Qualitative Analysis of Initial Palliative Care Consultations in Amyotrophic Lateral Sclerosis.

BACKGROUND: Palliative care (PC) benefits patients with amyotrophic lateral sclerosis (ALS), however the needs of patients and caregivers and the optimal timing of PC discussions remains unclear. This study reports the analysis of PC consult notes from a larger feasibility trial. The specific aims of this analysis were to i) identify the PC needs of patients with ALS via qualitative analysis and ii) identify characteristics of patients and caregivers that could predict specific PC needs. METHODS: This study was nested within a nonrandomized, prospective study of patients with ALS (and their caregivers) being treated at a multidisciplinary ALS clinic. Exclusion criteria of the main study were age <18 years, inability to complete questionnaires, and prior receipt of PC. All patients were offered a PC consultation (PCC); those who accepted were included in this nested study. Consultation notes were reviewed and thematic and content analyses were conducted. The occurrence of themes across patient and caregiver contextual variables were examined. RESULTS: Thirty-two PCCs were completed between October 2020 and April 2022. Six major themes were identified: PC roles (with subthemes encompassing the spectrum of specialist PC practice including symptom management and advance care planning), engagement with PC, patients' concerns for their caregivers, caregiver-specific concerns, finances, and COVID-19. An average of 12 topics were discussed per PCC (range = 3-22). Discussion of advance care planning, care coordination, and symptom management was common, and these topics were not discussed more frequently in PCCs with patients with lower functional status, more bulbar symptoms, or lower quality of life. Time from diagnosis did not impact topics of discussion. Patients reporting more symptoms of depression more frequently required psychological support, particularly regarding loss of independence, employment, and leisure activities. DISCUSSION: Patients with ALS and their caregivers have a wide range of PC needs. These needs vary irrespective of time from diagnosis, functional status, or quality of life, therefore PCC is recommended for all patients with ALS. PCC should be individualized based on patient and caregiver preferences. TRIAL REGISTRATION INFORMATION: The study was registered with ClinicalTrials.gov (NCT04257760; https://clinicaltrials.gov/ct2/show/NCT04257760) on February 6, 2020. The first enrollment occurred on October 20, 2020.

Identifying the Unmet Needs of People Living With Amyotrophic Lateral Sclerosis: A National Survey to Inform Interdisciplinary Palliative Care.

Introduction/Aims: This national survey builds on previous qualitative research examining potential palliative care needs among people living with ALS (pALS) by quantifying and investigating relationships among pALS' stage of illness progression; physical, emotional, social, spiritual, and intimacy-related concerns; advance care planning behaviors; perceptions of feeling heard and understood by healthcare providers; and overall quality of life. Methods: Researchers partnered with national organizations to recruit pALS to participate in a one-time survey comprising items from validated instruments (eg, the ALS Specific Quality of Life Instrument-Revised) and researcher-generated measures. Data were analyzed using logistic and linear regression. Results: Among pALS (n = 112), many respondents indicated they had discussed their wishes for end-of-life care with family or friends, shared their wishes with providers, and documented their wishes in writing (79.5%, 49.1%, and 63.4%, respectively). Mean (M) quality of life scores were moderate (M ≈ 6 of 10). Illness stage was associated with documentation of end-of-life care wishes but not with having discussed these wishes with others or with overall quality of life. Reported emotional intimacy received was comparable to that desired (difference = .01 of 10); however, a greater desire for physical intimacy relative to that received was indicated (difference = 1.75 of 10). Discussion: Interdisciplinary palliative care teams may enhance ALS care by promoting advance care planning behaviors (particularly discussing one's wishes with healthcare providers), providing interventions to improve quality of life, and supporting pALS in navigating challenges related to physical intimacy.

Optimizing breathlessness management in amyotrophic lateral sclerosis: insights from a comprehensive systematic review.

