ALS Care Research Articles

Provision of assistive technology devices among people with ALS in Germany: a platform-case management approach

Objective: The procurement of assistive technology devices (ATD) is an essential component of managed care in ALS. The objective was to analyze the standards of care for ATD and to identify challenges in the provision process. Methods: A cohort study design was used. We investigated the provision of 11,364 ATD in 1494 patients with ALS at 12 ALS centers in Germany over four years. Participants were patients that entered a case management program for ATD including systematic assessment of ATD on a digital management platform. Results: Wheelchairs (requested in 65% of patients), orthoses (52%), bathroom adaptations (49%), and communication devices (46%) were the most needed ATD. There was a wide range in the number of indicated ATD per patient: 1 to 4 ATD per patient in 45% of patients, 5 to 20 ATD in 48%, and >20 ATD in 7% of patients. Seventy percent of all requested ATD were effectively delivered. However, an alarming failure rate during procurement was found in ATD that are crucial for ALS patients such as powered wheelchairs (52%), communication devices (39%), or orthoses (21%). Leading causes for not providing ATD were the refusal by health insurances, the decision by patients, and the death of the patient before delivery of the device. Conclusions: The need for ATD was highly prevalent among ALS patients. Failed or protracted provision posed substantial barriers to ATD procurement. Targeted national strategies and the incorporation of ATD indication criteria in international ALS treatment guidelines are urgently needed to overcome these barriers.

Needs of informal caregivers across the caregiving course in amyotrophic lateral sclerosis: a qualitative analysis

ObjectivesAmyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a debilitating terminal condition. Informal caregivers are key figures in ALS care provision. The physical, psychological and emotional...

Experience with telemedicine in a multi-disciplinary ALS clinic

Objective: Telemedicine using video televisits is emerging as a means to provide care directly to patients. Here we report our experience using video televisits to provide follow-up care as a part of the Massachusetts General Hospital (MGH) Telemedicine for People with ALS (TelePALS) initiative.Methods: This was a retrospective chart review of all video televisit encounters conducted by the MGH ALS clinic between September 2014 and January 2016.Results: A total of 97 distinct ALS patients were seen using video televisits. Twenty-four percent of these patients had more than one video televisit. The median distance of the patients’ primary residence to MGH was 211 miles. The average video televisit lasted 32 min (±12). About half the patients (54%) were ambulatory at the time of their video televisit. The most commonly addressed issues were medication management (89%), discussion of goals of care (74%), research (55%) and equipment use (50%). Acute care issues were rarely discussed.Conclusion: Video televisits are feasible and can be a useful tool to supplement traditional clinic-based multidisciplinary ALS care.

Palliative care in ALS

Palliative care in ALS

Incorporation of telehealth into a multidisciplinary ALS Clinic: feasibility and acceptability

Objective: The practice of telehealth in the care of patients with ALS has received little attention, but has the potential to change the multidisciplinary care model. This study was carried out to assess the feasibility and acceptability of telehealth for ALS care via real-time videoconferencing from the clinic to patients’ homes. Methods: Patients and caregivers engaged in live telehealth videoconferencing from their homes with members of a multidisciplinary ALS care team who were located in an ALS clinic, in place of their usual in-person visit to the clinic. Participating patients, their caregivers, and health care providers (HCPs) completed surveys assessing satisfaction with the visit, quality of care, and confidence with the interface. Mixed methods analysis was used for survey responses. Results: Surveys from 11 patients, 12 caregivers, and 15 HCPs were completed. All patients and caregivers, and most HCPs, agreed that the system allowed for good communication, description of concerns, and provision of care recommendations. The most common sentiment conveyed by each group was that telehealth removed the burdens of travel, resulting in lower stress and more comfortable interactions. Caregivers and HCPs expressed more concerns than patients about the ways in which telehealth fell short of in-person care. Conclusions: Telehealth was generally viewed favourably by ALS patients, caregivers, and multidisciplinary team members. Improvements in technology and in methods to provide satisfactory remote care without person-to-person contact should be explored.

