Delivering bad news in amyotrophic lateral sclerosis: Proposal of a specific technique ALS ALLOW

2020 ◽  
pp. 10.1212/CPJ.0000000000000957
Author(s):  
Wesleigh F. Edwards ◽  
Sahana Malik ◽  
Jonathan Peters ◽  
Ivy Chippendale ◽  
John Ravits
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Communication Skills ◽  
Physician Communication ◽  
Emotional Trauma ◽  
Specific Technique ◽  
Als Patients ◽  
Delivering Bad News ◽  
Late Stages ◽  
Lateral Sclerosis

AbstractPurposeof Review: Physician communication skills are a critical part of care for amyotrophic lateral sclerosis (ALS) patients and caregivers. They shape the development of autonomy and quality of life, and they mitigate emotional trauma. Communication skills are especially critical at two different time points in the course of disease: early when delivering and establishing the diagnosis, and later when clarifying goals of care.Recent Findings:Several techniques for physician communication of difficult information are available, including SPIKES, ABCDE, and BREAKS. These emphasize the physician’s accountability and responsibility for communicating effectively. Formal training in these techniques is limited and their applicability specifically to ALS is inexact.Summary:We propose an ALS-specific technique which we call ALS ALLOW to guide physicians in conducting difficult communications with ALS patients and caregivers to develop their understanding, establish autonomy, set goals, and mitigate emotional trauma. The techniques are useful in discussions both early and late stages in disease.

scholarly journals Profile of Patients with Amyotrophic Lateral Sclerosis Across Continuum of Care

2014 ◽  
Vol 41 (2) ◽  
pp. 246-252 ◽  
Author(s):  
Vahe Kehyayan ◽  
Lawrence Korngut ◽  
Nathalie Jetté ◽  
John P. Hirdes
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Home Care ◽  
Comparison Group ◽  
Cross Sectional Study ◽  
Assessment Instrument ◽  
Cross Sectional ◽  
Home Care Nursing ◽  
Als Patients ◽  
Lateral Sclerosis

Objective:This study describes the socio-demographic and clinical profile of persons with amyotrophic lateral sclerosis (ALS) in home care, nursing homes and complex continuing care settings in several Canadian jurisdictions.Methods:A cross-sectional study was conducted using available Resident Assessment Instrument (RAI 2.0 and RAI Home Care) national databases from 1996- 2011. The profile of ALS patients was compared with patients without pre-specified neurological conditions.Results:There were 2,092 ALS patients identified in these settings. Persons with ALS were more likely than those in the comparison group to suffer from health instability (25.4%) and minor to major depressive symptoms (27.2%) , to experience falls (44.0%) and weight loss (22.9%), to require extensive assistance in activities of daily living (54.9%), and to receive rehabilitation services: physical (23.9%), speech language pathology (8.9%), and occupational therapy 43.3%).Conclusions:The ALS population in this study are greatly affected by a number of health issues. They are more likely than the comparison group to require therapies, medical interventions, and psychotropic drug use. While persons with ALS have a poor prognosis, a great deal could be done to enhance their quality of life and the quality of care they receive.

