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Overall survival from first ALS symptoms, inclusion and diagnosis (Kaplan Meier curves with 95% CI); n = 68 subjects. Median values provided in results section.
Source publication
Over a four-year period, ALS patients complied with the modalities of the multidisciplinary management follow-up without any drop-outs. The multidisciplinary management structure also contributes to increasing the experience and knowledge of the clinicians involved in managing patients suffering from this rare disease.
Contexts in source publication
Context 1
... evolution of the ALS-FRS-R scores over time in "bulbar" and "non-bulbar" subjects is detailed in appendix 1. Survival: Median survival time was 50 (34-95) months from the first symptoms, 35 (18-70) months from diagnosis and 24 (12-31) months from inclusion in the cohort ( fig. 3). Detailed survival probabilities are provided in table ...
Context 2
... evolution of the ALS-FRS-R scores over time in "bulbar" and "non-bulbar" subjects is detailed in appendix 1. Survival: Median survival time was 50 (34-95) months from the first symptoms, 35 (18-70) months from diagnosis and 24 (12-31) months from inclusion in the cohort ( fig. 3). Detailed survival probabilities are provided in table ...
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Citations
... 15 Alongside the person with ALS, multidisciplinary teams at ALS and neuromuscular centres support informal caregivers. 16,17 Further support comes from ALS patient organizations. 18 To the existing corpus of knowledge available on informal caregivers' needs, this article adds new information using interview data. ...
... 27 Multidisciplinary care for people with ALS and their families is provided primarily within neuromuscular centres specialized in ALS as well as neurological hospitals. 17 Generally, most people in Switzerland want to die at home, but they die in nursing homes and hospitals. 26 Palliative care support is varied throughout the country due to the federalist structure. ...
Objective: This study explores the supportive needs of informal caregivers of people with
amyotrophic lateral sclerosis (ALS) in Switzerland.
Method: We conducted semi-structured interviews with nine informal caregivers currently
providing care to a person with ALS, 14 bereaved informal caregivers, and 13 healthcare
professionals. Interviews were recorded on digital audio and analysed using an inductive
thematic analysis within a realist framework.
Result: Informal caregivers discussed five themes of support needs relating to being
overburdened by administrative demand, in contact with healthcare providers, home support,
especially during the terminal phase, and having or lacking social support. Healthcare
professionals discussed three themes of support needs of informal caregivers which related
to the general institutional support for informal caregivers, their own work as caring for
informal caregivers, and the challenges in healthcare for families with ALS they encountered.
Significance of results: Informal caregiving for people with ALS can be demanding. This
study provides evidence for improvements in supporting informal caregivers. It shows
administrative needs of informal caregivers, stresses their needs regarding advance care
planning early in the context of ALS, and underlines the importance of social support, be it in
peer-groups or community care.
... Team beruhende Betreuung einen Nutzen im Hinblick auf die Lebensqualität und das Überleben; dies trifft insbesondere auf ALS zu[5].Hinweis: Eine [HCO3 -]-Erhöhung legt den Verdacht auf eine nächtliche alveoläre Hypoventilation nahe. Bei einer neuromuskulären Erkrankung kann der Hustenstoss insuffizient werden, ausserdem steigt das Risiko von Aspirationen, die häufig stumm verlaufen. ...
... This cross-sectional controlled cohort study included adults diagnosed with ALS and participating in the clinical multidisciplinary follow-up of the Geneva University Hospitals (HUG) [16]. They were included at the start of their follow-up at the Geneva University Hospitals, while they had no enteral nutrition nor parenteral nutrition. ...
Background & Aims
Patients with amyotrophic lateral sclerosis (ALS) develop swallowing difficulties with the progression of the disease. The present study aimed at comparing oral function and body composition between ALS patients and healthy controls, and at evaluating which parameters are the most discriminant between both groups.
