Amyotrophic Lateral Sclerosis Care Research Articles

Narrative review of diagnosis, management and treatment of dysphagia and sialorrhea in amyotrophic lateral sclerosis.

The degenerative motor neuron disorder amyotrophic lateral sclerosis (ALS) frequently leads bulbar symptoms like dysarthria, dysphagia, and sialorrhea, in approximately one-third of cases being the initial symptom. Throughout the disease, more than two-thirds of ALS patients experience dysphagia, regardless of the region of onset. In this review, we aimed to offer an updated overview of dysphagia and sialorrhea in ALS, covering its diagnosis, monitoring, and treatment in clinical practice. Regular assessment of dysphagia and sialorrhea during each patient visit is essential and should be a standard aspect of ALS care. Early discussion of potential treatments such as high-calorie diets or percutaneous endoscopic gastrostomy (PEG) is crucial. Furthermore, this review highlights and discusses potential areas for improvement in both clinical practice and research.

Prognostic communication in amyotrophic lateral sclerosis: findings from a Nationwide Italian survey.

Amyotrophic Lateral Sclerosis (ALS) is a fatal motor neuron disease with a highly variable prognosis. Among the proposed prognostic models, the European Network for the cure of ALS (ENCALS) survival model has demonstrated good predictive performance. However, few studies have examined prognostic communication and the diffusion of prognostic algorithms in ALS care. To investigate neurologists' attitudes toward prognostic communication and their knowledge and utilization of the ENCALS survival model in clinical practice. A web-based survey was administered between May 2021 and March 2022 to the 40 Italian ALS Centers members of the Motor Neuron Disease Study Group of the Italian Society of Neurology. Twenty-two out of 40 (55.0%) Italian ALS Centers responded to the survey, totaling 37 responses. The model was known by 27 (73.0%) respondents. However, it was predominantly utilized for research (81.1%) rather than for clinical prognostic communication (7.4%). Major obstacles to prognostic communication included the unpredictability of disease course, fear of a negative impact on patients or caregivers, dysfunctional reaction to diagnosis, and cognitive impairment. Nonetheless, the model was viewed as potentially useful for improving clinical management, increasing disease awareness, and facilitating care planning, especially end-of-life planning. Despite the widespread recognition and positive perceptions of the ENCALS survival model among Italian neurologists with expertise in ALS, its implementation in clinical practice remains limited. Addressing this disparity may require systematic investigations and targeted training to integrate tailored prognostic communication into ALS care protocols, aligning with the growing availability of prognostic tools for ALS.

Patients’ and caregivers’ perception of multidimensional and palliative care in amyotrophic lateral sclerosis – protocol of a German multicentre study

IntroductionAmyotrophic lateral sclerosis (ALS) is an inevitably fatal condition that leads to a progressive loss of physical functioning, which results in a high psychosocial burden and organizational challenges related to medical care. Multidimensional and multiprofessional care is advised to meet the complex needs of patients and their families. Many healthcare systems, including Germany, may not be able to meet these needs because non-medical services such as psychological support or social counselling are not regularly included in the care of patients with ALS (pwALS). Specialised neuropalliative care is not routinely implemented nor widely available. Caregivers of pwALS are also highly burdened, but there is still a lack of support services for them.MethodsThis project aims to assess the perceptions and satisfaction with ALS care in Germany in pwALS and their caregivers. This will be achieved by means of a cross-sectional, multicentre survey. The examination will assess, to which extend the patients’ needs in the six domains of physical, psychological, social, spiritual, practical and informational are being met by current care structures. This assessment will be linked to mental well-being, subjective quality of life, attitudes toward life-sustaining measures and physician-assisted suicide, and caregiver burden. The study aims to recruit 500 participants from nationwide ALS centres in order to draw comprehensive conclusions for Germany. A total of 29 centres, mostly acquired via the clinical and scientific German Network for Motor Neuron Diseases (MND-NET), will take part in the project, 25 of which have already started recruitment.PerspectiveIt is intended to provide data-based starting points on how current practice of care in Germany is perceived pwALS and their caregivers and how it can be improved according to their needs. Planning and initiation of the study has been completed.Trial registrationThe study is registered at ClinicalTrails.gov; NCT06418646

