How should an amyotrophic lateral sclerosis (ALS) clinic be organized and managed, and which specialist disciplines are required?

Organization and Management of ALS Clinics

ALS clinics should be organized as multidisciplinary care centers operating within a hub-and-spoke model, with core specialist teams including neurologists, physiatrists, pulmonologists, allied health professionals (physical therapists, occupational therapists, speech-language pathologists, dietitians), clinical nurse specialists, social workers, and case managers, as this structure delivers superior survival outcomes and quality of life compared to single-physician care. 1

Organizational Structure

Hub-and-Spoke Model (2024 NASEM Framework)

The most current evidence-based organizational structure consists of three interconnected tiers: 1

• Comprehensive ALS Centers: NINDS-designated tertiary centers providing complex specialized support, research access, and workforce training 1
• Regional ALS Centers: Intermediate facilities offering multidisciplinary care with specialist partnerships 1
• Community-Based Centers: Local multidisciplinary clinics partnering with neurologists, physiatrists, pulmonologists, and community organizations to deliver care closer to patients' homes 1

This tiered system mirrors the National Cancer Institute model and ensures all ALS patients have access to specialized care and research opportunities regardless of geographic location. 1

Core Clinic Operations

Weekly clinic sessions staffed by a consistent core team provide the operational backbone: 2

• Core team members present at every visit: Neurologist, liaison/clinical nurse specialist, and patient advocacy representative 2
• Same-day on-site services: Pulmonary evaluation, physical therapy, occupational therapy, and speech-language pathology 2, 3
• Integrated scheduling: All specialists see the patient during a single visit to minimize travel burden 4, 5

The liaison nurse serves as the central coordinator, tracking all patients through disease registries and interfacing with community paramedical staff to ensure equipment and services are delivered within 1-2 weeks of clinic visits. 2

Required Specialist Disciplines

Medical Specialists

• Neurologist: Primary medical coordinator during diagnostic phase, responsible for diagnosis confirmation and disease-modifying therapy 1, 3
• Physiatrist (Physical Medicine & Rehabilitation): Care coordinator during rehabilitation phase, managing spasticity, mobility aids, and functional optimization 1, 3
• Pulmonologist: Respiratory management including NIV initiation, monitoring pulmonary function every 6 months minimum, and airway clearance strategies 6, 3

Allied Health Professionals

• Physical Therapist: Exercise prescription, mobility assessment, assistive device recommendations, and fall prevention 2, 3, 7
• Occupational Therapist: Activities of daily living adaptations, home modifications, and energy conservation techniques 2, 3, 7
• Speech-Language Pathologist: Dysphagia management, communication device implementation, and swallowing safety assessment 8, 2, 3
• Dietitian/Nutritionist: Nutritional assessment, meal texture modification, gastrostomy timing decisions, and weight monitoring 8, 3, 7

Support Services

• Clinical Nurse Specialist or Nurse Practitioner: Patient tracking, care coordination, symptom management, and liaison between hospital and community services 2, 3, 7
• Social Worker: Resource navigation, insurance coordination, caregiver support, and advance care planning facilitation 3, 7
• Psychologist: Cognitive screening (critical given 40% have cognitive impairment), mood disorder management, and coping strategies 1, 3
• Case Manager: Equipment procurement, home care coordination, and healthcare utilization optimization 3

Care Coordination Across Disease Phases

The Dutch protocol provides the clearest framework for phase-specific coordination: 1

• Diagnostic Phase: Neurologist serves as primary care coordinator 1
• Rehabilitation Phase: Physiatrist assumes care coordination role 1
• Terminal Phase: General practitioner becomes main coordinator, with palliative care integration 1

Critical caveat: Palliative care should be integrated from diagnosis, not reserved for the terminal phase, as early integration improves quality of life without limiting access to disease-modifying treatments. 8

Management Priorities That Drive Clinic Structure

Respiratory Support (Highest Impact Intervention)

• NIV initiation when FVC <80% with symptoms, FVC <50% regardless of symptoms, or PaCO₂ >45 mmHg delivers greater survival benefit than any pharmacologic therapy 6
• Pulmonary function testing every 6 months minimum with FVC/SVC and maximum expiratory pressure measurement 6
• Mechanical insufflation-exsufflation devices when peak cough flow becomes ineffective 6

Nutritional Management (Major Prognostic Factor)

• Videofluoroscopy at diagnosis to detect silent aspiration 8
• BMI and weight monitoring at every visit, as each 5% weight loss increases mortality risk by 34% 8
• Gastrostomy placement before >10% weight loss or severe respiratory compromise (FVC >50%) to avoid 4-fold mortality increase 8

Advance Care Planning

• Initiate discussions at diagnosis, before communication becomes limited 1, 6, 8
• Address preferences for NIV, tracheostomy ventilation, feeding tubes, and end-of-life care 6, 8
• Repeat discussions as disease progresses, as patient priorities change over time 1, 6

Equity and Access Considerations

Partnership with community organizations is essential to reach underrepresented populations, particularly Black Americans who experience significantly worse outcomes. 1 Multidisciplinary centers should establish targeted outreach programs and culturally appropriate care delivery models. 1

Reimbursement barrier: Insurance typically covers <50% of multidisciplinary clinic costs, creating financial disincentives for clinic establishment despite proven survival benefits. 1, 6 Value-based payment models aligned with quality indicators are needed to sustain this care model. 1

Common Pitfalls to Avoid

• Late palliative care referral: The most harmful error, negatively impacting quality of life for patients and caregivers 8
• Delaying respiratory assessment: NIV provides the greatest survival benefit of any ALS intervention and must be initiated based on objective criteria, not patient symptoms alone 6
• Overlooking cognitive screening: 40% have cognitive impairment affecting treatment decisions and NIV compliance; screen all patients before recommending complex interventions 1, 8
• Fragmented care delivery: Requiring multiple separate appointments defeats the integrated care advantage; all specialists must see patients during single visits 4, 5
• Inadequate caregiver support: Caregiver burden is substantial and worsens with patient behavioral deficits; structured support must begin at diagnosis 1, 8