Multidisciplinary Care In Amyotrophic Lateral Sclerosis Research Articles

Telehealth for amyotrophic lateral sclerosis in a multidisciplinary service in a Brazilian reference center.

Telehealth has been used in the treatment of different diseases, and it has been shown to provide benefits for patients with amyotrophic lateral sclerosis (ALS). Due to the social distancing measures put into effect during the coronavirus disease 2019 (COVID-19) pandemic, there was an urgent need for telehealth to ensure the provision of healthcare. To evaluate the feasibility of telehealth for the provision of multidisciplinary ALS care, and to assess its acceptability among patients and caregivers. We conducted a retrospective cohort study in which multidisciplinary evaluations were performed using the Teleconsulta platform. The patients included had ALS and at least one in-person clinical evaluation. The patients and the caregivers answered satisfaction questionnaires. The sample was composed of 46 patients, 32 male and 14 female subjects. The average distance from their residences to the reference services was of 115 km. Respiratory adjustment was the most addressed topic. The strategy is viable and well accepted in terms of satisfaction. It was even more positive for patients in advanced stages of the disease or for those living far from the referral center.

Palliative care in amyotrophic lateral sclerosis clinics: A survey of NEALS consortium membership.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative condition managed with a multidisciplinary approach. Palliative care is important for this approach, but there is a lack of recommendations for the role and involvement of palliative medicine (PM) specialists in multidisciplinary ALS care. This questionnaire-based survey assessed the state of palliative care in selective sites that are a part of Northeast Amyotrophic Lateral Sclerosis Consortium (NEALS). Twenty-seven percent of the sites included palliative care specialists as a part of their ALS clinics and they were introduced to the patients and family by ALS specialists (75.94%) usually at an advanced stage of ALS. Our study when compared with other study results having a similar aim showed that there is a variability in the practice of palliative care specialists in ALS clinics. Having evidence-based guidelines will help in the management of ALS patients more effectively.

Problem-focused coping underlying lower caregiver burden in ALS-FTD: implications for caregiver intervention

Objective: Amyotrophic lateral sclerosis (ALS) is a multisystem neurodegenerative disorder which includes cognitive and behavioral symptoms akin to frontotemporal dementia (FTD). Despite the necessity of caregiver intervention to assist with the management of cognitive and behavioral symptoms, there has been a lack of research on the topic. A focus on caregiver coping may offer a promising foundation to guide the development of interventions as part of ALS care. Accordingly, the aim of the present study was to examine the relationships between caregiver coping, psychological morbidity and burden of care in the context of ALS cognitive and behavioral symptoms. Methods: Fifty-five patient-caregiver dyads were recruited from specialized ALS and FTD clinics. Specific coping strategies were examined using the COPE Inventory/Brief COPE and psychological morbidity and burden were assessed using the Depression, Anxiety, and Stress Scale–21 and Zarit Burden Interview. The relationship between coping, psychological morbidity and burden of care were analyzed using univariate and multivariate methods. Results: High-burden caregivers were more likely to be caring for patients with a diagnosis of ALS-FTD (p =.0001). Caregivers used problem-focused strategies (particularly planning) more frequently (M = 71.4, SD = 15.3) compared to emotion-focused (M = 60.8, SD = 12.3) and dysfunctional coping strategies (M = 42.2, SD = 8.6). A diagnosis of ALS-FTD (p=.0001) and problem-focused strategies (p=.024) emerged as significant predictors of caregiver burden. Caregiver anxiety, depression and stress were not predictive of caregiver burden (p=.151). Conclusions: Timely provision of caregiver support optimizing problem-focused coping strategies as part of multidisciplinary ALS care, particularly for caregivers of ALS-FTD patients may mitigate caregiver burden.

An Interprofessional Approach to Amyotrophic Lateral Sclerosis Care.

Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disease that is characterized by the death of motor neurons in the spinal cord, brain stem, and motor cortex that are responsible for voluntary movement. For unknown reasons, military veterans are approximately twice as likely as the public to be given a diagnosis of ALS. Currently, there is no cure for ALS. The only US Food and Drug Administration-approved disease-modifying medication is riluzole (Rilutek), which may slow ALS progression but only minimally increases survival time. Multidisciplinary care in ALS has been shown to improve survival time and improve quality of life. The purpose of this article is to describe the development of an innovative evidence-based interprofessional healthcare program in a Veterans Administration medical center to address the needs of this vulnerable veteran population.

The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison

Background: Care for people with amyotrophic lateral sclerosis (ALS) has altered at King’s College Hospital over the last 20 years. The clinic has been a multidisciplinary, specialist, tertiary referral centre since 1995 with a large team with integrated palliative and respiratory care since 2006. We hypothesised that these changes would improve survival. Methods: In this retrospective observational study, patients diagnosed with El Escorial definite, probable and possible ALS between 1995–1998 and 2008–2011 were followed up. The primary outcome measure was a chi-square test for the proportion of each cohort surviving. Kaplan-Meier survival analysis and Cox multivariate regression were secondary analyses. Results: There was low reporting of some interventions. Five hundred and forty-seven people were included. Survival between the cohorts was significantly different (p = 0.022) with a higher proportion surviving during 2008–2011. Survival time was 21.6 (95% CI 19.2–24.0) months in the 2008–2011 cohort compared to 19.2 years (15.6–21.6) in the 1995–1998 cohort (log rank p = 0.018). Four hundred and ninety-three cases were included in the Cox regression. Diagnostic cohort was a significant predictor variable (HR 0.79 (0.64–0.97) p = 0.023). Conclusions: These results support the hypothesis that integrated specialist clinics with multidisciplinary input improve survival in ALS.

Amyotrophic lateral sclerosis: improving care with a multidisciplinary approach.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, leading to death within an average of 2–3 years. A cure is yet to be found, and a single disease-modifying treatment has had a modest effect in slowing disease progression. Specialized multidisciplinary ALS care has been shown to extend survival and improve patients’ quality of life, by providing coordinated interprofessional care that seeks to address the complex needs of this patient group. This review examines the nature of specialized multidisciplinary care in ALS and draws on a broad range of evidence that has shaped current practice. The authors explain how multidisciplinary ALS care is delivered. The existing models of care, the role of palliative care within multidisciplinary ALS care, and the costs of formal and informal care are examined. Critical issues of ALS care are then discussed in the context of the support rendered by multidisciplinary-based care. The authors situate the patient and family as key stakeholders and decision makers in the multidisciplinary care network. Finally, the current challenges to the delivery of coordinated interprofessional care in ALS are explored, and the future of coordinated interprofessional care for people with ALS and their family caregivers is considered.

Need and value of case management in multidisciplinary ALS care: A qualitative study on the perspectives of patients, spousal caregivers and professionals

Our objective was to explore the needs and value of case management according to patients with amyotrophic lateral sclerosis (ALS), their spousal caregivers, and health care professionals in the context of multidisciplinary ALS care. We undertook semi-structured interviews with 10 patients with ALS, their caregivers (n = 10) and their ALS health care professionals (n = 10), and held a focus group (n = 20). We transcribed the audio-taped interviews and analysed all data thematically. Participants indicated that in certain circumstances case management can have an added value. They identified factors for receptiveness to case management: adequacy of usual care, rate of disease progression, and degree of social network support and personal factors of patients and spousal caregivers. Participants valued the time for consultation, house calls and proactive approach of the case manager. Patients with ALS and caregivers appreciated emotional support, whereas professionals did not mention the importance of emotional support by the case manager.In conclusion, ALS teams can consider implementation of valued aspects of case management (accessibility, ample time, proactive approach, emotional support) in the usual multidisciplinary ALS care. Additional support might be provided to patients with rapidly progressive disease course, passive coping style and small social network.