Current practices in the nutrition management of people with amyotrophic lateral sclerosis (ALS): a survey of U.S. ALS care teams

Objective To assess current practices of U.S. professionals providing outpatient ALS nutrition care. Methods A cross-sectional survey assessing nutrition care practices was distributed in February/March 2023 through electronic mailing lists of relevant professional organizations. Results Of the 87 professionals completing the survey, 85.1% were registered dietitians and 50.6% had five or fewer years of experience in ALS care. Many (44.2%) professionals reported receiving no training on the nutrition care of people with ALS (PALS), and 40.2% reported having no other ALS dietitians in their close network. Methods utilized to estimate calorie and protein requirements in PALS varied widely. Although 95.4% of respondents reported that their clinic’s dietitian participates in feeding tube discussions, many practitioners may be waiting until ALS symptoms negatively impact PALS’ breathing, eating, swallowing, or weight to begin discussing feeding tubes. Additionally, few professionals reported institutional practices conducive for refeeding syndrome prevention or monitoring. Conclusions Many professionals providing outpatient nutrition care to PALS possess limited experience, received insufficient training, and are not connected to other ALS dietitians. Specific nutrition care practices, including nutrient need estimation, vary widely among health professionals. Practices surrounding feeding tube discussions and refeeding syndrome may be suboptimal at many institutions. These findings highlight the need for initiatives that educate and connect practitioners providing nutrition care to PALS.

Computational screening of damaging nsSNPs in human SOD1 genes associated with amyotrophic lateral sclerosis identifies destabilising effects of G38R and G42D mutations through in silico evaluation.

Amyotrophic lateral sclerosis (ALS), a complicated neurodegenerative disorder affected by hereditary and environmental variables, is a condition. In this study, the genetic makeup of ALS is investigated, with a focus on the SOD1 gene's single-nucleotide polymorphisms (SNPs) and their ability to affect disease risk. Eleven high-risk missense variations that may impair the functionality of the SOD1 protein were discovered after a thorough examination of SNPs in the SOD1 gene. These mutations were chosen using a variety of prediction approaches, highlighting their importance in the aetiology of ALS. Notably, it was discovered that the stability of the SOD1 wild-type protein structure was compromised by the G38R and G42D SOD1 variants. Additionally, Edaravone, a possible ALS medication, showed a greater affinity for binding mutant SOD1 structures, pointing to potential personalised treatment possibilities. The high-risk SNPs discovered in this investigation seem to have functional effects, especially on the stability of proteins and their interactions with other molecules. This study clarifies the complex genetics of ALS and offers insights into how these genetic variations may affect the effectiveness of therapeutic interventions, particularly in the context of edaravone. In this study advances our knowledge of the genetic mechanisms causing ALS vulnerability and prospective therapeutic strategies. Future studies are necessary to confirm these results and close the gap between individualised clinical applications and improved ALS care.

ALS Health care provider wellness

Background Interest in health care provider (HCP) wellness and burnout is increasing; however, minimal literature explores HCP wellness in the context of Amyotrophic Lateral Sclerosis (ALS) care. Objectives We sought to determine rates of burnout and resiliency, as well as challenges and rewards in the provision of ALS care. Methods A survey link was sent to physicians at all Canadian ALS centers for distribution to ALS HCPs in their network. The survey included demographics questions, and validated measures for resiliency and burnout; the Brief Resilient Coping Scale (BRCS) and the Single Item Burnout Score (SIBS). Participants were asked to describe challenges and rewards of ALS care, impact of COVID-19 pandemic, and how their workplace could better support them. Results There were 85 respondents across multiple disciplines. The rate of burnout was 47%. Burnout for female respondents was significantly higher (p = 0.007), but not for age, role, or years in ALS clinic. Most participants were medium resilient copers n = 48 (56.5%), but resiliency was not related to burnout. Challenges included feeling helpless while patients relentlessly progressed to death, and emotionally charged interactions. Participants found fulfillment in providing care, and through relationships with patients and colleagues. There was a strongly expressed desire for increased resources, team building/debriefing, and formal training in emotional exhaustion and burnout. Conclusions The high rate of burnout and challenges of ALS care highlight the need for additional resources, team-building, and formal education around wellness.

Quality-of-life assessment instruments used across ALS clinics.

