Remote Pulmonary Function Testing and Nurse Coaching in ALS

Not Applicable

Completed

Conditions: ALS

Interventions: Device: remote pulmonary function testing
Device: standard pulmonary function testing
Behavioral: Nurse Respiratory Health Coaching (NRHC)

Registration Number: NCT04490148

Lead Sponsor: Milton S. Hershey Medical Center

Brief Summary: Comparison of respiratory outcomes in patients receiving telemedicine-guided remote pulmonary function testing (rPFT) with or without the additional support of nurse coaching. This is a randomized controlled study which assesses the effects rPFT and coaching on respiratory outcomes and quality of life.

Detailed Description: This is a two-arm, randomized study to determine 1) whether weekly monitoring of respiratory function can lead to timelier initiation of NIV and 2) whether structured nurse coaching leads to improved self-efficacy for managing disease and better maintenance of respiratory health. For enrollees in both arms, standard FVC and MIP measurements obtained approximately every three months by the respiratory therapist during ALS Clinic are supplemented with self-administered rPFTs performed weekly. Enrollees in the rPFT+NRHC (nurse respiratory health coaching) arm will additionally receive monthly coaching with the study nurse via telehealth. Outcomes include measures of quality of life and self-efficacy for managing disease.

Recruitment & Eligibility

Status: COMPLETED

Sex: All

Target Recruitment: 34

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
Symptom onset within the last three years.
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).

Caregivers:

Be 18 years of age or older, of either gender.
Be able and willing to provide informed consent.

Exclusion Criteria

Patients:

Use of NIV or diaphragm pacer at time of obtaining informed consent.
FVC ≤50% predicted or MIP > -60 cm of water.
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

Study & Design

Study Type: INTERVENTIONAL

Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
remote PFT (rPFT) longitudinal	standard pulmonary function testing	Subjects will undergo standard pulmonary function testing as part of standard clinical procedure. They will also undergo weekly remote pulmonary function testing using the telemedicine interface and study equipment.
remote PFT (rPFT) + Nurse Coaching longitudinal	standard pulmonary function testing	Subjects will undergo standard pulmonary function testing as part of standard clinical procedure. They will also undergo weekly remote pulmonary function testing using the telemedicine interface and study equipment, and receive monthly coaching from an ALS nurse.
remote PFT (rPFT) longitudinal	remote pulmonary function testing	Subjects will undergo standard pulmonary function testing as part of standard clinical procedure. They will also undergo weekly remote pulmonary function testing using the telemedicine interface and study equipment.
remote PFT (rPFT) + Nurse Coaching longitudinal	remote pulmonary function testing	Subjects will undergo standard pulmonary function testing as part of standard clinical procedure. They will also undergo weekly remote pulmonary function testing using the telemedicine interface and study equipment, and receive monthly coaching from an ALS nurse.
remote PFT (rPFT) + Nurse Coaching longitudinal	Nurse Respiratory Health Coaching (NRHC)	Subjects will undergo standard pulmonary function testing as part of standard clinical procedure. They will also undergo weekly remote pulmonary function testing using the telemedicine interface and study equipment, and receive monthly coaching from an ALS nurse.

Primary Outcome Measures

Name	Time	Method
Date of Identification of Noninvasive Ventilation (NIV) Need From rPFT Monitoring	1 year	NIV is typically prescribed when an individual demonstrates signs of respiratory failure. In this study, forced vital capacity (FVC) of 50% or less of the predicted value serves as a surrogate for respiratory failure and constitutes identification of NIV need. First date of remote measurement of FVC 50% or less of the predicted value is reported here.
Date of Identification of NIV Need From Standard PFT Monitoring	From date of enrollment until first identification of non-invasive ventilation need, assessed up to 1.5 years	NIV is typically prescribed when an individual demonstrates signs of respiratory failure. In this study, forced vital capacity (FVC) of 50% or less of the predicted value serves as a surrogate for respiratory failure and constitutes identification of NIV need. First date of standard, in-clinic measurement of FVC 50% or less of the predicted value is reported here.
Self-efficacy for Managing Medications and Treatments	1 year	The self-efﬁcacy assessment contains questions from the Patient-Reported Outcomes Measurement Information System (PROMIS) item banks on Self-Efficacy for Managing Medications and Treatments (4 items). Cumulative T-scores from each test bank were reported at months 0, 3, 6, 9, and 12, with a mean score of 50±10, higher numbers indicating greater self-efficacy. The reported value is the slope of regression of self-efficacy on time, in T-score points per month.
Self-efficacy for Managing Social Interactions	1 year	The self-efﬁcacy assessment contains questions from the PROMIS item bank on Self-Efficacy for Managing Social Interactions (5 items). Cumulative T-scores from each test bank were reported at months 0, 3, 6, 9, and 12, with a mean score of 50±10, higher numbers indicating greater self-efficacy. The reported value is the slope of regression of self-efficacy on time, in T-score points per month.
Self-efficacy for Managing Symptoms	1 year	The self-efﬁcacy assessment contains questions from the PROMIS item bank on Self-Efﬁcacy for Managing Symptoms (9 items). Cumulative T-scores from each test bank were reported at months 0, 3, 6, 9, and 12, with a mean score of 50±10, higher numbers indicating greater self-efficacy. The reported value is the slope of regression of self-efficacy on time, in T-score points per month.

Secondary Outcome Measures

Name	Time	Method
Sleep Related Impairment	1 year	Patient-Reported Outcomes Measurement Information System (PROMIS) Item Bank v1.0 - Sleep Related Impairment - Short Form 8a (8 items), which uses Likert-type scaling that are summed to a total score. Participants complete this questionnaire each month (12 total administrations). Total score range 8-40, with higher numbers indicating more sleep-related impairment. The reported value is the slope of regression of total score on time, in point change per month.
rPFT Adherence	1 year	Participant adherence to rPFT protocol, defined as the percentage of eligible weeks with a submitted rPFT report.
Dyspnea Characteristics	1 year	Patient-Reported Outcomes Measurement Information System (PROMIS) Pool v1.0 - Dyspnea Characteristics (5 items), which uses Likert-type scaling that are summed to a total score. Participants complete this questionnaire each month (12 total administrations). Total score range 0-44, with higher numbers indicating more severe dyspnea. The reported value is the slope of regression of total score on time, in point change per month.
Respiratory-related Symptoms	1 year	Subjects were asked to report whether they experienced the following symptoms in the past month during each month on the study (12 administrations): excessive secretions, drooling, choking on secretions, morning headache. Reported value is the sum of experienced symptoms (0-4) summed over 12 months. Participants could experience none of these symptoms (0) up to experiencing all symptoms each month (48).
Dyspnea Functional Limitations	1 year	Patient-Reported Outcomes Measurement Information System (PROMIS) Item Bank v1.0 - Dyspnea Functional Limitations - Short Form 10a (10 items), which uses Likert-type scaling that are summed to a total score. Participants complete this questionnaire each month (12 total administrations). Total score range 0-30, with higher numbers indicating more functional limitations due to dyspnea. The reported value is the slope of regression of total score on time, in point change per month.