Amyotrophic Lateral Sclerosis (ALS)

Introduction:

ALS is commonly called Lou Gehrig’s disease and is a progressive, degenerative
disorder that involves both the upper and lower motor neurons. There is no change
in mental status or sensory function with the disease. The disease does result in
paralysis of the motor system, except the eyes. As the disease is more progressed,
families often can communicate with the patient through eye movements. Males
are affected more commonly than females. The disorder may present at any age,
but the age at onset is usually between 40 and late 60s. There is a familial form of
the disease that has been linked to an abnormality in chromosome 21.

PROGNOSIS
The disease is rapidly progressive and there is currently no known cure. As the
muscles weaken and atrophy, paralysis develops. Over time, the respiratory mus-
cles become involved. At first this results in poor air exchange, increasing the risk
for respiratory infections, such as pneumonia. Eventually, the respiratory compro-
mise leads to death from respiratory failure.

SIGNS AND SYMPTOMS

• Fatigue, especially with exertion
• Atrophy of muscles due to weakness
• Dysphagia (trouble swallowing) due to muscular weakness
• Weakness of muscles in the limbs
• Muscle twitching (fasciculation) due to changes within the muscles
• Slurred speech due to muscle weakness


TREATMENT
• Maintain adequate nutrition.
• Consult with speech pathologist for potential swallowing difficulties.
• Administer spasmolytic agent specific for amyotrophic lateral sclerosis, which
reduces the transmission of glutamine across the neural synapse. Use of this
drug appears to slow the progression of the disease:
• riluzole
• Administer medications to control symptoms.
• BIPAP (bi-level positive airway pressure) to assist respiration either at night-
time, intermittently as needed, or all day.
• Refer to hospice for end-of-life care.

NURSING DIAGNOSES
• Impaired physical mobility
• Ineffective airway clearance

NURSING INTERVENTION
• Develop a method of communication within the patient’s capabilities—ver-
bal communication may not be possible; patient may not be able to use call
bell system.
• Monitor vital signs—monitor respiratory function and cardiovascular status;
as muscular function decreases the respiratory muscles may be affected.
• Assess gag reflex—as muscular changes occur, normal protective gag reflex
will diminish.
• Explain to the patient:
• How to suction oral pharynx to remove secretions or food particles. As mus-
cle function decreases, the cough reflex will not be sufficient to remove these.
• How to tuck chin while drinking and eating to decrease chance of
aspiration.

• Monitor input and output.