Instruments have been developed to assess quality of life (QoL) among people with amyotrophic lateral sclerosis (ALS). It is unclear whether these are utilized regularly in the clinical setting to guide individual patient care. In this study we aimed to understand the current use of instruments and existing barriers to assessing QoL in clinical ALS care. An anonymous survey developed by Northeast ALS (NEALS) Consortium Palliative Committee members was distributed to all multidisciplinary NEALS members. Data were summarized via calculation of descriptive statistics. ALS Center characteristics were compared using chi-square and Fisher exact tests for categorical variables. Seventy-three (6.4%) of the 1132 NEALS members responded to the survey, representing 148 clinics, 49.3% of whom reported assessing QoL during clinic visits. The most used ALS-specific instruments were the ALS Assessment Questionnaire (19.4%) and Amyotrophic Lateral Sclerosis Specific Quality of Life scale (16.6%). Barriers reported were uncertainty regarding which instrument to use and length of visits. QoL assessment was not significantly correlated with length of clinic visit but with access to specialty palliative care. QoL assessments are performed by some, but not all, ALS centers during clinical visits. Although this study did have a low number of responding centers, the percentage, the proportion is similar to that seen in earlier studies, which limits the findings' generalizability. The value of QoL assessments' impact on outcomes should be further investigated and, if warranted, creative ways sought to increase the frequency of their use, including patient self-assessments before clinic and/or the use of teleheath to reduce the length of clinic visits.

Palliative Care in Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease of the motor neurons. Given the evolutive characteristics of this disease, palliative care principles should be a foundation of ALS care. A multidisciplinary medical intervention is of paramount importance in the different phases of disease. The involvement of the palliative care team improves quality of life and symptoms, and prognosis. Early initiation is of paramount importance to ensuring patient-centered care, when the patient has still the capability to communicate effectively and participate in his medical care. Advance care planning supports patients and family members in understanding and sharing their preferences according to their personal values and life goals regarding future medical treatment. The principal problems which require intensive supportive care include cognitive disturbances, psychological distress, pain, sialorrhrea, nutrition, and ventilatory support. Communication skills of health-care professionals are mandatory to manage the inevitability of death. Palliative sedation has peculiar aspects in this population, particularly with the decision of withdrawing ventilatory support.

Epidemiologic Trends of Amyotrophic Lateral Sclerosis in Ireland, 1996-2021.

The objective of this study was to examine changes to the incidence, prevalence, age at onset, and survival of patients diagnosed with amyotrophic lateral sclerosis (ALS) in the Republic of Ireland over 25 years. Incident and prevalent cases of ALS were estimated using the Irish population-based ALS Register, which has been in continuous operation since 1994. Incident cases were age standardized using the direct method and applied to 3 standard populations (Irish, European, and American). Survival was determined using Kaplan-Meier curves and Cox regression models. Non-normally distributed groups were compared using the Kruskal-Wallis test with a Bonferroni correction. A total of 2,771 patients with ALS were identified in the Republic of Ireland over 25 years. Incidence per 100,000 was determined for the population older than 15 years. Crude incidence increased from 2.64 to 5.46 per 100,000. Standardized incidence increased from 2.64 to 3.1 per 100,000. Prevalence increased from 5.83 to 8.10 per 100,000. The median age at onset increased from 64 to 67 years. The peak age of incidence increased from those between 70 and 74 years to those between 75 and 79 years. Overall, women had a consistently later median age at onset of 67 years compared with men at 65 years (p < 0.001). No significant difference in survival was noted between those captured across 3 different epochs (1996-2003, 2004-2012, 2013-2021). Older age at onset (hazard ratio [HR] 1.03, CI 1.02-1.04, p < 0.001) was a negative predictive factor of survival in multivariate Cox regression analysis. Riluzole use (HR 0.67, CI 0.50-0.90, p = 0.033) and diagnostic delay (HR 0.98, CI 0.98-0.99, p < 0.001) were positive predictive factors. Within the Republic of Ireland, the age-standardized overall incidence, peak incidence, prevalence, and age at onset of ALS have all increased over 25 years. Despite the widespread use of noninvasive ventilation, aggressive secretion management, and changes in ALS care, the mean survival within the Irish population has not changed.

