Amyotrophic Lateral Sclerosis Multidisciplinary Clinics Research Articles

A Clinical Focus on Shared Decision Making in Clinical Practice When Providing Dysarthria and Dysphagia Services to Individuals With Amyotrophic Lateral Sclerosis

Purpose: Traditional health care decision-making models center on clinicians making decisions for patients/caregivers based on the best available research evidence. However, this diminishes patient/caregiver involvement in their care and hinders the ability to align care plans with patient values and preferences. Shared decision making is a potentially beneficial process to implement with individuals with amyotrophic lateral sclerosis (ALS) to provide more holistic, patient-centered dysarthria and dysphagia treatment. Shared decision making promotes active involvement by patients/caregivers by informing them of potential treatment options, understanding their values and preferences, and aligning their desires with treatment options to determine the most optimal individualized care plan. Conclusions: The benefits and barriers to incorporating shared decision making within ALS multidisciplinary clinics are discussed in this clinical focus article. Furthermore, a fictional case study example of how to apply shared decision making to dysarthria and dysphagia management of individuals with ALS is provided.

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 3years. 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 90days was lower than of those alive beyond 90days (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.

“Re-identifying yourself”: a qualitative study of veteran views on implantable BCI for mobility and communication in ALS

Objectives Brain-computer interface (BCI) technology to assist with mobility and communication is an active area of research in amyotrophic lateral sclerosis (ALS). Implantable BCI offers promise for individuals with severe disease, such as locked-in syndrome, but also raises important ethical issues. We undertook in-depth qualitative interviews with ALS patients from a Veterans Administration hospital ALS multi-disciplinary clinic and explored their perspectives on issues of identity, privacy, enhancement, informed consent, and responsibility related to implantable BCI. Methods Semi-structured interviews were conducted with sixteen (n = 16) individuals, and transcripts were analysed using a modified grounded theory approach. Results Emergent themes included: (1) attitudes towards BCI were characterised by fear, hope, and hesitation about adoption of BCI technology; (2) analogies to other technologies were a useful tool in understanding and communicating opinions about ethical issues in BCI; (3) concerns about potentially socially stigmatising effects of BCI and the burden of adjustment to new therapeutic devices were important considerations to be weighed against the potential functional benefit of BCI use; (4) therapeutic decision-making in ALS often intimately involves loved ones; and (5) prospective decision-making about BCI was significantly affected by weighing the timing of the intervention with the progression of illness. Conclusion The interest in BCI and views on ethical issues raised by BCI is moderated by the experience of living with ALS. The findings from this study can help guide the development of implantable BCI technology for persons with ALS. Implications for rehabilitation Loved ones will play crucial roles in helping patients think through the possible benefits and burdens of getting a BCI device. Providers should consider how the ideal timing for getting an implantable BCI device will vary based on the priorities of persons with ALS and their disease stage. Concerns about social stigma, burden of adjustment, and the desire to maximise time left with loved ones may outweigh the potential functional benefits of BCI devices for some persons with ALS.

Practical implementation of noninvasive ventilation in Amyotrophic Lateral Sclerosis: lessons learned from a clinical case series.

PurposeNoninvasive ventilation (NIV) may improve survival and quality of life in Amyotrophic Lateral Sclerosis (ALS) patients. There is a surprising paucity of practical guidelines for office-based implementation and management of NIV outside of tertiary ALS centers. We saw the need for a clinical protocol to allow feasible and consistent NIV management in this patient population.MethodsWe created a clinical protocol for office-based initiation of NIV implemented on consecutive ALS patients referred from our regional ALS multidisciplinary clinic. The protocol provided initial empiric settings using a bilevel device in volume-assured pressure support mode. A respiratory therapist (RT) initiated NIV in an office setting and made adjustments according to patient tolerance and therapy targets outlined in the protocol. Later setting changes were performed at patient or provider request. We evaluated patient adherence and efficacy via device download at 30 days and 1 year.ResultsWe present data from a case series of the first 14 consecutive patients initiated on NIV over a 20-month period. Our protocol underwent iterative modification based on clinical experience and patient feedback. Early challenges included the significant time and resource burden required to coordinate device downloads and patient follow-up. Early 30-day NIV adherence was variable (median 20 out of 30 days), while 1-year NIV adherence was excellent (median 27.5 out of 30 days).ConclusionsOur RT-driven clinical NIV protocol was feasible but labor intensive. Achieving real-world adherence of NIV in our ALS patients required iterative protocol adjustment, significant RT provider time, and tele-based follow-up.