Pathogenesis and management of pain in amyotrophic lateral sclerosis

2021 ◽  
Vol 25 (2) ◽  
Author(s):  
Hanik Badriyah Hidayati ◽  
Celine Anindytha Pranata
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Population Aging ◽  
Advanced Stage ◽  
Key Word ◽  
Depression Management ◽  
Als Patients ◽  
Global Population ◽  
Cells Death ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease is characterized by deadly progressive motor neuron disease and other neuronal cells death, which is featured by progressive paralysis and leads to advanced disability and mortality due to respiratory failure.1,2 By the year of 2040, ALS is estimated to nearly double due to global population aging.3 Pain is one of the overlooked symptom, but widely complained by patients with ALS. It can arise at any stage of the disease; and the intensity of pain may increase with the course of the disease. The pain characteristic depends upon its pathogenesis in inducing pain such as nociceptive, neuropathic, or painful cramps. Pain intensity can be severe at an advanced stage of ALS thereby increasing the use of pain relievers and sedatives. It has been related to a declined functional status leading to a decreased quality of life and escalating the rate of depression. Management in ALS patients with complaints of pain differs according to the multifactorial character of pain.4,5 The different aspects of pain in ALS has not been much discussed. Therefore, this article will provide an overview about it. Key word: Pain; Amyotrophic lateral sclerosis; ALS; Pathogenesis; Pain management Citation: Hidayati HB, Pranata CA. Pathogenesis and management of pain in amyotrophic lateral sclerosis. Anaesth. pain intensive care 2021;25(2):236-243. DOI: 10.35975/apic.v25i2.1478 Received: 26 November 2020, Reviewed: 27 December 2021, Accepted: 21 February 2021

Quality of life, disability, and clinical variables in amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Mariana Asmar Alencar ◽  
Izaura Monique Moura da Silva ◽  
Stéfanie Marcelle Hilário ◽  
Marcela Ferreira de Andrade Rangel ◽  
Juliana Silva Abdo ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Amyotrophic Lateral Sclerosis ◽  
Progressive Increase ◽  
Clinical Aspects ◽  
Strongly Correlated ◽  
Functional Aspects ◽  
Fatigue Severity ◽  
Als Patients ◽  
Lateral Sclerosis

ABSTRACT Background: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease that results in a progressive increase in dysfunctions, limitations and restrictions over time, which can impact on quality of life (QoL). Therefore, expanding knowledge on QoL and possible factors associated with ALS can enable the development of actions to ensure greater wellbeing for the population. Objective: To investigate QoL in ALS and determine associations with demographic, functional and clinical aspects. Methods: Forty-five individuals with ALS (56.4±11.1 years) participated in the study. Demographic, clinical and functional aspects were investigated. Functioning and QoL were assessed using disease-specific tools (ALS Functional Ranting Scale-Revised/ALSFRS-R and ALS Assessment Questionnaire/ALSAQ-40). Fatigue was assessed using the Fatigue Severity Scale. Descriptive, correlation and stepwise multiple linear regression analyses were performed with the aid of the SPSS. Results: The mean ALSAQ-40 score was 279.0±118.3. QoL was significantly worse among women (p=0.001) and poor QoL was associated with the inability to walk (p=0.014), pain (p=0.021) and disease severity (p≤0.002). QoL was strongly correlated with the ALSFRS-R score (r=-0.82). Moderate to weak correlations were found for mobility [turning in bed (r=-0.62), locomotion (r=-0.33) and sit to stand (r=-0.40)], strength (r=-0.49), fatigue (r=0.35) and pain (r=-0.32) (p<0.03). The regression analysis revealed that the ALSFRS-R score (β=-0.76; p=0.00) and fatigue (β=0.20; p=0.04) were predictors of QoL. Conclusions: QoL was worse in women, older people, severe stages of ALS, patients with impaired mobility, those with a poorer physical performance and those who reported pain. Functional status and fatigue are predictors of QoL in ALS.

scholarly journals A Word Communication System with Partner Assist for Amyotrophic Lateral Sclerosis Patients in Late Stages

2021 ◽  
Author(s):  
Kuniaki Ozawa ◽  
Masayoshi Naito, ◽  
Naoki Tanaka ◽  
Shiryu Wada
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Communication System ◽  
Near Infrared ◽  
Active Role ◽  
Communication Aid ◽  
Wh Questions ◽  
The One ◽  
Als Patients ◽  
Late Stages ◽  
Lateral Sclerosis

