Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications

Andersen, Peter M.; Kuźma‐Kozakiewicz, Magdalena; Keller, Jürgen; Aho-Oezhan, Helena; Ciećwierska, Katarzyna; Szejko, Natalia; Vázquez, Cynthia; Böhm, Sarah; Badura-Lotter, Gisela; Meyer, Thomas; Petri, Susanne; Linse, Katharina; Hermann, Andreas; Semb, Olof; Stenberg, Erica; Nackberg, Simona; Dorst, Johannes; Uttner, Ingo; Häggström, Ann-Cristin; Ludolph, Albert C.; Lulé, Dorothée

doi:10.1007/s00415-018-8861-4

Cited by 41 publications

(46 citation statements)

References 29 publications

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“…Historical literature and current USA medical insurance standards recommend Bi-PAP usage be prescribed to patients only once their FVC %predict falls below 50%, unless a precipitous decline is noted or dyspnea is observed ( 11 , 41 ). However, other international ALS clinics have certainly promoted earlier non-invasive for several years based on their own clinical observations, which supported earlier non-invasive ventilation paradigms like Bi-PAP ( 42 ). The presented analyses showed significant ( p < 0.01) increases in survival duration for those starting ≥ 60, ≥ 70, ≥ 80, or ≥ 90 FVC %predict when compared to those starting at ≤ 50 FVC %predict.…”

Section: Discussionmentioning

confidence: 82%

Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols

et al. 2018

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Objective: It is hypothesized earlier non-invasive (NIV) ventilation benefits Amyotrophic Lateral Sclerosis (ALS) patients. NIV typically consists of the removable bi-level positive airway pressure (Bi-PAP) for adjunctive respiratory support and/or the cough assist intervention for secretion clearance. Historical international standards and current USA insurance standards often delay NIV until percent predicted forced vital capacity (FVC %predict) is <50. We identify the optimal point for Bi-PAP initiation and the synergistic benefit of daily Bi-PAP and cough assist on associative increases in survival duration.Methods: Study population consisted of a retrospective ALS cohort (Emory University, Atlanta, GA, USA). Primary analysis included 474 patients (403 Bi-PAP users, 71 non-users). Survival duration (time elapsed from baseline onset until death) is compared on the basis of Bi-PAP initiation threshold (FVC %predict); daily Bi-PAP usage protocol (hours/day); daily cough assist usage (users or non-users); ALS onset type; ALSFRS-R score; and time elapsed from baseline onset until Bi-PAP initiation, using Kruskal-Wallis one-way analysis of variance and Kaplan Meier.Results: Bi-PAP users' median survival (21.03 months, IQR = 23.97, N = 403) is significantly longer (p < 0.001) than non-users (13.84 months, IQR = 11.97, N = 71). Survival consistently increases (p < 0.01) with FVC %predict Bi-PAP initiation threshold: <50% (20.3 months); ≥50% (23.60 months); ≥80% (25.36 months). Bi-PAP usage >8 hours/day (23.20 months) or any daily Bi-PAP usage with cough assist (25.73 months) significantly (p < 0.001) extends survival compared to Bi-PAP alone (15.0 months). Cough assist without Bi-PAP has insignificant impact (14.17 months) over no intervention (13.68 months). Except for bulbar onset Bi-PAP users, higher ALSFRS-R total scores at Bi-PAP initiation significantly correlate with higher initiation FVC %predict and longer survival duration. Time elapsed since ALS onset is not a good predictor of when NIV should be initiated.Conclusions: The “optimized” NIV protocol (Bi-PAP initiation while FVC %predict ≥80, Bi-PAP usage >8 h/day, daily cough assist usage) has a 30. 8 month survival median, which is double that of a “standard” NIV protocol (initiation FVC %predict <50, usage >4 h/day, no cough assist). Earlier access to Bi-PAP and cough assist, prior to precipitous respiratory decline, is needed to maximize NIV synergy and associative survival benefit.

