'Talking lines': the stories of diagnosis and support as told by those with lived experience of rare forms of dementia.

BACKGROUND: People living with, or caring for someone with, rare forms of dementia can encounter issues while obtaining a diagnosis and trying to access appropriate support. This can affect their wellbeing, quality of life, social relationships and employment status. This study makes use of an arts-based narrative approach to explore individual accounts of these experiences whilst also exploring how, in telling their stories, those affected by rare forms of dementia might invoke, and situate their stories in relation to, broader cultural narratives around dementia and illness. METHODS: Semi-structured interviews were conducted via video-conferencing software with participants (N = 27), living with, or caring for someone with, a rare forms of dementia. Participants used line drawings to depict their journey from initial symptoms to the present day, followed by prompts to verbally narrate their experiences. All interview transcripts and line drawings were subjected to narrative analysis. Four sets of transcripts and drawings were then subjected to more in-depth analysis. RESULTS: Analysis shed light on the struggles encountered by both care-partners and people with a diagnosis, while navigating a health and social care system that does not always understand their needs. This often led to individuals feeling isolated and unsupported. Accounts also depicted challenges to identity brought on by the process. The moment of diagnosis was also drawn in a complicated light. Individuals found comfort in gaining understanding, but felt fear at recognising upcoming challenges. Participants situated their own accounts against mainstream cultural narratives around what good support for cognitive impairment and dementia might look like, whilst also demonstrating the influential role they took on in pursuing the right care. CONCLUSIONS: The use of line drawing, alongside narrative interviews, allowed participants to tell complicated, sometimes anachronistic, stories about difficult experiences, whilst also reflecting on, and attaching meaning to, them. These stories highlighted pressing gaps in healthcare services and shone a light on the various pieces of collective action individuals were engaged in in order to improve them. Finally, in modelling some elements of the participants' service provision which were working, the narratives pointed to future directions services might move in.

Comparing the Efficacy of Targeted and Blast Portal Messaging in Message Opening Rate and Anticoagulation Initiation in Patients With Atrial Fibrillation in the Preventing Preventable Strokes Study II: Prospective Cohort Study.

BACKGROUND: The gap in anticoagulation use among patients with atrial fibrillation (AF) is a major public health threat. Inadequate patient education contributes to this gap. Patient portal-based messaging linked to educational materials may help bridge this gap, but the most effective messaging approach is unknown. OBJECTIVE: This study aims to compare the responsiveness of patients with AF to an AF or anticoagulation educational message between 2 portal messaging approaches: sending messages targeted at patients with upcoming outpatient appointments 1 week before their scheduled appointment (targeted) versus sending messages to all eligible patients in 1 blast, regardless of appointment scheduling status (blast), at 2 different health systems: the University of Massachusetts Chan Medical School (UMass) and the University of Florida College of Medicine-Jacksonville (UFL). METHODS: Using the 2 approaches, we sent patient portal messages to patients with AF and grouped patients by high-risk patients on anticoagulation (group 1), high-risk patients off anticoagulation (group 2), and low-risk patients who may become eligible for anticoagulation in the future (group 3). Risk was classified based on the congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, stroke, vascular disease, age between 65 and 74 years, and sex category (CHA2DS2-VASc) score. The messages contained a link to the Upbeat website of the Heart Rhythm Society, which displays print and video materials about AF and anticoagulation. We then tracked message opening, review of the website, anticoagulation use, and administered patient surveys across messaging approaches and sites using Epic Systems (Epic Systems Corporation) electronic health record data and Google website traffic analytics. We then conducted chi-square tests to compare potential differences in the proportion of patients opening messages and other evaluation metrics, adjusting for potential confounders. All statistical analyses were performed in SAS (version 9.4; SAS Institute). RESULTS: We sent 1686 targeted messages and 1450 blast messages. Message opening was significantly higher with the targeted approach for patients on anticoagulation (723/1156, 62.5% vs 382/668, 57.2%; P=.005) and trended the same in patients off anticoagulation; subsequent website reviews did not differ by messaging approach. More patients off anticoagulation at baseline started anticoagulation with the targeted approach than the blast approach (adjusted percentage 9.3% vs 2.1%; P<.001). CONCLUSIONS: Patients were more responsive in terms of message opening and subsequent anticoagulation initiation with the targeted approach.

