Systematic Laryngoscopic Evaluation of Upper Airways in Ventilated ALS Patients in Portugal and in Norway

Study Overview
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Brief Summary
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The study examines if titration of Non-Invasive mechanical ventilation (NIV) settings during ongoing laryngoscopic visualization can improve the compliance of NIV in subjects with Amyotrophic Lateral Sclerosis (ALS). The study is a multicentre study between Norwegian National Advisory Unit on Long-term Mechanical Ventilation at the Thoracic Department, Haukeland University Hospital, Bergen, Norway and Centro Hospital Tras-os-Montes e Alto Douro, Vila Real, Portugal.

Detailed Description
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ALS, amyotrophic lateral sclerosis, is a rare and fatal disease causing gradual loss of motor neurons and thus loss of function in all skeletal muscle, eventually also in the breathing muscles, leading to respiratory failure and ultimately death. There is no causal treatment. The treatment provided aims to improve life quality as long as possible. Non-invasive ventilation (NIV) compensates for respiratory failure as breathing deteriorates. ALS patients with bulbar innervated muscle dysfunction (mouth, tongue, jaw, throat, larynx) have poor compliance to the use of NIV. It is not completely understood why bulbar ALS patients fails NIV, although the upper airways have been a suggested cause also of this treatment failure. The investigators in the Norwegian study group has previously examined laryngeal response patterns (using transnasal fiberoptic laryngoscopy ) during mechanically assisted cough in ALS. The studies revealed that Transnasal fiberoptic laryngoscopy was safely performed with ongoing mechanically assisted cough, and it appeared a feasible tool to visualize the laryngeal responses to mechanical cough therapy. The further hypothesis is that the use of transnasal fiberoptic laryngoscopy during ongoing NIV titration could improve the compliance of NIV treatment in ALS. Since ALS is a relatively rare disease and it is difficult to perform large clinical studies, a project group has been established and agreed to a common protocol of a population-based multicenter-study including two hospitals, in Bergen in Norway and Vila Real in Portugal. Aims of the study are: To investigate the feasibility of video-recorded Transnasal fiberoptic laryngoscopic evaluation of the upper airways applied during NIV titration, and to describe the upper airway responses to NIV treatment in patients with ALS. To explore whether the endoscopic view of the upper airways will influence the titration of NIV settings in ALS patients. To explore whether NIV titration with endoscopic evaluation will influence NIV compliance and ventilation effectiveness in ALS patients.

Official Title
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Systematic Laryngoscopic Evaluation of Upper Airways in Ventilated Amyotrophic Lateral Sclerosis (ALS) Patients in Portugal and in Norway

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Other: Transnasal Fiberoptic Laryngoscopy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients diagnosed Amyotrophic Lateral Sclerosis (ALS) due to El Escorial criteria Must have already established Non-invasive ventilation (NIV) Exclusion Criteria: Ages under 18 years Unstable ischemic heart disease Oncological disease Ventilatory support dependency (more than 16h per day)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Intervention Model: Single Group Assignment
Interventional Model Description: All the subjects will receive the same intervention, and they will be examined both prior to the intervention, one month after and three months after the intervention. They will act as they own controls.
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: NIV settings titration<br>Transnasal Fiberoptic Laryngoscopy will be used during ongoing NIV setting titration. Aim is to titrate NIV setting to be more optimal for laryngeal responses. | Other: Transnasal Fiberoptic Laryngoscopy<br>* Use of Transnasal Fiberoptic Laryngoscopy during NIV titration to optimalize the NIV settings to laryngeal responses during the NIV treatment.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Apnoea-hypopnea index (AHI) | Change in number of apnea and hypopnea events per hour of sleep | Baseline, one and three months after the intervention. | 
| Time of NIV use | Change in median daily and average daily of hours, minutes | Baseline, one and three months after the intervention. | 
| NIV Leaks | Change in median and 95% | Baseline, one and three months after the intervention. | 
| NIV time of use | Change in % of time of use > than 4 hours/day | Baseline, one and three months after the intervention. | 
| NIV compliance | Change in number of interruptions, complains and ventilator adjustments | Baseline, one and three months after the intervention. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Night-time pulseoximeter | Change in oxygen saturation of patients blood | Baseline, one and three months after the intervention. | 
| Night-time capnography | Change in concentration or partial pressure of carbon dioxide in the respiratory gases | Baseline, one and three months after the intervention. | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Noninvasive Ventilation, Laryngoscopy