BACKGROUND: Breathlessness is a prevalent symptom affecting the quality of life (QOL) of Amyotrophic Lateral Sclerosis (ALS) patients. This systematic review explored the interventions for controlling breathlessness in ALS patients, emphasizing palliative care (PALC), non-invasive ventilation (NIV), opioids, and non-pharmacological strategies. METHODS: A comprehensive search of PubMed, Cochrane Library, and Web of Science databases was conducted. Eligibility criteria encompassed adults with ALS or motor neuron disease experiencing breathlessness. Outcomes included QOL and symptom control. Study designs comprised qualitative studies, cohort studies, and randomized controlled trials. RESULTS: Eight studies were included, most exhibiting low bias risk, comprising one randomized controlled trial, three cohort studies, two comparative retrospective studies, and two qualitative studies (interviews). Most studies originated from Europe, with one from the United States of America. The participants totaled 3423, with ALS patients constituting 95.6%. PALC consultations significantly improved symptom assessment, advance care planning, and discussions about goals of care. NIV demonstrated efficacy in managing breathlessness, with considerations for device limitations. Opioids were effective, though predominantly studied in non-ALS patients. Non-pharmacological strategies varied in efficacy among patients. CONCLUSION: The findings underscore the need for individualized approaches in managing breathlessness in ALS. PALC, NIV, opioids, and non-pharmacological strategies each play a role, with unique considerations. Further research, especially ALS-specific self-management studies, is warranted.

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists.

OBJECTIVE: This paper examines neurologists' approaches to exit strategies (ESs), such as euthanasia and physician-assisted suicide, in patients with amyotrophic lateral sclerosis (PALS) in two European countries. METHODS: In a nationwide anonymous survey, we collected responses from 237 Polish and 228 German neurologists, focusing on their practices and beliefs about ESs, as well as their viewpoints on life-sustaining measures (LSMs) (percutaneous endoscopic gastrostomy, non-invasive, and invasive ventilation). To analyze the data, we employed statistical methods, including Mann-Whitney U, Kruskal-Wallis, chi-square tests, Spearman's rank correlation, and multiple regression analysis. RESULTS: One third of the neurologists initiated the discussion about ESs with PALS. Half were ready to have this conversation upon patient's request. Age, gender, religiousness, and nationality were closely associated with this approach. One in 9 neurologists received a request to terminate an LSM, whereas 1 in 10 to implement an ES. German neurologists and palliative care trainees acquired both demands more commonly. Neurologists quoted a low quality of life, decreased mood, and being a burden to the family/closest ones as primary reasons for a wish to hasten death among PALS. Although the majority expressed a willingness to terminate an LSM at a request of the patient, most opposed the legalization of euthanasia. Younger and less religious individuals were more likely to favor accepting euthanasia. CONCLUSION: Neurologists vary significantly in their approaches to terminal care. Complex relationships exist among personal indices, shared beliefs, and current practices.

Palliative care at any stage of amyotrophic lateral sclerosis: a prospective feasibility study.

Introduction: Many patients with amyotrophic lateral sclerosis (ALS) receive palliative care (PC) very late or not at all. The impact of PC on patients with ALS and caregivers has not been quantified. Study goals included (1) measuring the impact of early PC on quality of life and mood of patients/caregivers and (2) describing patient/caregiver satisfaction with PC. Methods: The study was a non-randomized, prospective feasibility study of patients with ALS being treated at The Ottawa Hospital ALS Clinic and their caregivers. Exclusion criteria were age < 18 years, inability to complete questionnaires, and prior receipt of PC. The ALS Specific Quality of Life-Revised (ALSSQOL-R) questionnaire (patients only) and Hospital Anxiety and Depression Scale (HADS) were completed at regular intervals for up to 2 years. Patients accepting a PC consultation completed a post-PC satisfaction survey. Primary outcome measures included ALSSQOL-R and HADS scores compared before and after PC consultation, and between groups receiving and not receiving a PC consultation. Secondary outcome measures included responses on the post-PC satisfaction survey (1 = strongly disagree, 5 = strongly agree). Results: 39 patients with ALS (age 66 ± 10 years, median time from diagnosis = 6 months) and 22 caregivers were enrolled. 32 patients had a PC consultation (30 were virtual). Patients and caregivers agreed with statements that the PC consult was helpful (mean ± SD = 4.54 ± 0.60, range = 3-5) and they would recommend PC to others with ALS (4.59 ± 0.59, range = 3-5). Participants disagreed with statements that the consult would have been better later in disease course (1.87 ± 0.80, range = 1-4) and that it took too much time/energy (1.44 ± 0.85, range = 1-4). Average ALSSQOL-R scores worsened significantly over time. HADS and ALSSQOL-R scores did not significantly differ between groups receiving and not receiving PC. Conclusion: Patients with ALS and their caregivers found virtual PC consultations beneficial irrespective of disease duration or severity. Offering routine PC to all patients with ALS is feasible and should be considered as part of standard care. Clinical trial registration: https://clinicaltrials.gov/ct2/show/NCT04257760, identifier NCT04257760.

