Je me sens en grande santé. Pourtant, quand le diagnostic de SLA est posé, toute ma vie est modifiée. Mon corps, lentement, me trahit. Par la musique, la littérature et la pratique de la peinture, j'ai trouvé une nouvelle façon de vivre. Bob Dylan, Kafka... Je replonge dans mes souvenirs de jeunesse, les confronte à l'ici et maintenant. J'ai décidé de faire un bon usage de ma maladie.
Background: despite being a terminal neurodegenerative disease, the role of palliative care is less recognised for motor neurone disease than for other life-limiting conditions. Understanding the experiences of, and need for, palliative care for patients and carers is key to configuring optimal policy and healthcare services.
Aim: To explore the experiences of, and need for, palliative care of people with motor neurone disease and their informal carers across the disease trajectory.
Design: A systematic review of qualitative research conducted using Thematic Synthesis – PROSPERO registration CRD42017075311.
Data Sources: four electronic databases were searched (MEDLINE, CINAHL, PsycINFO, Social Science Citation Index) using terms for motor neurone disease, amyotrophic lateral sclerosis, palliative care, and qualitative research, from inception to November 2018. Included papers were data extracted and assessed for quality.
Results: A total of 41 papers were included, representing the experiences of 358 people with motor neurone disease and 369 caregivers. Analytical themes were developed detailing patients’ and carers’ experiences of living with motor neurone disease and of palliative care through its trajectory including response to diagnosis, maintaining control, decision-making during deterioration, engaging with professionals, planning for end-of-life care, bereavement.
Conclusion: The review identified a considerable literature exploring the care needs of people with motor neurone disease and their carers; however, descriptions of palliative care were associated with the last days of life. Across the disease trajectory, clear points were identified where palliative care input could enhance patient and carer experience of the disease, particularly at times of significant physical change.
BACKGROUND: A developing body of evidence has provided valuable insight into the experiences of caregivers of people with motor neuron disease; however, understandings of how best to support caregivers remain limited.
AIM: This study sought to understand concepts related to the motor neuron disease caregiver experience which could inform the development of supportive interventions.
DESIGN: A qualitative thematic analysis of a one-off semistructured interview with caregivers was undertaken.
SETTING/PARTICIPANTS: Caregivers of people with motor neuron disease were recruited from a progressive neurological diseases clinic in Melbourne, Australia.
RESULTS: 15 caregivers participated. Three key themes were identified: (1) The Thief: the experience of loss and grief across varied facets of life; (2) The Labyrinth: finding ways to address ever changing challenges as the disease progressed; (3) Defying fate: being resilient and hopeful as caregivers tried to make the most of the time remaining.
CONCLUSIONS: Caregivers are in need of more guidance and support to cope with experiences of loss and to adapt to changeable care giving duties associated with disease progression. Therapeutic interventions which target these experiences of loss and change are worth investigation.
TRIAL REGISTRATION NUMBER: ACTRN12615000120572, pre-results.
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative disorder with enormous palliative care (PC) needs that begin at the time of diagnosis. Although it is an uncommon disease, clinicians who work in PC or hospice are likely to encounter ALS somewhat frequently given the needs of patients with ALS with regard to psychosocial support, symptom management, advance care planning (ACP), caregiver support, and end-of-life care. As such, PC clinicians should be familiar with the basic principles of ALS symptoms, treatments, disease course, and issues around ACP. This article, written by a team of neurologists and PC physicians, seeks to provide PC clinicians with tips to improve their comfort and skills caring for patients with ALS and their families.
AIMS AND OBJECTIVES: This study explores everyday life experience of relatives of people with ALS living at home with mechanical ventilation and formal caregivers.
BACKGROUND: ALS is a rapidly progressive disease affecting not only the patient but also close relatives. A burden is placed on relatives affecting their mental and physical health in settings where they provide care. Few studies have examined the everyday life challenges of close relatives with formal caregivers at home and home mechanical ventilation, which often prolongs survival.