A Case Report of Culture-Negative Necrotizing Fasciitis With Low Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) Score: A Diagnostic Dilemma.

The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score is a diagnostic tool used to help clinicians identify necrotizing fasciitis (NF) in its early stages. This tool uses six laboratory values including the patient's white blood cell count, C-reactive protein level, serum sodium level, creatinine level, and hemoglobin level to help with risk stratification. Each of these laboratory values is assigned a point value and the total score is used to determine the likelihood that a patient has NF, with a score of 6 or higher considered to be strongly indicative. The LRINEC score has gained popularity in recent years, having been included in guidelines and society recommendations for the management of NF. However, some studies have challenged the validity of the LRINEC score. Prompt and accurate diagnosis of NF is imperative given the associated mortality rate, which can be as high as 30%-40%, especially if the diagnosis is delayed. We present a case of a patient with a delayed diagnosis of NF that was initially missed in the early stages in the setting of a low LRINEC, however, growing clinical suspicion eventually led to an accurate diagnosis and management.

Clustering Gene Expressions Using the Table Invitation Prior.

A prior for Bayesian nonparametric clustering called the Table Invitation Prior (TIP) is used to cluster gene expression data. TIP uses information concerning the pairwise distances between subjects (e.g., gene expression samples) and automatically estimates the number of clusters. TIP's hyperparameters are estimated using a univariate multiple change point detection algorithm with respect to the subject distances, and thus TIP does not require an analyst's intervention for estimating hyperparameters. A Gibbs sampling algorithm is provided, and TIP is used in conjunction with a Normal-Inverse-Wishart likelihood to cluster 801 gene expression samples, each of which belongs to one of five different types of cancer.

Preventing preventable strokes: A study protocol to push guideline-driven atrial fibrillation patient education via patient portal.

Background: The main approach to preventing stroke in patients with atrial fibrillation (AF) is anticoagulation (AC), but only about 60% of at-risk individuals are on AC. Patient-facing electronic health record-based interventions have produced mixed results. Little is known about the impact of health portal-based messaging on AC use. Objective: The purpose of this study was describe a protocol we will use to measure the association between AC use and patient portal message opening. We also will measure patient attitudes toward education materials housed on a professional society Web site. Methods: We will send portal messages to patients aged ≥18 years with AF 1 week before an office/teleconference visit with a primary care or cardiology provider. The message will be customized for 3 groups of patients: those on AC; those at elevated risk but off AC; and those not currently at risk but may be at risk in the future. Within the message, we will embed a link to UpBeat.org, a Web site of the Heart Rhythm Society containing patient educational materials. We also will embed a link to a survey. Among other things, the survey will request patients to rate their attitude toward the Heart Rhythm Society Web pages. To measure the effectiveness of the intervention, we will track AC use and its association with message opening, adjusting for potential confounders. Conclusion: If we detect an increase in AC use correlates with message opening, we will be well positioned to conduct a future comparative effectiveness trial. If patients rate the UpBeat.org materials highly, patients from other institutions also may benefit from receiving these materials.

Durability of Changes in Biomarkers of Cardiometabolic Disease: 1-Year Family-Based Intervention in Children with Obesity.