Cluster RCT of case management on patients' quality of life and caregiver strain in ALS

To study the effect of case management on quality of life, caregiver strain, and perceived quality of care (QOC) in patients with amyotrophic lateral sclerosis (ALS) and their caregivers. We conducted a multicenter cluster randomized controlled trial with the multidisciplinary ALS care team as the unit of randomization. During 12 months, patients with ALS and their caregivers received case management plus usual care or usual care alone. Outcome measures were the 40-item ALS Assessment Questionnaire (ALSAQ-40), Emotional Functioning domain (EF); the Caregiver Strain Index (CSI); and the QOC score. These measures were assessed at baseline and at 4, 8, and 12 months. Case management resulted in no changes in ALSAQ-40 EF, CSI, or QOC from baseline to 12 months. ALSAQ-40 EF scores in both groups were similar at baseline and did not change over time (p = 0.331). CSI scores in both groups increased significantly (p < 0.0001). Patients with ALS from both groups rated their perceived QOC at baseline with a median score of 8, which did not change significantly during follow-up. Within the context of multidisciplinary ALS care teams, case management appears to confer no benefit for patients with ALS or their caregivers. This study provides Class III evidence that case management in addition to multidisciplinary ALS care does not significantly improve health-related quality of life of patients with ALS.

Recent advances in motor neuron disease

Amyotrophic lateral sclerosis (ALS) is the most common and most aggressive form of adult onset motor neuron degeneration. Despite intensive research, riluzole remains the only drug with proven efficacy. The cause and pathogenesis of the motor neuron degeneration in ALS appears to be a complex and multifactorial process. In this study, we review recent advances in our understanding of the molecular basis of sporadic and familial forms of ALS and improvements in the treatment of ALS patients. The discovery of mutations in the DNA/RNA-binding proteins transactive response DNA-binding protein 43 (TDP-43) and fused in sarcoma in familial ALS and the cytosolic sequestration of TDP-43 occurring in sporadic ALS opens new avenues in ALS research and suggests that altered RNA processing may be involved in the disease pathogenesis. The survival of ALS patients continues to increase most likely due to noninvasive respiratory ventilation, early percutaneous endoscopic gastrostomy and multidisciplinary care in ALS clinics. Recent insights in the pathology and genetics of ALS have improved our understanding of motor neuron degeneration and will hopefully lead to novel treatment strategies in ALS. Awaiting these, the beneficial effect of multidisciplinary care of ALS patients on survival and quality of life has become clear.

The costs of amyotrophic lateral sclerosis, according to type of care

Our objective was to estimate the economic burden of patients with amyotrophic lateral sclerosis (ALS) and to examine the effect of treatment in a multidisciplinary ALS treatment centre versus general care on costs and to describe differences in costs according to clinical characteristics. In a cross-sectional study 208 patients with ALS and their caregivers were interviewed and were asked to fill in a cost diary for six months. Patients were divided in groups according to type of treatment they received and according to clinical characteristics (site of onset, severity). The direct healthcare and non-healthcare costs were calculated according to Dutch guidelines for cost analysis in healthcare research, standardized for the year 2003. Ninety-eight patients were included when they had filled in the cost diary for at least three months and were affected for three years or less. Mean monthly costs were 1336 euros for the group receiving multidisciplinary care and 1271 euros for those receiving general care. This study shows that the costs of multidisciplinary ALS care were practically identical to the costs of general care. Earlier study showed that patients receiving multidisciplinary care had a better quality of life; therefore, the present study encourages the formation of multidisciplinary teams of professionals specialized in ALS care to further improve standards of care and QoL of patients suffering from ALS.

Racial differences in red cell indices.

A high prevalence of iron deficiency was found in apparently healthy Asian immigrant children in Harrow. After excluding children with overtly abnormal red cell indices, Asian and European children had identical haemoglobin values, but Asian children had much lower mean cell volume and mean corpuscular haemoglobin values and higher red blood cell values.