UNIT 2 ALS

Question 1

What is the epidemiology of ALS?

Answer 1

AKA Lou Gehrig’s disease
1. Unkown cause
2. Symptoms usually develop after 50.
3. Prevalence 3.9 per 100,000 people
4. More men than women affected

Question 2

What is the ALS pathophysiology?

Answer 2

1. Rapidly progressing, fatal CNS disease
2. Affects voluntary muscle control
3. DOES NOT IMPAIR SENSES OR ABILITY TO THINK
4. Both upper and lower neurons degenerate and die
5. Affects diaphragm and chest wall
   -Respiratory failure–> ventilator support

Question 3

What are the clinical manifestations of ALS?

Answer 3

1. Muscle cramps or stiffness, muscle weakness, slurred speech, difficulty swallowing
2. Upper motor neuron damage
   -Associated w/spasticity
3. Lower motor neurons damage
   -Associated w/flaccidity
4. Fatigue while talking
5. Tongue atrophy
6. Dysphagia
7. Dysarthria
8. Nasal quality of speech
9. Fasciculation of the face
10. Weakness of arm and hands
11. Spasticity
12. Muscle atrophy
13. Eventually muscle resp. involvement

Question 4

How is ALS diagnosed?

Answer 4

NO single test can be used to diagnose
1. Based on s/s
2. EMG and nerve conditions studies
3. CT scan and/or MRI of neck and head
4. Lumbar puncture
5. Genetic testing
6. Swallow studies
7. Rule out all other disorders

Question 5

What is the 1st drug approved to slow the progression of ALS symptoms?

Answer 5

1. Riluzole (Rilutek)

Question 6

What is the MOA for Riluzole?

Answer 6

Reduces damage to motor neurons by decrease release of glutamate

Question 7

Riluzole can delay the need for a tracheostomy by how long?

Answer 7

3-6 months

Question 8

What are the adverse effects of Riluzole?

Answer 8

1. Asthenia
2. GI reactions
3. Dizziness/Vertigo
4. Somnolence
5. Decreased lung function
6. neutropenia
7. Liver injury

Question 9

What are ALS complications?

Answer 9

1. Aspiration
2. Resp Failure
3. Pneumonia
4. Pressure ulcers
5. DVT
6. Pulmonary embolism (PE)
7. Constipation, contractures
8. Depression, weight loss
9. Loss of ability for self care

Question 10

Ineffective airway clearance is a nursing dx for ALS why?

Answer 10

R/T weak cough secondary to motor neuron death and muscle weakness

Question 11

Ineffective breathing pattern is a nursing dx for ALS why?

Answer 11

R/T weakness of resp. muscle

Question 12

Potential for injury is a nursing dx for ALS why?

Answer 12

R/T impaired physical immobility

Question 13

Impaired oral communication is a nursing dx for ALS why?

Answer 13

R/T dysarthria and tongue atrophy

Question 14

Ineffective coping is a nursing dx for ALS why?

Answer 14

R/T diagnosis of progressive disease that results in motor paralysis