Do ALS (Amyotrophic Lateral Sclerosis) patients require a tracheotomy tube with a cuff?

Tracheostomy Tube Selection for ALS Patients

Yes, ALS patients requiring tracheostomy should use a cuffed tracheostomy tube, particularly when they need mechanical ventilation or have aspiration risk, with high-volume/low-pressure cuffs being the preferred design. 1

Primary Indications for Cuffed Tubes in ALS

ALS patients typically require cuffed tracheostomy tubes for several critical reasons:

• Mechanical ventilation support: Cuffed tubes are essential for patients requiring ventilation with positive pressures, which is common in advanced ALS as respiratory muscle failure progresses 1
• Nocturnal ventilation needs: Many ALS patients require ventilation only at night, making cuffed tubes ideal as the cuff can be inflated during nocturnal ventilation and deflated during the day to allow speech and breathing around the tube 2
• Aspiration prevention: Chronic translaryngeal aspiration, which occurs in ALS patients with bulbar dysfunction, is a specific indication for cuffed tubes 1

Optimal Cuff Type and Management

High-volume/low-pressure cuffs are strongly preferred over low-volume/high-pressure cuffs to minimize tracheal wall damage and preserve airway epithelium perfusion 1:

• Cuff pressure must be maintained between 20-30 cmH₂O for air-filled cuffs to prevent both tidal volume loss and tracheal injury 3, 4
• Hyperinflation of cuffs to eliminate air leaks should be avoided despite being common practice, as it significantly increases the risk of tracheal injury 3
• Critical pitfall: A 2015 study of ALS patients with long-term tracheostomy found that 99% of caregivers were adding volume to cuffs without pressure measurement, resulting in dangerously high mean cuff pressures of 40 cmH₂O—double the recommended maximum 5

Cuff Management Protocol

The cuff management strategy should follow this algorithm:

• During mechanical ventilation: Keep cuff inflated with pressure maintained at 20-30 cmH₂O, checking pressure regularly with manometry 3, 4
• During ventilator weaning: Maintain cuff inflation throughout the weaning process from assisted to pressure-support mode 3
• After successful weaning: Once the patient no longer requires ventilation and is at low risk for aspiration, the cuff may be deflated or the tube changed to a cuffless design 3
• For speech facilitation: One-way speaking valves or capping can be considered only after the patient tolerates cuff deflation or transition to a cuffless tube 3, 2

Special Considerations for ALS Population

The decision for tracheostomy timing in ALS is particularly nuanced:

• Avoid emergency tracheostomy: Tracheostomy should not be performed during emergency situations in ALS patients; it requires a stable condition and prior reflection with the patient and family 6
• Glottic function is the key determinant: Long-term success with non-invasive ventilation and avoidance of tracheostomy depends primarily on preserved glottic function rather than inspiratory or expiratory muscle strength 7
• Oximetry-guided approach: When baseline SpO₂ drops below 95% and cannot be corrected by non-invasive ventilation or mechanically assisted coughing, tracheostomy or death typically occurs within 2 months 7

Long-Term Care Requirements

ALS patients with tracheostomy require intensive caregiver education and professional oversight 5:

• Caregivers must be trained in proper cuff pressure monitoring using manometry, not volume-based inflation 5
• Regular professional assessment is essential, as the mean duration of tracheostomy care in ALS patients is approximately 3 years 5
• Suctioning frequency is high (mean 27.5 times daily), requiring closed-circuit suctioning systems with inline catheters to minimize aerosolization 2, 5

Tube Features to Consider

Additional features that may benefit ALS patients include:

• Inner cannula: Dual-cannula tubes allow cleaning without changing the entire tube, beneficial for patients with thick secretions 1, 8
• Subglottic suction port: Tubes with suction ports above the cuff can significantly reduce aspiration volume, particularly with continuous suction 9
• Proper sizing: Select tube length extending at least 2 cm beyond the stoma while remaining 1-2 cm above the carina, with diameter avoiding tracheal wall damage 1