Patient perspectives on transitioning to amyotrophic lateral sclerosis multidisciplinary clinics.

PurposeMultidisciplinary clinics (MDC) have become the standard of care for management of amyotrophic lateral sclerosis (ALS). No studies however, have captured patients’ perspectives during a transition to ALS MDCs. Recently, an ALS MDC emerged from a single-physician clinic in Saskatoon, Canada, providing patients with a unique exposure to two different models of care.Patients and methodsFifteen patients with ALS participated in semi-structured interviews that were digitally recorded and transcribed. Two independent researchers performed an inductive thematic analysis. Information was coded based on emerging and a priori themes. An iterative process followed involving discussion and reexamination of the themes until consensus was reached.ResultsAll patients cited the convenience of integrated care as an advantage. Other advantages included clinical expertise and advocacy potential. Travel and reduced mobility were the most commonly discussed barriers/disadvantages of MDC attendance. The impact of geography and weather appeared to augment both the appreciation of an integrated approach and the impediment of travel, compared to the existing literature. The need for individualized care was demonstrated by the conflicting viewpoints obtained from participants. Most patients felt additional practitioners and supports for both patients and caregivers were required.ConclusionALS patients transitioned to MDCs reported many of the advantages and disadvantages reported elsewhere. A novel perspective of a MDC’s advocacy potential was recognized, and the need for an innovative approach to meet demands for individualized care was highlighted.

Perceptions of sexuality in individuals with Amyotrophic Lateral Sclerosis (ALS) and their treating clinicians.

Amyotrophic Lateral Sclerosis (ALS) may not affect an individual's sexual function directly, but it can indirectly impact their sexual activity. Sexual partners often become caregivers, diminishing sexuality within a relationship. This can result in decline of quality of life. The study aimed to explore the perspectives of individuals with ALS and their treating clinicians regarding the importance of sexuality in rehabilitation within a multidisciplinary ALS center. We hypothesize that individuals with ALS will express the need for sexuality-related discussions as a therapy. Electronic 11-item questionnaires were anonymously completed by individuals with ALS (n = 21) and ALS healthcare professionals (n = 81) between August 2014 to June 2016. Descriptive statistics were performed in STATA 14. Majority (90%, n = 92) of respondents stated that ALS impacts the sexuality of an individual with ALS, and agreed that sexuality-related discussion is needed as a complementary therapy. Over 75% of clinicians reported they were not familiar with any strategies or interventions to help the patients. ALS indirectly affects sexuality, thus confirming the need for promoting awareness regarding sexuality-related topics among individuals with ALS and healthcare professionals. ALS multidisciplinary clinics need to improve their delivery of care to address sexual rehabilitation as a complementary therapy.

Translation and Psychometric Evaluation of a Korean Version of the Amyotrophic Lateral Sclerosis-Specific Quality of Life – Revised

Objective: The objective was to translate the Amyotrophic Lateral Sclerosis-Specific Quality of Life - Revised instrument (ALSSQOL-R) into Korean and to examine the psychometric properties of the Korean amyotrophic lateral sclerosis (ALS) population.Methods: The translation involved forward and backward translation. The psychometric properties of the Korean version of the ALSSQOL-R (K-ALSSQOL-R) were tested by patients in an ALS multidisciplinary clinic in Korea (n = 120). The internal consistency, test-retest reliability, content validity, criterion-related validity, and construct validity were evaluated.Results: With regard to the reliability estimate, the internal consistency (Cronbach’s alpha = 0.92) and the test–retest reliability (ICC = 0.89) were good. With regard to the validity estimate, the K-ALSSQOL-R demonstrated concurrent validity with the McGill Quality of Life Single-Item Scale (r = 0.62) and the first question of the World Health Organisation QOL Instrument-Brief (WHOQOL-BREF) (r = 0.64). The convergent validity and discriminant validity were acceptable. The confirmatory factor analysis supported a six-factor model.Conclusion: The K-ALSSOQL-R can be used as a reliable and valid measure of QOL among Korean ALS patients.

