Incidence

Advanced Care Planning in ALS

Outline

• Introduction
• Epidemiology and Prognosis of ALS
• Diagnosis
• Diagnosis Communication
• Drug Treatment
  • Disease Modifying
  • Survival Prolongation
  • Symptomatic Treatment
• Multidisciplinary Care
  • Respiratory Care
  • Nutrition
  • Caregiver Fatigue
• End of Life Care

Introduction

What is ALS?

Epidemiology

• Median survival: 20-48 months from symptom onset
• Mean age of onset: 50 years old
•  Incidence: 1.4 cases per 100,000
•  M:F: 170:100
• Cumulative lifetime risk: 1:300 or 1:400
•  Philippine Data: None

Diagnosis

• Evidence of LMN degeneration by clinical, neurophysiological and
  neuropathologic means
• Evidence of UMN degeneration by clinical means
•  Progressive symptoms affecting a region or regions
•  Absence of other disease that can explain the above symptoms

 

Upper Motor Signs (Brain)	Lower Motor Signs (Brainstem and Spinal Cord)
• Spasticity	• Weakness
• Hyperreflexia	• Fasciculations
• Spreading reflexes	• Atrophy
• Pathologic Reflexes

• El Escorial Criteria
• Revised El Escorial Criteria
• Awaji Criteria

Segment	Muscles Involved	UMN	LMN
Bulbar	Facial muscles, Tongue, Muscles for speech and deglutition	Exaggerated gag, snout reflex, pseudobulbar affect and forced yawn	Atrophic tongue and facial muscles, Dysphonia, Dysphagia
Cervical	Muscles in the Upper Extremities	Hoffman, Tromner and finger flexor reflexes	Weakness, atrophy, fasciculations in the Upper Extremities
Thoracic	Muscles of respiration and paraspinal muscles	Loss of superficial abdominal reflexes	Respiratory problem
Lumbosacral	Lower extremities	Extensor plantar reflexes	Weakness, atrophy and fasciculations in the lower extremities

Diagnosis

• Clinically Definite
• Clinically Probable
• Clinically Probable- Laboratory Supported
• Clinically Possible

Diagnosis

•  Clinical signs with high sensitivity and specificity
  • Split Hand Syndrome
  • Split Hand Index
• Neuromuscular ultrasound in detecting fasciculation
• Clinically Probable- Laboratory Supported
• Axonal Excitability Studies

Disease Communication/Breaking the News in ALS

• Represents the beginning of treatment
• Basis of the patient’s autonomy
• The first things said are often the most remembered

Why breaking bad news is difficult for doctors?

1. Fear of messenger being blamed for the bad news
2. Perceived lack of time
3. Lack of training
4. Fear of being asked difficult questions causing distress
5. Invoking fears of one’s own mortality

Process of Diagnosis Communication

• Quiet and comfortable location, convenient time, permitting the
  patient to question or express doubts with the patients relatives or
  caregivers present
• Clear language
• Non verbal communication is important

Content of Diagnosis Communication

Preference:

• 87% prefers to have as much information as possible
•  5% prefers to have additional information only if it was optimistic
•  8% leave to the discretion of their physician

Content:

• 60% wants to know the disease (not the name)
• 54% wants to know about treatment and outcome
•  8% leave to the discretion of their physician

Drug Treatment

• Disease Modifying Drugs: None
• Survival Prolonging Drugs
  • Riluzole: offers 2-3 months survival benefit which may be greater in those
  with bulbar onset and younger patients
  • Edaravone: decline on the ALS FR by 2.49 points fewer than placebo group
  • Downside: stringent inclusion criteria, limited duration of data
•  Symptomatic Treatment

Symptomatic Treatment

Multidisciplinary Care

• Respiratory Care
• Nutrition
• Communication
• Caregiver Fatigue

Respiratory Car

Goals:

1. Prolong survival
2. Improve quality of life

Mechanism of respiratory failure in ALS:
-Loss pf phrenic nerve function

Signs and symptoms of respiratory failure

• Difficulty in speech
• Difficulty in airway clearance
• Aspiration as bulbar muscle coordination declines

Surrogates for respiratory function

1. Forced Vital Capacity
2. Maximum Inspiratory Pressure
3. Maximum Expiratory Pressure

Guidelines for qualification of respiratory assist device

• Diagnosis of ALS plus
• O2 sats of <89% for 5 mins on overnight oximetry
• Increased PaCO2 of >45 mmHg or
• FVC <50%
• MIP < -60 cm H20

Respiratory Assistive Devices

• Non- Invasive Ventilation (NIV)
  • Standard of care for respiratory support in patients with ALS
  • Appropriate time to start: unclear but new data suggest that early NIV
    adaption in the disease course (FVC >80%) may provide additional mortality
    benefit
• Diaphragmatic Pacing
  • Not recommended
• Mechanical Insufflation- Exsufflation
  • Augment respiratory mechanics and produce an adequate cough
• Tracheostomy
  • Only 2-5% undergo tracheostomy
  • In theory, best done prior to emergent/urgent need but in reality, often occur in times of acute respiratory failure
  • Patients who choose tracheostomy:
    • Younger
    • Have young children
    • Higher degrees of education
    • High socio economic status
    • Definitely not doctors ?

Nutrition

• Dysphagia and aspiration
  1. Malnutrition
  2. Severe weight loss
  3. Dehydration
  4. Aspiration pneumonia

PEG

• No firm rules regarding the timing of feeding tube

AAN Guidelines: should be offered at the onset of symptomatic
dysphagia and accelerated weight loss:

1. Preferably done prior t significant respiratory functional decline (FVC<50%)
2. PEG may still be offered in patients with declining respiratory function

*No studies: oral vs enteral feeding

Nutrition

• High calorie and protein enriched diets combined with snacks and nutritional shakes are encouraged

Communication

• Successful communication strategies can improve quality of life especially as some patients with ALS will lose effective verbal communication
• Options:
  • Writing (Pen and Paper) or E-writers
  • Text to speech applications
  • Eye gaze technologies
  • Voice banking options
  • One push solution for medical emergencies

End of Life Care

• Conversations regarding end of life decision-making should be held early in the disease course before a respiratory or nutritional crisis occurs
• Quality of life discussions include defining what interventions are consistent with the patient’s wishes
• Palliative and hospice care may have a role
• Caregiver fatigue and burden should be assessed on a ongoing basis