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

INTRODUCTION/AIMS: 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. METHODS: 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. RESULTS: 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. DISCUSSION: 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.

Radiologically Inserted Gastrostomy Tube Placement Guided by the Assessment and Primary Palliative Care Provided by an Amyotrophic Lateral Sclerosis Multidisciplinary Clinic: A Single-Arm Retrospective Clinical Study.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a median survival of about 3 years. An ALS multidisciplinary team can provide primary palliative care and improve outcomes and quality of life for patients. Feeding tube insertion may be considered for patients with significant weight loss, or respiratory insufficiency. While radiologically inserted gastrostomy (RIG) tube placement may be an option, further studies are required to determine its best timing and appropriateness. This study's objectives were to evaluate the feasibility and outcomes of RIG tube placement in ALS patients over a 90-day follow-up period through the assessment and primary palliative care provided by the multidisciplinary team. This retrospective study reviewed the placement of 16 or 18 French RIG-tube without intubation or endoscopy for 36 ALS patients at a single center between April 2019 and December 2021. Measures included ALS Functional Rating Scale-Revised (ALSFRS-R) scores to determine the ALS stage. Demographic, clinical, procedural, and follow-up data were reviewed. Results showed that the RIG tube placement had a low rate of minor adverse events (11%) and no major procedure-related adverse events. The mean ALSFRS-R score at the time of procedure in subjects who died within 90 days was lower than of those alive beyond 90 days (P = .04). This study found that RIG-tube placement is a safe and effective way to manage dysphagia in ALS patients and highlights the importance of educating members of the multidisciplinary clinic in palliative care principles to determine the appropriateness of RIG tube placement.

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.

'Intensive palliative care': a qualitative study of issues related to nurses' care of people with amyotrophic lateral sclerosis at end-of-life.

Background: Amyotrophic lateral sclerosis (ALS) is currently an incurable and fatal disease, which often comes with a high symptom burden at the end-of-life stage. Little is known about nurses' experiences in this context. Objective: To explore the experience of nurses caring for people with ALS at end-of-life. Design: A qualitative multiple-case study design. Method: Individual semi-structured interviews were conducted between February and August 2022 with nurses from Quebec, Canada, who had provided care to at least one person living with ALS at the end-of-life in the past 12 months. The content analysis method was used for data analysis and within-case and cross-case analyses were conducted, as well as comparative analyses according to the type of position held by the participants that determined the cases: (1) home care, (2) hospital and (3) palliative care home. Results: Participating in the study were 24 nurses: 9 were from home care, 8 from hospitals and 7 from palliative care homes. Five main themes were identified: (1) identifying the end-of-life period, (2) communication issues, (3) supporting the need for control, (4) accompanying in the fight culture and (5) the extent of the need for care. A sixth theme was also added in order to report the need expressed by nurses to improve their care of patients living with ALS at end-of-life. Conclusions: Although nurses' experiences varied among the different settings, the study identifies the pressing need for better education and, above all, more resources when caring for a person living with ALS at end-of-life. Future research should explore the experiences of other members of the healthcare team and test interventions designed to improve the quality of life and end-of-life of people living with ALS.

A Smart System for Remote Monitoring of Patients in Palliative Care (HumanITcare Platform): Mixed Methods Study.

