Clinical trajectory of hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders in older adults.

BACKGROUND: Research on hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorder (hEDS/HSD) has described its natural history and clinical course in children, adolescents, and young to middle-aged adults. However, more research is needed on the clinical trajectory of hEDS/HSD into older age. Therefore, clinicians, including nurse practitioners, know little about identifying older adults with undiagnosed hEDS/HSD. OBJECTIVE: This review sought to identify studies regarding aging in hEDS/HSD. DATA SOURCES: This scoping review included PubMed, Cumulative Index to Nursing and Allied Health Literature, and Scopus and found 15 studies that mentioned age or aging on the symptoms and health-related quality of life. CONCLUSIONS: No study had a stated aim regarding aging in hEDS/HSD, but all studies corroborated earlier natural history studies describing the age-related trajectory of manifestations in younger people. Studies found that symptom progression was heterogeneous, multisystemic, and unpredictable. Studies also noted prolonged diagnosis delays and long symptom duration, but the impact of these factors on outcomes was unclear. The high variability in patient outcomes precludes the prediction of outcomes based on the included studies. The clinical impact of aging on hEDS/HSD remains mostly speculative. IMPLICATIONS FOR PRACTICE: Nurse practitioners, especially those in primary care, should consider that older adults presenting with multimorbidity may have undiagnosed hEDS/HSD. More research is needed to identify symptom patterns and clinical history that may suggest an underlying connective tissue disorder.

Data-Driven Analytics to Discover APRN's Impact on Nursing Home Hospitalization: Causal Discovery Analysis.

OBJECTIVES: Research shows advanced practice registered nurses (APRNs) embedded in nursing homes (NHs) reduce resident hospitalizations. However, the specific APRN activities that reduce hospitalizations have not been adequately investigated. This study aims to identify the causal links between APRN activities and NHs resident hospitalization. The study also examined relationships among other variables, including advanced directives, clinical diagnosis, and length of hospitalization. DESIGN: Secondary data analysis. SETTING AND PARTICIPANTS: Residents of NHs participating in the Missouri Quality Initiative for Nursing Homes, 2016-2019. METHODS: We performed a secondary analysis of data from the Missouri Quality Initiative for Nursing Homes Intervention using causal discovery analysis, a machine learning, data-driven technique to determine causal relationships across data. The resident roster and INTERACT resident hospitalization datasets were combined to create the final dataset. Variables in the analysis model were divided into before and after hospitalization. Expert consensus was used to validate and interpret the outcomes. RESULTS: The research team analyzed 1161 hospitalization events and their associated NH activities. APRNs evaluated NH residents before a transfer, expedited follow-up nursing assessments, and authorized hospitalization when necessary. No significant causal relationships were found between APRN activities and the clinical diagnosis of a resident. The analysis also showed multifaceted relationships related to having advanced directives and duration of hospitalization. CONCLUSIONS AND IMPLICATIONS: This study demonstrated the importance of APRNs embedded in NHs to improve resident outcomes. APRNs in NHs can facilitate communication and collaboration among the nursing team, leading to early identification and treatment for resident status changes. APRNs can also initiate more timely transfers by reducing the need for physician authorization. These findings emphasize the crucial role of APRNs in NHs and suggest that budgeting for APRN services may be an effective strategy to reduce hospitalizations. Additional findings regarding advance directives are discussed.

Hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders in older women: A feasibility study.