Background: The sustainability of health benefits in response to lifestyle-based interventions remains unclear in children with overweight and obesity, and cardiometabolic disease (CMD). We determined the changes in novel biomarkers of CMD in a 1-year family-based intervention (FBI) program, during 6-month active monitoring phase and at 12-month follow-up. Methods: Children with an age-adjusted body mass index (BMI) percentile ≥85 (N = 130; age 8-11 years) were recruited for a 1-year (6-month monitored and 6-month unmonitored) randomized controlled FBI program. Anthropometry and selected biomarkers of CMD were measured in 87 participants, randomly allocated to intervention (INT) and education-only (EDU) groups, at baseline, immediately after a 6-month active intervention or control period, and at 12-month unmonitored follow-up. Results: Samples from 87 participants (age 10.00 ± 0.11 years and Tanner stage ≤3) with obesity (BMI%ile = 97.45 ± 0.15) were available. Overall intervention effect (between groups), was observed for total (T) and high molecular weight (HMW) adiponectin, ratio of total to HMW adiponectin, fibrinogen, and interleukin (IL)-6 (P < 0.05 for all). However, between-group beneficial changes after adjusting for baseline levels were limited to BMI percentile, T and HMW adiponectin and their ratio, IL-6, and fibrinogen (P < 0.05 for all) mainly during the 6-month period of monitored intervention. Changes in traditional risk factors such as lipids and triglycerides were inconsistent. During the 6-month follow-up period, the changes in biomarkers leveled-off, except for T and HMW adiponectin, IL-6, and fibrinogen that continued to show benefits (P < 0.05) from the 6- to 12-month follow-up. Conclusions: The FBI program beneficially altered novel biomarkers of CMD during the monitored intervention phase in school-age children with obesity, but they mostly moved back toward baseline during the unmonitored follow-up phase. The changes in novel biomarkers of CMD appear to be more sensitive compared to the traditional risk factors. The study implies the need for refinements in lifestyle-based approaches in the preservation of cardiovascular health and calls for robust biomarkers to monitor the changes. The study was registered at ClinicalTrials.gov (NCT01146314).

Profiles in paint: contrasting responses to a common artistic exercise by people with different dementias.

Paintings could offer insight into the varied experiences of people with different dementias. In this project, a single exercise - the painting of a group of objects in still-life - was used to capture artistic production in four artists with different diagnoses of dementia and four healthy artists. Whilst quantitative studies provide important insights into the neuroanatomical supports for artistic actions, autonomous art exercises may yield deeper understanding of the individual creative experience in the context of neurodegenerative disease.

Conceptualising and Understanding Artistic Creativity in the Dementias: Interdisciplinary Approaches to Research and Practise.

Creativity research has a substantial history in psychology and related disciplines; one component of this research tradition has specifically examined artistic creativity. Creativity theories have tended to concentrate, however, on creativity as an individual phenomenon that results in a novel production, and on cognitive aspects of creativity, often limiting its applicability to people with cognitive impairments, including those with a dementia. Despite growing indications that creativity is important for the wellbeing of people living with dementias, it is less well understood how creativity might be conceptualised, measured and recognised in this population, and how this understanding could influence research and practise. This paper begins by exploring prevailing concepts of creativity and assesses their relevance to dementia, followed by a critique of creativity and dementia research related to the arts. Perspectives from researchers, artists, formal and informal caregivers and those with a dementia are addressed. We then introduce several novel psychological and physiological approaches to better understand artistic-related creativity in this population and conclude with a conceptualisation of artistic creativity in the dementias to help guide future research and practise.

The lines are open: An artist's experience of working with dementia research.

This paper offers a first-hand account from a visual artist working with people living with a rare dementia. It explores the processes and motivations involved in situating an individual artistic practice in the field of dementia research. The paper discusses the potential role of creative art-making for neuropsychological testing and some of the complexities of interpretation that this involves. This account also highlights the ways in which working with people with dementia can be personally and artistically transformative.

Preparatory planning framework for Created Out of Mind: Shaping perceptions of dementia through art and science.

Created Out of Mind is an interdisciplinary project, comprised of individuals from arts, social sciences, music, biomedical sciences, humanities and operational disciplines. Collaboratively we are working to shape perceptions of dementias through the arts and sciences, from a position within the Wellcome Collection. The Collection is a public building, above objects and archives, with a porous relationship between research, museum artefacts, and the public.  This pre-planning framework will act as an introduction to Created Out of Mind. The framework explains the rationale and aims of the project, outlines our focus for the project, and explores a number of challenges we have encountered by virtue of working in this way.

