Management of ALS Patients Using Volume-Controlled Ventilation (VCV)

Respiratory Assessment and Monitoring

The American Thoracic Society recommends performing pulmonary function tests (PFTs) at minimum every 6 months to track respiratory decline, including measurements of maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and forced vital capacity (FVC) in patients with Amyotrophic Lateral Sclerosis (ALS) using Volume-Controlled Ventilation (VCV) 1
Screening for sleep disturbances at each clinical visit is important as these are indicators of respiratory insufficiency in ALS patients 1
Waveform capnography is used to confirm and continuously monitor airway and ventilation status during respiratory support in ALS patients 2, 3

Adjusting ventilator parameters based on patient comfort and tolerance is crucial for effective VCV in ALS patients 4
Sleep quality assessment is essential for optimizing VCV settings, as poor sleep quality can impact respiratory function in ALS patients 4, 5
Digital downloads from the ventilator and monitoring of unintentional leaks are important for adjusting ventilator parameters in ALS patients 4
Oximetry readings and capnography (when available) are used to adjust ventilator parameters and optimize VCV settings in ALS patients 4

The American Academy of Neurology recommends implementing lung volume recruitment techniques (breath stacking) for patients with reduced lung function or cough effectiveness 1
Mechanical insufflation-exsufflation devices are considered for secretion clearance in ALS patients 1
Anticholinergic medication is recommended as first-line therapy for sialorrhea (excessive saliva) in ALS patients 1
Botulinum toxin therapy to salivary glands is considered if anticholinergics are inadequate or poorly tolerated in ALS patients 1

Mouthpiece ventilation (MPV) is considered as a daytime ventilatory support option for patients with preserved bulbar function 1, 4
MPV allows ventilatory support through an angled mouthpiece and has been used successfully to avoid tracheostomy in ALS patients 4

Screening for malnutrition (BMI, weight loss) at diagnosis and every 3 months during follow-up is essential for ALS patients 1
Percutaneous endoscopic gastrostomy (PEG) is considered for nutritional support when dysphagia develops in ALS patients 6
PEG is recommended as the preferred approach for gastrostomy, with radiologically inserted gastrostomy (RIG) as an alternative for more frail ALS patients 6

Transitioning to invasive mechanical ventilation via tracheostomy is considered when NIV fails or is not tolerated, bulbar function worsens significantly, frequent aspiration occurs, insufficient cough persists, or recurrent chest infections occur in ALS patients 1, 4

Delaying NIV initiation in patients with respiratory symptoms should be avoided, as early intervention may improve outcomes in ALS patients 1, 4
Progressive bulbar symptoms may limit the use of non-invasive options in ALS patients 4