Delivering tertiary centre specialty care to ALS patients via telemedicine: a retrospective cohort analysis

Objective: This study was undertaken to determine if ALS patients evaluated via telemedicine received the same quality of care as patients evaluated by traditional face-to-face encounters.Methods: A retrospective cohort study design was used. Participants were patients diagnosed with ALS that received multidisciplinary care at the tertiary Cleveland VA ALS Centre between 1 March 2008- and 31 anuary 2015. Participants were not randomised, but chose telemedicine based on preference, disability level or distance from the clinic. Telemedicine in this study consisted of a video conferencing platform enabling remote rather than face-to-face encounters with participants.Results: There was no significant association between receiving quality ALS care and the mode of care. There was a trend for telemedicine patients to utilise home health care less often than those that received clinic care (AOR 0.50; 95% CI 0.16–1.59). There was no significant difference in survival time between the two groups (log-rank test χ2 = 3.62, df = 1, p = 0.05). Patients receiving telemedicine had a higher probability of remaining stable or having <30% decrease in ALSFRS-R over time (log-rank test χ2 = 4.46, df = 1, p = 0.03). There was a significantly lower risk of disease progression for patients receiving telemedicine (HR = 0.39, 95% CI = 0.16–0.93).Conclusions: Patients managed by telemedicine received the same quality of care and had similar outcomes to those patients seen via traditional face-to-face encounters. Telemedicine is an effective platform for delivering high quality tertiary ALS care.

神経難病（ALS）の地域連携の課題―臨床現場の問題点―

神経難病（ALS）の地域連携の課題―臨床現場の問題点―

E.11 Non-invasive ventilation in patients with amyotrophic lateral sclerosis: practice patterns amongst Canadian care providers

Background: The purpose of this study was to: 1) describe current non-invasive ventilation (NIV) usage patterns amongst Canadian ALS healthcare providers; 2) compare/contrast with previous practice patterns; and 3) explore barriers to NIV access encountered by current practitioners. Methods: Healthcare professionals (including physicians, respiratory therapists, and nurses) at major Canadian ALS care centres were sent a web-based survey. Participants were asked to provide input on practice demographics, access and initiation of NIV, and follow-up of NIV. Quantitative data were analyzed with descriptive and comparative statistics, while qualitative data were analyzed using interpretative phenomenological analysis method to identify emergent themes. Results: 26 participants responded. Median NIV usage was 39% (range 10-100%), about double of what was previously reported (18%). Mean times from referral to routine and urgent NIV initiation were 13 (95% CI 9-17) and 5 (95% CI 3-7) days respectively. NIV was most commonly initiated in clinic (68%), while 38% report having access to home-NIV initiation. Lack of social support (62%) and cognitive impairment (46%) were the most common deterrents to initiating NIV. Similar to what is previously reported, barriers to access can be stratified to patient, clinical, institutional, and regional levels. Conclusions: Despite increased usage and improved access, there remain considerable barriers for ALS patients to receive NIV.

National Characteristics of Emergency Medical Services in Frontier and Remote Areas

