Remote Pulmonary Function Testing and Nurse Coaching in ALS

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.

Inclusion Criteria:
Patients:
1. Possess a diagnosis of definite, probable, probable laboratory-supported, or
possible ALS by revised El Escorial research criteria [Brooks2000].
2. Be 18 years of age or older.
3. Have a caregiver available to participate in the study
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).
Caregivers:
1. Be 18 years of age or older, of either gender.
2. Be able and willing to provide informed consent.
Exclusion Criteria:
Patients:
1. Use of NIV or diaphragm pacer at time of obtaining informed consent.
2. FVC ≤50% predicted or MIP > -60 cm of water.
3. 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.
4. Cognitive impairment, as judged by the ALS clinic neurologist, that prevents
participation in the study.
Caregivers: None