Amyotrophic Lateral Sclerosis
Conditions
Keywords
amyotrophic lateral sclerosis, non-invasive ventilation, mask, custom, 3D-printing
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.
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.
Interventions
DEVICECustom Mask Interface
Subjects enrolled in the study will received a custom designed, 3D printed, non-invasive ventilation mask interface.
Sponsors
ALS Association
University of Michigan
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DEVICE_FEASIBILITY
Masking
NONE
Masking description
There is no masking.
Intervention model description
All trial enrollees will receive the custom-designed 3D printed non-invasive ventilation mask interface.
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Average number of hours the custom NIV mask interface is used per night | 1 month | Average number of hours the custom NIV mask interface is used per night |
| Leak parameters from device | 1 month | Average minutes in large leak or leak measured from the device in liters per minute and averaged over the month |
| Tidal volume measurements | 1 month | (average measured tidal volume/average set tidal volume)\*100% |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Interviews with subjects regarding their experience with the custom mask Qualitative assessment of custom NIV mask efficacy | 1 month | Interviews with subjects regarding their experience with the custom mask |
Other
| Measure | Time frame | Description |
|---|---|---|
| PROMIS Neuro-Quality of Life Fatigue Scale | 1 month | Fatigue Scale. Scales: For each question, Always (5), Often (4), Sometimes (3), Rarely (2), and Never (1) is selected. |
| ALS Depression Inventory-12 (ADI-12) | 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 |
| PROMIS Neuro-Quality of Life Sleep Disturbance Scale | 1 month | Sleep Disturbance Scale. Scales: For each question, Always (5), Often (4), Sometimes (3), Rarely (2), and Never (1) is selected. |
| ALS Functional Rating Scale, Revised (ALSFRS-R) | 1 month | Functional Rating Scale. Scales: Customized for each question, but 4 is the maximum and generally means 'normal', and 0 represents significant impairment. |
| ALS Specific Quality of Life-20 (ALSSQOL20) | 1 month | Quality of Life Scale. Scales: For each question, 1-10 is selected, where 10 means extremely and 0 means not at all. |
| Epworth Sleepiness Scale (ESS) | 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. |
Countries
United States
Outcome results
None listed