Information Needs and Preferences of Family Caregivers of Patients With Amyotrophic Lateral Sclerosis.

OBJECTIVE: The aim of this study was to explore the information needs and preferred sources of information of Korean family caregivers of patients with amyotrophic lateral sclerosis (ALS). METHODS: Family caregivers of patients with ALS (n = 108) completed a structured questionnaire to assess their information needs and preferred sources of information. RESULTS: Most of the caregivers obtained health information from Internet searches (89.8%) and healthcare professionals (85.2%). The source rated most helpful was healthcare professionals, and that rated least helpful was broadcast media. Family caregivers who were younger than 50 years and well educated and caregivers of bulbar-onset ALS patients had higher scores of information needs. CONCLUSION: Providing information through healthcare professionals and self-support groups could enhance family caregiver satisfaction. Family caregivers who are older and less educated need to be more empowered to be involved in information-based caring, and caregivers of patients with bulbar-onset ALS have substantial information needs.

Skill, confidence and support: conceptual elements of a child/youth caregiver training program in amyotrophic lateral sclerosis - the YCare protocol.

Aim: Pilot study of a novel caregiving training and support intervention for children and youth <19 years, who provide care for person living with amyotrophic lateral sclerosis. Materials & methods: Youth (n = 19) between the ages of 8-19 years, participated in skills training and support program (basic care, feeding/communication, assistive devices and social support). Results: Participants reported significant increase in confidence in tasks, including communication systems and respiratory equipment. Participants identified goal setting and creating behaviors to reach those goals. Benefits of training included that the day changed their perceptions of care and meeting 'like' peers. Conclusion: Results of the pilot YCare intervention underscores the need to assess how young caregivers feel and respond to new tasks when receiving support from developmentally similar peers.

Psychometric properties of the Korean version of the positive aspects of caregiving scale for family caregivers of people with amyotrophic lateral sclerosis.

OBJECTIVE: Many caregivers report finding positive meanings in their caregiving roles and activities. The positive aspects of caregiving (PAC) scale was designed to measure positive appraisals of caregiving. This study assessed the reliability and validity of the Korean version of the PAC for family caregivers of people with amyotrophic lateral sclerosis (ALS). METHOD: The instrument's content and semantic equivalence were established using translation and back translation of the PAC. A convenience sample of 127 family caregivers of patients with ALS in Korea was recruited. Content, construct, and convergent validity of the Korean PAC were evaluated. Cronbach's α was used to assess reliability. This study used secondary data; the primary study received approval from the Institutional Review Board of H Hospital, from where data were collected. The consent forms did not mention the future use of data. Therefore, we have applied for approval regarding this study's protocol and exemption from informed consent. RESULTS: The Cronbach's α was 0.92, and internal consistency was acceptable. Exploratory factor analysis supported the construct validity with a two-factor solution explaining 74.73% of the total variance. Regarding convergent validity, the Korean PAC score negatively correlated with caregiver burden and depression and positively with self-rated health status. We were unable to evaluate the suitability of the suggested structural dimensionality through confirmatory factor analysis. Furthermore, as we used secondary data, we could not assess retest reliability for the evaluation of the scale's stability. SIGNIFICANCE OF RESULTS: The Korean PAC was found to be an applicable instrument with satisfactory reliability and validity and suitable for further use as a measure for positive appraisals of caregiving for family caregivers of people with ALS. It may be effective for measuring caregivers' psychological resources.

Amyotrophic lateral sclerosis: Nursing care and considerations.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease that is always fatal, although treatment can help slow disease progression. This article discusses the etiology and pathophysiology, signs and symptoms, diagnosis, and clinical management of ALS, with special nursing considerations to help patients at the end of life.

Everyday life experiences of close relatives of people with amyotrophic lateral sclerosis receiving home mechanical ventilation-A qualitative study.

AIMS AND OBJECTIVES: To explore everyday life experience of relatives of people with amytrophic lateral sclerosis (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.

Psychological distress in partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: what's the role of care demands and perceived control?

