Amyotrophic Lateral Sclerosis (ALS) Nursing Care Plan and Management

Definition
  • Amyotrophic lateral sclerosis (ALS) is a disease of unknown cause in which there is a loss of motor neurons (nerve cells controlling muscles) in the anterior horns of the spinal cord and the motor nuclei of the lower brain stem.
  • It is often referred to as Lou Gehrig’s disease.
Risk Factors
  • Autoimmune
  • Free radical damage
  • Oxidative stress
  • Cigarette smoking
Pathophysiology
  • As motor neuron cells die, the muscle fibers that they supply undergo atrophic changes. Neuronal degeneration may occur in both the upper and lower motor neuron systems.
  • The leading theory held by researchers is that over excitation of nerve cells by the neurotransmitter glutamate leads to cell injury and neuronal degeneration.
Amyotrophic-Lateral-Sclerosis
Assessment/Clinical Manifestations/Signs and Symptoms
The signs and symptoms presented depend on the location of the affected neuron. Generally, the following presentations are evident:
  • Fatigue
  • Progressive muscle weakness
  • Cramps
  • Twitching
  • Incoordination
Anterior horns
  • Progressive weakness
  • Muscle atrophy (arms, trunk, legs)
  • Spasticity
  • Brisk or overreactive muscle reflexes
Cranial nerves
  • Muscle weakness
  • Difficulty talking
  • Difficulty swallowing
  • Difficulty breathing
  • Soft palate and upper esophageal weakness
  • Weakness on the posterior tongue
Bulbar muscles
  • Progressive difficulty in speaking
  • Difficulty in swallowing
  • Articulation and speech effects
  • Compromised respiratory function
Diagnostic Tests
The following tests and assessment tools are used to verify the presence of Amyotrophic Lateral Sclerosis:
  • EMG studies of affected muscles indicate reduction in the number of functioning motor units
  • MRI may show high signal intensity in the corticospinal tracts
Medical Management
No specific therapy exists for ALS. The main focus of medical and nursing management is on interventions to maintain or improve function, well-being and quality of life.
  • Symptomatic treatment and rehabilitative measures are employed to support the patient and improve the quality of life.
  • Baclofen (Lioresal), dantrolene sodium (Dantrium), or diazepam (Valium) may be useful for patients troubled by spasticity, which causes pain and interferes with self-care.
  • A patient experiencing problems with aspiration and swallowing may require enteral feeding.
  • Mechanical ventilation (using negative-pressure ventilators) is an option if alveolar hypoventilation develops.
  • Patients are encouraged to complete an advance directive or “living will” to preserve their autonomy in decision making.
Nursing Diagnosis
  • Impaired mobility related to muscle wasting, weakness, and spasticity
  • Impaired communication related to impairment of the muscles of speech
  • High risk for aspiration related to impaired muscles of swallowing
  • Ineffective breathing pattern related to impaired muscles of breathing
Nursing Management
  1. Provide intellectual stimulating activities, because the client typically experiences no cognitive deficits and retains mental abilities.
  2. Provide client and family teaching.
  3. Promote measures to enhance body image.
  4. Promote client and family coping as the client and his family deal with the poor prognosis and the grieving process
  5. Provide referrals.
  6. Maximize functional abilities
    • Prevent complications of immobility
    • Promote self-care
    • Maximize effective communication
  7. Ensure adequate nutrition
  8. Prevent respiratory complications
    • Promote measures to maintain adequate airway
    • Promote measures to enhance gas exchange, such as oxygen therapy and ventilator assistance.
    • Promote measures to prevent respiratory infection

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