DESIGN: We undertook a qualitative study with a phenomenological-hermeneutic approach inspired by Ricoeur. Eleven close relatives were interviewed and the three-level analysis method developed by Dreyer and Pedersen was conducted. The Consolidated Criteria for Reporting Qualitative Research checklist was used.
RESULTS: Four themes were derived from the data during analysis: Vulnerable relatives fighting to keep track of everything, Formal caregivers - a distressing relief, A prison without personal space, and We are in this together until the end.
CONCLUSIONS: Close relatives of persons with ALS are burdened with everyday life challenges despite having formal caregivers at home, and they feel imprisoned. Despite these challenges and concerns about the end of life of their relative, they stay until the end.
RELEVANCE TO CLINICAL PRACTICE: There is clearly a need to investigate interventions in clinical practice supporting close relatives to prepare them for what to expect during a prolonged disease course.
PURPOSE: To describe key stakeholders' perspectives on specialist palliative care and its integration into the management of amyotrophic lateral sclerosis (ALS).
METHODS: The study conducted was a qualitative, noninterventional, descriptive study. Data were collected via individual interviews of 42 stakeholders (n = 14 patients, n = 16 family caregivers, and n = 12 health-care providers). Transcribed interviews were analyzed using inductive thematic analysis techniques.
RESULTS: Stakeholders' general impressions of specialist palliative care were highly variable. Many expressed limited or inaccurate understandings of palliative care's definition and purpose. Perceptions of palliative care as hospice were common. Stakeholders generally supported the integration of specialist palliative care into ALS management, and many recognized the value of early integration of palliative services in both the community and the clinical setting.
CONCLUSION: Key stakeholders readily identified a meaningful role for specialist palliative care in ALS management. Integration of specialist palliative care into existing systems of support would be facilitated by a more comprehensive understanding of the service among patients, family caregivers, and health-care providers.
A major unresolved problem in neurodegenerative disease is why and how a specific set of neurons in the brain are highly vulnerable to neuronal death. Multiple pathways and mechanisms have been proposed to play a role in Alzheimer disease (AD), Parkinson disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington disease (HD), yet how they contribute to neuronal vulnerability remains far from clear. In this review, various mechanisms ascribed in AD, PD, ALS, and HD will be briefly summarized. Particular focus will be placed on Rhes-mediated intercellular transport of the HD protein and its role in mitophagy, in which I will discuss some intriguing observations that I apply to model striatal vulnerability in HD. I may have unintentionally missed referring some studies in this review, and I extend my apologies to the authors in those circumstances.
Palliative care is an approach to the care of patients, affected by serious illness, and their families that aims to reduce suffering through the management of medical symptoms, psychosocial issues, spiritual well-being, and setting goals of care. Patients and families affected by a neurodegenerative illness have significant palliative care needs beginning at the time of diagnosis and extending through end-of-life care and bereavement. We advocate an approach to addressing these needs where the patient's primary care provider or neurologist plays a central role. Key skills in providing effective palliative care to this population include providing the diagnosis with compassion, setting goals of care, anticipating safety concerns, caregiver assessment, advance care planning, addressing psychosocial concerns, and timely referral to a hospice. Managing distressing medical and psychiatric symptoms is critical to improving quality of life throughout the disease course as well as at end-of-life. Many symptoms are common across illnesses; however, there are issues that are specific to the most common classes of neurodegenerative illness, namely dementia, parkinsonism, and motor neuron disease. Incorporating a palliative approach to care, although challenging in many ways, empowers physicians to provide greater support and guidance to patients and families in making the difficult journey through a neurodegenerative illness.
The psychological phenotype in amyotrophic lateral sclerosis (ALS) is less negative than in other neurodegenerative diseases, manifested by a lower prevalence of psychopathology, such as anxiety and major depression, and a higher perceived quality of life by patients, irrespective of physical impairment. We hypothesized that positive psychological factors such as hope, optimism, and self-efficacy in people with ALS (PALS) were key determinants of satisfaction with life (SWL), despite physical impairment, and were protective against psychopathology. Forty PALS, at different functional levels, completed objective questionnaires to evaluate psychological factors of hope, optimism, self-efficacy, and SWL. Approximately 41% of the variance in SWL was accounted for by the Agency factor of hope. The results indicated that SWL was significantly correlated to specific positive psychological factors of hope and self-efficacy. Physical impairment was not correlated with positive psychological factors or SWL. These results support the role of hope and self-efficacy in maintaining satisfaction with life in PALS and consideration of these potentially modifiable factors could improve palliative therapy.
