Info

Medication or Drug Class

Dosage

Description

Rationale

Riluzole

50 mg PO q 12 hours

Central nervous system agent

Inhibits presynaptic release of glutamic acid in the central nervous system; prolongs tracheostomy-free survival by decreasing injury of neurons by glutamic acid

Baclofen

5 mg PO tid

Skeletal muscle relaxant

Manages muscle spasticity

Tizanidine

4-8 mg PO qid

Skeletal muscle relaxant

Manages muscle spasticity

Other Therapies: Quinine can also be used for relief of painful muscle cramps and spasms.

Independent

MANAGING THE DISEASE. Teach the patient breathing exercises, methods to change positions, chest physical therapy techniques, and incentive spirometry. Explore measures to reduce the risk of aspiration. Encourage rest periods prior to meals to decrease muscle fatigue. Have the patient sit in an upright position with her or his neck slightly flexed during meals, use a neck support such as a cervical collar, and serve foods with a soft consistency. Encourage the patient to remain in an upright position for at least 30 minutes after a meal. If the patient is having problems handling oral secretions, teach him or her how to use oral suction.

As the disease worsens, the patient may lose the ability to speak. Work with the patient and family to develop alternate methods of communication, such as eye blinks, a picture or word chart, or computers with artificial speech or synthesizers. When the patient's immobility increases, teach the family to provide skin care to all pressure points. The patient needs to be turned and positioned frequently. The use of a pressure-reducing mattress will also help maintain skin integrity.

ACTIVITIES OF DAILY LIVING. To achieve maximum mobility and independence, institute an exercise regimen with active or passive range-of-motion (ROM) exercises. Use supportive devices for mobility and transfer, and instruct the patient on the use of splints. Establish regular bowel and bladder routines. Work with the patient and significant others to develop a pattern for activities of daily living that allows the patient to participate but not to become overly fatigued. As mobility decreases, help the patient obtain equipment such as a walker, a wheelchair, or a lift. Ask the patient or family to describe the living environment (or perform a home assessment) to identify areas that may cause potential injury or to recommend modifications to the environment.

EMOTIONAL SUPPORT. Early in the disease process, expect the patient and family to be angry, deny the probable disease outcome (death), or show extreme anxiety. The patient and family will most likely experience periods of depression and may need a referral to a counselor or support group. Recognize that the disease is a catastropic event in the family's life and will change the family forever. Most communities have local chapters of the Amyotrophic Lateral Sclerosis Association.

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