Patient journey to a specialist amyotrophic lateral sclerosis multidisciplinary clinic: an exploratory study.

BackgroundThe multidisciplinary approach in the management of Amyotrophic Lateral Sclerosis (ALS) has been shown to provide superior care to devolved care, with better survival, improved quality of care, and quality of life. Access to expert multidisciplinary management should be a standard for patients with ALS. This analysis explores the patient journey from symptom onset and first engagement with health services, to the initial visit to a specialist ALS Multidisciplinary Clinic (MDC) in Dublin, Ireland.MethodsA retrospective exploratory multi-method study details the patient journey to the MDC. Data from medical interviews and systematic chart review identifies interactions with the health services and key timelines for thirty five new patients presenting with a diagnosis of ALS during a 6 month period in 2013.ResultsThe time from first symptom to diagnosis was a mean of 16 months (median 13 months), with a mean interval of 19 months (median 14.6) from first symptoms to arrival at the MDC. The majority of patients were seen by a general practitioner, and subsequently by neurology services. There was an average of four contacts with health services and 4.8 investigations/tests, prior to their first Clinic visit. On the first visit to the MDC patients are linked into an integrated ‘system’ that can provide specialist care and link with voluntary, palliative and community services as required.ConclusionsEngagement with a multidisciplinary team has implications for service utilization and quality of life of patients and their families. We have demonstrated that barriers exist that delay referral to specialist services. Comprehensive data recording and collection, using multiple data sources can reconstruct the timelines of the patient journey, which can in turn be used to identify pathways that can expedite early referral to specialist services.

Development of a model to guide decision making in amyotrophic lateral sclerosis multidisciplinary care

Patients with amyotrophic lateral sclerosis (ALS) face numerous decisions for symptom management and quality of life. Models of decision making in chronic disease and cancer care are insufficient for the complex and changing needs of patients with ALS . The aim was to examine the question: how can decision making that is both effective and patient-centred be enacted in ALS multidisciplinary care? Fifty-four respondents (32 health professionals, 14 patients and eight carers) from two specialized ALS multidisciplinary clinics participated in semi-structured interviews. Interviews were transcribed, coded and analysed thematically. Comparison of stakeholder perspectives revealed six key themes of ALS decision making. These were the decision-making process; patient-centred focus; timing and planning; information sources; engagement with specialized ALS services; and access to non-specialized services. A model, embedded in the specialized ALS multidisciplinary clinic, was derived to guide patient decision making. The model is cyclic, with four stages: 'Participant Engagement'; 'Option Information'; 'Option Deliberation'; and 'Decision Implementation'. Effective and patient-centred decision making is enhanced by the structure of the specialized ALS clinic, which promotes patients' symptom management and quality of life goals. However, patient and carer engagement in ALS decision making is tested by the dynamic nature of ALS, and patient and family distress. Our model optimizes patient-centred decision making, by incorporating patients' cyclic decision-making patterns and facilitating carer inclusion in decision processes. The model captures the complexities of patient-centred decision making in ALS. The framework can assist patients and carers, health professionals, researchers and policymakers in this challenging disease environment.

What are the roles of carers in decision-making for amyotrophic lateral sclerosis multidisciplinary care?