OBJECTIVE: To describe health care service utilization and cost for decedents with and without amyotrophic lateral sclerosis (ALS) in the last year of life.
METHODS: Using linked health administrative data, we conducted a retrospective, population-based cohort study of Ontario, Canada, decedents from 2013 to 2015. We examined demographic data, rate of utilization, and cost of health care services in the last year of life.
RESULTS: We identified 283,096 decedents in Ontario, of whom 1,212 (0.42%) had ALS. Decedents with ALS spent 3 times as many days in an intensive care unit (ICU) (mean 6.3 vs 2.1, p < 0.001), and twice as many days using complex continuing care (mean 12.7 vs 6.0, p < 0.001) and home care (mean 99.1 vs 41.3, p < 0.001). A greater percentage of decedents with ALS received palliative home care (44% vs 20%, p < 0.001) and palliative physician home visits (40% vs 18%, p < 0.001) than decedents without ALS. Among decedents with ALS, a palliative physician home visit in the last year of life was associated with reduced adjusted odds of dying in hospital (odds ratio 0.65, 95% confidence interval 0.48-0.89) and fewer days spent in the ICU. Mean cost of care in the last year of life was greater for those with ALS ($68,311.98 vs $55,773.48, p < 0.001).
CONCLUSIONS: In this large population-based cohort of decedents, individuals with ALS spent more days in the ICU, received more community-based services, and incurred higher costs of care in the last year of life. A palliative care physician home visit was associated with improved end of life outcomes; however, the majority of patients with ALS did not access such services.
PURPOSE OF REVIEW: In this review, we summarize the recently published literature that demonstrates the efficacy and safety of autologous haematopoietic stem cell transplantation (AHSCT) in multiple sclerosis (MS) and highlight the importance of supportive care required for the safe and well-tolerated delivery of AHSCT.
RECENT FINDINGS: MS is an autoimmune inflammatory and degenerative disorder of the central nervous system (CNS). In the majority of patients, the illness runs a relapsing remitting course (RRMS), culminating in a secondary progressive phase with gradual accumulation of fixed disabilities. Currently available disease-modifying therapies suppress CNS inflammation but have a limited effect on preventing disease progression for which there remains no effective therapy. Over the last two decades, there has been increasing evidence that AHSCT is a highly effective therapeutic strategy for treatment-resistant inflammatory types of MS, especially RRMS. Concerns about the safety of AHSCT in MS, usually a nonlife-threatening disease, have previously limited its use. However, AHSCT can now be delivered safely with major long-term benefits because of increasing transplant centre experience, judicious patient selection and good supportive care.
SUMMARY: MS is currently the fastest growing indication for AHSCT in Europe. Supportive care before, during and after the transplant period is key to its successful delivery of AHSCT.
BACKGROUND: People with multiple sclerosis (MS) have complex symptoms and different types of needs. These demands include how to manage the burden of physical disability as well as how to organise daily life, restructure social roles in the family and at work, preserve personal identity and community roles, keep self-sufficiency in personal care, and how to be part of an integrated care network. Palliative care teams are trained to keep open full and competent lines of communication about symptoms and disease progression, advanced care planning, and end-of-life issues and wishes. Teams create a treatment plan for the total management of symptoms, supporting people and families on decision-making. Despite advances in research and the existence of many interventions to reduce disease activity or to slow the progression of MS, this condition remains a life-limiting disease with symptoms that impact negatively the lives of people with it and their families.
OBJECTIVES: To assess the effects (benefits and harms) of palliative care interventions compared to usual care for people with any form of multiple sclerosis: relapsing-remitting MS (RRMS), secondary-progressive MS (SPMS), primary-progressive MS (PPMS), and progressive-relapsing MS (PRMS) We also aimed to compare the effects of different palliative care interventions.
