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Lesson 34. Amyotrophic Lateral Sclerosis

2-34. AMYOTROPHIC LATERAL SCLEROSIS

a.  Definition. Amyotrophic lateral sclerosis (ALS) is a progressive, incapacitating, and fatal disease of unknown cause. It is characterized by loss of motor neurons in the anterior horns of the spinal cord and lower brain stem. Amyotrophic lateral sclerosis is commonly known as Lou Gehrig's Disease.

b Signs and Symptoms.

(1) Symptoms vary, depending upon the location of affected motor neurons.
(2) Progressive weakness and atrophy of muscles of arms, trunk, or legs.
(3) Progressive difficulty in speaking and swallowing, speech may be nasal and unintelligible.
(4) Excessive drooling.
(5) Muscle twitching.
(6) Mental facilities are not affected.
(7) Death usually occurs 3-5 years after onset.

c. Medical and Nursing Management

(1) Objective--to support the patient and improve quality of life.
(2) Instruct the patient to perform active exercises and range of motion exercises to strengthen uninvolved muscles and prevent disuse atrophy.
(3) Utilize braces, splints, canes, etc., to keep patient mobile as long as possible.
(4) Assist the patient to prevent complications that may result from symptoms.
(a) Keep suction apparatus at bedside, as aspiration is a constant danger.      
(b) Instruct patient to drink and eat in an upright position with the neck flexed.        
(c) Use soft cervical collar if patient has difficulty holding head up.
(d) Give semi-soft foods. Avoid easily-aspirated pureed foods and mucous producing foods (milk).
(e) Keep in mind that patient may have frequent outbursts of laughing and crying.
(f)   Develop communication system when speech is lost.

(5) Give patient and family compassionate and caring support.

(a) Allow expressions of feelings and frustrations about losses and eventual outcome.
(b)   Remember that the patient is alert and retains vision, ocular movement, intelligence, and consciousness even though he/she is paralyzed.
(c)    Advise patient's family of helping services of ALS Society of America.

David L. Heiserman, Editor

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Revised: June 06, 2015