Although much is known about EMS care in urban, suburban, and rural settings, only limited national data describe EMS care in isolated and sparsely populated frontier regions. We sought to describe the national characteristics and outcomes of EMS care provided in frontier and remote (FAR) areas in the continental United States (US). We performed a cross-sectional analysis of the 2012 National Emergency Medical Services Information System (NEMSIS) data set, encompassing EMS response data from 40 States. We linked the NEMSIS dataset with Economic Research Service-identified FAR areas, defined as a ZIP Code >60 minutes driving time to an urban center with >50,000 persons. We excluded EMS responses resulting in intercepts, standbys, inter-facility transports, and medical transports. Using odds ratios, t-tests and the Wilcoxon rank-sum test, we compared patient demographics, response characteristics (location type, level of care), clinical impressions, and on-scene death between EMS responses in FAR and non-FAR areas. There were 15,005,588 EMS responses, including 983,286 (7.0%) in FAR and 14,025,302 (93.0%) in non-FAR areas. FAR and non-FAR EMS events exhibited similar median response 5 [IQR 3–10] vs. 5 [3–8] min), scene (14 [10–20] vs. 14 [10–20] min), and transport times (11 [5.,24] vs. 12 [7,19] min). Air medical (1.51% vs. 0.42%; OR 4.15 [95% CI: 4.03–4.27]) and Advanced Life Support care (62.4% vs. 57.9%; OR 1.25 [1.24–1.26]) were more common in FAR responses. FAR responses were more likely to be of American Indian or Alaska Native race (3.99% vs. 0.70%; OR 5.04, 95% CI: 4.97–5.11). Age, ethnicity, location type, and clinical impressions were similar between FAR and non-FAR responses. On-scene death was more likely in FAR than non-FAR responses (12.2 vs. 9.6 deaths/1,000 responses; OR 1.28, 95% CI: 1.25–1.30). Approximately 1 in 15 EMS responses in the continental US occur in FAR areas. FAR EMS responses are more likely to involve air medical or ALS care as well as on-scene death. These data highlight the unique characteristics of FAR EMS responses in the continental US.

The being-in-the-world in the final trimester of a low-risk gestation reveals a being-with needing care

The goal of this study was to: understand the pregn ant Being-in-the-world (Dasein) in the final trimes ter of a low risk pregnancy. The present article is the result of qualitative research based on Heidegger' s phenomenology. Ten interviews were conducted with p regnant women in their third trimester of a low risk gestation. Results: due to their limitations, the pregnant Dasein becomes dependant of her partne r and family. The pregnant Dasein longs for the momen t that she will take care of her baby, be in its company, because she already fosters feelings for t he baby. The pregnant Dasein in the final trimester of a low risk pregnancy requires care and support f rom her partner and family members during the pregnancy. It was noted that this being has doubts, fears and longings, and is more vulnerable. Therefore, a nurse that carries out low risk prenat al care during this period should be present, improving the quality of the approach to women and thus meet their needs.

Prospective study of cost of care at multidisciplinary ALS centers adhering to American Academy of Neurology (AAN) ALS practice parameters

Multidisciplinary care in ALS is associated with longer survival, improved quality of life, and reduced hospital admissions, but there are no published data on institutional costs associated with multidisciplinary ALS care at U.S. centers. We prospectively examined institutional costs, adherence to AAN Practice Parameters and patient satisfaction in multidisciplinary ALS clinics at 18 U.S. ALS centers. Centers reported patient volumes; direct costs for staff salary/benefits, supplies and equipment; and institutional non-salary and overhead costs over a three-month period. In 1117 patients seen during this period, mean age was 61.5 years (range 25–91 years), 56% were male, and mean ALSFRS-R score was 29. Mean total salary/benefit cost per clinic day for all providers was $2964 (range $1692–$5236 across centers). Mean salary/benefit cost per patient per clinic was $507 (range $258–$806 across centers). Differences among centers in reporting non-salary costs prevented meaningful analysis. Practice parameter adherence and patient satisfaction were high. This prospective collaborative study demonstrates the direct financial burden of evidence-based multidisciplinary ALS care in the U.S.; more refined non-salary and overhead cost data are needed to evaluate the full cost impact of care. These data may be useful in supporting evidence-based models of patient centered care for ALS.

Comprehensive rehabilitative care across the spectrum of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) is a neurodegenerative disease that results in progressive muscle weakness and wasting. There is no known cure and the disease is uniformly fatal. This review discusses current concepts in ALS care, from breaking the diagnosis to end-of-life care. People with ALS have several multidisciplinary needs due to a complex and dynamic disease process. They benefit from rehabilitation interventions that are individualized and have the goal of optimizing independence, function, and safety. These strategies also help minimize symptomatic burden and maximize quality of life. Patient-centered, multidisciplinary care has a significant impact on the life of people with ALS and is the current standard of care for this patient population.