SEARCH METHODS: On 31 October 2018, we conducted a literature search in the specialised register of the Cochrane MS and Rare Diseases of the Central Nervous System Review Group, which contains trials from CENTRAL, MEDLINE, Embase, CINAHL, LILACS, Clinical trials.gov and the World Health Organization International Clinical Trials Registry Platform. We also searched PsycINFO, PEDro and Opengrey. We also handsearched relevant journals and screened the reference lists of published reviews. We contacted researchers in palliative care and multiple sclerosis.
SELECTION CRITERIA: Randomised controlled trials (RCTs) and cluster randomised trials were eligible for inclusion, as well as the first phase of cross-over trials. We included studies that compared palliative care interventions versus usual care. We also included studies that compared palliative care interventions versus another type of palliative interventions.
DATA COLLECTION AND ANALYSIS: We used standard Cochrane methodological procedures. We summarised key results and certainty of evidence in a 'Summary of Finding' table that reported outcomes at six or more months of post-intervention.
MAIN RESULTS: Three studies (146 participants) met our selection criteria. Two studies compared multidisciplinary, fast-track palliative care versus multidisciplinary standard care while on a waiting-list control, and one study compared a multidisciplinary palliative approach versus multidisciplinary standard care at different time points (12, 16, and 24 weeks). Two were RCTs with parallel design (total 94 participants) and one was a cross-over design (52 participants). The three studies assessed palliative care as a home-based intervention. One of the three studies included participants with 'neurodegenerative diseases', with MS people being a subset of the randomised population. We assessed the risk of bias of included studies using Cochrane's 'Risk of Bias' tool. We found no evidence of differences between intervention and control groups in long-time follow-up (> six months post-intervention) for the following outcomes: mean change in health-related quality of life (SEIQoL - higher scores mean better quality of life; MD 4.80, 95% CI -12.32 to 21.92; participants = 62; studies = 1; very low-certainty evidence), serious adverse events (RR 0.97, 95% CI 0.44 to 2.12; participants = 76; studies = 1, 22 events, low-certainty evidence) and hospital admission (RR 0.78, 95% CI 0.24 to 2.52; participants = 76; studies = 1, 10 events, low-certainty evidence).The three included studies did not assess the following outcomes at long term follow-up (> six months post intervention): fatigue, anxiety, depression, disability, cognitive function, relapse-free survival, and sustained progression-free survival.We did not find any trial that compared different types of palliative care with each other.
AUTHORS' CONCLUSIONS: Based on the findings of the RCTs included in this review, we are uncertain whether palliative care interventions are beneficial for people with MS. There is low- or very low-certainty evidence regarding the difference between palliative care interventions versus usual care for long-term health-related quality of life, adverse events, and hospital admission in patients with MS. For intermediate-term follow-up, we are also uncertain about the effects of palliative care for the outcomes: health-related quality of life (measured by different assessments: SEIQoL or MSIS), disability, anxiety, and depression.
Stress is a commonly reported concern of individuals with chronical diseases, including multiple sclerosis (MS). This study sought to investigate the relationships between self-transcendence, death anxiety, and perceived stress among individuals with MS from Iran. A second aim of the study was to assess the buffering effect of self-transcendence in the relationship between death anxiety and perceived stress. Two hundred and fifteen participants with MS from four hospitals completed measures assessing self-transcendence, death anxiety, and perceived stress. Using structural equation modeling, death anxiety was found to be positively related to perceived stress. In addition, there was a negative relationship between self-transcendence and perceived stress. Results of the study suggest that self-transcendence is a buffer in the link between death anxiety and perceived stress for individuals with MS. The findings demonstrate the importance of self-transcendence in decreasing the effects of death anxiety on perceived stress and have clinical implications for health professionals.