Accessing and Receiving Speech-Language Pathology Services at the Multidisciplinary Amyotrophic Lateral Sclerosis Clinic: An Exploratory Qualitative Study of Patient Experiences and Needs.

This study sought to explore how patients with amyotrophic lateral sclerosis (ALS) presenting with coexisting bulbar and cognitive impairments and their caregivers experienced the speech-language pathologist (SLP) services provided in multidisciplinary ALS clinics in Canada and identified their perceived needs for bulbar symptom management. This qualitative study was informed by interpretive description. Seven interviews were conducted with patients with severe bulbar dysfunction or severe bulbar and cognitive dysfunction due to ALS or ALS-frontotemporal dementia, respectively, and/or their caregivers. Purposive sampling was used to recruit individuals with severe bulbar or bulbar and cognitive disease. Thematic analysis was used to analyze interview data. Patients and caregivers reported difficulties with accessing and receiving SLP services at the multidisciplinary ALS clinic. These difficulties were further exacerbated in those with severe cognitive disease. Participants expressed a need for more specific (i.e., disease and service-related) information and personalized care to address their changing needs and preferences. Engaging caregivers earlier in SLP appointments was perceived as vital to support care planning and provide in-time caregiver education. This study highlighted the challenges experienced by patients and caregivers in accessing and receiving SLP services. There is a pressing need for a more person-centered approach to ALS care and a continuing need for education of SLPs on care provision in cases of complex multisymptom diseases within a multidisciplinary ALS clinic. https://doi.org/10.23641/asha.24069222.

The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis.

To highlight the complexities surrounding respiratory care in ALS.To alert clinicians to the risk that complexity of ALS care may modify the effectiveness of any specific, evidence-based therapy for ALS.To describe the importance of person-centred care and shared decision-making in optimising care in ALS.

Withdrawal of mechanical ventilation in amyotrophic lateral sclerosis patients: a multicenter Italian survey

BackgroundLaw 219/2017 was approved in Italy in December 2017, after a years-long debate on the autonomy of healthcare choices. This Law, for the first time in Italian legislation, guarantees the patient’s right to request for withdrawal of life-sustaining treatments, including mechanical ventilation (MV).ObjectiveTo investigate the current status of MV withdrawal in amyotrophic lateral sclerosis (ALS) patients in Italy and to assess the impact of Law 219/2017 on this practice.MethodsWe conducted a Web-based survey, addressed to Italian neurologists with expertise in ALS care, and members of the Motor Neuron Disease Study Group of the Italian Society of Neurology.ResultsOut of 40 ALS Italian centers, 34 (85.0%) responded to the survey. Law 219/2017 was followed by an increasing trend in MV withdrawals, and a significant increase of neurologists involved in this procedure (p 0.004). However, variations across Italian ALS centers were observed, regarding the inconsistent involvement of community health services and palliative care (PC) services, and the intervention and composition of the multidisciplinary team.ConclusionsLaw 219/2017 has had a positive impact on the practice of MV withdrawal in ALS patients in Italy. The recent growing public attention on end-of-life care choices, along with the cultural and social changes in Italy, requires further regulatory frameworks that strengthen tools for self-determination, increased investment of resources in community and PC health services, and practical recommendations and guidelines for health workers involved.

Iranian clinical practice guideline for amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegeneration involving motor neurons. The 3-5 years that patients have to live is marked by day-to-day loss of motor and sometimes cognitive abilities. Enormous amounts of healthcare services and resources are necessary to support patients and their caregivers during this relatively short but burdensome journey. Organization and management of these resources need to best meet patients' expectations and health system efficiency mandates. This can only occur in the setting of multidisciplinary ALS clinics which are known as the gold standard of ALS care worldwide. To introduce this standard to the care of Iranian ALS patients, which is an inevitable quality milestone, a national ALS clinical practice guideline is the necessary first step. The National ALS guideline will serve as the knowledge base for the development of local clinical pathways to guide patient journeys in multidisciplinary ALS clinics. To this end, we gathered a team of national neuromuscular experts as well as experts in related specialties necessary for delivering multidisciplinary care to ALS patients to develop the Iranian ALS clinical practice guideline. Clinical questions were prepared in the Patient, Intervention, Comparison, and Outcome (PICO) format to serve as a guide for the literature search. Considering the lack of adequate national/local studies at this time, a consensus-based approach was taken to evaluate the quality of the retrieved evidence and summarize recommendations.