INTRODUCTION: Hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders cause joint instability, chronic pain, fatigue and progressive multisystemic dysfunction, increasing symptom burden and decreasing quality of life. Researchers know little about how these disorders progress in women as they age. OBJECTIVE: This research aimed to determine the feasibility of an internet-based study to understand the clinical characteristics, symptom burden and health-related quality of life in older women with symptomatic hypermobility disorders. METHODS: This cross-sectional, internet-based survey studied recruitment methods, suitability and usability of survey instruments and obtained baseline data on women aged 50 and older with hEDS/HSD. Researchers recruited participants from a Facebook group for older adults with Ehlers-Danlos syndrome. Outcome measures included health history, the Multidimensional Health Assessment Questionnaire and the RAND Short Form 36 health survey. RESULTS: Researchers recruited 32 participants from a single Facebook group within 2 weeks. Nearly all participants were satisfied with the survey length, clarity and navigation, with 10 participants providing free-text recommendations for survey improvement. The survey suggests a high symptom burden and poor quality of life in older women with hEDS/HSD. CONCLUSION: The results support the feasibility and importance of a future internet-based comprehensive study about hEDS/HSD in older women.

Utilisation of Digital Applications for Personal Recovery Amongst Youth with Mental Health Concerns.

There is an increasing population of youths that report mental health issues. Research has shown that youths are reluctant to seek help for various reasons. A majority of those who do seek help are using digital mental health supports. Subsequently, efforts to promote youth mental health have focused on the use of digital applications as a means of overcoming barriers related to factors including stigma and lack of available services. The worldwide move toward recovery-oriented care led to emerging research on personal recovery amongst youths with mental health concerns. This study sought to address the need for recovery-oriented digital resources for youths. It utilised a qualitative design methodology to develop a rich interpretation of how youths are using digital interventions to support their mental health recovery journey. It sought to understand how existing digital applications are useful for youth recovery and identified characteristics associated with recovery and engagement. The content analysis generated five categories that represent facilitators of youth recovery and the thematic analysis identified key elements of digital applications that support youth recovery. The results offer complimentary support and guidance for recovery-oriented care and the use of digital mental health interventions in the promotion of personal recovery amongst youths.

Longitudinal analysis of aging in place at TigerPlace: Resident function and well-being.

This paper reports on a longitudinal eight-year analysis (2011-2019) of trajectory of function and well-being residents of TigerPlace Aging in Place (AIP) model of care. Residents were routinely assessed using standard health assessment instruments. Average scores from each measure were examined for changes or trends in resident function; decline over time was calculated. Scores for depression, mental health subscale Short Form Health Survey-12 (SF-12) remained stable over time. Mini Mental State Exam declined to mild dementia range (21-24). Physical measures SF-12 physical health subscale, ADLs, and IADLs declined slightly, while fall risk increased over time. When yearly trends in AIP were modeled with a referent group there was no significant worsening of functioning. The length of stay for TigerPlace residents continued to remain stable at nearly 30 months. Residents maintained function in the environment of their choice longer at cost less than nursing homes, and just above residential care cost.

An initiative to improve pneumococcal immunization counseling in children with nephrotic syndrome.

BACKGROUND: Immunization is essential in preventing life-threatening pneumococcal infections in children with nephrotic syndrome. An additional 23-valent pneumococcal polysaccharide vaccine (PPSV23) series is required for children with nephrotic syndrome. Despite current practice guidelines, many children with nephrotic syndrome do not receive PPSV23. METHODS: Our nephrology clinic conducted a quality improvement project to improve the overall rate of PPSV23 counseling to more than 70% within a 12-month period by applying several targeted interventions to raise providers' awareness, improve communication with primary care providers, and increase provider adherence. Data was collected from the electronic health record (EHR), and monthly performance was tracked via monthly control charts and overall immunization counseling rate charts. RESULTS: We increased adherence to PPSV23 vaccination counseling from a baseline of 12 to 86%. The first intervention that effectively increased the vaccine counseling rate from 12 to 30% was improving a provider's awareness of the PPSV23 literature and vaccine guidelines. Other interventions included regular performance reviews at division meetings, creating an immunization protocol, posting performance charts on the office bulletin board, and unifying vaccine recommendation templates. Lastly, specific and timely EHR reminders improved the total counseling rate from 52 to 86% and maintained adherence until the completion of the project. CONCLUSION: Bridging the knowledge gap in provider awareness and using specific EHR reminders can improve adherence to PPSV23 counseling in children with nephrotic syndrome. Such interventions could be applied to similar groups of immunocompromised patients in whom additional vaccines are indicated. A higher resolution version of the Graphical abstract is available as Supplementary information.

