What treatment should be done for a patient with Amyotrophic Lateral Sclerosis (ALS) and Chronic Obstructive Pulmonary Disease (COPD) presenting with increased clear secretions, cough, and difficulty clearing secretions, with hypercapnia?

Management of Respiratory Complications in ALS and COPD with Increased Secretions

For a patient with ALS and COPD presenting with increased clear secretions, cough difficulty, and hypercapnia (pCO2 of 54 mmHg), noninvasive positive pressure ventilation (NPPV) combined with airway clearance techniques should be the primary treatment approach. 1, 2

Initial Assessment and Management

Airway Clearance

• Secretion management techniques:
  • Coughing and forced expiratory maneuvers to aid secretion clearance 1
  • Consider mechanical insufflation/exsufflation devices for airway clearance in ALS 3
  • Physiotherapy with postural drainage and chest percussion
  • Adequate hydration to maintain thin secretions

Ventilatory Support

• Implement NPPV as the patient has:

  • Hypercapnia (PaCO2 > 45 mmHg)
  • Difficulty clearing secretions
  • Combined ALS and COPD pathology 1

• NPPV settings:

  • Start with CPAP 4-8 cmH2O plus pressure support 10-15 cmH2O 1
  • Target normalization of PaCO2 1
  • Consider pressure-controlled settings for better patient synchrony

Contraindications to NPPV

Be aware that copious secretions can be a relative contraindication to NPPV 1. Monitor closely for:

• Inability to clear secretions despite assistance
• Risk of aspiration
• Impaired mental status

Pharmacological Management

COPD Component

• Bronchodilators:

  • Long-acting bronchodilator therapy (LABA+LAMA) 4
  • Consider short-acting bronchodilators (SABA/SAMA) for rescue

• Anti-inflammatory therapy:

  • If blood eosinophilia (≥300 cells/μL) or recent hospitalization for exacerbation, consider triple therapy (LABA+LAMA+ICS) 4
  • If signs of infection (change in sputum characteristics), initiate antibiotics based on local resistance patterns 1

• Corticosteroids:

  • If exacerbation suspected: Prednisone 30-40 mg orally daily for 10-14 days 1
  • If unable to take oral medication, equivalent IV dose

Oxygen Therapy

• Provide supplemental oxygen if hypoxemic (target SpO2 88-90%)
• Caution with high-flow oxygen in hypercapnic patients
• Monitor closely for worsening hypercapnia with oxygen therapy 1

Monitoring and Escalation of Care

Regular Assessment

• Monitor arterial blood gases (ABGs)
• If pH < 7.35 with hypercapnia, deliver NPPV in a controlled environment (intermediate ICU/high-dependency unit)
• If pH < 7.25, consider ICU admission with intubation readily available 1

Criteria for Escalation

Consider invasive ventilation if:

1. NPPV failure: worsening ABGs/pH within 1-2 hours or lack of improvement after 4 hours
2. Severe acidosis (pH < 7.25) and severe hypercapnia (PaCO2 > 60 mmHg)
3. Life-threatening hypoxemia
4. Tachypnea > 35 breaths/min 1

Long-term Management Considerations

For ALS Component

• Offer NPPV to all ALS patients with forced vital capacity < 50% 2
• Consider early discussion about advance directives and end-of-life care 5
• Evaluate for percutaneous endoscopic gastrostomy if dysphagia present 5

For COPD Component

• Reassess need for long-term oxygen therapy
• Consider pulmonary rehabilitation after acute episode resolves 1
• Implement smoking cessation if applicable

Special Considerations

Timing of NPPV Initiation

• If this represents acute-on-chronic respiratory failure, do not initiate long-term NIV during this admission
• Reassess for long-term NIV 2-4 weeks after resolution of acute episode 1

Screening for Sleep Apnea

• Consider screening for obstructive sleep apnea before initiating long-term NIV 1

Pitfalls to Avoid

• Do not delay NPPV if indicated - early implementation improves outcomes 6
• Do not use excessive sedation which may worsen respiratory drive
• Do not withdraw oxygen therapy abruptly in chronically hypercapnic patients 1
• Avoid high-carbohydrate diets and extremely high caloric intake to reduce excess CO2 production 1

This approach addresses both the immediate respiratory compromise and the underlying neuromuscular and pulmonary pathologies, with the goal of improving gas exchange, reducing work of breathing, and enhancing secretion clearance.