Optimum Insufflation Capacity in NMD

Study Overview
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Brief Summary
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Patients with underlying neuromuscular disorder (NMD) often suffer from weakness in the inspiratory and expiratory muscles. Consequently they do not have the strength to generate the minimum flow of 160 to 300 liters/minute for an efficient cough function. The restricted cough function allows secretion to accumulate, which in turn causes narrowing of the airway lumen and makes ventilation of the neuromuscular patient even more difficult. The patient's susceptibility to infection increases again and the vicious circle repeats itself. Severe secretion retention may even lead to ventilator failure. Effective secretion and cough management instead reduces the risk for stay in hospital. Therefore, secretion and cough management is a mandatory part of the therapeutic concept for treating patients with neuromuscular disease. The therapeutic efficacy of the Lung Insufflation Assist Maneuver(LIA) integrated in the ventilator VENTIlogic LS-plus manufactured by Weinmann GmbH+Co KG was studied in a pilot study carried out by the Dep. for Pediatric Pulmonology and Sleep Medicine at the University Hospital of Essen/Germany in cooperation with Research & Development at Weinmann GmbH &Co KG, Germany . The objective of the pilot study was to examine the therapeutic efficacy of LIAM as a cough support function in patients with neuromuscular disease and indications for mechanical ventilation. We hypothesized that i) a certain insufflation maneuver pressure may be optimal to achieve the highest individual peak cough flow and ii) that this pressure is below the pressure needed to achieve the maximum insufflation capacity. We define the lowest insufflation capacity at which the best individual PCF can be achieved as optimum insufflation capacity (OIC). The study was performed using two different techniques in order to demonstrate that findings are not dependent on maneuver details but are rather based on effects of maneuver pressure. The protocol was limited to techniques which do not require breath stacking: i) insufflation with an Intermittend Positive Pressure (IPPB) device and ii) with the VENTIlogic LS using LIAM.

Official Title
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Optimum Insufflation Capacity and Peak Cough Flow Augmentation in Patients With Neuromuscular Disease

Conditions
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Duchenne Muscular Dystrophy, Spinal Muscular Atrophy, Chronic Respiratory Insufficiency

Intervention / Treatment
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* Procedure: IPPB
* Procedure: LIAM


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: neuromuscular disorder respiratory insufficiency use of home mechanical ventilation Exclusion Criteria: acute illness history of pneumothorax

Ages Eligible for Study
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Minimum Age: 6 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: Diagnostic
Allocation: Non-Randomized
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Lung Insufflation Volume<br>Measurement of the lung volume after hyperinsufflation with positive pressure by IPPB or LIAM | Procedure: IPPB<br>* Lung insufflation with positive pressure using IPPB up from 10 to 40 mbar in 5 mbar increments and consecutive measurement of the insufflation volume<br>* Other names: Intermittent Positive Pressure Breathing = IPPB;Procedure: LIAM<br>* Lung insufflation using the Lung Insufflation Assist maneuver of the Ventilogic LS mechanical ventilator (Weinmann Germany) with positive pressure up from 10 to 40 mbar in 5 mbar increments and consecutive measurement of the insufflation volume<br>* Other names: LIAM = Lung Insufflation Assist Maneuver;|
| Experimental: Peak Cough Flow<br>Measurement of the peak cough flow after hyperinsufflation with positive pressure by IPPB or LIAM | Procedure: IPPB<br>* Lung insufflation with positive pressure using IPPB up from 10 to 40 mbar in 5 mbar increments and consecutive measurement of the insufflation volume<br>* Other names: Intermittent Positive Pressure Breathing = IPPB;Procedure: LIAM<br>* Lung insufflation using the Lung Insufflation Assist maneuver of the Ventilogic LS mechanical ventilator (Weinmann Germany) with positive pressure up from 10 to 40 mbar in 5 mbar increments and consecutive measurement of the insufflation volume<br>* Other names: LIAM = Lung Insufflation Assist Maneuver;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| insufflation capacity | Lung volume was measured during spontaneous breathing and after a lung insufflation assist maneuver. | change of lung volume with the procedure; during hospital stay on average 3 days | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Peak cough flow | Peak cough flow was measured during spontaneous breathing and after a lung insufflation assist maneuver. | change of peak cough flow with the procedure; during hospital stay on average 3 days | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Cough augmentation, Peak cough flow, hyperinsufflation