BACKGROUND: Due to the complexities of advanced illnesses and their treatments, it can be difficult for patients in palliative care to maintain their quality of life. Telemedicine interventions in chronic disease management engage patients in their care, provide continuous follow-up by their health care providers, identify symptoms earlier, and allow a quick response to illness-related decline. OBJECTIVE: We aimed to detail and reflect on the design of an app and evaluate its feasibility to monitor the clinical situation of patients with advanced illnesses. METHODS: This study used a mixed methods design using qualitative methods to inform app development and design and quantitative methods for data collection and analysis of patient evaluations. Palliative care units in 2 Spanish university hospitals (Nuestra Señora de la Candelaria in Santa Cruz de Tenerife and University Hospital Complex of Ferrol in A Coruña) carried out a literature review, designed the study protocol, and obtained approval from the Ethics Committee from June to December 2020. In addition, focus group meetings were held, and the design and technical development of the app were elaborated on and subsequently presented in the participating palliative care units. From January to March 2021, the app was made public on the App Store and Play Store, and a pilot study with patients was carried out in April to September 2021. RESULTS: Six focus group meetings were held that included doctors, nurses, app developers, technology consultants, and sponsors. In addition, the technology consultants presented their results 3 times in the participating palliative care units to obtain feedback. After the app's final design, it was possible to publish it on the usual servers and begin its evaluation in patients (n=60, median age 72 years). Sixty percent (n=36) of the participants were women and 40% (n=24) were men. The most prevalent advanced pathology was cancer (n=46, 76%), followed by other diseases (n=7, 12%) and amyotrophic lateral sclerosis (n=5, 8%). Seventy percent (n=42) of the patients were already in follow-up prior to the start of the study, while 30% (n=18) were included at the start of their follow-up. The information in the app was collected and entered by relatives or caregivers in 60% (n=36) of the cases. The median follow-up was 52 (IQR 14-104) days. In all, 69% (n=41) had a follow-up >30 days (10 were deceased and 9 were missing data). The use of the different sections of the app ranged from 37% (n=22) for the glycemic record to 90% (n=54) for the constipation scale). Patients and caregivers were delighted with its ease of use and usefulness. CONCLUSIONS: Incorporating an intelligent remote patient monitoring system in clinical practice for patients in palliative care can improve access to health services and provide more information to professionals.

Palliative care principles in ALS.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease associated with progressive dysphagia, dysarthria, extremity weakness, and dyspnea. Although there are some disease-modifying pharmacological treatments available which can modestly slow disease progression, the disease is relentlessly progressive and is ultimately fatal. Patients living with ALS should be supported using the principles of palliative care, and in particular, the use of a holistic approach to support the patients and their families. Evidence would support management of patients living with ALS by a multidisciplinary ALS specialty clinic. These multidisciplinary clinics will help support the multitude of symptoms a patient living with ALS can experience, including dysphagia, communication impairments, dexterity impairments, mobility deficits, and respiratory insufficiency. Formal involvement of specialist-trained palliative practitioners can occur throughout the course of the illness, or when the patient is open to their involvement. There are several models of palliative care that can be followed, including integration of palliative care into the multidisciplinary ALS clinic, separate involvement of a palliative care specialty team, home-based palliative care, telemedicine supported care, and hospice care. Key components of palliative care in ALS are goals-of-care discussions advance directive planning, symptoms management, and end-of-life support.

The involvement of palliative care with neurology - a comparison of UK, Switzerland and Italy.

OBJECTIVES: To ascertain the involvement of palliative care with neurology services in the care of people with amyotrophic lateral sclerosis (ALS) in the United Kingdom, Italy and Switzerland, in particular the collaboration with and referral from neurology, the involvement in multidisciplinary team care and in the respiratory support of ALS patients. METHODS: In 2019, two online surveys were undertaken of palliative care specialists, using specialist groups of the European Academy of Neurology, European Association of Palliative Care and the Association of Palliative Medicine for Great Britain and Ireland. RESULTS: The respondents were specialist palliative care professionals, predominantly senior doctors, involved in the care of people with ALS. As the numbers of respondents from many countries were in single figures the analysis was restricted to the United Kingdom, Italy and Switzerland. The time of involvement varied, with early involvement commonest in the UK. Barriers to referral included neurologists not referring and financial issues, particularly in Switzerland. The reluctance of patients and families to see palliative care services was reported as less than 20% in all countries. Respondents were often involved in the care of people receiving noninvasive ventilation (NIV), in all countries. and with tracheostomy ventilation (TV), particularly in Italy. CONCLUSIONS: Palliative care services are often involved in the care of people with ALS, but the extent and timing of involvement varies. The use of clinical guidelines and education on palliative care for neurology services may encourage collaboration, for the benefit of people with ALS and their families.

