Percorso assistenziale e scelte terapeutiche nella Sclerosi Laterale Amiotrofica (SLA) / Care pathway and therapeutics choices for Amyiotrophic Lateral Sclerosis (ALS)

2018 ◽  
Vol 67 (2) ◽  
pp. 157-174
Author(s):  
Mario Sabatelli

La Sclerosi laterale amiotrofica (SLA) è una malattia devastante caratterizzata da una paralisi progressiva dei muscoli scheletrici compresi quelli degli arti, della deglutizione, della fonazione e della respirazione. La morte si verifica entro 3-5 anni nella gran parte dei casi e in un contesto di grande sofferenza a causa dei sintomi della insufficienza respiratoria. La introduzione di tecnologie innovative per supportare la funzione respiratoria con la ventilazione meccanica ha cambiato notevolmente l’approccio assistenziale alle persone con SLA. Il presente contributo intende approfondire, sulla base dell’esperienza maturata presso il Centro Clinico NeMOFondazione Policlinico Universitario A. Gemelli - IRCCS, alcuni aspetti problematici nel percorso assistenziale e delle relative scelte terapeutiche per i pazienti affetti da SLA. ---------- Amyiotrophic Lateral Sclerosis (ALS) is a devastating disease characterized by progressive muscular weakness, leading to limb palsy, difficulties in swallowing, speaking and breathing. Death occurs within 3-5 years in most patients in the context of significant suffering due to symptoms of respiratory failure. The availability of novel technology to support respiratory function by mechanical ventilation has profoundly changed the management of people with ALS. The present article addresses some critical aspects of both care pathway and the related therapeutics choices for patients with ALS on the basis of the experience of the Centro Clinico NeMO-Fondazione Policlinico Universitario A. Gemelli - IRCCS.

2020 ◽  
Vol 7 (1) ◽  
Author(s):  
Yulya Mauliddina ◽  
Ferryal Basbeth ◽  
Muhammad Arsyad

Background: A mechanical ventilator is a device used to help with respiratory function. Its use is indicated for patients with hypoxemia, severe hypercapnia and respiratory failure. Mechanical ventilator is one of the important and widely used aspects for critical patient care in the Intensive Care Unit (ICU). Methods: This research was conducted with non-probability sampling techniques. Non-probability sampling techniques was determined by purposive method, which is to determine the criteria first, then the samples are taken according to predetermined criteria. Results: As much as 98 medical records taken from the Juwita Bekasi Hospital ICU from  2013-2017  showed  that 3 patients showed effective results for ventilator installation and 95 patients showed ineffective results. Conclusion: Based on medical record in Juwita Bekasi Hospital from 2013 to 2017, The mechanical ventilation installation was not effective and only has 1% effectivity.


2017 ◽  
Vol 9 (1) ◽  
Author(s):  
Hiroshi Kataoka ◽  
Hitoki Nanaura ◽  
Kaoru Kinugawa ◽  
Yuto Uchihara ◽  
Hiroya Ohara ◽  
...  

If invasive ventilation can be avoided by performing noninvasive mechanical ventilation (NIV) in patients with acute respiratory failure (ARF), the disease can be effectively managed. It is important to clarify the characteristics of patients with neuromuscular diseases in whom initial NIV is likely to be unsuccessful. We studied 27 patients in stable neuromuscular condition who initially received NIV to manage fatal ARF to identify differences in factors immediately before the onset of ARF among patients who receive continuous NIV support, patients who are switched from NIV to invasive ventilation, and patients in whom NIV is discontinued. Endpoints were evaluated 24 and 72 hours after the initiation of NIV. After 24 hours, all but 1 patient with amyotrophic lateral sclerosis (ALS) received continuous NIV support. 72 hours later, 5 patients were switched from NIV to invasive ventilation, and 5 patients continued to receive NIV support. 72 hours after the initiation of NIV, the proportion of patients with a diagnosis of ALS differed significantly among the three groups (P=0.039). NIV may be attempted to manage acute fatal respiratory failure associated with neuromuscular diseases, but clinicians should carefully manage the clinical course in patients with ALS.


1993 ◽  
Vol 7 (4_suppl) ◽  
pp. 49-64 ◽  
Author(s):  
Edward Anthony Oppenheimer

As respiratory function starts to deteriorate in those with amyotrophic lateral sclerosis, one of the principal questions that has to be answered is whether it is appropriate to provide ventilatory support. Although expensive, it is perfectly feasible to provide this at home, and this article examines many of the issues surrounding home mechanical ventilation.


2015 ◽  
Vol 75 (2) ◽  
Author(s):  
A. Estella ◽  
L. Pérez Bello Fontaiña

400,000 incidents of sea drowning take place every year. We report the case of a woman, 34 years old, attended on the beach by external hospital emergency services after suffering immersion in seawater resulting in drowning due to acute respiratory failure. She was admitted to the critical care unit and an emergency fiberoptic bronchoscopy was performed, extracting a large quantity of sand and algae. The respiratory function improved and FiO2 requirements were gradually reduced following the procedure. After 8 days of mechanical ventilation the patient was extubated, with good clinical tolerance and was transferred to a ward.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Benoît Misset ◽  
Eric Hoste ◽  
Anne-Françoise Donneau ◽  
David Grimaldi ◽  
Geert Meyfroidt ◽  
...  

Abstract Background The COVID-19 pandemic reached Europe in early 2020. Convalescent plasma is used without a consistent evidence of efficacy. Our hypothesis is that passive immunization with plasma collected from patients having contracted COVID-19 and developed specific neutralizing antibodies may alleviate symptoms and reduce mortality in patients treated with mechanical ventilation for severe respiratory failure during the evolution of SARS-CoV-2 pneumonia. Methods We plan to include 500 adult patients, hospitalized in 16 Belgian intensive care units between September 2020 and 2022, diagnosed with SARS-CoV-2 pneumonia, under mechanical ventilation for less than 5 days and a clinical frailty scale less than 6. The study treatment will be compared to standard of care and allocated by randomization in a 1 to 1 ratio without blinding. The main endpoint will be mortality at day 28. We will perform an intention to treat analysis. The number of patients to include is based on an expected mortality rate at day 28 of 40 percent and an expected relative reduction with study intervention of 30 percent with α risk of 5 percent and β risk of 20 percent. Discussion This study will assess the efficacy of plasma in the population of mechanically ventilated patients. A stratification on the delay from mechanical ventilation and inclusion will allow to approach the optimal time use. Selecting convalescent plasmas with a high titer of neutralizing antibodies against SARS-CoV-2 will allow a homogeneous study treatment. The inclusion in the study is based on the consent of the patient or his/her legal representative, and the approval of the Investigational Review Board of the University hospital of Liège, Belgium. A data safety monitoring board (DSMB) has been implemented. Interim analyses have been planned at 100, 2002, 300 and 400 inclusions in order to decide whether the trail should be discontinued prematurely for ethical issues. We plan to publish our results in a peer-reviewed journal and to present them at national and international conferences. Funding and registration The trial is funded by the Belgian Health Care Knowledge Center KCE # COV201004 Trial registration Clinicaltrials.gov registration number NCT04558476. Registered 14 September 2020—Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT04558476


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