What is the management of Amyotrophic Lateral Sclerosis (ALS)?

Management of Amyotrophic Lateral Sclerosis (ALS)

ALS management requires immediate integration of multidisciplinary palliative care from the moment of diagnosis, not at end-of-life, with proven benefits for both survival and quality of life. 1, 2

Multidisciplinary Team Approach

Establish a multidisciplinary team immediately upon diagnosis that includes neurology, pulmonology, nutrition, speech therapy, physical therapy, occupational therapy, psychology, and palliative care specialists. 1, 3

• Meta-analysis demonstrates that multidisciplinary care extends survival by a mean of 141.67 days compared to general neurology care alone (p=0.0005), with even greater benefit for bulbar-onset patients. 2
• This approach improves both survival duration and quality of life outcomes, particularly mental health scores. 2

Disease-Modifying Pharmacotherapy

Two FDA-approved medications provide modest disease modification: 4, 5

• Edaravone (intravenous): 60 mg IV infusion over 60 minutes. Initial cycle consists of daily dosing for 14 days followed by 14-day drug-free period. Subsequent cycles involve daily dosing for 10 days out of 14-day periods, followed by 14-day drug-free periods. 4
• Riluzole: A glutamate receptor antagonist that modestly extends survival. 5

Common pitfall: Edaravone contains sodium bisulfite and may cause allergic reactions including anaphylaxis in susceptible individuals; screen for sulfite allergies before initiating. 4

Respiratory Management

Non-invasive ventilation (NIV) is the single most important intervention for improving both quality of life and survival. 1, 3

• Initiate NIV when respiratory dysfunction develops, typically indicated by symptoms of nocturnal hypoventilation, orthopnea, or declining forced vital capacity. 1
• NIV compliance may be reduced in patients with cognitive impairment; assess cognitive function before recommending. 6, 1
• Invasive mechanical ventilation decisions require careful advance planning, as only 4-9% of patients choose this option, with wide cultural variation (4% UK, 0% USA, 9% Japan). 6

Nutritional Support

Implement early nutritional interventions before significant weight loss occurs. 1, 3

Dysphagia Management Strategies:

• Food texture modification to prevent aspiration and improve intake, recognizing that both oral and pharyngeal swallowing stages may be compromised even without obvious clinical signs. 1
• Chin-tuck posture as primary airway protection technique to prevent laryngeal penetration. 1
• Head rotation for hypertonicity or incomplete upper esophageal sphincter closure. 1
• Throat clearing every 3-4 swallows to prevent post-swallow aspiration in patients with laryngeal penetration. 1

Feeding Tube Considerations:

• Gastrostomy placement rates vary significantly by country (23% UK, 50% USA, 35% Japan), with mean feeding duration of 11-18 months. 6
• Consider gastrostomy when oral intake becomes inadequate, but carefully evaluate in patients with significant cognitive or behavioral deficits, as compliance may be poor. 6, 1

Nutritional Supplementation:

• Advise multiple small meals throughout the day for patients with fatigue. 1
• Recommend high-calorie meal enrichment before resorting to commercial supplements. 1
• Add dietary fiber for constipation related to abdominal muscle weakness. 1

Cognitive and Behavioral Assessment

Screen all patients for cognitive impairment, as up to 40% have evidence of cognitive dysfunction that significantly impacts treatment decisions and prognosis. 6, 1

• Patients with frontotemporal dementia or executive dysfunction have significantly shorter survival. 6, 1
• Cognitive impairment reduces likelihood of choosing long-term mechanical ventilation and decreases NIV compliance. 6, 1
• Executive dysfunction increases risk of falls, choking episodes, and injuries, and reduces compliance with assistive devices. 6
• Critical consideration: Insistence on NIV or feeding tube placement in patients with significant behavioral and cognitive deficits may be inappropriate and should be evaluated individually. 6

Advance Care Planning

Initiate advance directive discussions early in the disease course, ideally at diagnosis, before communication becomes limited. 6, 1

Timing and Triggers for End-of-Life Discussions:

• Patient distress 6
• Disease progression milestones 6
• Patient's expressed desire to discuss these issues 6

Key Discussion Points:

• Preferences regarding ventilatory support (non-invasive vs. invasive) 1
• Feeding tube placement 1
• End-of-life care preferences 1

Important caveat: Although advance directives are considered useful in 78% of European centers, only 30% of patients actually complete them, highlighting the need for persistent, gentle encouragement. 1

Symptom Management

Sialorrhea:

• Anti-muscarinic therapy or botulinum toxin A injections, though these do not improve dysphagia itself. 1

Spasticity and Cramps:

• Address with appropriate pharmacotherapy and physical therapy interventions. 7

Pseudobulbar Affect:

• Recognize and treat this common symptom that impacts quality of life. 7

Pain:

• Despite being a motor neuron disease, pain management is an important component of care. 7

Depression:

• Screen regularly and treat aggressively, as depression is common and treatable. 7

Caregiver Support

Implement structured caregiver support from diagnosis, as caregiver burden is substantial and worsens with behavioral deficits in patients. 6, 1

• Provide counseling services 1
• Facilitate support groups 1
• Establish crisis management systems 1
• Recognize that behavioral deficits in ALS patients have significant negative impact on caregiver quality of life. 6

Coordination of Care Across Disease Phases

The Dutch model provides a clear framework for care coordination: 6

• Diagnostic phase: Neurologist serves as care coordinator 6
• Rehabilitation phase: Rehabilitation medicine consultant coordinates 6
• Terminal phase: General practitioner becomes primary coordinator 6

Critical Pitfalls to Avoid

Late referral to palliative services is the most common and harmful error, negatively impacting quality of life for both patients and caregivers. 6, 1

• Early palliative care integration allows relationship building with staff before communication becomes severely limited. 1
• Paradoxical barrier: In some healthcare systems, formal palliative care enrollment may actually limit access to assistive equipment, necessitating careful integration within broader multidisciplinary care rather than complete transition. 1
• Do not wait for "terminal phase" to involve palliative care; the palliative approach should begin at diagnosis. 6, 1