A Palliative Care Approach to Amyotrophic Lateral Sclerosis.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a degenerative disease characterized by motor dysfunction. Currently, treatment options are limited and management is based mostly on symptom control and quality of life optimization, so palliative care plays a fundamental role. Our objective was to characterize the ALS population in Madeira Island that was referenced and/or followed by a palliative care unit over a five-year period. METHODS: Longitudinal, retrospective, descriptive, and observational study to analyze patients with ALS who were referred and/or followed by a palliative care unit during a five-year period, between 2017 and 2021. Patient's medical electronic and physical records were analyzed to gather data. Descriptive and inferential statistical analysis was done using Microsoft Excel and Statistical Package for the Social Sciences (version 28.0.1). RESULTS: During this five-year period, a total of 38 patients were diagnosed with ALS in Madeira Island and 23 (60.53%) were referred to palliative care. Three patients died before assessment, so 20 (50.63%) were followed by the palliative care team. They had a median life expectancy of 425 days and the median time spent in palliative care was 137 days. Of this population, 56.52% (n=13) was male with an average age of 64 years. The median period from diagnosis to referral was 167 days, with most referrals being made by family medicine (39.13%; n=9) motivated by uncontrolled symptoms (95.65%; n=22). The median period from referral to first assessment by a palliative care physician was 19 days. The Palliative Performance Scale (PPS) and Confusion Assessment Method (CAM) applied on the first visit had a median score of 40% in the former and was negative in 95.00% (n=19) of patients in the latter. Advanced care directives were present in 55.00% (n=11) of patients and all provided care was in accordance with the patient's wishes. The most common symptoms were dysphagia, dyspnea, pain, anxiety, and sialorrhea. The most used drugs were morphine, riluzole, butylscopolamine, bisacodyl, and midazolam. As for other interventions, 55.00% (n=11) of patients underwent noninvasive ventilation (NIV), 15.00% (n=3) were submitted to percutaneous endoscopic gastrostomy (PEG), and one patient (5.00%) was nasogastrically intubated. The death rate was 95.00% (n=19) with 73.68% (n=14) of deaths occurring in the palliative care unit. CONCLUSION: Literature has shown that there are many advantages to the early inclusion of palliative care in ALS management, achieving effective symptom control and greater quality of life, but also reducing caregiver burden. However, in this study, we found that referrals to palliative care were late and included mostly cases of advanced disease with uncontrolled symptoms.

Attitudes of caregivers towards prolonging and shortening life in advanced stages of amyotrophic lateral sclerosis.

INTRODUCTION: Inevitable disease progression in amyotrophic lateral sclerosis (ALS) forces patients and their caregivers (CGs) to reflect on end-of-life treatment. The CGs are often heavily burdened with their role of surrogate decision-makers. The aim of the study was to analyze attitudes of CGs and presumable attitudes of ALS patients from the CGs' perspective towards palliative care in advanced disease stages. MATERIAL AND METHODS: One hundred and sixty four CGs from Germany and Poland were interviewed regarding their own preferences and patients' ideational attitudes towards life-prolonging (invasive and non-invasive ventilation, tube feeding) and life-shortening methods (termination of measures, active measures if permitted by law). The data were correlated with patient- and CG-related factors: demographic and clinical data, care commitment, depression and quality of life (QoL). RESULTS: The CGs were mostly female spouses of ALS patients, with secondary/higher education. Nearly 70% (81% in Poland, 57% in Germany; p = 0.0001) reported positive attitudes towards life-prolonging methods, which positively correlated with religiousness and negatively with patients' age. Approximately 40% of CGs (25% and 51% respectively; p = 0.001) reported positive attitudes towards life-shortening methods. It positively correlated with time since diagnosis and negatively with the CG's QoL, religiosity and religious/spiritual faith as factors that significantly influenced end-of-life decisions. There was a strongly positive correlation between CGs' positive attitudes towards life-shortening methods and presumed positive patients' attitudes assessed by their CGs ( p < 0.000001). CONCLUSIONS: Although attitudes towards treatment differed between countries, the CGs of ALS patients were generally positive towards life-prolonging treatment. A greater acceptance of life-shortening methods in the case of longer disease duration and poorer QoL may indicate worse coping with disease progression and weaker adaptation mechanisms in CGs compared to those previously reported in ALS patients. A close resemblance of the CGs' answers to probable patients' attitudes reported by the CGs indicates that many GCs might actually express their own culturally shaped attitudes towards end-of-life methods. In light of earlier-reported discrepancies between presumed opinions of the CGs and of patients themselves, a greater focus should be placed on thorough discussions on future treatment options with ALS patients in the presence of their CGs, to stay in line with the patient's authentic will.

