Question
Why is noninvasive ventilation (NIV) recommended for patients with neuromuscular diseases experiencing chronic respiratory failure?
Answer
The panel's recommendation of employing non-invasive ventilation aligns logically with this context. However, it is worth highlighting that while clinical indications for non-invasive ventilation are present, they can vary based on factors such as the specific neuromuscular disease, patient age, and the pace of disease progression. In essence, these multifaceted variables influence the suitability of non-invasive ventilation.
The evidence was primarily derived from adults with ALS, given its prevalence as the most common NMD. However, its application should be approached thoughtfully when considering younger children, adolescents, and adults with non-ALS diagnoses. The panel further suggests that its applicability remains subject to these diverse factors even with established indications for non-invasive ventilation. The panel highlights key indicators, such as a decrease in forced vital capacity (FVC) to less than 80% of predicted accompanied by symptoms or an FVC below 50% of predicted symptoms. Similarly, a SNIP or MIP measurement indicative of a reserve of -40 centimeters of water can signify the need for non-invasive ventilation.
The guidelines have transitioned from recommendations and marked a significant development in 2023. Specifically, if a patient's FVC falls below 80% with symptoms or below 50% without symptoms, the situation warrants the implementation of non-invasive ventilation.
This Ask the Expert is an edited excerpt from the course, Respiratory Management of Patients With Neuromuscular Weakness Series: Review Recommendations and Best Practice, presented by presented by Dr. Duane Reed, EdD RRT, RCP.
