ALS

Question 1

ALS

Answer 1

Deteriorating disease of the motor system characterized by muscle weakness progressing to muscle atrophy and eventually paralysis and death.
Does not involve autonomic changes, sensory alterations or cognitive changes.
Affects motor neurons in the brainstem and spinal cord. Dead neurons can’t activate muscles

Question 2

possible causes of ALS

Answer 2

Exposure to heavy metals, viral infection.
Is associated with HIV infection
10% of ALS patients inherit it on an autosomal dominant gene that can result in the disease,
Autoimmune destruction and neurotransmitter depletion

Question 3

statistics of ALS

Answer 3

Rare
Onset usually 40-70 yrs. Peak 55.
Twice as common in men as women
Progresses rapidly. No cure. 50% patients die within 2-5 years of diagnosis due to respiratory failure..

Question 4

clinical symptoms of ALS

Answer 4

Weakness in extremities. Twitching in arms, trunk or legs. Uncoordinated movements.
Loss of fine motor control of hands. Spasticity is usually present and stretch reflexes are brisk and overactive.
Eventually, paresis, paralysis, loss moa ADL ability, total immobility.
Difficulty speaking, chewing, swallowing
Voice has nasal sound. Eventually can’t speak.
Regurgitation of liquids through nose.
Dyspnea, difficulty clearing airway with complications of pneumonia and respiratory failure.
Emotional effects: Patient is cognitively intact while wasting away.

Question 5

diagnostic evaluation of ALS

Answer 5

First r/o other disorders
EMG to differentiate between neuropathy or myopathy
CK increased
Muscle biopsy shows atrophy
Serial muscle testing
Pulmonary function tests

Question 6

medical management of ALS

Answer 6

Basically supportive
a. Quinine for muscle cramps
b. Cervical esophogotomy to bypass larynx
May use drugs like Baclofen to decrease spasticity.
Riluzole ( Rilutek )
a. Only drug approved for treatment ALS
b. Has neuroprotective effect. Extends life
for several months.
Referral to VNS services

Question 7

Nursing Care for ALS

Answer 7

Improve respiratory function and maintain patent airway:
a. Assess respiratory function and
swallowing ability. Position change
1-2hrs. Suctioning, CPT, spirometry,
oxygen as needed, elevate HOB, breathing
exercises.
b. Treat respiratory infections promptly
c. Portable ventilatory support.

Question 8

higher incidence of ALS in

Answer 8

HIV positive patients

Question 9

nursing care for ALS

Answer 9

Maximize independence:
a. AROJM to PROJM q 2hrs. Contractures
can develop within a week.
b. Utilize energy conservation measures.
c. Stretching exercises, splints, assistive
devices.
d. Wheelchair when too weak

Question 10

nursing care for ALS

Answer 10

Nutritional support:
a. Maintain nitrogen balance and
hydration. Monitor albumin, hgb & hct,
total protein levels.
b. Calorie count, I&O, daily weight, and
Dietary consult.
c. Standby suction. Keep upright for 15-20
minutes after eating. Keep head slightly
flexed. Don’t wash down foods with liquids.
d. Will eventually need enteral feedings.

Question 11

nursing care for ALS

Answer 11

Communication:
a. Work out plan for this before patient
can’t talk.
b. Eye movements and muscles remain
intact so signals can be prearranged.
Skin integrity:
a. Assess, lubricate, keep clean & dry.
b. Special beds, turn & position q 1-2 hrs.
c. Treat skin breakdown immediately.
d. Good catheter care. Monitor for infection.

Question 12

nursing care for ALS

Answer 12

Individual / Family coping:
a. Requires realistic planning.
b. Consider hospice referral, VNS,
Physical & occupational & speech therapy,
and dietary consult.
c. Teaching of suctioning and home car e
if family wants patient at home.
d. Address patient’s interest in an Advance
Directive/ Living Will.
e. Refer to a support group, psychologist, social
worker.