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Section: Discussionmentioning

confidence: 82%

Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols

et al. 2018

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“…Although there is a significant and growing body of literature on decision making within the MND population, the impact of communication and/or cognitive impairments does not emerge with any clarity. Instead, the broader extant literature focuses on particular interventions (namely, ventilation and gastrostomy) [33][34][35][36][37][38][39] , the broad process of decision making (from the perspective of the patient, carer, or health care professional) 10,12,13,32,[40][41][42] , or cognitive assessment of decision-making ability. 43,44 However, this information is rarely extrapolated to consider patients' personal decision making.…”

Section: Amyotrophic Lateral Sclerosis and Assisted Ventilationmentioning

confidence: 99%

Communication and cognitive impairments and health care decision making in MND: A narrative review

Paynter

Cruice

Mathers

et al. 2019

Evaluation Clinical Practice

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Rationale Motor neurone disease (MND) is a neurodegenerative disease presenting with progressive weakness of voluntary muscles. For any condition, person‐centred health care relies on the sharing of information and a mutual understanding of the person's needs and preferences. Decision making in MND becomes more complex as there is no cure and a high prevalence of co‐morbid communication and/or cognitive difficulties. Objective To identify the reported impact of communication and/or cognitive impairment on patient and carer involvement in health care decision making in MND. Methods A review and synthesis of studies addressing issues of communication impairment and/or cognitive impairment in relation to decision making focussed on MND was conducted. Articles were excluded if they were reviews, case studies, conference papers, or commentaries. To be included studies needed to address issues of communication impairment or cognitive impairment specifically in relation to decision making. Relevant data were extracted verbatim and subjected to content analysis to support the narrative summary. Results Seventy‐six articles were identified, and 35 articles screened. Six articles met inclusion criteria each describing examples of decision making in MND. There was limited data related to communication and/or cognitive impairment, and the impact these impairments may have on decision making despite recognition that many people with MND may lose verbal communication or develop subtle cognitive impairments. The literature is primarily from the perspective of others. Conclusion This review highlights that the current body of literature exploring decision making within the MND population presents us with extremely limited insights into the impact of communication and/or cognitive impairments on health care decision making. Extant literature focuses on interventions (namely, ventilation and gastrostomy), the broad process of decision making, or cognitive assessment of decision‐making ability. Whilst most studies acknowledge that deficits in communication or cognition impact the decision‐making process, this issue is not the focus of any study.

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“…This was consequently considered during the regular appointments at our center, because it requires early counselling of patients and their relatives, even in stages when no dysphagia is present, in order to foster an informed decision making. In addition, decisions of patients may shift, e.g., as a complex result of their dynamic emotional response to the disease as well as their socio-cultural background [38][39][40]. Thus, a minority of the 15 patients who decided against a gastrostomy, might change their decision.…”

Section: Discussionmentioning

confidence: 99%

Triage of Amyotrophic Lateral Sclerosis Patients during the COVID-19 Pandemic: An Application of the D50 Model

Steinbach

Prell

Gaur

et al. 2020

JCM

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Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease, the management of which requires the continuous provision of multidisciplinary therapies. Owing to the novel coronavirus disease (COVID-19) pandemic, regular contact with ALS patients at our center was severely restricted and patient care was at risk by delay of supportive therapies. We established a triage system based on the D50 disease progression model and were thus able to identify a prospective cohort with high disease aggressiveness (D50 < 30). Thirty-seven patients with highly aggressive disease were actively offered follow-up, either via telephone or on-site, depending on their disease-specific needs and abilities. We describe here the procedures, obstacles, and results of these prescient efforts during the restrictions caused by COVID-19 in the period between March and June 2020. In conclusion, four patients with highly aggressive disease were initiated with non-invasive ventilation and two received a gastrostomy. We could show that a comparable amount of advanced care was induced in a retrospective cohort within a similar time period one year prior to the COVID-19 outbreak. Our workflow to identify high-risk patients via D50 model metrics can be easily implemented and integrated within existing centers. It helped to maintain a high quality of advanced care planning for our ALS patients.

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Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications

Cited by 41 publications

References 29 publications

Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols

Associative Increases in Amyotrophic Lateral Sclerosis Survival Duration With Non-invasive Ventilation Initiation and Usage Protocols

Communication and cognitive impairments and health care decision making in MND: A narrative review

Triage of Amyotrophic Lateral Sclerosis Patients during the COVID-19 Pandemic: An Application of the D50 Model

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