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.
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.
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.
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.
Background: The absence of curative medication for amyotrophic lateral sclerosis (ALS) makes palliative care and understanding quality of life (QoL) in ALS a clinical priority. Previous qualitative research has explored the concept of QoL in terms of illness impact on life perspectives and sense of self.
Objective: In this research, we explored 'concerns' - one of the four aspects in the World Health Organisation's conceptualisation of QoL - towards adding to the literature.
Methods: In-depth interviews with 26 individuals with ALS were subjected to thematic analysis involving both inductive and deductive approaches to explore participant's concerns, and to evaluate the relevance of their concerns for understanding QoL in ALS.
Findings: The analysis showed that concerns for significant others contribute to participant's QoL because of their existential value. It was important for participants to minimise the impact of limitations and burdens associated with ALS on significant others, even at a cost to self.
Discussion: The current study supports a holistic approach in service provision, ensuring the inclusion of relevant significant others. It is further suggested that clinicians explore the specifics of burdens perceived by patients in order to support them in minimising the burdens for their significant others.
Un témoignage de résilience et de courage d'un homme atteint de la maladie de Charcot, syndrome neurodégénératif également appelé sclérose latérale amyotrophique (SLA). Lorsque lui est annoncé qu'il n'a plus que trois ans à vivre, l'auteur, déployant des ressources inespérées, décide de se battre et de continuer à vivre pour ses proches et pour lui-même malgré sa tétraplégie.
Hope is an important factor in coping and adjusting to life-threatening disease. In this study, we examined the meaning of hope among people with amyotrophic lateral sclerosis (ALS). Semi-structured interviews with 12 people revealed that hope had two contradictory meanings. For some, hope was considered an obstacle to achieving control and to actively cope with the disease. Alternatively, other people with ALS regarded hope as a crucial resource, which empowered their coping and control. These findings are discussed in the context of research and theoretical literature about hope and coping processes.
BACKGROUND: There is increasing awareness of the need for an integrated palliative care approach in chronic progressive neurological diseases. Advance care planning (ACP) is an integral part of this approach. As a systematically organized and ongoing communication process about patients' values, goals and preferences regarding medical care during serious and chronic illness, ACP aims to involve patients in decision-making before they become cognitively and communicatively incapable. However, it remains underutilized in daily neurological practice except for speciality clinics such as ALS centers. Our aim was to study ACP in the tertiary ALS center Amsterdam and to investigate patients' reflections on it. Subsequently we used this knowledge to formulate recommendations for integration of ACP in the care of patients with other chronic progressive neurological diseases.
METHODS: Non-participating observations of all appointments of patients with amyotrophic lateral sclerosis (ALS) or progressive muscular atrophy (PMA) with the treating physician, in various stages of disease, during 6 consecutive months, followed by single in-depth interviews, and an inductive analysis.
RESULTS: Twenty-eight Dutch patients participated, varying in age, gender, disease onset and severity of physical decline. ACP started directly when the diagnosis was given, by means of a general outlook on the future with progressive disability and immediate introduction to a customized multidisciplinary team. During follow-up ACP was realized by regular appointments in which monitoring of the patient's status and clear communication strategies formed the basis of tailor-made discussions on treatment options. Patients accepted this policy as careful professional guidance.
CONCLUSIONS: ACP is a professional communication process throughout the whole course of progressive disease. It is feasible to integrate ACP into follow-up of patients with ALS and PMA from diagnosis onwards. Supported by recent literature, we argue that such a well-structured approach would also enhance the quality of care and life of patients with other chronic progressive neurological diseases.
Integration of a Palliative Care Specialist in ALS Clinic: Observations from one Center Palliative care specialists can aid in the care of patients with amyotrophic lateral sclerosis (ALS). In this article, we describe our one-year experience incorporating a palliative care specialist into the ALS multidisciplinary team. We describe our integration model, patient selection, and visit content. Of 500 total clinic patients, 74 (14.8%) were seen by the palliative care specialist in one year. Referral was most often triggered by advance care planning needs (91%). In the initial visit with the palliative care specialist, topics most frequently covered included goals of care (84%), anxiety/depression (35%), and medical decision making about feeding tubes (27%) or tracheostomy (31%). Symptom management comprised a relatively small number of the visits, and duration of visits was limited by patient fatigue. Patients with complex goals of care cmay benefit from the input of a palliative care specialist, and unique integration models may help to facilitate care delivery.