Caregivers of Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA) patients often experience psychological distress. Yet, it is unclear which factors explain the variance in psychological distress. This study seeks to evaluate how care demands and perceived control over caregiving influence psychological distress using moderation and mediation analysis. Data were collected as part of a RCT and 148 partners of patients with ALS or PMA were included. Psychological distress was assessed using the Hospital Anxiety and Depression Scale. Care demands were operationalized as physical functioning (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised) and behavioural changes of the patient (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire). Perceived control over caregiving was assessed using items adapted from the Job Content Questionnaire. Results showed that more behavioural changes and lower perceived control over caregiving were associated with higher levels of psychological distress in caregivers. Patients' physical functioning was not significantly related to caregivers' psychological distress. No moderation or mediation effects were found of perceived control over caregiving on the relationship between demand and psychological distress. Monitoring, psychoeducation and caregiver support with regard to behavioural changes in patients, seem to be important for the wellbeing of caregivers. Caregivers' perceived control might be a target for future interventions.

[Neuropsychopathology of amyotrophic lateral sclerosis]. / Neuropsychopathologie de la sclérose latérale amyotrophique.

Amyotrophic lateral sclerosis is a serious neuro-degenerative disease related to involvement of central motor neurons (cerebral cortex) and peripheral neurons (anterior horn of the spinal cord and motor nuclei of the medulla). Clinical progression is marked by extensive paralysis leading to death, most frequently by respiratory failure. Specific nursing care is required at each stage of the illness.

Caregiver burden and family functioning in different neurological diseases.

Aim of this study is to examine caregiver burden and family functioning in different neurological conditions. Forty-two primary caregivers of patients with Amyotrophic Lateral Sclerosis (ALS), Alzheimer's Disease and other dementia (AD), Parkinson's Disease (PD), Acquired Brain Injuries (ABI) and Multiple Sclerosis (MS) were administered scales for the evaluation of caregiver burden (CBI) and family functioning (FACES IV). Caregiver burden was overall high, with caregivers of patients with ALS and ABI having exceeded the CBI cut-off score for possible burn-out. The average scores of caregivers of patients with AD or other dementia and PD were close to the cut-off score, whereas those of caregivers of patients with MS were significantly lower than the others. Family cohesion, family satisfaction and the quality of family communication were associated with reduced levels of caregiver burden, whereas disengagement was associated with a higher burden. The data from the present study confirm that caregiver burden is a relevant issue in the context of neurological diseases, especially for those causing higher degrees of impairment. Significant correlations with family functioning emerged as well, highlighting the importance of studying and treating caregiver burden within the context of family relations.

The burden of apathy for caregivers of patients with amyotrophic lateral sclerosis.

OBJECTIVES: Apathy is the most common behavioral symptom of amyotrophic lateral sclerosis (ALS). Despite its known impact on caregiver wellbeing, apathy is typically considered a unitary construct making assessment and targeting treatment problematic. The aim of this study was to explore the relationship between caregiver burden and the behavioral, cognitive, and emotional symptoms of apathy in ALS. METHODS: Fifty-one ALS patient-caregiver dyads from an ALS/frontotemporal dementia Clinic were assessed with the Apathy Evaluation Scale which measured the cognitive, behavioral, emotional, and nonspecific symptoms of apathy as well as the Zarit Burden Interview, a measure of perceived burden among caregivers of cognitively impaired older adults. The relationship between apathy and caregiver burden were analyzed using univariate and multivariate methods. RESULTS: Apathy was identified in 18% of ALS patients. Greater behavioral (p = 0.011) and nonspecific (p = 0.010) symptoms of apathy exhibited by patients were reported by caregivers with higher levels of burden compared to caregivers with lower levels of burden. Of the cognitive, behavioral, emotional, and nonspecific symptoms of apathy, only the behavioral symptoms explained a significant amount of variance in caregiver burden (p = 0.031). CONCLUSIONS: Apathy, specifically the behavioral symptoms of apathy was associated with higher burden of care among ALS caregivers, highlighting the importance of multidimensional assessment of apathy and provision of behavior management support as part of ALS care.