Considerações da percepção dos cuidadores diante do cuidado com pacientes com esclerose lateral amiotrófica / Considerations of the caregivers' perception regarding the care of patients with amyotrophic lateral sclerosis / Consideraciones de percepción profesional antes de cuidar a pacientes con esclerosis amiotrófica lateral

Objetivo: Analisar as considerações dos cuidadores de pacientes diagnosticados com esclerose lateral amiotrófica para obtenção de melhor compreensão quanto sua saúde mental. Método: Trata-se de uma revisão integrativa da literatura de caráter narrativo, realizada através das bases BVS Brasil, BVSalud, Lilacs, Pepsic e site da ABRELA, entre abril e maio de 2020. Foram incluídos artigos no idioma português onde considera-se cuidadores, saúde mental relacionados a pacientes diagnosticados com esclerose lateral amiotrófica. Resultados: Desde os primeiros sintomas de esclerose lateral amiotrófica até o momento da morte, o paciente e seu cuidador, passam por perturbações emocionais e sociais significativas, das quais contribuem negativamente na qualidade de vida de ambos. Os cuidadores, em sua maioria mulheres e parentes, apresentaram níveis de sobrecarga percebida e atenção negligenciada a si mesmos. Conclusão: Conclui-se que os cuidadores necessitam de maiores ferramentas que possibilitem a manutenção de seus aspectos psicológicos, gerando maior qualidade de vida (AU).

Objective: To analyze the caregivers' considerations regarding patients diagnosed with amyotrophic lateral sclerosis to obtain a better understanding of their mental health. Method: This is an integrative review of the literature of a narrative character, carried out through the VHL Brazil, BVSalud, Lilacs, Pepsic databases and ABRELA, between April and May 2020. Articles in Portuguese were included, including caregivers, mental health related to patients diagnosed with amyotrophic lateral sclerosis. Results: From the first symptoms of amyotrophic lateral sclerosis to the moment of death, the patient and his caregiver undergo significant emotional and social disturbances, which contribute negatively to the quality of life of both. Caregivers, mostly women and relatives, had levels of perceived overload and neglected attention to themselves. Conclusion: It is concluded that caregivers need greater tools that enable the maintenance of their psychological aspects, generating greater quality of life (AU).

Objetivo: Analizar las consideraciones de los cuidadores de pacientes diagnosticados de esclerosis lateral amiotrófica para conocer mejor su salud mental. Método: Se trata de una revisión integradora de la literatura de carácter narrativo, realizada a través de las bases de datos BVS Brasil, BVSalud, Lilacs, Pepsic y sitio web ABRELA, entre abril y mayo de 2020. Se incluyeron artículos en portugués, incluyendo cuidadores, salud mental relacionados con pacientes diagnosticados con esclerosis lateral amiotrófica. Resultados: Desde los primeros síntomas de la esclerosis lateral amiotrófica hasta el momento de la muerte, el paciente y su cuidador sufren importantes trastornos emocionales y sociales, que contribuyen negativamente a la calidad de vida de ambos. Los cuidadores, en su mayoría mujeres y familiares, tenían niveles de sobrecarga percibida y descuidaban la atención hacia ellos mismos. Conclusión: Se concluye que los cuidadores necesitan mayores herramientas que permitan el mantenimiento de sus aspectos psicológicos, generando mayor calidad de vida (AU).

Manejo multidisciplinario y avances terapéuticos en la esclerosis lateral amiotrófica / Multidisciplinary care and therapeutic advances in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the motor system, resulting in progressive weakness and muscle wasting. Despite the tremendous advances in physiopathological and clinical characterization, we do not have a curative treatment yet. The progressive and fatal course of ALS makes its management particularly complex and challenging given the diversity of symptoms presenting during the disease progression. The main goal in the treatment of ALS patients is to minimize morbidity and maximize the quality of life. Currently, a series of therapeutic interventions improve the quality of life and prolong survival, including multidisciplinary care, respiratory management, and disease-modifying therapy. Within the supportive interventions, weight maintenance through nutritional and metabolic support is critical. In addition, the management of neuropsychiatric manifestations and preservation of communicative capacity before speech loss are also crucial. Lastly, early palliative care intervention is essential to optimize symptomatic management. Anticipatory guidelines to face the inevitable patient deterioration should be devised. This article updates the main therapeutic strategies used in these patients, including evolving clinical trials with promising novel therapies.