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.

The economic evaluation of ALS care: quality and cost

Objective: The study aims to analyze the quality of studies that make economic evaluations for amyotrophic lateral sclerosis (ALS). Assessing the quality of studies can guide policy-making and planning. Methods: One of the most recognized checklists “The Consensus on Health Economic Criteria” (CHEC)-list designed by Evers et al. in 2005 aims to answer two important questions: is the methodology of the study appropriate, and are the results of the study valid? We reviewed studies focusing on ALS and its economic costs, and evaluated the studies with (CHEC)-list. Results: We examined 25 articles in terms of their cost evaluation and quality. It is seen that they mainly focus on medical costs, ignoring social care costs. When the quality of the studies is examined, it is seen that the studies overall achieve high scores in terms of their purpose and research question, but some of the studies score low in terms of ethical dimension, comprehensiveness of expenditure items, their application of sensitivity analyses and their study design. Conclusions: The main recommendation of our study for future cost evaluation studies is that they should focus on the questions in the checklist that are scored low overall by the 25 articles, and consider the social care costs as well as medical costs. Our recommendations when designing cost studies can be applied to other chronic diseases with long-term economic costs like ALS.

Pulmonary care for ALS: Progress, gaps, and paths forward.

Adults with amyotrophic lateral sclerosis (ALS) have been using home mechanical ventilation for over 50 years. More recently, home respiratory care has evolved to include portable home ventilators, airway clearance devices, and physiological assessments with telemonitoring capability. National organizations currently offer incentives for providing a pulmonary care specialist within a multidisciplinary ALS clinic; however, several critical gaps exist between the available technology and employing a clinician with the necessary expertise. Lack of formal training and poor financial incentives have led to a paucity of both clinicians and active clinical research engaging in the home respiratory care of ALS. Criteria for noninvasive ventilation (NIV) initiation are controversial, and few guidelines exist on the ideal subsequent adjustments of NIV with evolving disease. Consequently, many patients with ALS tolerate NIV poorly and must face the harrowing decision of hospice vs tracheostomy. Advancement of respiratory care in ALS has been hindered by critical gaps in pulmonologist availability, training in chronic respiratory failure, financial support, clinical research, and clarity on ventilation management beyond initiation. Only a multifaceted approach will suffice for addressing the voids in ALS respiratory care, including various education initiatives, financial incentives, clinical research programs, and elevating the standard of respiratory care.

"Revolutionizing Amyotrophic Lateral Sclerosis (ALS) Management: A Case Report on Muscle Regeneration and Sustained Relaxation through Placental Injection Therapy at Rodem Hospital"

This case report from the Rodem Hospital introduces a pioneering intervention for muscle rigidity in Amyotrophic Lateral Sclerosis (ALS), featuring a unique placental extract injection and glucose injection therapy combined with lidocaine. This novel approach has demonstrated significant muscle regeneration and sustained relaxation in 47 ALS patients. Unlike traditional treatments, this protocol offers a more sustainable and regenerative outcome. The treatment involved injections of a mixture containing glucose, lidocaine, and placental extract, targeting severely rigid muscles. Remarkably, 42 of the 47 patients showed considerable improvements in knee flexion and a dramatic reduction in pain. The other two also experienced notable progress. This method stands out for its cost efficiency, impact on muscle suppleness, and reduced pain, suggesting a potential paradigm shift in ALS management. This case series highlights the importance of continued innovation and personalized treatment strategies in ALS care, aiming to improve patient quality of life and functional abilities. This case report from the Rodem Hospital introduces a pioneering intervention for muscle rigidity in Amyotrophic Lateral Sclerosis (ALS), featuring a unique placental extract injection and glucose injection therapy combined with lidocaine. This novel approach has demonstrated significant muscle regeneration and sustained relaxation in 47 ALS patients. Unlike traditional treatments, this protocol offers a more sustainable and regenerative outcome. The treatment involved injections of a mixture containing glucose, lidocaine, and placental extract, targeting severely rigid muscles. Remarkably, 42 of the 47 patients showed considerable improvements in knee flexion and a dramatic reduction in pain. The other two also experienced notable progress. This method stands out for its cost efficiency, impact on muscle suppleness, and reduced pain, suggesting a potential paradigm shift in ALS management. This case series highlights the importance of continued innovation and personalized treatment strategies in ALS care, aiming to improve patient quality of life and functional abilities.

