Objective:

To quantify practice patterns in respiratory support among ALS Centers

Background:

Substantial increases in available methods of assessing ventilation and treatment options for people with ALS (PALS) has led to wide variations in practice patterns. Payors, such as Medicare (CMS), dictate some patterns, though these vary among payers and are different by country. Since noninvasive ventilation (NIV) has survival and quality of life benefits in PALS, but opinions on timing and methods of ventilation vary, we sought to better understand practices for ventilatory support among ALS Centers.

Design/Methods:

The Northeast ALS Consortium (NEALS) Ventilation and Respiratory Care Committee conducted an online survey of ALS Centers in the U.S., Canada, Mexico, Japan and Australia on respiratory practices. The survey was completed by 70 sites from January to March 2024.

Results:

The most common means of testing for ventilatory impairment was in-office spirometry. The vast majority at least sometimes used nocturnal oximetry with or without capnography or overnight polysomnography.  The least commonly used tools were transcutaneous CO2 monitoring and in-home unsupervised spirometry, with 60-70% of respondents never using these modalities. The majority of US respondents favored the European FVC guidelines for NIV justification (FVC<80% predicted with pulmonary symptoms) over CMS guidelines (FVC<50% predicted regardless of pulmonary symptoms). Respiratory muscle strength training was commonly used by over 60% of centers. NIV daytime usage with mouthpiece ventilation was used by about half the centers. Non-invasive ventilators (NIV) rather than respiratory assist devices (RAD) was the preferred first treatment for respiratory insufficiency. 

Conclusions:

Even among specialized centers, practice patterns vary substantially for PALS respiratory assessment and treatment. Consensus was clear for preference of the most established measure of ventilatory function and treatment of ventilation failure. Further investigation is needed for broader access for spirometry, NIV, and other ventilatory support for PALS.