Amyotrophic Lateral Sclerosis, Motor Neuron Disease, Respiratory Failure
Conditions
Brief summary
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.
Interventions
DEVICENIMV with sigh breaths
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
DEVICEStandard NIMV
Will be treated by standard NIMV with no sigh
Sponsors
Ospedale San Raffaele
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
SUPPORTIVE_CARE
Masking
TRIPLE (Subject, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | 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
| Measure | Time frame |
|---|---|
| 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 the arterial blood PCo2 concentration assessed by arterial blood gas test | 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 |
| 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 |
Countries
Italy
Outcome results
None listed