Phase II trial of combination bevacizumab and temsirolimus in the treatment of recurrent or persistent endometrial carcinoma: a Gynecologic Oncology Group study.

OBJECTIVE: This two-stage phase II study was designed to assess the activity of the combination of temsirolimus and bevacizumab in patients with recurrent or persistent endometrial carcinoma (EMC). METHODS: Eligible patients had persistent or recurrent EMC after receiving 1-2 prior cytotoxic regimens, measurable disease, and Gynecologic Oncology Group performance status ≤ 2. Treatment consisted of bevacizumab 10 mg/kg every other week and temsirolimus 25 mg IV weekly until disease progression or prohibitory toxicity. Primary end points were progression-free survival (PFS) at six months and overall response rate using RECIST criteria. RESULTS: Fifty-three patients were enrolled. Forty-nine patients were eligible and evaluable. Median age was 63 years, and prior treatment consisted of one or two regimens in 40 (82%) and 9 (18%), respectively. Twenty (41%) received prior radiation. Adverse events were consistent with those expected with bevacizumab and temsirolimus treatment. Two gastrointestinal-vaginal fistulas, one grade 3 epistaxis, two intestinal perforations and 1 grade 4 thrombosis/embolism were seen. Three patient deaths were possibly treatment related. Twelve patients (24.5%) experienced clinical responses (one complete and 11 partial responses), and 23 patients (46.9%) survived progression free for at least six months. Median progression-free survival (PFS) and overall survival (OS) were 5.6 and 16.9 months, respectively. CONCLUSION: Combination of temsirolimus and bevacizumab is deemed active based on both objective tumor response and PFS at six months in recurrent or persistent EMC. However, this treatment regimen was associated with significant toxicity in this pretreated group. Future study will be guided by strategies to decrease toxicity and increase response rates.

The relationship between serum sodium and intracranial pressure when using hypertonic saline to target mild hypernatremia in patients with head trauma.

INTRODUCTION: Limited data suggest mild hypernatremia may be related to lower intracranial pressure (ICP) in patients with traumatic brain injury (TBI). The practice at the study center has been to use hypertonic saline (HTS) to generate a targeted serum sodium of 145 to 155 mEq/l in patients with TBI. The purpose of this study was to determine the relationship between serum sodium values and ICP, and to evaluate the acute effect of HTS on ICP. METHODS: A retrospective review of patients who were admitted to the trauma ICU for TBI, had an ICP monitor placed, and received at least one dose of HTS between January 2006 and March 2011 was performed. Data were collected for up to 120 hours after ICP monitor placement. The primary outcome was the relationship between serum sodium and maximum ICP. Secondary outcomes were the relationship between serum sodium and the mean number of daily interventions for ICP control, and the acute effect of HTS on ICP during the 6 hours after each dose. Linear regression was used to analyze the primary outcome. Analysis of variance on ranks and repeated measures analysis of variance were used to evaluate the number of interventions and the acute effect of HTS on ICP, respectively. RESULTS: Eighty-one patients were enrolled with mean ± standard deviation age of 36 ± 15 years and median Glasgow Coma Scale score of 7 (interquartile range, 4 to 7). A total of 1,230 serum sodium values (range, 118 to 174 mEq/l) and 7,483 ICP values (range, 0 to 159 mmHg) were collected. There was no correlation between serum sodium and maximum ICP (R(2) = 0.0052). The overall mean ± standard deviation number of interventions for elevated ICP per day was 4.2 ± 2.9, 2.9 ± 2.0, and 2.6 ± 2.3 for patients with a mean serum sodium of < 145, 145 to 155, and > 155 mEq/l, respectively (P < 0.001). Regarding the acute effect of HTS on ICP, there was no statistical difference in mean ICP compared with baseline during hours 1 through 6 following HTS doses (baseline, 13.7 ± 8.4 mmHg; hour 1, 13.6 ± 8.3 mmHg; hour 2, 13.5 ± 8.8 mmHg; hour 3, 13.3 ± 8.7 mmHg; hour 4, 13.4 ± 8.7 mmHg; hour 5, 13.4 ± 8.3 mmHg; hour 6, 13.5 ± 8.3 mmHg; P = 0.84). CONCLUSIONS: Serum sodium concentrations did not correlate with ICP values. These results warrant further evaluation and possible reassessment of sodium goals for ICP management in patients with TBI.