Cette étude présente un patient atteint d’une sclérose latérale amyotrophique (SLA) bulbaire en phase palliative symptomatique. Le Projet de Vie du patient était de participer à un hommage sportif organisé en son honneur dans les 10 jours. Lors de l’hospitalisation, le patient a présenté une détresse respiratoire aiguë sur pneumonie impliquant une démarche décisionnelle collégiale. L’adaptation des interventions thérapeutiques a permis de réaliser le Projet de Vie malgré une fin de vie proche. Le décès est survenu deux jours après l’hommage. Ce cas clinique permet d’illustrer l’interdisciplinarité au sein des équipes, et de discuter l’intrication synergique entre technique et relation.
This paper recounts the author's conversations with Ryan Farnsworth, a 30-year-old ALS patient who consented to be interviewed for the purpose of improving communication between physicians and patients. Under the California End of Life Option (ELOA), the patient had been prescribed medication that would allow him to end his life at a time of his choosing. He describes coping with the challenges of the illness, how he will make the decision when to take the drugs and what he hopes will be his legacy.
BACKGROUND: ALS is an incurable neurodegenerative disorder, with the recommendation that symptom management and palliative care start immediately or soon after diagnosis. However, little is known about healthcare utilization at the end of life in this patient group.
AIM: To describe healthcare utilization at the end of life in patients who died from ALS.
DESIGN: We performed a retrospective cohort study using population-level administrative databases. The description of healthcare utilization was based on (1) validated quality indicators for end-of-life care, and (2) the European Federation of Neurological Societies guidelines on the clinical management of ALS.
SETTING: We included all people who died from ALS in Belgium between 2010 and 2015 (using ICD-10 code G12.2).
RESULTS: 1636 people died from ALS in Belgium between 2010 and 2015. The mean age at death was 71 years (SD11.3), and 56% were men. Specialized palliative care was used by 44% at some point in the last two years of life. In the last month of life, 13% received tube feeding, 48% received diagnostic testing, 41% were admitted to a hospital, and 25% were admitted to an emergency department. Medications were used mainly to treat pain (43%), insomnia and fatigue (33%) and thrombosis (32%); 39% used riluzole. Non-invasive ventilation was used by 18%. 39% died at home.
CONCLUSION: Administrative data provide a valuable source to describe healthcare utilization in small populations such as ALS, but more clinical evidence is needed on the advantages and disadvantages initiating or terminating treatments at the end of life.
OBJECTIVE: This is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme-Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com).
METHODS: In this cross-sectional study, patients with ALS in LIS (n = 19) were interviewed on well-being (quality of life, depression) as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments (ventilation, percutaneous endoscopic gastroscopy) and hastened death. Also, clinical data were recorded (ALS Functional Rating Scale-revised version). Standardized questionnaires (Anamnestic Comparative Self-Assessment [ACSA], Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), ALS Depression Inventory-12 items [ADI-12], schedule of attitudes toward hastened death [SAHD], Motor Neuron Disease Coping Scale) were used, which were digitally transcribed; answers were provided via eye-tracking control. In addition, caregivers were asked to judge patients' well-being.
RESULTS: The majority of patients had an ACSA score >0 and a SEIQoL score >50% (indicating positive quality of life) and ADI-12 <29 (indicating no clinically relevant depression). Physical function did not reflect subjective well-being; even more, those with no residual physical function had a positive well-being. All patients would again choose the life-sustaining techniques they currently used and their wish for hastened death was low (SAHD <10). Caregivers significantly underestimated patient's well-being.
INTERPRETATION: Some patients with ALS in LIS maintain a high sense of well-being despite severe physical restrictions. They are content with their life-sustaining treatments and have a strong will to live, which both may be underestimated by their families and public opinion.
Non-invasive ventilation (NIV) has become an important cornerstone of symptomatic treatment in amyotrophic lateral sclerosis (ALS), improving survival and quality of life. In this review, we summarize the most important recent developments and insights, including evidence of efficacy, indication criteria and time of initiation, ventilation parameters and adaptation strategies, treatment of complicating factors, transition from NIV to invasive ventilation, termination of NIV and end-of-life management. Recent publications have questioned former conventions and guideline recommendations, especially with regard to timing and prognostic factors; therefore, a fresh look and re-evaluation of current evidence is needed.