Remote Pulmonary Function Testing in Amyotrophic Lateral Sclerosis (Pilot)

Not Applicable

Completed

Interventions: Device: remote pulmonary function testing
Device: standard pulmonary function testing

Brief Summary: 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: 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.

Recruitment & Eligibility

Inclusion Criteria

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:

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:

Symptom onset within the last three years. 5) Have a computer and home internet service sufﬁcient for engaging in telemedicine sessions.
Have a second device capable of downloading the spirometer application from an app store (Android- or iOS-based smartphone or tablet).

Exclusion Criteria

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

Arm && Interventions

Group	Intervention	Description
remote PFT (rPFT) validation	remote pulmonary function testing	Subjects in this arm perform both standard and remote PFT assessments in order to validate the procedure.
remote PFT (rPFT) validation	standard pulmonary function testing	Subjects in this arm perform both standard and remote PFT assessments in order to validate the procedure.

Primary Outcome Measures

Name	Time	Method
Standard PFT - Forced Vital Capacity	One administration - 10 minutes	Respiratory therapist will administer three valid maneuvers of forced vital capacity (FVC) The best FVC value is the outcome.
Standard PFT - Maximal Inspiratory Pressure	One administration - 10 minutes	Respiratory therapist will administer three valid maneuvers of maximal inspiratory pressure (MIP). The best MIP value is the outcome.
Remote PFT - Forced Vital Capacity	One administration - 10 minutes	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.
Remote PFT - Maximal Inspiratory Pressure	One administration - 10 minutes	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.

Secondary Outcome Measures

Name	Time	Method
Therapist Reported Outcomes	10 minute survey administered following completion of standard and remote PFT of Part 1	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 \[0-5 (worst-best)\]
Patient and Caregiver Reported Outcomes	10 minute survey administered following completion of standard and remote PFT of Part 1	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 \[0-5 (worst-best)\]