How Should Physicians Care for Dying Patients with Amyotrophic Lateral Sclerosis?

We discuss physician aid in dying, euthanasia, and other dimensions of palliative care decision making and define relevant terms raised by this case of a dying patient with amyotrophic lateral sclerosis in Washington State who is unable to self-administer a lethal prescription. We then present a concrete framework that clinicians can directly apply when faced with difficult cases such as this one. We outline how exploring motivations, obtaining informed consent, defining goals, and examining alternatives can help guide physicians like the one in this case. We conclude by summarizing one way in which physicians might balance these issues while still remaining within the constraints of the law.

A Journey with Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Motor neurons are affected in certain patterns, such as cervical, thoracic, lumbar, and bulbar (facial) regions. Although initial presentations can vary, eventually upper and lower motor neurons are lost in the two types of ALS: familial and sporadic. A case study highlighting the reality of living with bulbar ALS relays Sister A's journey from early to late ALS. Etiology, pathophysiology, diagnosis, treatment, and clinical care are discussed.

Circumstances and Signs of Approaching Death in Patients With Amyotrophic Lateral Sclerosis Undergoing Noninvasive Ventilation in Home Care Settings.

The purpose of this study was to elucidate the circumstances and symptoms of patients with amyotrophic lateral sclerosis (ALS) using noninvasive positive pressure ventilation (NPPV) in whom death was approaching, to understand how to provide palliative care to dying patients with ALS receiving NPPV management. The participants were 6 home visiting nurses who were head or subhead nurses of a home visiting nursing agency. Data were collected conducting individual semistructured interviews and then group interviews. The results were based on qualitative analysis of the circumstances of death and prognostication and showed 5 categories: difficulties with knowing about approaching death, several signs and symptoms of knowing about approaching death, importance of feeling prepared and provision of palliative care to die at home, death caused by accident, and fate determined by the caregiver's ability. Further research is needed to collect rich data about predicting approaching death to improve end-of-life care for NPPV-dependent ALS patients who do not receive sufficient benefits from NPPV.

Longitudinal predictors of caregiver burden in amyotrophic lateral sclerosis: a population-based cohort of patient-caregiver dyads.

OBJECTIVE: Caregiver burden is a recognised consequence of caring for a patient with neurodegeneration. Amyotrophic lateral sclerosis (ALS) differs from other neurodegenerations by its rapid progression and impairment of motor, cognitive, and behavioural function, which contribute to caregiver burden. However, longitudinal factors that determine the extent of caregiver burden, and in particular the impact of psychological distress among caregivers, have not been fully established. METHODS: Patients with ALS (n = 85) and their primary caregivers (n = 85) completed three serial evaluations. Caregiver burden was measured using the Zarit Burden Interview (ZBI). Anxiety and depression were evaluated using the Hospital Anxiety and Depression Scale (HADS). The Edinburgh Cognitive-Behavioural ALS Screen (ECAS) was used to determine cognitive function in patients. The ALS Functional Rating Scale (ALSFRS-R) measured disease progression. RESULTS: Using the ZBI, caregivers were categorised as high or low burden. In the low burden group, anxiety scores from the HADS predicted caregiver burden (r = 0.410, F = 3.73, p = 0.033), whereas the depression sub-score from the HADS was predictive of caregiver burden in the high burden group (r = 0.501, F = 5.87, p = 0.006) for cross-sectional analyses. Longitudinally, an elevated score on the HADS at Time 1 was the largest predictor of caregiver burden across serial assessments. CONCLUSION: In a patient cohort with relatively preserved cognitive function (65%), anxiety and depression at Time 1, as measured by the HADS, were the best predictors of caregiver burden at Time 3. This observation provides a mechanism by which caregiver burden can be identified by health-care professionals and a stepped care programme of intervention initiated.

The amyotrophic lateral sclerosis supportive care needs assessment instrument: Development and psychometric evaluation.

