An Overview of Respiratory Treatments for Individuals With Amyotrophic Lateral Sclerosis 1

2013 ◽  
Vol 22 (1) ◽  
pp. 17-25
Author(s):  
Stuart Cleary ◽  
Janice Richman-Eisenstat

Given the limited life expectancy of individuals diagnosed with amyotrophic lateral sclerosis (ALS) and the high likelihood that patients will suffer from a breathing difficulty and an encumbered airway, promoting the highest level in the ability to maintain an open and clear airway is of the utmost importance to patients and their families. In this article, authors describe the basic mechanisms of neuromuscular-induced respiratory failure and explain the underlying physiological rationale for four commonly used respiratory treatments in cases of advanced ALS. The article also includes research on the use of manual breath stacking to improve coughing and airway clearance while swallowing.

2020 ◽  
Author(s):  
Antonio Faraone ◽  
Chiara Beltrame ◽  
Andrea Crociani ◽  
Paolo Carrai ◽  
Elena Lovicu ◽  
...  

Abstract BackgroundThe role of non-invasive positive pressure ventilation (NIPPV) in COVID-19 patients with acute hypoxemic respiratory failure (AHRF) is uncertain. This study was aimed to assess the effectiveness and safety of NIPPV in patients with COVID-19-associated AHRF admitted to non-ICU wards.MethodsWe retrospectively evaluated all COVID-19 patients consecutively admitted to the COVID-19 general wards of a medium-size Italian hospital, from March 6 to May 7, 2020. Healthcare workers (HCWs) caring for COVID-19 patients were monitored, undergoing naso-pharyngeal swab for SARS-CoV-2 in case of onset of COVID-19 symptoms, and periodic SARS-CoV-2 screening serology.ResultsOverall, 50 of 143 patients (mean age 74.6 years) were treated with NIPPV, and 22 (44%) were successfully weaned. Due to limited life expectancy, 25 (50%) of 50 NIPPV-treated patients received a “do not intubate” order. Among these, only 6 (24%) were weaned from NIPPV. Of the remaining 25 NIPPV-treated patients, 16 (64%) were successfully weaned, 9 (36%) underwent delayed endotracheal intubation and, among these, 3 (33.3%) died. NIPPV success was predicted by the use of corticosteroids (OR 15.4, p 0.013), the PaO2/FiO2 ratio measured 24-48 hours after NIPPV initiation (OR 1.02, p 0.015), and the presence of a “do not intubate” order (OR 0.03, p 0.020). During the study period, 2 of 124 (1.6%) HCWs caring for COVID-19 patients were diagnosed with mild SARS-CoV-2 infection.ConclusionsApart from patients with limited life expectancy, NIPPV was effective in a substantially high percentage of patients with COVID-19-associated AHRF. The risk of SARS-CoV-2 infection among HCWs was low.


2019 ◽  
Vol 34 (12) ◽  
pp. 2720-2722 ◽  
Author(s):  
Nancy L. Schoenborn ◽  
Jin Huang ◽  
Cynthia M. Boyd ◽  
Sarah Nowak ◽  
Craig E. Pollack

2018 ◽  
Vol 145 ◽  
pp. 1-7 ◽  
Author(s):  
Capucine Morélot-Panzini ◽  
Thierry Perez ◽  
Kamila Sedkaoui ◽  
Elodie de Bock ◽  
Bernard Aguilaniu ◽  
...  

2020 ◽  
pp. 571-578
Author(s):  
Miles Witham ◽  
Jacob George ◽  
Denis O’Mahony

The use of pharmacological agents is often a central component of medical therapy for older people. Medications can relieve symptoms, improve function, and prevent illness, but they also have the capacity to inflict great harm. Older people are at particular risk of such harms as a result of impaired homeostatic reserve, of altered drug metabolism, the presence of multimorbidity and consequent polypharmacy, which increases both exposure to potentially harmful agents and the chance of drug–drug interactions. The therapeutic priorities for older, frail people may differ when compared to younger, robust patients; limited life expectancy means that attempts to prolong life may become relatively less important than the relief of symptoms and avoidance of side effects and medication burden.


2020 ◽  
Vol 10 (3) ◽  
pp. 58 ◽  
Author(s):  
Owen Connolly ◽  
Laura Le Gall ◽  
Gavin McCluskey ◽  
Colette G Donaghy ◽  
William J Duddy ◽  
...  

Amyotrophic lateral sclerosis is a rare and fatal neurodegenerative disease characterised by progressive deterioration of upper and lower motor neurons that eventually culminates in severe muscle atrophy, respiratory failure and death. There is a concerning lack of understanding regarding the mechanisms that lead to the onset of ALS and as a result there are no reliable biomarkers that aid in the early detection of the disease nor is there an effective treatment. This review first considers the clinical phenotypes associated with ALS, and discusses the broad categorisation of ALS and ALS-mimic diseases into upper and lower motor neuron diseases, before focusing on the genetic aetiology of ALS and considering the potential relationship of mutations of different genes to variations in phenotype. For this purpose, a systematic review is conducted collating data from 107 original published clinical studies on monogenic forms of the disease, surveying the age and site of onset, disease duration and motor neuron involvement. The collected data highlight the complexity of the disease’s genotype–phenotype relationship, and thus the need for a nuanced approach to the development of clinical assays and therapeutics.


2019 ◽  
Vol 28 (6) ◽  
pp. 501-508 ◽  
Author(s):  
Luca Pasina ◽  
Barbara Brignolo Ottolini ◽  
Laura Cortesi ◽  
Mauro Tettamanti ◽  
Carlotta Franchi ◽  
...  

Objective: Older people approaching the end of life are at a high risk for adverse drug reactions. Approaching the end of life should change the therapeutic aims, triggering a reduction in the number of drugs.The main aim of this study is to describe the preventive and symptomatic drug treatments prescribed to patients discharged with a limited life expectancy from internal medicine and geriatric wards. The secondary aim was to describe the potentially severe drug-drug interactions (DDI). Materials and Methods: We analyzed Registry of Polytherapies Societa Italiana di Medicina Interna (REPOSI), a network of internal medicine and geriatric wards, to describe the drug therapy of patients discharged with a limited life expectancy. Results: The study sample comprised 55 patients discharged with a limited life expectancy. Patients with at least 1 preventive medication that could be considered for deprescription at the end of life were significantly fewer from admission to discharge (n = 30; 54.5% vs. n = 21; 38.2%; p = 0.02). Angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers, calcium channel blockers, lipid-lowering drugs, and clonidine were the most frequent potentially avoidable medications prescribed at discharge, followed by xanthine oxidase inhibitors and drugs to prevent fractures. Thirty-seven (67.3%) patients were also exposed to at least 1 potentially severe DDI at discharge. Conclusion: Hospital discharge is associated with a small reduction in the use of commonly prescribed preventive medications in patients discharged with a limited life expectancy. Cardiovascular drugs are the most frequent potentially avoidable preventive medications. A consensus framework or shared criteria for potentially inappropriate medication in elderly patients with limited life expectancy could be useful to further improve drug prescription.


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