Remote Pulmonary Function Testing in Amyotrophic Lateral Sclerosis (Pilot)

Study Overview
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Brief Summary
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The specific objective of this study is to validate the practice of remote pulmonary function testing (rPFT) conducted in the home through the use of connected mobile health devices and the Penn State Hershey ALS Telemanagement program.

Detailed Description
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The specific objective of this study is to validate the practice of remote pulmonary function testing (rPFT) conducted in the home through the use of connected mobile health devices and the Penn State Hershey ALS Telemanagement program. The central hypothesis is that guided home assessment of respiratory function is a valid method for detecting respiratory insufficiency leading to noninvasive ventilation (NIV) recommendation. This study has the potential to transform the current practice of conducting breathing assessments every three months, resulting in timelier detection of respiratory insufficiency, thereby staining quality of life and lengthening survival. This protocol has the potential to demonstrate telemanagement exceeding the standards of ALS care. This is a self-controlled study which will enroll 40 patients from the ALS clinic. On the day of their clinical visit, study participants will perform both a standard PFT as well as a simulated rPFT, both generating three valid repetitions of forced vital capacity (FVC) and maximal inspiratory pressure (MIP) procedures. The simulated rPFT will mimic the practice of home telemonitoring by having patients be instructed by a respiratory therapist over the telemanagement portal while in a research room within the ALS clinic. The primary hypothesis is that there is no difference in the results of PFT and rPFT for respiratory assessment of FVC and MIP.

Official Title
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Remote Pulmonary Function Testing in Amyotrophic Lateral Sclerosis (Pilot)

Conditions
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ALS

Intervention / Treatment
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* Device: remote pulmonary function testing
* Device: standard pulmonary function testing


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Part 1 Patients: Possess a diagnosis of definite, probable, probable laboratory-supported, or possible ALS by revised El Escorial research criteria [Brooks2000]. Be 18 years of age or older. Have a caregiver available to participate in the study Caregivers: Be 18 years of age or older, of either gender. Be able and willing to provide informed consent. Respiratory Therapist Be a member of the Hershey Medical Center ALS multidisciplinary care team. Be able and willing to provide verbal informed consent after receiving a summary explanation of research (SER). Part 2 imposes additional inclusion criteria for patients only. Patients: 4) Symptom onset within the last three years. 5) Have a computer and home internet service sufﬁcient for engaging in telemedicine sessions. 6) Have a second device capable of downloading the spirometer application from an app store (Android- or iOS-based smartphone or tablet). Exclusion Criteria: Exclusion criteria are the same for both parts of the study. Patients: Use of NIV or diaphragm pacer at time of obtaining informed consent. FVC ≤50% predicted or MIP > -60 cm H2O. ALS Functional Rating Scale (ALSFRS-R) [Cedarbaum1999] score on day of screening of ≥2 on items for speech, swallowing, and salivation. These items are indicators of bulbar dysfunction, which limits the reliability of PFT administration. Cognitive impairment, as judged by the ALS clinic neurologist, that prevents participation in the study. Caregivers: None Respiratory Therapists: None

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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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Health Services Research
Intervention Model: Single Group Assignment
Interventional Model Description: A self-controlled validation study of experimental remote PFT assessment vs standard of care (non-randomized)
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: remote PFT (rPFT) validation<br>Subjects in this arm perform both standard and remote PFT assessments in order to validate the procedure. | Device: remote pulmonary function testing<br>* Telemedicine delivery of pulmonary function testing in ALS, including Forced Vital Capacity (FVC) and Maximal Inspiratory Pressure (MIP)<br>Device: standard pulmonary function testing<br>* Standard clinical delivery of pulmonary function testing in ALS, including Forced Vital Capacity (FVC) and Maximal Inspiratory Pressure (MIP)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Standard PFT - Forced Vital Capacity | Respiratory therapist will administer three valid maneuvers of forced vital capacity (FVC) The best FVC value is the outcome. | One administration - 10 minutes | 
| Standard PFT - Maximal Inspiratory Pressure | Respiratory therapist will administer three valid maneuvers of maximal inspiratory pressure (MIP). The best MIP value is the outcome. | One administration - 10 minutes | 
| Remote PFT - Forced Vital Capacity | Respiratory therapist will use the telehealth interface to guide the patient and caregiver to self-administer three valid FVC maneuvers. The best FVC value is the outcome. | One administration - 10 minutes | 
| Remote PFT - Maximal Inspiratory Pressure | Respiratory therapist will use the telehealth interface to guide the patient and caregiver to self-administer three valid MIP maneuvers. The best MIP value is the outcome. | One administration - 10 minutes | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Patient and Caregiver Reported Outcomes | Survey responses from the patient/caregiver pair. Likert-type scales are used to generate subscores pertaining to: General Acceptability, Forced Vital Capacity Acceptability, and Maximal Inspiratory Pressure Acceptability. Subscales (evaluated separately): General Acceptability [0-5 (worst-best)] Forced Vital Capacity Acceptability [0-5 (worst-best) Maximal Inspiratory Pressure Acceptability [0-5 (worst-best)] | 10 minute survey administered following completion of standard and remote PFT of Part 1 | 
| Therapist Reported Outcomes | Survey responses from the respiratory therapist. Likert-type scales are used to generate subscores pertaining to: General Acceptability, Forced Vital Capacity Acceptability, and Maximal Inspiratory Pressure Acceptability. Subscales (evaluated separately): General Acceptability [0-5 (worst-best)] Forced Vital Capacity Acceptability [0-5 (worst-best) Maximal Inspiratory Pressure Acceptability [0-5 (worst-best)] | 10 minute survey administered following completion of standard and remote PFT of Part 1 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Telemedicine, Pulmonary Function Testing, Telehealth