Respiratory Management in Patients with ALS

Assessment of Respiratory Function

The American Thoracic Society recommends performing pulmonary function tests (PFTs) at a minimum of every 6 months to monitor respiratory decline in ALS patients, including measurements such as maximum inspiratory pressure (MIP) or maximum expiratory pressure (MEP) 1, 2
Sleep disturbances should be screened for at each visit, as they are an important indicator of respiratory insufficiency 1, 2

The American College of Chest Physicians recommends considering NIV when any of the following are present: FVC < 80% of predicted in symptomatic patients, FVC < 50% of predicted, MIP < -60 cm H₂O, MEP < 40 cm H₂O, PCF < 270 L/min, evidence of sleep-disordered breathing on polysomnography, or hypercapnia (awake PaCO₂ > 45 mm Hg) 3
Polysomnography or overnight oximetry can help guide NIV initiation when resources allow, but is not necessary for adult patients 2

The American Thoracic Society recommends individualizing NIV treatment to achieve optimal ventilation goals, including adjusting parameters such as mode of ventilation, inspiratory time, and inspiratory/expiratory pressures, and assessing sleep quality, digital downloads, unintentional leaks, and oximetry (capnography when available) 2
For patients with preserved bulbar function, the American College of Chest Physicians recommends considering mouthpiece ventilation (MPV) for daytime ventilatory support as an adjunct to nocturnal mask NIV 2

The American Thoracic Society recommends starting with a trial of anticholinergic medication as first-line therapy for patients with sialorrhea (excessive saliva), and considering botulinum toxin therapy to salivary glands if anticholinergics are inadequate or poorly tolerated 2
For patients with reduced lung function or cough effectiveness, the American College of Chest Physicians recommends implementing lung volume recruitment techniques (breath stacking) and considering mechanical insufflation-exsufflation devices for secretion clearance 2

The European Society for Clinical Nutrition and Metabolism recommends screening for malnutrition (BMI, weight loss) at diagnosis and every 3 months during follow-up, and considering gastrostomy for nutritional support when dysphagia develops 4, 5

The American College of Chest Physicians recommends considering invasive home mechanical ventilation via tracheostomy when NIV fails or is not tolerated, bulbar function worsens, frequent aspiration occurs, insufficient cough persists despite adequate secretion management, recurrent chest infections occur despite secretion management, or lung function continues to decline 2, 5
The American Thoracic Society recommends starting discussions regarding invasive ventilation early in the disease course, including goals of care, potential need for institutionalization, and caregiver burden 2