Evaluation of remote pulmonary function testing in motor neuron disease

Abstract

Introduction: Motor neuron disease (MND) causes respiratory insufficiency, which is managed in part through use of noninvasive ventilation (NIV). Guidelines for the initiation of NIV are based on pulmonary function tests (PFTs), usually performed once every three months. In the setting of MND telemedicine, remote monitoring of respiratory health may permit earlier intervention, but proof of equivalence to conventional PFTs is lacking. Methods: We implemented delivery of remote PFTs (rPFTs), based on our institution’s telemedicine platform, with the goals of validating measurement equivalence to conventional forced vital capacity (FVC) and maximal inspiratory pressure (MIP) assessments, and assessing process acceptability from both patients and therapists. Results: When remotely guided by a respiratory therapist, 40 patient/caregiver teams produced respiratory parameters that were tightly correlated with those acquired through the standard evaluation. Both patients and therapists generally rated the setup and use of the devices positively, with patient ratings higher than those of the therapists. Discussion: This study suggests that rPFTs are accurate and acceptable, and thus may be incorporated into MND telemedicine for clinical and research use.

Keywords:

• Telemedicine
• forced vital capacity
• maximal inspiratory pressure
• pulmonary function testing
• motor neuron disease

Acknowledgments

The authors thank Susan Walsh and Anne Haulman for their assistance in study design.

Declaration of interest

Andrew Geronimo, None.

Dr. Simmons receives research support from Cytokinetics, Mallinckrodt, and Biogen, and has received consulting fees from Biohaven, none of which relate directly to this research.

Additional information

Funding

This project was supported by the National Center for Advancing Translational Sciences, National Institutes of Health [Grants UL1TR000127 and UL1TR002014], the Paul and Harriett Campbell Fund for ALS Research, and the ALS Association, Greater Philadelphia Chapter.