Palliative Care Management Options for End-Stage ALS
A palliative care approach should be adopted from the time of ALS diagnosis, with early referral to specialized palliative services to improve quality of life and reduce suffering for patients with worsening ALS. 1, 2
Timing of Palliative Care Intervention
- The European Federation of Neurological Sciences recommends palliative care should be integrated from diagnosis, not just at end-of-life 1
- Early referral is essential as patients with end-stage ALS rapidly develop distressing symptoms and may lose ability to communicate 1
- Despite recommendations, palliative care is often initiated too late, with studies showing terminal phase management is unsatisfactory in 33-61% of cases 1
Multidisciplinary Care Framework
- Evidence shows multidisciplinary care improves both survival and quality of life in ALS 1, 2, 3
- Key team members should include:
- Neurologist
- Palliative care specialist
- Respiratory therapist
- Nutritionist
- Speech-language pathologist
- Physical/occupational therapist
- Social worker/psychologist
- Home care coordinator
Symptom Management in End-Stage ALS
Respiratory Management
- Monitor for signs of respiratory insufficiency (orthopnea, dyspnea, morning headaches)
- Options include:
- Non-invasive ventilation (NIV) - improves quality of life and may extend survival
- Invasive ventilation via tracheostomy (requires extensive discussion about long-term implications)
- Oxygen therapy (use cautiously as it may suppress respiratory drive)
- Medications for dyspnea (opioids, benzodiazepines)
Nutritional Support
- For dysphagia:
- Modify food textures and liquid consistency
- Fractionate and enrich meals
- Consider oral nutritional supplements 2
- Gastrostomy tube placement before respiratory function significantly declines
Pain Management
- Pain is common but often undertreated in ALS 1, 4
- Address specific causes:
- Musculoskeletal pain (positioning, physical therapy, analgesics)
- Neuropathic pain (gabapentin, pregabalin)
- Spasticity (baclofen, tizanidine)
- Cramps (quinine, magnesium)
Secretion Management
- For excessive saliva:
- Anticholinergics (glycopyrrolate, scopolamine)
- Botulinum toxin injections to salivary glands
- Suction devices for home use
- For thick secretions:
- Adequate hydration
- Mucolytics
- Mechanical insufflation-exsufflation devices
Communication Support
- Augmentative and alternative communication (AAC) devices
- Eye-tracking technology
- Brain-computer interfaces for severely impaired patients
Psychosocial and Spiritual Support
- Regular assessment for depression and anxiety
- Psychological support for both patient and caregivers 3
- Address spiritual needs and existential distress
- Support groups for patients and caregivers
- Respite care options for family caregivers
End-of-Life Planning
- Advance care planning should begin early in disease course 3
- Discuss and document preferences regarding:
- Resuscitation status
- Ventilatory support
- Artificial nutrition and hydration
- Preferred place of death
- Regular reassessment of goals of care as disease progresses
Medication Management
- Riluzole (50mg twice daily) remains the primary disease-modifying medication, though it only extends life by approximately 2-3 months 5, 6
- Consider discontinuing medications that no longer provide benefit or may cause burden
- Focus on medications that improve comfort and quality of life
Home-Based vs. Facility-Based Care
- Most ALS patients prefer to die at home 1
- Community palliative care programs can provide valuable home-based services and respite care
- Hospice enrollment should be considered when:
- Critically impaired breathing
- Rapid disease progression
- Severe nutritional impairment
- Life-threatening complications 1
Common Pitfalls in End-Stage ALS Care
- Late referral to palliative services (only 28% of patients in one study successfully accessed palliative care) 1
- Focusing only on respiratory and nutritional issues while neglecting other distressing symptoms like pain, frozen shoulder, and mouth ulcers 1
- Limiting access to supportive equipment once formal palliative care begins 1
- Inadequate preparation for loss of communication ability
- Insufficient support for family caregivers
Palliative care for ALS should be integrated throughout the disease course, not just at the end of life. Early involvement of palliative specialists alongside the multidisciplinary ALS team provides the best opportunity to address the complex physical, emotional, and existential needs of patients with this devastating disease.