Methods
We included ALS patients at the start of their multidisciplinary follow-up at the Geneva University Hospitals and healthy age-, gender-, and dental status-matched adults. We assessed the severity of the disease through the ALS Functional Rating Scale and the swallowing difficulties through the EAT-10 score. We performed an intraoral examination of the dental status, and measured chewing performance, bite, lip and tongue force, saliva weight, and body composition. Group comparisons were performed with t-tests or Mann-Whitney tests as appropriate. Linear discriminant analysis was used to determine the most discriminant parameters between groups.
Results
Twenty-six ALS patients (bulbar onset: n=7, median (IQR) ALS Functional Rating Scale: 37(11)) were included. The ALS patients had a significantly lower chewing performance (p<0.001), lip force (p<0.001), tongue force (p=0.002), saliva weight (p<0.004) and fat-free mass index (p<0.001) as compared to the healthy individuals, and a higher EAT-10 score (p<0.001). In ALS patients, a low chewing performance was correlated with a low bite (r= −0.45, p<0.05)) and tongue force (r=−0.59, p<0.05). The most discrimant parameters between both groups, by order of importance, were chewing performance, fat-free mass index and saliva weight and allowed the calculation of a discriminant function.
Conclusion
Compared to healthy controls, ALS patients have significant alterations of oral function and body composition. The most discrimant parameters between both groups were chewing performance, fat-free mass index and saliva volume. It remains to be demonstrated whether oral parameters predict outcome.
Keywords
oral function
chewing efficiency
saliva
tongue force
lip force
fat-free mass
... Details of the multidisciplinary management structure have been previously reported [11]. Briefly, patients are seen every 3 months for a 1-day clinical evaluation in an outpatient day-care centre. ...
... Four patients suffered from dementia and were excluded, leaving 64 patients for analysis. The median time between initial symptoms and the first multidisciplinary management visit was 11.1 months (IQR [8][9][10][11][12][13][14][15][16][17][18]. The median number of visits was 4 (IQR 2-6), and the median number of palliative care consultations was 3 (IQR 2-4). ...
Introduction:
Although recommended, the implementation of early advance care planning is suboptimal in amyotrophic lateral sclerosis (ALS) patients. Barriers to advance care planning include healthcare professionals’ and patients’ reluctance, and uncertainty about the right time to initiate a discussion.
Aim of the study:
To determine how often advance care planning was initiated, and the content of the discussion in a first routine palliative care consultation integrated within a multidisciplinary management programme.
Methods:
Between June 2012 and September 2016, a prospective cohort study was conducted in Geneva University Hospitals. Sixty-eight patients were seen every 3 months for a 1-day clinical evaluation in a day care centre.
Results:
The patients’ mean ± standard deviation age was 68.6 ± 11.9 years, 50% were women. Four patients were excluded because of dementia. Advance care planning was initiated with 49 (77%) patients in the first palliative care consultation. Interventions most often addressed were cardiopulmonary resuscitation (49%), intubation and tracheostomy (47%) and palliative sedation (36.7%). Assisted suicide was discussed with 16 patients (36.6%). Functional disability was the only factor associated with initiation of advance care planning. Nearly half of the patients wrote advance directives (45%) or designated a healthcare surrogate (41%). Bulbar onset, functional disability and noninvasive ventilation were not associated with the completion of advance directives.
Conclusion:
Early initiation of advance care planning is feasible in most ALS patients during a routine consultation, and relevant treatment issues can be discussed. All ALS patients should be offered the opportunity to write advance directives as completion was not associated with disease severity. .
Purpose
This study sought to explore how patients with amyotrophic lateral sclerosis (ALS) presenting with coexisting bulbar and cognitive impairments and their caregivers experienced the speech-language pathologist (SLP) services provided in multidisciplinary ALS clinics in Canada and identified their perceived needs for bulbar symptom management.
Method
This qualitative study was informed by interpretive description. Seven interviews were conducted with patients with severe bulbar dysfunction or severe bulbar and cognitive dysfunction due to ALS or ALS-frontotemporal dementia, respectively, and/or their caregivers. Purposive sampling was used to recruit individuals with severe bulbar or bulbar and cognitive disease. Thematic analysis was used to analyze interview data.