People with severe physical impairment such as amyotrophic lateral sclerosis (ALS) in a completely locked-in state (CLIS) suffer from inability to express their thoughts to others. To solve this problem, many brain-computer interface (BCI) systems have been developed, but they have not proven sufficient for CLIS. In this paper, we propose a word communication system: a BCI with partner assist, in which partners play an active role in helping patients express a word. We report here that five ALS patients in late stages (one in CLIS and four almost in CLIS) succeeded in expressing their own words (in Japanese) in response to wh-questions that could not be answered “yes/no.” Each subject sequentially selected vowels (maximum three) contained in the word that he or she wanted to express, by using a “yes/no” communication aid based on near-infrared spectroscopy. Then, a partner entered the selected vowels into a dictionary with vowel entries, which returned candidate words having those vowels. When there were no appropriate words, the partner changed one vowel and searched again or started over from the beginning. When an appropriate word was selected, it was confirmed by the subject via “yes/no” answers. Two subjects confirmed the selected word six times out of eight (credibility of 91.0% by a statistical measure); two subjects, including the one in CLIS, did so five times out of eight (74.6%); and one subject did so three times out of four (81.3%). We have thus taken the first step toward a practical word communication system for such patients.

scholarly journals A Word Communication System with Partner Assist for Amyotrophic Lateral Sclerosis Patients in Late Stages

2021 ◽  
Author(s):  
Kuniaki Ozawa ◽  
Masayoshi Naito, ◽  
Naoki Tanaka ◽  
Shiryu Wada
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Communication System ◽  
Near Infrared ◽  
Active Role ◽  
Communication Aid ◽  
Wh Questions ◽  
The One ◽  
Als Patients ◽  
Late Stages ◽  
Lateral Sclerosis

People with severe physical impairment such as amyotrophic lateral sclerosis (ALS) in a completely locked-in state (CLIS) suffer from inability to express their thoughts to others. To solve this problem, many brain-computer interface (BCI) systems have been developed, but they have not proven sufficient for CLIS. In this paper, we propose a word communication system: a BCI with partner assist, in which partners play an active role in helping patients express a word. We report here that five ALS patients in late stages (one in CLIS and four almost in CLIS) succeeded in expressing their own words (in Japanese) in response to wh-questions that could not be answered “yes/no.” Each subject sequentially selected vowels (maximum three) contained in the word that he or she wanted to express, by using a “yes/no” communication aid based on near-infrared spectroscopy. Then, a partner entered the selected vowels into a dictionary with vowel entries, which returned candidate words having those vowels. When there were no appropriate words, the partner changed one vowel and searched again or started over from the beginning. When an appropriate word was selected, it was confirmed by the subject via “yes/no” answers. Two subjects confirmed the selected word six times out of eight (credibility of 91.0% by a statistical measure); two subjects, including the one in CLIS, did so five times out of eight (74.6%); and one subject did so three times out of four (81.3%). We have thus taken the first step toward a practical word communication system for such patients.

scholarly journals A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

2021 ◽  
Vol 11 (3) ◽  
pp. 372
Author(s):  
Tara Peseschkian ◽  
Isabell Cordts ◽  
René Günther ◽  
Benjamin Stolte ◽  
Daniel Zeller ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Amyotrophic Lateral Sclerosis ◽  
Rating Scale ◽  
Severe Disease ◽  
Disease Stage ◽  
Individualized Care ◽  
Als Patients ◽  
The Impact ◽  
Lateral Sclerosis

Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL (HRQoL) in 325 ALS patients was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5) and EuroQol Five Dimension Five Level Scale (EQ-5D-5L), together with disease severity (captured by the revised ALS Functional Rating Scale (ALSFRS-R)) and the current care and therapies used by our cohort. At inclusion, the mean ALSAQ-5 total score was 56.93 (max. 100, best = 0) with a better QoL associated with a less severe disease status (β = −1.96 per increase of one point in the ALSFRS-R score, p < 0.001). “Limb-onset” ALS (lALS) was associated with a better QoL than “bulbar-onset” ALS (bALS) (mean ALSAQ-5 total score 55.46 versus 60.99, p = 0.040). Moreover, with the ALSFRS-R as a covariate, using a mobility aid (β = −7.60, p = 0.001), being tracheostomized (β = −14.80, p = 0.004) and using non-invasive ventilation (β = −5.71, p = 0.030) were associated with an improved QoL, compared to those at the same disease stage who did not use these aids. In contrast, antidepressant intake (β = 5.95, p = 0.007), and increasing age (β = 0.18, p = 0.023) were predictors of worse QoL. Our results showed that the ALSAQ-5 was better-suited for ALS patients than the EQ-5D-5L. Further, the early and symptom-specific clinical management and supply of assistive devices can significantly improve the individual HRQoL of ALS patients. Appropriate QoL questionnaires are needed to monitor the impact of treatment to provide the best possible and individualized care.

