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

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.

Study Overview

• Status: Terminated
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Device: Custom Mask Interface

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 Type: Interventional
• Enrollment (Actual): 5
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • Michigan
    • Ann Arbor, Michigan, United States, 48109
      • Michigan Medicine

Participation Criteria

Eligibility Criteria

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

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Device Feasibility
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / 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.

What is the study measuring?

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

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
ALS Depression Inventory-12 (ADI-12)	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"	1 month
ALS Functional Rating Scale, Revised (ALSFRS-R)	Functional Rating Scale. Scales: Customized for each question, but 4 is the maximum and generally means 'normal', and 0 represents significant impairment.	1 month
ALS Specific Quality of Life-20 (ALSSQOL20)	Quality of Life Scale. Scales: For each question, 1-10 is selected, where 10 means "extremely" and 0 means "not at all".	1 month
Epworth Sleepiness Scale (ESS)	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".	1 month
PROMIS Neuro-Quality of Life Fatigue Scale	Fatigue Scale. Scales: For each question, "Always" (5), "Often" (4), "Sometimes" (3), "Rarely" (2), and "Never" (1) is selected.	1 month
PROMIS Neuro-Quality of Life Sleep Disturbance Scale	Sleep Disturbance Scale. Scales: For each question, "Always" (5), "Often" (4), "Sometimes" (3), "Rarely" (2), and "Never" (1) is selected.	1 month

Collaborators and Investigators

• Sponsor: University of Michigan
• Collaborators: ALS Association
• Investigators: Principal Investigator: Stephen A Goutman, MD, University of Michigan

Study record dates

Study Major Dates

• Study Start (Actual): March 14, 2017
• Primary Completion (Actual): March 14, 2020
• Study Completion (Actual): March 14, 2020

Study Registration Dates

• First Submitted: April 16, 2018
• First Submitted That Met QC Criteria: May 8, 2018
• First Posted (Actual): May 9, 2018

Study Record Updates

• Last Update Posted (Actual): April 6, 2021
• Last Update Submitted That Met QC Criteria: April 5, 2021
• Last Verified: April 1, 2021

More Information

Terms related to this study

• Keywords: amyotrophic lateral sclerosis, non-invasive ventilation, mask, custom, 3D-printing
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: HUM00112433

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No