A Pilot Study of the Utility of 3D Printed Masks for ALS Subjects

Study Overview
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Brief Summary
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Non-invasive ventilation (NIV) is an important therapy for patients with a number of neurological diseases. Specifically, NIV has been shown to be an effective treatment for people with amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease), which is a fatal, non-curable, progressive disease of the motor neurons. However, due to changes in facial structure associated with the disease, many ALS patients find that traditional NIV masks don't fit well. In this study, investigators will perform a feasibility study on NIV mask interfaces which are custom designed for each ALS patient and then manufactured via 3D printing.

Detailed Description
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Non-invasive ventilation (NIV) is an important therapy for patients with a number of neurological diseases. Specifically, for amyotrophic lateral sclerosis (ALS)--a fatal, non-curable, progressive disease of the motor neurons--NIV represents one of the most effective treatments with a survival benefit of greater than 1 year. Despite this survival advantage, and the corresponding improvement in Quality of Life (QoL), compliance with NIV can be poor. Factors contributing to poor compliance to NIV include bulbar onset disease and mask leaks. In addition to reduced adherence to therapy, elevated mask leaks can compromise the pressure adjustment algorithm in average volume assured pressure support (AVAPS) ventilation, a commonly used mode of bilevel (positive airway pressure) PAP therapy in ALS. In an effort to increase NIV compliance in subjects with neurological conditions, and specifically ALS, investigators hypothesize that a better fit achieved through 3D printed NIV mask interfaces may improve utilization by decreasing mask leaks, decreasing the required delivery pressures and improving compliance with therapy. Investigators therefore propose a feasibility study to investigate the use of 3D printing to manufacture customized NIV mask interfaces for subjects with ALS who encounter difficulty using NIV because of poorly fitting mask interfaces despite exhausting available commercial mask options.

Official Title
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A Pilot Study of the Utility of 3D Printed Masks for ALS Subjects

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Device: Custom Mask Interface


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age greater than 18 Ability to communicate in English Diagnosis of ALS Subject followed in the ALS Clinic at University of Michigan Current use of NIV. Problematic mask leak defined as one of the following: A. A clinical complaint of mask leak impeding use of PAP AND PAP use <6 hours per night AND/OR B. Average percentage of the night in large leak on download of >10% (AVAPS) At least 1 month of NIV use Exclusion Criteria: Subjects successfully using a commercially available mask without objective or subjective leak with good compliance (average use >6 hours per night) Silicone allergy

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: Device Feasibility
Intervention Model: Single Group Assignment
Interventional Model Description: All trial enrollees will receive the custom-designed 3D printed non-invasive ventilation mask interface.
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Custom Mask Interface<br>Patients use a custom mask interface for one month with an option to use for a year if it performs better than a commercial mask. | Device: Custom Mask Interface<br>* Subjects enrolled in the study will received a custom designed, 3D printed, non-invasive ventilation mask interface.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Average number of hours the custom NIV mask interface is used per night | Average number of hours the custom NIV mask interface is used per night | 1 month | 
| Leak parameters from device | Average minutes in large leak or leak measured from the device in liters per minute and averaged over the month | 1 month | 
| Tidal volume measurements | (average measured tidal volume/average set tidal volume)*100% | 1 month | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Interviews with subjects regarding their experience with the custom mask Qualitative assessment of custom NIV mask efficacy | Interviews with subjects regarding their experience with the custom mask | 1 month | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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amyotrophic lateral sclerosis, non-invasive ventilation, mask, custom, 3D-printing