PurposeFamily carers of patients with amyotrophic lateral sclerosis (ALS) are presumed to have frequent involvement in decision-making for symptom management and quality of life. To better understand and improve decision-making, we investigated the range and extent of carer participation in decision-making. By focusing on the perspectives of ALS support carers, the study aimed to explore carer participation in decision-making, to identify carer roles, and determine the facilitators and barriers to carer participation in decision-making for ALS multidisciplinary care.Participants and methodsAn exploratory, in-depth study was conducted with eight carers of ALS patients from two specialized ALS multidisciplinary clinics. Carers participated in semi-structured interviews that were audio recorded and transcribed then coded and analyzed for emergent themes.ResultsCarers made a significant contribution to ALS decision-making. Their roles were: promoting the patient voice, promoting patient health literacy, and providing emotional support and logistical assistance. Facilitators of carer participation in decision-making were perceived to be: health professional endorsement of patients’ decision-making style; access to credible information sources; evidence-based information from the ALS clinic, ALS support association, and health practitioners; supportive relationships with family and friends; spiritual faith; ease of contact with ALS services; and availability of physical and practical support for carers. Barriers to carer participation included: changes to patient communication and cognition; conflict between respect for patients’ independence and patients’ best interest; communication breakdown between patient, carer, and service providers; the confronting nature of disease information; credibility of Internet sites; carer coping strategies; lack of support for the carer; and the burden of care.ConclusionCarers enhance ALS patient-centered care through their participation in decision-making. They collaborate with patients and health professionals to form a decision-making triad within specialized multidisciplinary ALS clinical care. Nevertheless, health professional engagement with carers as collaborative partners is acknowledged to be a significant challenge.

What influences patient decision-making in amyotrophic lateral sclerosis multidisciplinary care? A study of patient perspectives

BackgroundPatients with amyotrophic lateral sclerosis (ALS) are required to make decisions concerning quality of life and symptom management over the course of their disease. Clinicians perceive that patients’ ability to engage in timely decision-making is extremely challenging. However, we lack patient perspectives on this issue. This study aimed to explore patient experiences of ALS, and to identify factors influencing their decision-making in the specialized multidisciplinary care of ALS.MethodsAn exploratory study was conducted. Fourteen patients from two specialized ALS multidisciplinary clinics participated in semistructured interviews that were audio recorded and transcribed. Data were analyzed for emergent themes.ResultsDecision-making was influenced by three levels of factors, ie, structural, interactional, and personal. The structural factor was the decision-making environment of specialized multidisciplinary ALS clinics, which supported decision-making by providing patients with disease-specific information and specialized care planning. Interactional factors were the patient experiences of ALS, including patients’ reaction to the diagnosis, response to deterioration, and engagement with the multidisciplinary ALS team. Personal factors were patients’ personal philosophies, including their outlook on life, perceptions of control, and planning for the future. Patient approaches to decision-making reflected a focus on the present, rather than anticipating future progression of the disease and potential care needs.ConclusionDecision-making for symptom management and quality of life in ALS care is enhanced when the patient’s personal philosophy is supported by collaborative relationships between the patient and the multidisciplinary ALS team. Patients valued the support provided by the multidisciplinary team; however, their focus on living in the present diverged from the efforts of health professionals to prepare patients and their carers for the future. The challenge facing health professionals is how best to engage each patient in decision-making for their future needs, to bridge this gap.

ALS multidisciplinary clinic and survival

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motorneurons, for which there is no effective cure. Because of the multifactorial nature of impairment and disablity in ALS, multidisciplinary clinics (MDC) have been recently introduced in the management of ALS patients; their effects on survival remain, however, largely debated. To compare survival of ALS patients who received their care at MDC with that of patients followed by general neurology clinics. Source of the study was a prospective population-based registry of ALS established in Puglia, Southern Italy, in 1997. We examined survival of 126 out of 130 incident ALS cases that were diagnosed during the period 1998-99. 84 patients (67%) were enrolled and followed by MDC and the remaining 42 (33%) by general neurological clinics. No difference in median survival time from the diagnosis was observed between patients followed by ALS multidisciplinary (17.6 months) and general clinics (18 months). No beneficial effect was present among bulbar onset ALS (11.7 versus 23 months). In multivariate analysis management by ALS MDC was associated with only a 10% increase in survival probability at 12 months (HR: 0.91; 95%CI: 0.44-1.89; p = 0.9). In this population-based series, we found that in Southern Italy management of ALS by multidisciplinary clinics does not improve survival, regardless of site of symptoms onset.