ALS/Lou Gehrig Disease (mod4) Flashcards
ALS Etiology
Unknown, suspected to be hereditary.
- huge cost on care because they fail quickly and need supportive care
Pathophysiology of ALS
Two main subtypes Bulbar onset vs limb onset
- Rapid progression of degeneration of motor neurons…nothing we can do
- Brain = Bulbar: upper motor neurons is a bulbar onset (less % survival rate)
- lower motor neurons is in the limbs (most common)
- weakness, atrophy, fasciculations, spasticity (stiffening of muscles)
Contributors of mortality rate for ALS?
Leading cause of resp failure; due to weakness
- Patient functionally diagnosed as dementia
- patients can have symptoms of both bulbar and limb onset
Diagnosis for ALS?
Mostly based on clinical symptoms; tests may be performed to rule out other neuromuscular disorders such as: Electromyogram, nerve conduction study, MRI, LP, Muscle Biopsy, Nerve Biopsy
Bulbar ALS symptoms
Dysphagia starting at the face (inability to swallow, excessive drooling, inability to speak bc of weakness in facial muscles, risk of aspirations, choking on food)
- Dysarthria (trouble speaking)
- Weakness and spasticity (tightening) of muscles above the neck. advance quickly when they lose control of the upper airway
Limb ALS symptoms
- Cramps and stiffness
- fasciculations
- Muscle atrophy
- loss of strength in hands (losing grip strength)
- balance problems
ALS management?
Multidisciplinary team approach model:
- Early palliative care; holistic approach for diverse needs
- Team that will manage communication, swallowing, mobility, daily living, resp care, cognition
ALS symptom management
Largely involves pulmonary hygiene and mechanical ventilation of some sort:
- Heated high flow humidity especially during infection
- Airway clearance/cough assist via MIE, MLVRM, or manually assisted cough
- Every resp procedure should follow peak cough flows and VC
- Avoid deep suction when possibel
Airway clearance model for ALS
- Monitoring peak cough flow <270 then initiate lung volume recruitment
- manually assisted cough, lung volume if not met
-stacked and peak cough flow <160 with the therapies the patient becomes at risk for trach and you should talk to them about goals of care and what they want for treatment
Benefits of NIV for ALS?
Widely used in first line care; helps with quality of life and sleep.
- As resp muscles decline, they have a harder time sleeping and have issues with daytime sleepiness
- **improves survival, some aspects of HRQOL and sleep
NIV indications for ALS?
When FVC < 65% of predicted
- NIP <40 (negative insp pressure)
- daytime hypercapnia (typically starts as nocturnal) bipap at night nocturnally and measure CO2
- Orthopnea symptoms
- Abnormal nocturnal oximetry
What does invasive ventilation involve for ALS patients?
Usually tracheostomy’s and continued pulmonary hygiene
- must align with patients goals of care
- Risk of locked in state occurring (brain intact but can’t move or communicate with outside world)
NIV parameters for ALS patients?
Spontaneous timed; s/t mode over S when pressure targeted device
- volume targeted ventilation modes (like AVAPS) if pt can’t tolerate pressure targeted modes
- AVAP is used as volume targeted, pressure limited, giving the pt a guaranteed volume at variable pressure
- consider Ti 1.2-1.5 to ensure adequate volumes, set higher to ensure volumes get into lungs
Mask vs mouthpiece for ALS ventilation?
Consider mouthpiece ventilation if patient with bulbar symptoms advances to daytime NIV (can’t tolerate mask on face)
- decreases risk of infection
ALS medications?
- Riluzole: may slow disease progression by months, could slow need for ventilation support
- Edaravone: could slow disease progression
- Anticholinergics: manage hypersalivation
