Amyotrophic Lateral Sclerosis: ALS (Lou Gerig's disease)
what is ALS?
-involves progressive degeneration of the motor neurons of the spinal cord, brainstem, and motor cortex -messages form the brain cannot reach the muscles
what are the risk factors for ALS?
-no known cure -rare -40-70 years old males related to autoimmune disorder, free radical damage, and oxidative stress
what is the medical management of ALS?
1. Rituzole (Ritutek) - VERY EXPENSIVE decreases the amount of glutamate in the brain has a neuroprotective effect w early ALS extends surviuval time -monitor for liver toxicity symptomsatic treatment 2.to decrease spasticity: -baclofen (Lioresal) -dantrolene sodium (Dantrium) -diazepam (Valium)
what is important to remember about people with ALS?
CLIENT REMAINS ALERT AND MENTALLY INTACT THROUGHOUT THE COURSE OF THE DISEASE
What does ALS result in?
death
what are the nursing diagnoses for ALS?
disturbed body image disabled family coping anxiety powerlessness risk for aspirations ineffective breathing patterns imbalanced nutrition, less than body impaired physical mobility impaired verbal communication self care deficits
what are the management/interventions for ALS?
goal: maointain oor improve function, well-being, and quality of life PT prevent complications or respiratory involvment and immobility promote comfort
what is the diagnosis for ALS?
history physical exam -motor strength, speech, swallowing, faciculations (spontaneous movements), atrophy electromyelogram -normal sensory conduction; abnormal motor conduction muscle biopsy (VERY PAINFUL) -atrophic fibers Abnormal pulmonary function tests
what are the EARLY physical assessment findings for ALS?
mild clumsiness fatigue while walking tongue atrophy weakness of hands/arms fasciculations of the face dysphagia dysarthria (difficulty saying words) nasal quality of speech
what are the progression of symptoms for ALS?
muscle atrophy, particularly the trapezius, sternocleidomastoid muscles bulbar paralysis (dysarthria, dysphagia) flaccid quadriplegia reapiratory compromise*** (this is what kills the patient)