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

• ClinicalTrials.gov Identifier: NCT03519880
• Recruitment Status: Terminated
• First Posted: May 9, 2018
• Last Update Posted: April 6, 2021
• Sponsor: University of Michigan
• Collaborator: ALS Association
• Information provided by (Responsible Party): Stephen Goutman, University of Michigan

Study Description

Brief Summary:

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.

Condition or disease	Intervention/treatment	Phase
Amyotrophic Lateral Sclerosis	Device: Custom Mask Interface	Not Applicable

Detailed Description:

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.

Study Design

• Study Type: Interventional (Clinical Trial)
• Actual Enrollment: 5 participants
• Allocation: N/A
• Intervention Model: Single Group Assignment
• Intervention Model Description: All trial enrollees will receive the custom-designed 3D printed non-invasive ventilation mask interface.
• Masking: None (Open Label)
• Masking Description: There is no masking.
• Primary Purpose: Device Feasibility
• Official Title: A Pilot Study of the Utility of 3D Printed Masks for ALS Subjects
• Actual Study Start Date: March 14, 2017
• Actual Primary Completion Date: March 14, 2020
• Actual Study Completion Date: March 14, 2020

Arms and Interventions

Arm	Intervention/treatment
Experimental: Custom Mask Interface; 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; Subjects enrolled in the study will received a custom designed, 3D printed, non-invasive ventilation mask interface.

Outcome Measures

Primary Outcome Measures :

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

Secondary Outcome Measures :

1. Interviews with subjects regarding their experience with the custom mask Qualitative assessment of custom NIV mask efficacy [ Time Frame: 1 month ]
   Interviews with subjects regarding their experience with the custom mask

Other Outcome Measures:

1. ALS Depression Inventory-12 (ADI-12) [ Time Frame: 1 month ]
   Depression Scale. Scales: For each question, the following is selected: "I fully agree", "I agree", "I don't agree", "I do not agree at all"
2. ALS Functional Rating Scale, Revised (ALSFRS-R) [ Time Frame: 1 month ]
   Functional Rating Scale. Scales: Customized for each question, but 4 is the maximum and generally means 'normal', and 0 represents significant impairment.
3. ALS Specific Quality of Life-20 (ALSSQOL20) [ Time Frame: 1 month ]
   Quality of Life Scale. Scales: For each question, 1-10 is selected, where 10 means "extremely" and 0 means "not at all".
4. Epworth Sleepiness Scale (ESS) [ Time Frame: 1 month ]
   Sleepiness Scale. Scales: 3 means "high chance of dozing", 2 means "moderate chance of dozing", 1 means "slight chance of dozing", and 0 means "would never doze".
5. PROMIS Neuro-Quality of Life Fatigue Scale [ Time Frame: 1 month ]
   Fatigue Scale. Scales: For each question, "Always" (5), "Often" (4), "Sometimes" (3), "Rarely" (2), and "Never" (1) is selected.
6. PROMIS Neuro-Quality of Life Sleep Disturbance Scale [ Time Frame: 1 month ]
   Sleep Disturbance Scale. Scales: For each question, "Always" (5), "Often" (4), "Sometimes" (3), "Rarely" (2), and "Never" (1) is selected.

Eligibility Criteria

• Ages Eligible for Study: 18 Years and older (Adult, Older Adult)
• Sexes Eligible for Study: All
• Accepts Healthy Volunteers: No

Criteria

Inclusion Criteria:

1. Age greater than 18
2. Ability to communicate in English
3. Diagnosis of ALS
4. Subject followed in the ALS Clinic at University of Michigan
5. Current use of NIV.

6. 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)

7. At least 1 month of NIV use

Exclusion Criteria:

1. Subjects successfully using a commercially available mask without objective or subjective leak with good compliance (average use >6 hours per night)
2. Silicone allergy

Contacts and Locations

Locations

United States, Michigan

Michigan Medicine

Ann Arbor, Michigan, United States, 48109

Sponsors and Collaborators

University of Michigan

ALS Association

Investigators

• Principal Investigator: Stephen A Goutman, MD; University of Michigan

More Information

• Responsible Party: Stephen Goutman, Assistant Professor of Neurology, University of Michigan
• ClinicalTrials.gov Identifier: NCT03519880
• Other Study ID Numbers: HUM00112433
• First Posted: May 9, 2018
• Last Update Posted: April 6, 2021
• Last Verified: April 2021

Individual Participant Data (IPD) Sharing Statement:

• Plan to Share IPD: No

• Studies a U.S. FDA-regulated Drug Product: No
• Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Stephen Goutman, University of Michigan:

amyotrophic lateral sclerosis, non-invasive ventilation, mask, custom, 3D-printing

Additional relevant MeSH terms:

Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Sclerosis; Pathologic Processes; Neurodegenerative Diseases; Nervous System Diseases; Neuromuscular Diseases; Spinal Cord Diseases; Central Nervous System Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Metabolic Diseases