scholarly journals Slow vital capacity

2020 ◽  
Vol 8 (1) ◽  
pp. 144
Author(s):  
Shruti Singh ◽  
Sara Z. Khan ◽  
Bhakti Patel ◽  
Rammohan Gumpeni ◽  
Sameer Verma ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Respiratory Function ◽  
Vital Capacity ◽  
Neuromuscular Disorders ◽  
Respiratory Muscle Fatigue ◽  
Expiratory Muscle ◽  
Non Invasive ◽  
Als Patients ◽  
Lateral Sclerosis

Forced vital capacity (FVC) and slow vital capacity (SVC) are non-invasive tests of respiratory function. Although FVC has been extensively studied and is used in most PFT (pulmonary function test) labs, SVC can also be used in clinical practice as it is a more comfortable and convenient test to perform. SVC-based diagnostic criteria can lead to earlier detection of obstructive lung disease. In contrast to FVC, SVC is less affected by respiratory muscle fatigue, airflow patency, expiratory muscle weakness and air leakage making it an appropriate test of respiratory function in patients of amyotrophic lateral sclerosis (ALS) and other neuromuscular disorders. As respiratory insufficiency is the major cause of mortality in ALS patients, regular SVC measurement provides the respiratory functional status, so that early treatment can be started which improves the survival and quality of life in these patients. The purpose of this article is to highlight the importance of considering SVC in clinical practice.

scholarly journals Detection of early nocturnal hypoventilation in neuromuscular disorders

2017 ◽  
Vol 46 (3) ◽  
pp. 1153-1161 ◽  
Author(s):  
Federica Trucco ◽  
Marina Pedemonte ◽  
Chiara Fiorillo ◽  
Hui-leng Tan ◽  
Annalisa Carlucci ◽  
...  
Keyword(s):  
Vital Capacity ◽  
Clinical Symptoms ◽  
Neuromuscular Disorders ◽  
Invasive Ventilation ◽  
Additional Tool ◽  
Non Invasive ◽  
Respiratory Involvement ◽  
Nocturnal Hypoxemia ◽  
Nocturnal Hypoventilation ◽  
Non Invasive Ventilation

Objective Nocturnal hypoventilation (NH) is a complication of respiratory involvement in neuromuscular disorders (NMD) that can evolve into symptomatic daytime hypercapnia if not treated proactively with non-invasive ventilation. This study aimed to assess whether NH can be detected in the absence of other signs of nocturnal altered gas exchange. Methods We performed nocturnal transcutaneous coupled (tc) pCO2/SpO2 monitoring in 46 consecutive cases of paediatric-onset NMD with a restrictive respiratory defect (forced vital capacity < 60%). Nocturnal hypoventilation was defined as tcPCO2 > 50 mmHg for > 25% of recorded time, and hypoxemia as tcSpO2 < 88% for > 5 minutes. Daytime symptoms and bicarbonate were recorded after overnight monitoring. Results Twenty-nine of 46 consecutive patients showed NH. Twenty-three patients did not have nocturnal hypoxemia and 18 were clinically asymptomatic. In 20 patients, PaCO2 in daytime blood samples was normal. Finally, 13/29 patients with NH had isolated nocturnal hypercapnia without nocturnal hypoxia, clinical NH symptoms, or daytime hypercapnia. Conclusions Paediatric patients with NMD can develop NH in the absence of clinical symptoms or significant nocturnal desaturation. Therefore, monitoring of NH should be included among nocturnal respiratory assessments of these patients as an additional tool to determine when to commence non-invasive ventilation.

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.

Effect of non‐invasive ventilation on survival, quality of life, respiratory function and cognition: A review of the literature

2006 ◽  
Vol 7 (4) ◽  
pp. 195-200 ◽  
Author(s):  
Sanne Piepers ◽  
Jan‐Paul Van Den Berg ◽  
Sandra Kalmijn ◽  
W‐Ludo Van Der Pol ◽  
John H. J. Wokke ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Respiratory Function ◽  
Invasive Ventilation ◽  
Review Of The Literature ◽  
Non Invasive ◽  
Non Invasive Ventilation

scholarly journals Teaching an Old Dog New Tricks: Serum Troponin T as a Biomarker in Amyotrophic Lateral Sclerosis