Results
Patients and caregivers reported difficulties with accessing and receiving SLP services at the multidisciplinary ALS clinic. These difficulties were further exacerbated in those with severe cognitive disease. Participants expressed a need for more specific (i.e., disease and service-related) information and personalized care to address their changing needs and preferences. Engaging caregivers earlier in SLP appointments was perceived as vital to support care planning and provide in-time caregiver education.
Conclusions
This study highlighted the challenges experienced by patients and caregivers in accessing and receiving SLP services. There is a pressing need for a more person-centered approach to ALS care and a continuing need for education of SLPs on care provision in cases of complex multisymptom diseases within a multidisciplinary ALS clinic.
Supplemental Material
https://doi.org/10.23641/asha.24069222
Objective: This retrospective (case-control) collaborative study evaluates tendon reflex recordings combined with transcranial magnetic stimulation motor evoked potentials recordings (T-MEPs) at lower limbs in amyotrophic lateral sclerosis (ALS).
Methods: T-MEPs were recorded in 97 ALS patients distinguished according to their patellar reflex briskness at first consultation. Patients’ electrophysiological data were compared with values measured in 60 control patients matched for age and height. Correlations studies between parameters or with some patients’ clinical characteristics were also performed.
Results: The central motor conduction time yields the highest sensitivity (82%) and specificity (93%), allowing twice more upper motor neuron (UMN) dysfunction detection than clinical examination, and being more altered in late stages of the disease. The T response to MEP response amplitude ratio (T/MEP ar) is nearly as sensitive to detect ALS and better identifies abnormal hyperreflexia. It is not correlated with evolutive stage, contrarily to conduction time-related parameters. In addition, T-MEPs detects asymmetries escaping clinical examination.
Conclusion: The corticospinal conduction to lower limbs is slowed in ALS. The T/MEP ar helps deciding when patellar reflexes are abnormal in a given patient suspected of ALS.
Significance: The T-MEP technique provide powerful electrophysiological biomarkers of UMN involvement in ALS. This simple and painless procedure introduces the clinically useful concept of electrophysiological hyperreflexia and might be expanded to future exploration of proximal upper limbs and bulbar territories.
Introdução: A esclerose lateral amiotrófica (ELA) é uma doença degenerativa que provoca a destruição dos neurônios responsáveis pelo movimento dos músculos voluntários e leva, progressivamente, à paralisia. Os cuidados paliativos objetivam amparar o enfermo e sua família devido à impossibilidade de cura da doença. Objetivo: Este artigo objetivou responder a seguinte pergunta-guia: em pacientes portadores de Esclerose Lateral Amiotrófica, como é que os cuidados paliativos, comparados com nenhuma outra intervenção amenizadora, afetam os enfermos em seu estado terminativo de doença? Metodologia: A metodologia aplicada balizou-se no levantamento bibliográfico sistemático em duas bases de dados: Biblioteca Virtual em Saúde (BVS) e Pubmed. Os fatores de inclusão concentraram-se em trabalhos que avaliaram a relação entre ELA e intervenções paliativas ou ELA e qualidade de vida dos enfermos. Resultados: Constatou-se que a ventilação não-invasiva, o planejamento, a modulação medicamentosa ou comunicativa e a gastrostomia foram métodos importantes para prover a adaptação dos pacientes. No desfecho, identificou-se um múltiplo estado de satisfação, posto que a égide da humanização ascendeu a noção do bem-estar como pilar efervescente da condição de sobrevida e a carência desses cuidados engatilhou um decréscimo ao estado motor e psicológico dos relegados à não-assistência. Conclusão: Novos estudos são necessários para aprofundar o entendimento entre delimitações prévias de decisões dos pacientes e o peso da equipe multidisciplinar nesse contexto decisório.
