Tolerability, Safety and Efficacy of Sigh Breaths During NIMV in Motor Neuron Disease

Study Overview
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Brief Summary
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Non-invasive mechanical ventilation (NIMV) is the recommended standard of care as initial therapy for patients with motor neuron disease (MND) with deterioration of the respiratory function. SIGH_01 study is aimed at investigating the tolerability, safety profile and efficacy of sigh breaths during non-invasive mechanical ventilation in patients with MND in comparison to the standard ventilation support protocol.

Official Title
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Tolerability, Safety and Efficacy of Sigh During Non-invasive Mechanical Ventilation in Patients With Motor Neuron Disease- A Pilot Study

Conditions
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Motor Neuron Disease, Respiratory Failure, Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Device: NIMV with sigh breaths
* Device: Standard NIMV


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Willing and able to give informed consent MND diagnosis according to El-Escorial criteria Non-invasive ventilation indications in accordance with the international guidelines Exclusion Criteria: Inability to adhere to study visit schedule or lack of reliable caretaker Presence of dementia History of arrhythmia, heart failure or pneumothorax
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
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Triple


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Standard NIMV protocol with sigh breaths<br> | Device: NIMV with sigh breaths<br>* Sigh breaths: Maximal Inspiratory pressure (IPAPmax) increased of at least 25% ( ≥ 125%) with a frequency of at least 1/200 breaths. The sigh breaths will be delivered as part of the standard NIMV protocol<br>|
| Active Comparator: Standard NIMV protocol without sigh breaths<br> | Device: Standard NIMV<br>* Will be treated by standard NIMV with no sigh<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in nocturnal oxygen desaturation index (ODI) assessed by transcutaneous nocturnal oximetric registration |  | Evaluation will be performed before NIMV initiation and two months after | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in the arterial blood PCo2 concentration assessed by arterial blood gas test |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in the arterial blood PO2 concentration assessed by arterial blood gas test |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in the arterial blood Ph assessed by arterial blood gas test |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in the forced vital capacity (FVC) assessed by spirometry test |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in self-perceived quality of life(QoL) assessed by 5-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-5). Score between 0 and 100 pts (higher score denotes worse QoL) |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in sleep quality assessed by Pittsburg Sleep Quality Index (PSQI) questionnaire. Score between 0 to 21, where higher scores denote a worse sleep quality. |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in sleep quality assessed by Numeric Rating Scale (NRS). Score between 0 to 10, where higher scores denote a worse sleep quality. |  | Evaluation will be performed before NIMV initiation and two months after | 
| Changes in nocturnal dyspnoea assessed by Numeric Rating Scale (NRS). Score between 0 to 10, where higher scores denote a worse nocturnal dyspnoea. |  | Evaluation will be performed before NIMV initiation and two months after |