Unit 1: Amyotrophic Lateral Sclerosis (ALS) Flashcards
Amyotrophic Lateral Sclerosis (ALS)
“Lou Gehrig’s Disease”
- rapidly progressing; fatal CNS (brain and spinal cord) disease that affects voluntary muscle control
- gradual degeneration and death of motor neurons; muscle weakness, atrophy, and twitch
- loss of ability of brain to initiate muscle movement
- does not impair the senses or ability to think
- eventually, all muscles are affected, and patients are paralyzed, but sensation is intact
- most die from respiratory failure within 3 to 5 years of onset of manifestations
Pathophysiology of ALS
- motor neurons are located throughout the CNS and serve as controlling and communication links between the nervous system and voluntary muscles
- messages are passed from upper motor neurons (located in brain) to lower motor neurons (located in spinal cord) and on to particular muscles
- in ALS, both upper and lower neurons degenerate and die
- unable to function, muscles gradually weaken, atrophy, and twitch (fasciculation)
- the progressive degeneration leads to death of the cells, resulting in brain losing the ability to initiate and control muscle movement
Bulbar ALS
“progressive bulbar palsy”
- form of ALS
- affects the muscles involved in speech, swallowing, and tongue movements
Why do patients with ALS eventually have respiratory compromise?
ALS affects the diaphragm and chest wall
-respiratory failure and need for placement of an artificial airway and ventilator support
Clinical Manifestations of ALS
-any muscle group may first be affected
-described first as general complaints of:
>muscle cramps or stiffness
>muscle weakness on one part of the body first
>slurred speech
>difficulty swallowing (dysphagia)
-early clinical manifestations depend on which part of the body is damaged first
-as disease progresses, experiences weakness and atrophy in other parts of the body
To be diagnoses with ALS
- must have clinical manifestations of both upper and lower motor neuron damage that cannot be attributed to other causes
- Upper motor neuron damage: spasticity (increase in muscle tone/ stiffness)
- Lower motor neuron damaged: flaccidity (flabby; muscle weakness)
Medical Management for ALS
- no single test is used to diagnose
- needs complete history and physical exam
- includes: muscle strength and endurance testing to see if weakness, spasticity, atrophy, and hyperreflexia (increased reflexes) are increasing
- CT scan and/or MRI of the neck and head to r/o other conditions that mimic ALS (spinal cord tumor, herniated disk, spondylosis, brain tumor, MS etc.)
- Genetic testing if there is a known family hx
- Electromyography (EMG) and nerve conduction studies used to determine which nerves are damaged and to r/o other conditions (peripheral neuropathy)
- Swallow studies to see if the muscles controlling the patient’s ability to swallow have been affected b/x the patient is at risk for aspiration w/ dysphagia
- Lumbar puncture needed to evaluate CSF and cytology
- no cure exists
- management focuses on slowing disease progression and managing clinical manifestations
- Riluzole (Rilutek) first medication approved to slow disease progression
Treatment
- medications for symptom relief
- physical, occupational, and speech therapies used to maximize function
- as disease progresses and muscles weaken, assistive and breathing devices may be used
- nutrition important component; patients tend to loose weight; gastric feeding tube must be placed to help meet nutritional requirements
- as the chest and diaphragm muscles weaken, need to consider forms of mechanical ventilation requiring a tracheotomy
Medications used in the Treatment of ALS
- Baclofen: for muscle cramps
- laxatives and stool softeners for constipation (Milk of magnesia, Miralax, Lactulose) or Dulcolax, Senokot
- Analeptics (CNS stimulants) for fatigue and weakness
- Tricyclic antidepressants for excessive salivations, pain, and depression
- Riluzole (Rilutek)
- Edaravone (Radicava)
Medications: Baclofen
antispasmodic
- muscle relaxant
- depresses CNS to reduce pain and inhibit reflexes at the spinal level to decrease muscle spasm
- for ALS, used for muscle cramps
- Side Effects: seizures, dizziness, drowsiness, fatigue, weakness, nausea
Medications: Riluzole (Rilutek)
- glutamate inhibitor; decreases glutamate levels, an amino acid that affects the nerves that sends messages from the brain to muscles
- used for ALS
Medications: Edaravone (Radicava)
- glutamate inhibitor
- improved functional ability
- for ALS
- Side Effects: hypersensitivity reactions, headache, contusion, gait abnormality
- Nursing: monitor for S/S of hypersensitivity reactions (redness, wheals, erythema multiforme, urticaria (hives), decreased BP, dyspnea) w/ each infusion
Complications of ALS
ALS is a progressive disease that leads to the inability to move -aspiration of food or fluid -respiratory failure -pneumonia -pressure injuries -deep vein thrombosis (DVT) -pulmonary embolism (PE) -constipation -contractures -depression -weight loss -loss of the ability to care for self >important to make decisions about enteral feedings, intubation and mechanical ventilation, and end-of-life decisions
Nursing Management: Assessment and Analysis
first clinical manifestations of ALS are general complaints b/c of weakness of the involved muscles
>Clinical presentation r/t the progressive wasting of the motor neurons:
-difficulty breathing
-difficulty swallowing; choking, drooling
-speech problems; slurring words, slow speech
-voice changes; hoarseness
-muscle cramps
-head drops d/t weak neck muscles
-muscle twitching (fasciculation)
-increasing muscle weakness- localized to one part of the body and then spreads
-paralysis
-weight loss
-tongue atrophy
>although muscle weakness is the initial hallmark sign of ALS, clinical manifestations vary w/ each individual, and can begin in the muscles of the arms, legs, hands, or feet, or in the muscles of speech and swallowing
>although everyone w/ ALS does not experience the same clinical manifestations or progression of the disease, progressive muscle weakness and paralysis are universally experienced
Nursing Diagnoses for ALS
- ineffective airway clearance r/t weak cough secondary to motor neuron death and muscle weakness
- risk for aspiration r/t weakness of muscles of swallowing
- ineffective breathing pattern r/t weakness of the respiratory muscles secondary to motor neuron dysfunction
- potential for injury r/t impaired physical mobility secondary to motor neuron death and muscle weakness
- impaired oral communication r/t dysarthria (motor speech disorder) and tongue atrophy secondary to motor neuron death
- ineffective coping r/t the diagnosis of a progressive disease that results in motor paralysis