2021 ◽  
Author(s):  
Sergio Castro-Gomez ◽  
Barbara Radermacher ◽  
Pawel Tacik ◽  
Sandra R. Mirandola ◽  
Michael T. Heneka ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Troponin I ◽  
Troponin T ◽  
Case Reports ◽  
Neuromuscular Disorders ◽  
University Hospital ◽  
Biological Origin ◽  
Muscle Involvement ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by progressive loss of upper and lower motor neurons. Diagnosis, management and therapeutic trials are hampered by a lack of informative biomarkers. Troponins (Tn) are components of skeletal and cardiac muscles. Acute elevation of cardiac isoforms of troponin I (cTnI) and T (cTnT) in serum indicates myocardial injury. Case reports suggested that serum levels of cTnT, but not cTnI are chronically elevated in ALS and other neuromuscular disorders.Using standard clinical laboratory methodologies we studied serum troponin levels in a multicentric cross-sectional cohort of 75 ALS patients and sixty controls (DESCRIBE-ALS cohort) and in a real-world cohort of 179 consecutive patients from our ALS clinic at the University Hospital Bonn.We found that serum cTnT, is elevated in >60% of ALS patients while cTnI is always normal. Serum cTnT levels increase over time and correlate with disease severity as measured with the revised ALS FRS score. There was no correlation with the phosphorylated neurofilament heavy chain (pNfH) levels in the cerebrospinal fluid. We propose that cTnT elevations in ALS are of non-cardiac origin and may serve as a proxy of lower motor neuron or skeletal muscle involvement. They potentially help to stratify patients according to lower motoneuron involvement. Further research will determine the biological origin of the cTnT elevation and its validity as a diagnostic and/or prognostic marker. Our finding also serves as a reminder to interpret cTnT with caution elevations in patients with neuromuscular diseases.

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.

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

Use of nasal pillows in amiotrophic lateral sclerosis (ALS) patients with high non invasive ventilation (NIV) dependence

2018 ◽  
Author(s):  
Luana Focaraccio ◽  
Giulia Rovere ◽  
Roberta Di Tria ◽  
Renza Ambrosanio ◽  
Elisa Clivati ◽  
...  
Keyword(s):  
Invasive Ventilation ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Als Patients ◽  
Lateral Sclerosis

scholarly journals NIPPV: Prevalence, Approach and Barriers to Use at Canadian ALS Centres

2010 ◽  
Vol 37 (1) ◽  
pp. 54-60 ◽  
Author(s):  
Benjamin R. Ritsma ◽  
Michael J. Berger ◽  
David A. Charland ◽  
Michael A. Khoury ◽  
Joel T. Phillips ◽  
...  
Keyword(s):  
Positive Pressure Ventilation ◽  
Invasive Mechanical Ventilation ◽  
Positive Pressure ◽  
Optimal Care ◽  
Non Invasive ◽  
Morning Headache ◽  
Barriers To Use ◽  
Als Patients ◽  
Descriptive Survey ◽  
Lateral Sclerosis

Objective:The purpose of this study was to evaluate Canadian amyotrophic lateral sclerosis (ALS) centres with respect to: 1) the prevalence of Non-invasive positive pressure ventilation (NIPPV) and invasive mechanical ventilation via tracheostomy (TV) utilization, 2) the approach to NIPPV use, focusing upon the currently employed initiation criteria and 3) the barriers influencing NIPPV administration.Methods:A descriptive survey research design aimed to obtain quantitative data and open-ended responses from an active physician at each of the 15 multidisciplinary Canadian ALS centres.Results:The principal findings of this study were: 1) NIPPV and TV are used in 18.3% and 1.5% of patients at Canadian ALS centres, respectively, 2) symptoms of respiratory insufficiency, namely orthopnea (clinical significance rated at 9.00/10 ± 1.48), dyspnea (8.27 ± 1.95) and morning headache (7.55 ± 1.21) are the most significant indicators for NIPPV initiation, 3) the primary barriers to NIPPV utilization are patient intolerance (70% of centres) and inaccessibility of respirologists and ventilation technologists (50% of centres).Conclusions:Variability in NIPPV use has an impact upon the management of Canadian ALS patients. The establishment of more definitive NIPPV initiation criteria, emphasizing respiratory symptoms, and the attenuation of barriers to NIPPV use should be targeted so as to ensure optimal care for all ALS patients.

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