The diagnostic journey in adults with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders.

BACKGROUND: Researchers have identified lengthy diagnosis delays in patients with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders (hEDS/HSD), but the reason for these delays is unclear. OBJECTIVE: This review seeks to synthesize the existing qualitative research about hEDS/HSD to understand the reasons for diagnosis delay. DATA SOURCES: We searched PubMed, Scopus, CINAHL, Google Scholar, and Dissertations and Theses databases for all qualitative studies about hEDS/HSD that mentioned the diagnosis process. A total of 283 studies were retrieved, from which we identified 13 studies to include in this synthesis. CONCLUSIONS: The reviewers identified and organized diagnosis delay themes under four overarching categories: disease, patient, provider, and system. Disease factors included the nature of the symptoms and lack of a confirmatory test. Patient factors included psychological and emotional responses, seeing multiple providers, and receiving multiple diagnoses. Provider factors related to limited knowledge and attitudes. System factors included silo-based health care systems and bureaucratic barriers. IMPLICATIONS FOR PRACTICE: Diagnosis delays result from complex, overlapping, and interacting factors. Nurse practitioners have a critical role in improving care and reducing diagnosis delays in patients with hEDS/HSD. Further research is needed to understand the causes and consequences of diagnosis delays in hEDS/HSD.

Introducing an innovative model of acute paediatric mental health and addictions care to paediatric emergency departments: a protocol for a multicentre prospective cohort study.

INTRODUCTION: Children and youth with mental health and addiction crises are a vulnerable patient group that often are brought to the hospital for emergency department care. We propose to evaluate the effect of a novel, acute care bundle that standardises a patient-centred approach to care. METHODS AND ANALYSIS: Two paediatric emergency departments in Alberta, Canada are involved in this prospective, pragmatic, 29-month interventional quasi-experimental study. The acute care bundle comprises three components, applied when appropriate: (1) assessing self-harm risk at triage using the Ask Suicide-Screening Questionnaire (ASQ) to standardise the questions administered, enabling risk stratification; (2) use of the HEADS-ED (Home, Education, Activities/peers, Drug/alcohol, Suicidality, Emotions and behaviour, Discharge Resources) to focus mental health evaluations for those who screen high risk on the ASQ; and (3) implementation of a Choice And Partnership Approach to enable shared decision making in care following the emergency department visit. The overarching goal is to deliver the right care at the right place and time for the patients. The study design involves a longitudinal collection of data 12 months before and after the introduction of the bundle and the use of quality improvement strategies such as Plan-Do-Study-Act cycles during a 5-month run-in period to test and implement changes. The primary study end-point is child/youth well-being 1 month after the emergency department visit. Secondary outcomes include family functioning, child/youth well-being at 3 and 6 months, satisfaction with emergency department care, and health system outcomes (hospital admissions, length of emergency department stays, emergency department revisits). ETHICS AND DISSEMINATION: The study is registered at www.ClinicalTrials.gov and has received ethics and operational approvals from study sites. The results of the study will be reported in accordance with the Strengthening the Reporting of Observational Studies in Epidemiology statement. Results will be shared broadly with key policy and decision makers and disseminated in peer-reviewed academic journals and presentations at conferences. TRIAL REGISTRATION NUMBER: NCT04292379.

Characteristics of falls and recurrent falls in residents of an aging in place community: A case-control study.

