What is the management approach for Amyotrophic Lateral Sclerosis (ALS)?

Management of Amyotrophic Lateral Sclerosis (ALS)

The management of ALS requires a comprehensive multidisciplinary approach focusing on disease-modifying therapies, symptom management, nutritional support, respiratory care, and early palliative care integration from the time of diagnosis.

Disease-Modifying Therapies

• FDA-approved medications:

  • Riluzole (50 mg twice daily): First-line therapy that extends survival by approximately 2-3 months 1, 2

    • Monitor liver function tests during the first year of treatment
    • Common side effects include nausea, asthenia, and elevated liver enzymes 3

  • Edaravone (60 mg IV): Administered as an intravenous infusion over 60 minutes 4

    • Initial treatment cycle: daily dosing for 14 days followed by a 14-day drug-free period
    • Subsequent cycles: daily dosing for 10 days out of 14-day periods, followed by 14-day drug-free periods
    • Monitor for hypersensitivity reactions and sulfite allergic reactions

Bulbar Symptom Management

• Dysarthria and communication:

  • Early referral to speech-language pathologists for comprehensive evaluation 5
  • Plan for augmentative and alternative communication (AAC) methods before speech is severely affected

• Dysphagia management:

  • Adapt food texture and liquid consistency to facilitate swallowing and prevent aspiration 6, 5
  • Recommend postural maneuvers (chin-tuck posture) to protect airway during swallowing 6
  • Fractionate meals and enrich with high-calorie foods to combat fatigue and weight loss 6
  • Consider oral nutritional supplements when weight loss progresses 6
  • Implement gastrostomy (PEG preferred, RIG for frail patients) when significant dysphagia develops or weight loss >10% occurs 5

Respiratory Management

• Monitor for signs of respiratory insufficiency:
  • Consider non-invasive ventilation (NIV) when respiratory symptoms develop 5
  • Note that bulbar dysfunction makes NIV failure more likely 5
  • Consider controlled ventilation in patients with neuromuscular disease 5
  • Discuss invasive ventilation options (tracheostomy) as disease progresses

Palliative Care

• Integrate palliative care from diagnosis:
  • Focus on symptom management including pain control, spasticity management, and psychological support 5
  • Early referral to palliative services is essential 6, 5
  • Advance care planning should begin early, discussing preferences regarding:
    • Resuscitation status
    • Ventilatory support
    • Artificial nutrition and hydration
    • Preferred place of death 5

Multidisciplinary Care

• Coordinate care through specialized ALS clinics when available:
  • Neurologist
  • Pulmonologist
  • Gastroenterologist
  • Speech-language pathologist
  • Physical and occupational therapists
  • Nutritionist
  • Palliative care specialist
  • Social worker
  • Mental health professional

Common Pitfalls and Caveats

1. Delayed diagnosis: The average time from symptom onset to diagnosis is approximately 12 months, delaying beneficial interventions 7

   • Early diagnosis allows earlier initiation of disease-modifying therapy and multidisciplinary care

2. Inadequate palliative care: Common issues include 6:

   • Late referral to palliative services
   • Focus only on respiratory and nutritional issues while neglecting other distressing symptoms
   • Limited access to supportive equipment
   • Inadequate preparation for loss of communication ability
   • Insufficient support for family caregivers

3. Respiratory management challenges:

   • Deterioration in patients with neuromuscular disease can be very sudden 5
   • Patients with bulbar dysfunction may have more difficulty with NIV 5

4. Nutritional challenges:

   • Weight loss >10% is associated with 2.5 times higher mortality risk 5
   • Implement nutritional interventions before significant weight loss occurs

By implementing this comprehensive approach to ALS management, clinicians can help improve quality of life, potentially extend survival, and provide appropriate supportive care throughout the disease course.