Clinical trial NCT03887338: Systematic Laryngoscopic Evaluation of Upper Airways in Ventilated ALS Patients in Portugal and in Norway
Official title: Systematic Laryngoscopic Evaluation of Upper Airways in Ventilated Amyotrophic Lateral Sclerosis (ALS) Patients in Portugal and in Norway
Study type: INTERVENTIONAL
Overall status: UNKNOWN
Phase: NA
Conditions: Amyotrophic Lateral Sclerosis
Keywords: Noninvasive Ventilation
Laryngoscopy
Lead sponsor: Haukeland University Hospital
Sponsor class: OTHER
Collaborators: name: University of Trás-os-Montes and Alto Douro
name: Universidade do Porto
name: Haukeland University Hospital
First posted: 2019-03-22
Enrollment: 60
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Intervention 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.
Primary purpose: SUPPORTIVE_CARE

Brief summary: 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: 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.

Eligibility criteria: 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)

Primary outcomes:
measure: Apnoea-hypopnea index (AHI)
timeFrame: Baseline, one and three months after the intervention.
measure: Time of NIV use
timeFrame: Baseline, one and three months after the intervention.
measure: NIV Leaks
timeFrame: Baseline, one and three months after the intervention.
measure: NIV time of use
timeFrame: Baseline, one and three months after the intervention.
measure: NIV compliance
timeFrame: Baseline, one and three months after the intervention.
Secondary outcomes:
measure: Night-time pulseoximeter
timeFrame: Baseline, one and three months after the intervention.
measure: Night-time capnography
timeFrame: Baseline, one and three months after the intervention.
Other outcomes:
Locations:
city: Bergen
country: Norway
facility: Norwegian National Advisory Unit on Long-term Mechanical Ventilation at Thoracic Department, Haukeland University Hospital
state: Hordaland
city: Vila Real
country: Portugal
facility: Centro Hospitalar Tras-os-Montes e Alto Douro
Study officials:
affiliation: Haukeland University Hospital
name: Tiina M Andersen, PhD
role: PRINCIPAL_INVESTIGATOR
affiliation: Centro Hospitalar Tras-os-Montes e Alto Douro
name: Bebiana Conde, PhD fellow
role: PRINCIPAL_INVESTIGATOR
References:
citation: Andersen T, Sandnes A, Brekka AK, Hilland M, Clemm H, Fondenes O, Tysnes OB, Heimdal JH, Halvorsen T, Vollsaeter M, Roksund OD. Laryngeal response patterns influence the efficacy of mechanical assisted cough in amyotrophic lateral sclerosis. Thorax. 2017 Mar;72(3):221-229. doi: 10.1136/thoraxjnl-2015-207555. Epub 2016 May 12.
citation: Andersen TM, Sandnes A, Fondenes O, Nilsen RM, Tysnes OB, Heimdal JH, Clemm HH, Halvorsen T, Vollsaeter M, Roksund OD. Laryngeal Responses to Mechanically Assisted Cough in Progressing Amyotrophic Lateral Sclerosis. Respir Care. 2018 May;63(5):538-549. doi: 10.4187/respcare.05924. Epub 2018 Apr 17.
citation: Andersen TM, Sandnes A, Fondenes O, Clemm H, Halvorsen T, Nilsen RM, Tysnes OB, Heimdal JH, Vollsaeter M, Roksund OD. Laryngoscopy Can Be a Valuable Tool for Unexpected Therapeutic Response in Noninvasive Respiratory Interventions. Respir Care. 2018 Nov;63(11):1459-1461. doi: 10.4187/respcare.06674. No abstract available.
citation: Conde B, Martins N, Brandao M, Pimenta AC, Winck JC. Upper Airway Video Endoscopy: Assessment of the response to positive pressure ventilation and mechanical in-exsufflation. Pulmonology. 2019 Sep-Oct;25(5):299-304. doi: 10.1016/j.pulmoe.2019.02.008. Epub 2019 Apr 16.
citation: Andersen TM, Halvorsen T, Fondenes O, Heimdal JH, Roksund OD, Vollsaeter M, Roksund OD. Larynx: The Complex Gateway to the Lungs. Respir Care. 2019 Jul;64(7):866-869. doi: 10.4187/respcare.06989. No abstract available.