A quality process study of lymph node evaluation in endometrial cancer.

Our objective was to analyze the reported lymph node counts between surgeons, histology prosectors, and pathologists using a cohort of patients enrolled on a national protocol that standardized surgical intent.This is a retrospective review of patients with uterine cancer who underwent a standardized formal staging procedure as dictated by a National Cancer Institute sponsored protocol. Patients were staged using the International Federation of Gynecology and Obstetrics 1988 guidelines. All patients required a hysterectomy, bilateral salpingo-oophorectomy, and bilateral pelvic and para-aortic lymphadenectomy. Lymphadenectomy specimens were separated by the following regions: external iliac, obturator, common iliac, and periaortic. Lymph node counts were analyzed by region, surgeon, histology prosector, and pathologist.There were 78 patients enrolled in the protocol during the study period. Of them, 72 (92%) patients met the inclusion criteria. A total of 2397 lymph nodes were counted, with an average total number of 33 (SD=9) lymph nodes dissected per patient. Surgeons A, B, and C had an average lymph node count of 32, 33, and 35, respectively, with no significant difference in mean node count (P=0.66). Prosectors 1 to 4 dissected an average of 34, 33, 28, and 35 lymph nodes, respectively (P=0.091). There were 2 pathologists with ≥ 10 cases. Their mean lymph node counts were 35 and 30, respectively, with no significant difference in mean node count (P=0.079).This systematic review did not identify a discrepancy in nodal count among surgeons, prosectors, or pathologists at our institution. The methods used may be helpful in structuring interdepartmental reviews for completeness of nodal dissections in cases where surgical intent has been standardized.

Bilateral and multinodular signet-ring stromal tumor of the ovary.

BACKGROUND: Signet-ring stromal tumor of the ovary is a rare, benign sex cord stromal tumor that is typically unilateral. Its distinction from malignant signet-ring cell adenocarcinoma metastatic to the ovary (Krukenberg tumor), is critical. CASE: A 69-year-old gravida 5 woman presented with a 1-week history of right lower abdominal pain. Ultrasonography revealed a 4-cm right adnexal mass. Serum CA 125 and carcinoembryonic antigen were within normal limits. An exploratory laparotomy, partial omentectomy, bilateral salpingo-oophorectomy and peritoneal washing were performed. Solid multinodular masses in both ovaries were identified that histologically corresponded to bilateral signet-ring stromal tumors. CONCLUSION: We report a rare example of signet-ring stromal tumor that is bilateral and multinodular mimicking a Krukenberg tumor. Awareness of this unusual presentation can help prevent incorrect diagnosis.

Identification of components responsible for the odor of cigar smoker's breath.

Following smoking 1/2 of a cigar, the most odorous cigar tobacco smoke components extracted from the surface of the tongue by nylon-meshed swabs and then extracted from the swab headspace by solid phase microextraction were ethyl pyrrole, 2,3-dimethyl pyrazine, and 2-ethyl pyridine. Similar classes of compounds were identified from the headspace of an aqueous simulated saliva solution treated with cigar smoke. The most odorous compounds were 2,3,5-trimethyl pyridine, 2,5-dimethyl pyrazine, and 2-ethyl-3,5-dimethyl pyridine. Pyridines and pyrazines, the most prominent classes of odorous compounds identified in this experiment, may be generated during cigar pyrrolysis by cleavage of nicotine or by Maillard reaction.