BACKGROUND: Falls and fall-related injuries remain an ongoing and serious health problem in older adults. Many clinical and environmental factors have been implicated in falls and recurrent falls, including sleep disturbances, sensory deficits, balance problems, incontinence, comorbid conditions, and certain categories of medications. We undertook this study to determine if there was an association between these factors and falls or recurrent falls in older adult residents of an aging in place community. METHODS: Our retrospective case-control study compared residents who did and did not fall in an aging in place community, as well as those who experienced recurrent versus single falls in a single year. RESULTS: A total of 50 residents met the criteria for inclusion in this study, with 30 participants (60%) having experienced one or more falls during the observation period. Of the 30 participants who fell, 21 (70%) experienced more than one fall in a single year. Variables associated with falls included marital status and bowel incontinence; variables associated with recurrent falls included self-reported sleep difficulty, balance with sitting to standing and surface-to-surface transfer, use of a walker, and use of antidepressant medications. DISCUSSION: Our study supports the existing nursing research that falls, and recurrent falls are the result of multiple, interrelated factors. Further research is needed into preventative measures for both falls and recurrent falls, particularly in the context of aging in place.

Reducing Door-to-Reperfusion Time for Mechanical Thrombectomy With a Multitiered Notification System for Acute Ischemic Stroke.

OBJECTIVE: To reduce door-to-angiographic reperfusion (DTR) time to 120 minutes for patients presenting with acute ischemic stroke attributed to anterior circulation large-vessel occlusion amenable to endovascular mechanical thrombectomy. PATIENTS AND METHODS: Patients treated with mechanical thrombectomy before (April 10, 2015, through April 11, 2016) and after (April 12, 2016, through May 10, 2017) implementation of a multitiered notification system were studied. Lean process mapping was used to assess inefficiencies with multidisciplinary triage. A 3-tiered paging platform, which rapidly alerts essential personnel of the acute ischemic stroke team at advancing decision points, was introduced. RESULTS: Sixty-two patients were analyzed before and after implementation (34 vs 28, respectively). Following intervention, DTR time was reduced by 43 minutes (mean DTR, 170 minutes vs 127 minutes; P=.02). At 90-day follow up, 5 of the 28 patients in the postintervention cohort (19%) had excellent neurologic outcomes, defined as a modified Rankin Scale score of 0, compared to 0 of 34 (0%) in the preintervention cohort (P=.89). Reductions were also seen in the length of stay on the neurocritical care service (mean, 6 vs 3 days; P=.006), and total hospital charges for combined groups (mean, $100,083 vs $161,458; P<.001). CONCLUSION: The multitiered notification system was a feasible solution for improving DTR within our institution, resulting in reductions of overall DTR time, neurocritical care service length of stay, and total hospital charges.

Public Health Interventions: School Nurse Practice Stories.

This study describes and analyzes school nurses' (SN) experiences with understanding and using public health interventions from the Public Health Intervention Wheel. The Wheel offers a model for naming interventions provided by SNs from a public health perspective. Research teams from academic and SN practice settings conducted six focus groups with school nurses from Minnesota. Participants were asked to share experiences through telling stories from their practice that represented a specific wedge of the Wheel. Researchers organized data by intervention; often stories represented more than one intervention. Stories represented all levels of practice. This study highlights important contributions of school nurses to promote the health of school populations through the use of Wheel interventions. The integration of Wheel interventions in the application of the Framework for 21st-Century School Nursing Practice™ provides SNs with a language to document and communicate their expert professional practice.

Second Life(®) Virtual Learning in Public Health Nursing.

BACKGROUND: With limited available public health nursing clinical placements, nurse educators are challenged to create alternative learning experiences that engage students. METHOD: Nursing faculty developed three Second Life® virtual learning scenarios in family health, disaster preparedness, and home safety areas of interest as a supplement to clinical experiences for undergraduate senior nursing students in a public health nursing course. Student perceptions of their learning were evaluated by a survey and focus group. RESULTS: Although students perceived learning public health nursing knowledge, they were less likely to agree that the experience was as effective for learning about public health nursing when compared with on-site clinical experiences. CONCLUSION: The use of Second Life is an effective learning platform for teaching undergraduate nursing students public health nursing when clinical experiences are difficult to obtain. Nursing faculty need to ensure technological challenges are addressed and that scenarios are realistic, and engage students through interactive collaboration. [J Nurs Educ. 2016;55(9):536-540.].