Amyotrophic lateral sclerosis (ALS) health charities are central to ALS care: perspectives of Canadians affected by ALS

Objective Expert consensus guidelines recommend referral of people with amyotrophic lateral sclerosis (ALS) to ALS health charities for support. Limited research indicates that patients and families value interaction with these volunteer sector organizations. We investigated how patient support from Canadian ALS health charities (ALS Societies) is experienced by those affected by ALS, and whether patient-centered outcomes validate recommendations for referral. Methods Data were drawn from the ALS Talk Project, an asynchronous online focus group study. Patients and family caregivers were recruited from regions across Canada. Seven groups met online for 14 weeks between January and July 2020. Seventy-eight participants made statements about ALS Societies. Data were qualitatively analyzed using directed content analysis and the constant-comparative approach. Results Participants viewed ALS Societies as integral to the healthcare system. The Societies acted as patient navigators and filled perceived care gaps, including psychological support. They provided critical practical assistance, particularly equipment loans and peer support groups; comprehensive disease-related and real-life information; and personal connections. They facilitated knowledge of research, emerging therapies, and research opportunities. Delayed referral to ALS Society supports and information resources was a concern for some participants. Conclusions ALS Societies provide patients with critical practical, informational, and emotional support and play an overarching role as patient/research navigators. Patient-centred outcomes support patient referral to ALS Societies. Communication about the services provided should be a standard component of clinical care, with choice of access left to individuals. Clinical conversations should be supplemented with information resources developed by these voluntary sector organizations.

Impact of the covid-19 pandemic on amyotrophic lateral sclerosis care in the UK

The Covid-19 pandemic has impacted healthcare. Our aim was to identify how amyotrophic lateral sclerosis (ALS) care in the UK has been affected by the pandemic by exploring the experiences of people living with ALS (plwALS), healthcare professionals (HCPs) working with plwALS, and ALS care centers. Three surveys were carried out to explore the experiences of plwALS, HCPs and ALS care centers during the pandemic. Quantitative data were analyzed using descriptive and inferential statistics and triangulated with the qualitative data which were analyzed thematically. Responses from 53 plwALS, 73 HCPs and 23 ALS care centers were analyzed. Five main themes were identified: keeping safe, losses, negative emotions, delivering care and alternative care delivery in a pandemic. PlwALS and HCPs felt that care was sub-optimal as a result of the pandemic. Changes to care included longer waiting times and face-to-face appointments being canceled or replaced by virtual consultations. While benefits of virtual consultations were reported, concerns were raised about incomplete clinical assessments and the disruption of provision of testing and interventions. ALS care has changed as a result of the pandemic. Patients have had a lack of face-to-face contact with HCPs and have experienced delays to investigations and treatments. PlwALS and HCPs were concerned about the impact of this change, but the long-term implications remain unclear. We propose recommendations for HCPs caring for plwALS, that will promote continuity of evidenced based care in the context of a pandemic.

Caregivers' View of Socio-Medical Care in the Terminal Phase of Amyotrophic Lateral Sclerosis-How Can We Improve Holistic Care in ALS?

Multidimensional socio-medical care with an early integration of palliative principles is strongly recommended in amyotrophic lateral sclerosis (ALS), but provided inconsistently. We conducted telephone interviews with 49 former caregivers of deceased ALS patients to examine their experience of care in the terminal phase including caregiver burden. Patients who received specialized palliative care (45% of patients) were more likely to die at home (p = 0.004) and without burdening symptoms (p = 0.021). The majority of caregivers (86%) reported deficits in socio-medical care. Most frequently mentioned were problems receiving medical aids (45%) and a lack of caregiver support (35%). A higher level of deficits experienced by caregivers was associated with negative health outcomes on the side of the caregivers (reported by 57% of them; p = 0.002) and stronger caregiver burden (p = 0.004). To provide good quality of dying to patients and reduce the burden on caregivers, multidimensional—including palliative—care in ALS urgently needs to be strengthened in the healthcare structures.