed more frequently. CONCLUSIONS: The implementation of the consensus-derived OM toolkit will improve monitoring and assessment of adult SMA patients, and enrich the quality of real-world evidence. Regular updates to the toolkit must be considered as new evidence becomes available.

Changes in nursing students' knowledge, attitudes and interest in caring for older adults: A longitudinal cohort study.

Nursing education programs have a responsibility to prepare future nurses to care for the growing number of older adults who will be accessing health care services. Preparation involves ensuring students have the knowledge, skills and desire to provide quality care to older adults. A longitudinal cohort study was conducted to collect data on students' knowledge of, attitudes towards, and interest in working with older adults during each year of an undergraduate nursing program. Data were collected using the Kogan Attitudes Towards Older People Scale, Palmore's Fact on Aging Quiz and a demographic instrument designed specifically for this study. Results showed slight improvements in students' overall attitudes towards older adults at the completion of their nursing degree. Increases in knowledge were seen in 12 of 24 areas measured. Gerontology as a preferred career choice was highest immediately following a clinical placement focusing on caring for older adults. Integrating gerontological clinical experiences into a beginning fundamental nursing course does not necessarily improve students' learning in the area of caring for older adults. Nursing programs have a responsibility to design, monitor and update curricula to ensure students are being adequately prepared to care for older adults.

Implementation of pain best practices as part of the spinal cord injury knowledge mobilization network.

Context/objective: The spinal cord injury (SCI) knowledge mobilization network (KMN) is a community of practice formed in 2011 as part of a national best practice implementation (BPI) effort to improve SCI care. This study objective was to determine whether completion and documentation of pain practices could be improved in a neurorehabilitation setting using the KMN implementation approach. Design: Single site, pre-post intervention study. Setting: Neurorehabilitation hospital. Participants: Twenty sequential consenting inpatients with SCI, with retrospective comparative analysis of 50 sequential SCI admissions pre-KMN. Interventions: A local Site Implementation Team (SIT) was formed to develop an implementation plan, including acceptable timeframes for completion and documentation of four specific pain best practices: (1) pain assessment on admission, (2) development of an Inter-Professional Pain Treatment Plan (IPTP), (3) pain monitoring throughout admission, and (4) a pain discharge plan. Outcomes: Provider adherences to pain best practices were the primary outcomes. The secondary outcome was patient satisfaction. Results: Provider adherence for most outcomes exceeded 70% completion within acceptable timeframes, with improvements found for all outcomes as compared to the retrospective cohort. Notably, pain education as part of the IPTP improved from 12% completion to 74%, documenting pain onset from 4.5% to 80% and pain discharge plan from 40% to 74%. Overall, participants were satisfied with their pain management. Conclusions: Pain best practices were more consistently documented after the KMN implementation. Pain practices in all four areas have now been expanded to all inpatient diagnoses using the same forms and framework created in the implementation process.

An Analysis of Falls and Those who Fall in a Chronic Care Facility.

OBJECTIVES: Falls in hospitals lead to adverse patient outcomes and prevention of falls is of upmost importance. Little is known about fall and injury rates in chronic care facilities, which are similar to skilled nursing facilities in the United States. Current fall risk tools in this setting are not well studied. Enhancing the understanding of how patient characteristics relate to fall circumstances is also needed. DESIGN: Retrospective analysis of falls over 3 years on consecutive admissions and discharges. SETTING AND PARTICIPANTS: A 104-bed geriatric chronic care facility. MEASURES: Fall and injury data, descriptive data for patients measuring mobility, balance, cognition, function, and frailty in relation to risk of falls and fall circumstances were analyzed. RESULTS: There were 1141 falls, with an overall fall rate of 8.48 falls per 1000 occupied bed days. The overall injury rate was 37.2 injuries per 100 falls. Being male and frail, having a mobility aid, poor mobility, balance, or cognition were associated with falling. Patients with good balance but poor cognition was more likely to fall outside their room, while those with poor mobility/balance fell more often in their room. The Clinical Frailty Scale performed modestly well at predicting falls with an odds ratio of 2.5 (95% confidence interval 1.9-3.2). CONCLUSIONS AND IMPLICATIONS: Fall rates in chronic care facilities differ from what is reported in other settings. Patient characteristics such as male, use or misuse of a mobility aid, and poor cognition are more common in fallers. Fall circumstances differ in those with poor cognition compared with those with poor mobility and balance. More research focusing on frailty, cognition, and mobility/balance is needed to develop accurate tools that can predict those at a high risk of falls in these facilities.