Public Health Interventions for School Nursing Practice.

School nurses (SNs) use public health nursing knowledge and skills to provide nursing services to school populations. The Public Health Intervention Wheel is a practice framework that can be used to explain and guide public health nursing interventions. SNs who were also members of the National Association of School Nurses completed an electronic survey on their use of public health interventions as defined by the wheel. Although 67% of the participants were not familiar with the Public Health Intervention Wheel, respondents reported conducting activities that were consistent with the Wheel interventions. Screening, referral and follow-up, case management, and health teaching were the most frequently performed interventions. Intervention use varied by educational level, age of nurse, years of practice, and student population. The Public Health Intervention Wheel is a relevant and useful framework that provides a language to explain population-based school nursing practice.

CE: Nursing Management of Patients with Ehlers-Danlos Syndrome.

OVERVIEW: Ehlers-Danlos syndrome (EDS), a hereditary connective tissue disorder, has historically been misunderstood and underdiagnosed by health care providers. Because of the high degree of phenotypic variability, patients are often correctly diagnosed only after years of seemingly unrelated but debilitating injuries and illnesses. Specific genetic mutations have been identified for some, but not all, EDS types; patients presenting with a high index of suspicion should be referred to a geneticist. As awareness and recognition of the syndrome improve, nurses are increasingly likely to care for patients with EDS. This article gives a brief overview of the syndrome and provides guidance on ways to manage symptoms, recognize and prevent serious complications, and improve patients' quality of life.

An integrative literature review of interventions to reduce violence against emergency department nurses.

AIM: To critique the evidence that underpins interventions intended to minimise workplace violence directed against emergency department nurses, to inform researchers and policy makers regarding the design, development, implementation and evaluation of emergency nursing anti-violence and counter-violence interventions. BACKGROUND: Workplace violence perpetrated against emergency department nurses is at least continuing and at worst increasing. Occupational violence has detrimental effects on job satisfaction, retention and recruitment, and the quality and cost of patient care. DESIGN: An integrated literature review. METHOD: Searches of the Cochrane Library, CINAHL, MEDLINE and the Joanna Briggs Institute between 1986-May 2007. Included articles were appraised and then synthesised into a narrative summary. RESULTS: Ten primary research studies were included. Interventions were classified as environmental, practices and policies, or skills. While each study has useful information regarding the implementation of interventions, there is no strong evidence for their efficacy. CONCLUSIONS: The weight of effort is still directed towards defining the phenomenon rather than addressing solutions. Studies that assessed the efficacy of a single intervention failed to take account of context; and participatory context-driven studies failed to provide generalisable evidence. Concerted multi-site and multi-disciplinary, action-oriented research studies are urgently needed to provide an evidence base for the prevention and mitigation of violence perpetrated against emergency department nurses. RELEVANCE TO CLINICAL PRACTICE: The investigation of interventions rather than repeatedly redefining the problem and directing resources into debating semantics or differentiating 'degrees' of violence and aggression is recommended. This review unambiguously identifies the gap in research-based interventions.

Diagnostic assays for active infection with human herpesvirus 6 (HHV-6).