Riluzole Exhibits No Therapeutic Efficacy on a Transgenic Rat model of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 17 (3) ◽  
pp. 275-285 ◽  
Author(s):  
Si Chen ◽  
Qiao Liao ◽  
Ke Lu ◽  
Jinxia Zhou ◽  
Cao Huang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Rat Model ◽  
Microglial Activation ◽  
Transgenic Rat ◽  
Intragastric Administration ◽  
Behavioral Deficits ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Transgenic Rat Model

Background: Amyotrophic lateral sclerosis (ALS) is a neurological disorder clinically characterized by motor system dysfunction, with intraneuronal accumulation of the TAR DNAbinding protein 43 (TDP-43) being a pathological hallmark. Riluzole is a primarily prescribed medicine for ALS patients, while its therapeutical efficacy appears limited. TDP-43 transgenic mice are existing animal models for mechanistic/translational research into ALS. Methods: We developed a transgenic rat model of ALS expressing a mutant human TDP-43 transgene (TDP-43M337V) and evaluated the therapeutic effect of Riluzole on this model. Relative to control, rats with TDP-43M337V expression promoted by the neurofilament heavy subunit (NEF) gene or specifically in motor neurons promoted by the choline acetyltransferase (ChAT) gene showed progressive worsening of mobility and grip strength, along with loss of motor neurons, microglial activation, and intraneuronal accumulation of TDP-43 and ubiquitin aggregations in the spinal cord. Results: Compared to vehicle control, intragastric administration of Riluzole (30 mg/kg/d) did not mitigate the behavioral deficits nor alter the neuropathologies in the transgenics. Conclusion: These findings indicate that transgenic rats recapitulate the basic neurological and neuropathological characteristics of human ALS, while Riluzole treatment can not halt the development of the behavioral and histopathological phenotypes in this new transgenic rodent model of ALS.

Plasma Creatinine Level Does Not Predict Respiratory Function in Amyotrophic Lateral Sclerosis

2021 ◽  
pp. 1-5
Author(s):  
João Morgadinho ◽  
Ana Catarina Pronto-Laborinho ◽  
Vasco A. Conceição ◽  
Marta Gromicho ◽  
Susana Pinto ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Creatinine Level ◽  
Cox Regression ◽  
Functional Decline ◽  
Plasma Creatinine ◽  
Plasma Creatinine Level ◽  
Decline Rate ◽  
Respiratory Outcome ◽  
Als Patients ◽  
Lateral Sclerosis

In amyotrophic lateral sclerosis (ALS) lower plasma creatinine level has been associated with shorter survival and faster functional decline. It has not been clear if creatinine is associated with respiratory outcome. We analyzed retrospectively a population of unselected ALS patients. Multiple-regression and Cox-regression analyses were performed. We included 233 patients, mean age 62.8, mean disease duration of 18.6 months. At baseline, creatinine was significantly associated with ALSFRS-R, but not with its decline rate. No predictive value was disclosed for FVC, or their decline rate, or with survival. We did not confirm that creatinine is a marker of respiratory outcome.

Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review

2021 ◽  
Author(s):  
Georgiana Soares Leandro ◽  
Mário Emílio Teixeira Dourado Júnior ◽  
Glauciane Costa Santana ◽  
Luan Samy Xavier Dantas
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Coping Strategies ◽  
Integrative Review ◽  
Als Patients ◽  
Lateral Sclerosis
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