OBJECTIVE: The aim of the study is to develop an amyotrophic lateral sclerosis supportive care needs (ALSSCN) instrument based on Fitch's Supportive Care Needs Framework and to test its psychometric properties. METHOD: This study consists of three parts: (1) item generation from the literature review and qualitative interview; (2) content validation; and (3) psychometric evaluation of the instrument. Participants who were diagnosed with ALS (n = 139) were recruited from two ALS clinics in Seoul, Korea, and Busan, Korea for the psychometric testing.ResultThe ALSSCN consisted of 37 items with seven domains: physical, psychological, emotional, spiritual, social, informational, and practical needs. The Cronbach's alpha of each domain ranged from 0.61 (social needs) to 0.90 (emotional needs). The intra-class correlation coefficient for test-retest was 0.89, indicating good test-retest reliability. The overall ALSSCN was significantly negatively correlated with the quality of life, which supported convergent validity. Confirmatory factor analysis of the ALSSCN supported a seven-factor model.Significance of resultsThe ALSSCN has acceptable internal consistency, stability, and content and construct validity in a Korean ALS population. ALSSCN is a psychometrically sound measure and can be adopted by healthcare professionals, researchers, and administrators to comprehensively assess the perceived supportive care needs of patients with ALS.

Factor analysis of the Zarit Burden Interview in family caregivers of patients with amyotrophic lateral sclerosis.

OBJECTIVE: The Zarit Burden Interview has been used in many studies to assess caregiver burden in family caregivers of patients with amyotrophic lateral sclerosis, but the factor structure of the Zarit Burden Interview in the caregivers of amyotrophic lateral sclerosis patients is unknown. The aim of this study was to explore the factor structure of the Zarit Burden Interview in family caregivers of amyotrophic lateral sclerosis patients using exploratory factor analysis. METHODS: The exploratory factor analysis was performed using generalized least squares with oblique rotation in a sample of 202 family caregivers. RESULTS: Three factors had an eigenvalue greater than 1 and accounted for 60.33% of the total variance. The three factors were named as follows: (factor 1) "Social restrictions" (items 2, 3, and 10-15); (factor 2) "Self-criticism" (items 20-21); and (factor 3) "Anger and frustration" (items 1, 4-6, 9, and 16-19). The correlation between factors 1 and 3 was much higher (r = 0.79) than that between factors 1 and 2 (r = 0.14) or factors 2 and 3 (r = 0.15). CONCLUSION: The findings of this study enriched our understanding of several meaningful dimensions of the caregiving burden in caregivers of an amyotrophic lateral sclerosis population and provided opportunities for future intervention.

Caregiver burden in amyotrophic lateral sclerosis: A systematic review.

BACKGROUND: Informal caregivers of patients with amyotrophic lateral sclerosis experience increased levels of caregiver burden as the disease progresses. Insight in the factors related to caregiver burden is needed in order to develop supportive interventions. AIM: To evaluate the evidence on patient and caregiver factors associated with caregiver burden in amyotrophic lateral sclerosis informal caregivers. DESIGN: A systematic review. DATA SOURCES: Four electronic databases were searched up to 2017. Studies that investigated quantitative relations between patient or caregiver factors and caregiver burden were included. The overall quality of evidence for factors was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach. RESULTS: A total of 25 articles were included. High quality of evidence was found for the relation between caregiver burden and the factor "behavioral impairments." Moderate quality of evidence was found for the relations between caregiver burden and the factors "feelings of depression" of the caregiver and "physical functioning" of the patient. The remaining rated caregiver factors-"feelings of anxiety," "distress," "social support," "family functioning," and "age"-and patient factors-"bulbar function," "motor function," "respiratory function," "disease duration," "disinhibition," "executive functioning," "cognitive functioning," "feelings of depression," and "age"-showed low to very low quality of evidence for their association with caregiver burden. CONCLUSION: Higher caregiver burden is associated with greater behavioral and physical impairment of the patient and with more depressive feelings of the caregiver. This knowledge enables the identification of caregivers at risk for caregiver burden and guides the development of interventions to diminish caregiver burden.

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

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