Informal family caregivers make a significant contribution to the U.S. health care system, and the need for caregivers will likely increase. Gaining deeper insights into the caregiver experience will provide essential knowledge needed to support the future caregiver workforce delivering care. Discourse analysis is a viable approach in analyzing textual caregiver data that focuses on the end-of-life caregiving experience. The purpose of this study was to conduct an in-depth discourse analytic examination of 13 hours of caregiver interview data, which reveal the multiplicity of shifting stances and perceptions of one caregiver in the midst of end-of-life care, specifically with regard to his perceptions of self (caregiver) and other (care recipient). By isolating a specific but limited set of reference terms used throughout the discourse, we gained systematic glimpses into the mind and perceptions of this single caregiver in relation to his role as caregiver for his terminally ill wife.
OBJECTIVE: The aims of this study were to identify the unmet care needs and to examine the mediating effect of unmet supportive care needs in the relationship between functional status and quality of life (QOL) in Korean patients with amyotrophic lateral sclerosis (ALS).
METHOD: This was a cross-sectional study conducted among 186 patients with ALS recruited from a tertiary hospital in Seoul, South Korea. ALS patients' functional status, unmet supportive care needs, and QOL were assessed by Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, the Amyotrophic Lateral Sclerosis Supportive Care Needs Instrument, and the Amyotrophic Lateral Sclerosis Specific Quality of Life - Revised Instrument, respectively. Mediation analysis was tested using Baron and Kenny's regression analysis and a Sobel test.
RESULT: The mean score for functional status was 33.35 ± 8.89; for unmet supportive care needs it was 2.40 ± 0.66; and for QOL it was 4.95 ± 1.29. Functional status was significantly correlated with unmet care needs and QOL. Unmet care needs satisfaction demonstrated a complete mediating effect on the relationship between functional status and QOL of the patients with ALS (ß = –0.53, p < 0.001) and the effect was significant (Sobel test; Z = 5.48, p < 0.001).
SIGNIFICANCE OF RESULTS: Although QOL was negatively affected by the functional status in our sample, the relationship was fully mediated via unmet supportive care needs. Because there is no cure for ALS, and the condition is rapidly progressive with a lethal outcome, providing care by meeting patients’ needs is a critical aspect of caring for these patients. Early assessment of supportive care needs, providing services, and referring ALS patients to appropriate resources could enhance their QOL.
Background: Cannabis is increasingly used by persons at end of life to ameliorate symptoms such as pain, spasticity, anorexia, or anxiety. Cannabis hyperemesis is a distressing adverse effect of chronic use and may cause significant morbidity. Unfortunately, the clinical presentation of this syndrome may be subtle in a person with complex medical issues or disability. Providers must remain vigilant for possible variations in presentation in these populations.
Aim: To assess literature on cannabis hyperemesis and present unique considerations for clinical assessment and treatment for patients at end of life.
Design: Initial literature scoping yielded limited evidence on the subject in the setting of chronic disease and disability. A case of cannabis hyperemesis in a person with advanced amyotrophic lateral sclerosis is presented to illustrate challenges in diagnosis and management in this setting. A narrative synthesis of current literature on assessment and management and special considerations for evaluation and treatment for patients under palliative care was performed.
Results: Several unique considerations for the diagnosis and management of cannabis hyperemesis in palliative care patients are highlighted in the case presented, including: (1) Symptoms may possibly be abolished through decrease rather than complete abstinence from cannabis, (2) Frequent hot baths may not be present in patients with physical impairments in activities of daily living, and (3) Management of primary symptoms (pain, spasticity, nausea, and anxiety) in the end-of-life care patient must be considered to maximize comfort.
Conclusion: The presentation of cannabis hyperemesis may be atypical in palliative care patients due to disability. More work is needed to improve risk stratification for patients using cannabis for palliative care.
La sclérose latérale amyotrophique (SLA) est une maladie neurodégénérative incurable. Elle est responsable d'une dépendance croissante au cours de son évolution et nécessite le recours à des techniques de suppléance vitales. Elle peut constituer un modèle relevant d'une prise en charge palliative. Celle-ci est pourtant tardive. Il existe de véritables enjeux légaux et éthiques à aborder précocement ce type de soins. L'élaboration de directives anticipées permet de respecter l'autonomie du patient. La maladie grave rend pourtant leur élaboration difficile. L'information du patient sur les possibilités thérapeutiques curatives mais aussi palliatives peut faciliter l'expression de ses souhaits. L'approche palliative précoce dans la SLA facilite le dialogue autour de la fin de vie et pourrait faciliter les choix du patient mais aussi du médecin.