The Current Status of End-of-Life Care in ALS: Progress and Personal Experience from the Past Ten Years in the USA

End-of-life (EOL) care and management in ALS is one of the least studied subjects within ALS management. In early 2000, Robert Wood Johnson and the ALS Association assembled an advisory committee to develop recommendations for the promotion of excellence during EOL care. We have reviewed this 10-year-old publication¹⁾ and describe the progress we have made in 16 different areas of EOL recommendations. Although there are some areas with little progress over the past 10 years, a few areas, particularly those regarding psychosocial management, symptomatic treatments, and ethical issues of EOL, have made major advancements during this time. the statements and recommendations for promoting excellence in EOL made 10 years ago appear to still be valid. We also review hastening death, a newly recognized issue of EOL, and discuss the potential reasons why Japanese and American patients chose tracheostomy-invasive ventilator at different rates. We conclude that EOL management is still evolving and in progress. Until we have a cure for ALS, every effort is being made to improve EOL care for patients with ALS.

Developing brain-computer interfaces from a user-centered perspective: Assessing the needs of persons with amyotrophic lateral sclerosis, caregivers, and professionals

By focus group methodology, we examined the opinions and requirements of persons with ALS, their caregivers, and health care assistants with regard to developing a brain-computer interface (BCI) system that fulfills the user's needs. Four overarching topics emerged from this analysis: 1) lack of information on BCI and its everyday applications; 2) importance of a customizable system that supports individuals throughout the various stages of the disease; 3) relationship between affectivity and technology use; and 4) importance of individuals retaining a sense of agency. These findings should be considered when developing new assistive technology. Moreover, the BCI community should acknowledge the need to bridge experimental results and its everyday application.

Optimizing Patient Autonomy in Amyotrophic Lateral Sclerosis: Inclusive Decision-Making in Multidisciplinary Care

and may impede the timing and quality of their decisions. Several interventions for ALS care, including nutrition, hydration and respiratory support, are contingent on well-timed implementation [5,6]. However, optimal timing of symptom management may be at odds with patients’ readiness to consider these complex interventions. Patients and families need time to come to terms with their changing situation before they can fully participate in decision-making. Reactions to the diagnosis can have an influence on patients’ willingness to engage with healthcare services [7] and their preferred role in decisionmaking. Desire for autonomy is an individual choice, reflecting patients’ attitudes to life, personal circumstances and healthcare experiences [7]. Patients and families are confronted by escalating degeneration and loss, over which they have no control. Autonomy over choices of symptom management and quality of life may become a way for patients to exert control over their continually changing situation [8]. Three aspects of ALS service delivery have been found to facilitate patient autonomy in decision-making: access to specialized ALS multidisciplinary care [7];

A Poisson Regression Model to Examine Spatial Patterns in Antenatal Care Utilisation in Nigeria

ABSTRACTDespite the benefits of antenat]al care to the mother and the child, it is available less often in sub‐Saharan Africa than in other regions. Coverage of at least four visits is lower, with a slower rate of increase, than in other regions of the world. Using data from 2008 Nigeria Demographic and Health Survey, we applied a Poisson regression model with geo‐additive predictors to analyse individual‐level and community‐level determinants of antenatal care utilisation and quantify district specific spatial effect. The spatial structure was modelled using conditional autoregressive prior, whereas the metrical covariates were assumed to be non‐linear. Findings reveal substantial spatial variation with a distinct north–south divide in antenatal care utilisation in Nigeria. The effects of mother's age at birth, partner's age, and marital duration were non‐linear, and utilisation was found to differ significantly between never‐married and ever‐married women respondents. Results further show that older women, particularly those above 35 years old, utilise antenatal care services during pregnancy more than the younger ones. Policymakers and public health providers need to put in more efforts to address these differentials as a minimum of four visits for antenatal care during pregnancy is required by all women in all parts of the country. Allocations for antenatal care services should also take into account the differentials so that those states lagging behind can be brought up, at least to scale with others. Copyright © 2013 John Wiley &amp; Sons, Ltd.

