ALS Flashcards
most common form of _____ disease
-motor neuron
more common in ____
- males (below age 50)
- after age 50, males=females
Forms of ALS
- Classical Sporadic (most common)
- Pacific (guam)
- Familial (genetic)
Cause of ALS
- unknown
- environmental (diet?)
- trauma
- physical/emotional stress
Average Onset Age
-60 years
Onset
-typically starts in one limb
- Upper Limb
- Lower Limb
- Bulbar (CN S/Sx)
Clinical Course
-progressive deterioration
Bulbar signs
- poor prognosis
- CN symptoms/signs
Life expectancy
-2-5 years from diagnosis
Riluzole
-drug extends life by about 3 months
Pathology
- loss of selective motor neurons
- UMN and LMN signs
CNS Areas Affected
- Cortex, brainstem, spinal cord
- frontotemporal cortex (mental changes)
- posterior columns (sensory)
Mixed UMN & LMN signs
- UMN: spasticity
- LMN: flaccidity
collateral sprouting can cause
- reinnervation
- not as effective as other diseases
UMN S/Sx
- spasticity
- hyperreflexia
- loss of dexterity & speed
- pathological reflex
LMN S/Sx
- weakness/atrophy
- hyporeflexia
- fasciculations
- muscle cramps
Bulbar related S/Sx
- speech/swallowing
- enhanced gag reflex
- suck/snout reflexes
Cognitive Involvement:
Frontotemporal
- verbal/nonverbal fluency
- abstract reasoning
- memory for verbal and nonverbal material
- working memory
- mental flexibility
Activity Limitations
- moility
- ADL/IADL
- Speech production
- Swallowing
- Respiration
-Bowel/bladder spared
Better QoL Associated with
-psychological and existential scales, religious faith and support
QoL not associated with
-physical funciton/strength
ALS
-amyotrophic lateral sclerosis
PT Exam
- cognition
- pain
- joint integrity/ROM
- muscle performance
- motor function
- tone/reflexes
- CN integrity
- balance, gait, function
- respiratory function
- integumentary integrity
- environmental barriers
- endurance/fatigue
ALS Functional Rating Scale
- self-report
- assess various aspects of function
Intervention Goals
-maintain fullest physical, psychological and social function and independence for as long as possible
Unrealistic Goals
- Increase strength
- prevent strength loss over long-term
- alter disease course
Intervention Approaches
- Restorative
- Preventative
- Compensatory
Stages of ALS
-I through VI
Early Disease Challenges
- emotional impact of diagnosis
- uncertain future
- inevitable loss
How Denial can be beneficial
- might help accept horrible diagnosis easier
- digest it in smaller chunks
Middle Disease Challenges
- multiple losses experienced
- great need to make decisions
- may be reluctant to use compensatory strategies
Late Disease Challenges
- confronted with terminal aspects of disease
- worries about family/loved ones
- decisions regarding life-saving measures
Psychological Support
- Education
- stage appropriate hope
- reassure
- caution with web searches
- interdisciplinary approach
- give opportunities for decision making
- emphasize abilities
Remediation
- strengthening
- stretching
- aerobic conditioning
- leisure/recreation
Prevention
- pressure
- respiratory
- spasticity
- DVT
- contractures
- ROM
- bracing
- positioning
Compensation
- AD
- orthotics
- respiratory aids/vents
- home modification
- caregiver training
Spasticity Management
- meds: limited use
- stretching
- positioning
- exercise
Strengthening Guidelines
- early stages only
- don’t strengthen muscles with grade <3/5
- aware of overuse signs
- can strengthen unaffected Mm
- short bouts (10 min, 2-3x/day)
Signs of Respiratory Mm Weakness
- dyspnea
- orthopnea
- sleep disturbance
- daytime hypersomnolence
- morning headaches
- abnormal breathing patterns
Leading cause of death
-respiratory failure
Managing Secretions
- suction catheters
- assisted or mechanical coughing techniques
- pulmonary hygiene methods
- tracheostomy
Trach Tubes
- QoL better for pts
- QoL worse for caregivers (lost jobs, lack of sleep to care for pt etc)
Maintaining Oxygen
-non-invasive positive pressure
Tracheostomy/Mechanical Ventilation
-
Palliative Care
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