Well-being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective.

OBJECTIVE: To investigate intercultural neurologists' perception of well-being in patients with amyotrophic lateral sclerosis (ALS) using gastrostomy (PEG), non-invasive, and/or invasive ventilation (NIV/IV) and to analyse the determinants and impact on the management of the above medical interventions (MIs). METHODS: The study was based on anonymous questionnaires addressing the clinical approach and personal attitude towards the use of PEG, NIV and IV in ALS patients completed by 465 neurologists: 228 from Germany and 237 from Poland. RESULTS: The German and Polish neurologists estimated the quality of life in ALS patients using PEG and NIV as neutral, whilst low in individuals using IV. A regression model revealed an independent influence of palliative care training (PCT) and age on that attitude in the German group. Higher values of estimated patients' depressiveness on PEG, NIV and IV were found amongst the Polish neurologists. Marital status, experience in ALS and being a parent independently influenced the perception of patients' depressiveness in the German, whilst marital status, age and PCT were factors in the Polish group. Amongst German neurologists, a higher perception of patients' depressiveness in individuals using PEG, NIV and IV was linked to the later timing of the MIs discussion. In the Polish group, it was a lower estimation of QoL in patients using PEG. CONCLUSION: Neurologists' perception of ALS patients' well-being on MIs reflects their demographic status, professional experience and potentially their cultural background. This perception plays an important role in the timing of MIs discussion, possibly influencing the decision-making process.

Quality of life and mental health in the locked-in-state-differences between patients with amyotrophic lateral sclerosis and their next of kin.

For both patients with amyotrophic lateral sclerosis (ALS) and their next of kin (NOK), the maintenance of quality of life (QoL) and mental health is particularly important. First studies suggest significant discrepancies between QoL reports by patients and NOK, but little is known for advanced ALS stages. To address this issue, we screened 52 ALS patients in incomplete locked-in state (iLIS). Final results were obtained for 15 couples of iLIS patients and NOK. We assessed patients' and NOK's subjective QoL, depression and anxiety and NOK's caregiver burden. Gaze controlled questionnaires allowed direct assessment of patients. Patients and NOK self-reported comparable, mostly moderate to high levels of QoL. Of note, NOK indicated stronger anxiety symptoms. Higher anxiety levels in NOK were associated with stronger caregiver burden and reduced QoL. No significant misjudgment of patient's QoL by the NOK was evident, while patients overestimated NOK's global QoL. However, NOK with severe caregiver burden and depression symptoms gave poorer estimations of patients' QoL. This relationship is relevant, considering NOK's impact on life critical treatment decisions. While the daily time NOK and patient spend together was positively correlated with NOK's QoL and mental health, this was not reversely found for the patients. Our results suggest that NOK adapt less successfully to the disease and concomitant experience of loss and point to an urgent need for specialized psychosocial support. The findings emphasize the importance of direct psychological wellbeing assessment of both patients and NOK in clinical practice, enabled by eye-tracking technology for patients in iLIS.

Pain in amyotrophic lateral sclerosis: a narrative review.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative condition characterized by loss of motor neurons, resulting in motor weakness of the limbs and/or bulbar muscles. Pain is a prevalent but neglected symptom of ALS, and it has a significant negative impact on the quality of life of patients and their caregivers. This review outlines the epidemiology, clinical characteristics, underlying mechanisms, and management strategies of pain in ALS to improve clinical practice and patient outcomes related to pain. Pain is a prevalent symptom among patients with ALS, with a variable reported prevalence. It may occur at any stage of the disease and can involve any part of the body without a specific pattern. Primary pain includes neuropathic pain and pain from spasticity or cramps, while secondary pain is mainly nociceptive, occurring with the progression of muscle weakness and atrophy, prolonged immobility causing degenerative changes in joints and connective tissue, and long-term home mechanical ventilation. Prior to treatment, the exact patterns and causes of pain must first be identified, and the treatment should be tailored to each patient. Treatment options can be classified into pharmacological treatments, including nonsteroidal anti-inflammatory drugs, antiepileptic drugs, drugs for cramps or spasticity, and opioid; and nonpharmacological treatments, including positioning, splints, joint injections, and physical therapy. The development of standardized and specific assessment tools for pain-specific to ALS is required, as are further studies on treatments to reduce pain, diminish suffering, and improve the quality of life of patients with ALS.