The impact of clinical factors, riluzole and therapeutic interventions on ALS survival: A population based study in Modena, Italy

The prognostic role of riluzole, enteral nutrition (EN), non-invasive ventilation (NIV) and interdisciplinary care in ALS is still debated. A population based study has been performed focusing on ALS survival, with particular attention to prognostic factors and therapeutic intervention. All patients diagnosed with ALS between 2000 and 2009 and residing in Modena, Italy, have been registered. A centre for motor neuron disease (MND) has been active in our province since 2000, in addition to a prospective registry collecting all incident cases. One hundred and ninety-three incident cases have been collected during the 10 years of the study. Results demonstrated that median survival was 41 months (the overall three-year and five-year survival rates being 54.36% and 28.81%, respectively). Based on univariate analysis, factors related to survival were: age at diagnosis, gender, site of onset, phenotype, riluzole treatment and tracheostomy. In the Cox multivariable model, the factors independently related to a longer survival were age (p < 0.01), site of onset (p = 0.02) and riluzole treatment (p < 0.01), with a median gain in survival of 29 months (patients aged < 55 years compared with patients ≥ 55 years), 20 months (spinal versus bulbar onset), and 12 months (riluzole, yes vs. no), respectively. In conclusion, the study has confirmed the prognostic role of clinical features, but has surprisingly demonstrated that riluzole prolonged life significantly longer than NIV and EN. This observational study described the effects of ALS management in a setting that may approximate routine clinical practice more closely than randomized controlled trial (RCT); effects of uncontrolled potential confounders, however, cannot be excluded.

耐熱マグネシウム合金ダイカスト凝固割れ発生の予測

This study was performed to develop the predicting method of solidification cracking during die-casting of tentative heat-resistant magnesium alloys and commercial ones such as Mg–Al–Ca–RE, Mg–Al–Ca–Mn, Mg–Al–Ca–Sn, AE44 and AZ91D. Both high temperature ductility curve and strain near cracking area of each alloy at liquidus-solidus temperature range were required in order to predict the solidification cracking. High temperature strain was calculated by a casting simulation code,“ProCAST”. High temperature ductility curve of each alloy was measured by the experiment. Comparing with this high temperature strain and ductility at liquidus and solidus temperature range, it was confirmed whether solidification cracking occurs or not in each magnesium alloy. Finally, this predicting method was verified using simple shape of die-casting. Consequently, occurrence of solidification cracking in the simple shape of die-casting in all magnesium alloys used can be predicted by this predicting method.

Multidisciplinary Care in ALS: Expanding the Team

Optimal management of ALS requires a multidisciplinary team that operates in an integrated manner. Health care professionals that are unaccustomed to managing ALS may not recognise the significance...

Helicopter EMS: Research Endpoints and Potential Benefits.

Patients, EMS systems, and healthcare regions benefit from Helicopter EMS (HEMS) utilization. This article discusses these benefits in terms of specific endpoints utilized in research projects. The endpoint of interest, be it primary, secondary, or surrogate, is important to understand in the deployment of HEMS resources or in planning further HEMS outcomes research. The most important outcomes are those which show potential benefits to the patients, such as functional survival, pain relief, and earlier ALS care. Case reports are also important “outcomes” publications. The benefits of HEMS in the rural setting is the ability to provide timely access to Level I or Level II trauma centers and in nontrauma, interfacility transport of cardiac, stroke, and even sepsis patients. Many HEMS crews have pharmacologic and procedural capabilities that bring a different level of care to a trauma scene or small referring hospital, especially in the rural setting. Regional healthcare and EMS system's benefit from HEMS by their capability to extend the advanced level of care throughout a region, provide a “backup” for areas with limited ALS coverage, minimize transport times, make available direct transport to specialized centers, and offer flexibility of transport in overloaded hospital systems.