BACKGROUND: Human herpesvirus 6 (HHV-6) causes ubiquitous infection in early childhood with lifelong latency or persistence. Reactivation of HHV-6 has been associated with multiple diseases including encephalitis. Chromosomal integration of HHV-6 also occurs. Previous studies have suggested that the detection of HHV-6 DNA in plasma is an accurate marker of active viral replication. OBJECTIVE: We sought to determine whether PCR assays on plasma could correctly differentiate between primary HHV-6 infection, chromosomal integration of HHV-6 and latent HHV-6 infection. STUDY DESIGN: We performed qualitative PCR, real-time quantitative PCR (RQ-PCR), and reverse-transcriptase PCR (RT-PCR) assays on samples of peripheral and cord blood mononuclear cells, as well as plasma, from groups of subjects with well defined HHV-6 infection, including subjects with chromosomally integrated HHV-6. RESULTS AND CONCLUSIONS: The detection of HHV-6 DNA in plasma was 92% sensitive compared to viral isolation for the identification of primary infection with HHV-6. All plasma samples from infants with chromosomally integrated HHV-6 had HHV-6 DNA detectable in plasma while only 5.6% were positive by RT-PCR. The specificity of plasma PCR for active replication of HHV-6 was 84% compared to viral culture while the specificity of RT-PCR was 98%. Our results demonstrate that qualitative or quantitative PCR of plasma is insufficient to distinguish between active viral replication and chromosomal integration with HHV-6. We found a higher specificity of RT-PCR performed on PBMC samples compared to PCR or RQ-PCR performed on plasma when evaluating samples for active HHV-6 replication.

Development of human cloned blastocysts following somatic cell nuclear transfer with adult fibroblasts.

Nuclear transfer stem cells hold considerable promise in the field of regenerative medicine and cell-based drug discovery. In this study, a total of 29 oocytes were obtained from three young (20-24 years old) reproductive egg donors who had been successful in previous cycles. These oocytes, deemed by intended parents to be in excess of their reproductive needs, were donated for research without financial compensation by both the egg donor and intended parents after receiving informed consent. All intended parents successfully achieved ongoing pregnancies with the oocytes retained for reproductive purposes. Mature oocytes, obtained within 2 hours following transvaginal aspiration, were enucleated using one of two methods, extrusion or aspiration, after 45 minutes of incubation in cytochalasin B. Rates of oocyte lysis or degeneration did not differ between the two methods. Somatic cell nuclear transfer (SCNT) embryos were constructed using two established adult male fibroblast lines of normal karyotype. High rates of pronuclear formation (66%), early cleavage (47%), and blastocyst (23%) development were observed following incubation in standard in vitro fertilization culture media. One cloned blastocyst was confirmed by DNA and mitochondrial DNA fingerprinting analyses, and DNA fingerprinting of two other cloned blastocysts indicated that they were also generated by SCNT. Blastocysts were also obtained from a limited number of parthenogenetically activated oocytes. This study demonstrates, for the first time, that SCNT can produce human blastocyst-stage embryos using nuclei obtained from differentiated adult cells and provides new information on methods that may be needed for a higher level of efficiency for human nuclear transfer.

Prediction of early progression in recently diagnosed IgA nephropathy.

BACKGROUND: Most studies of prognosis in IgA nephropathy (IgAN) have tried to predict dichotomous outcomes based on a small number of clinical or semi-quantitative histological variables in large numbers of patients. METHODS: We pursued a quite different approach. We measured GFR annually for 4-5 years in 22 adult patients with recently diagnosed IgAN. Quantitative morphology was performed on the diagnostic biopsy specimens and baseline glomerular filtration dynamics were performed at study entry. An initial set of 30 plausible predictor variables (half demographic or physiological, half structural) was reduced to 22 using phylogenetic trees. Least-angle regression (LARS) was used to predict the rate of GFR change from these variables RESULTS: The rate of GFR change ranged from a loss of 41 ml/min/year to a gain of 8.6 ml/min/year. We found an optimum predictor set of five baseline variables: the percentage of glomeruli with global sclerosis, the fractional interstitial area, the serum creatinine, the average tuft volume of non-sclerotic glomeruli and the renal plasma flow. CONCLUSIONS: The strong predictive relationship of the three structural variables with the slope of GFR in our subjects suggests that even at the time of their initial diagnosis many patients with IgAN already manifest a 'remnant kidney' phenomenon. The distinctive pathophysiological insights derived from this study suggest some of the advantages of intense quantitative investigations applied to a small number of subjects.