I. Muscle Weakness/Skin Status
- Encourage continuation of daily routines and activities.
- Maintain exercise program to tone and strengthen unaffected muscle groups without fatiguing affected muscles.
- Range-of-Motion (ROM) exercises to prevent contracture and pain in joints; first Active ROM, then passive. Refer to physical therapist for exercise program and see recommended passive range of motion description in Managing ALS (MALS) Manual II.
- When weakness in the extremities begins to compromise mobility, safety, or independence in ADL, refer to a physical or occupational therapist.
- Use as many different positions as possible when in bed. Change positions every two hours, or on skin tolerance. After each change of position, check for redness over bony
- prominences, and provide an eggshell or circulating mattress when immobility prevents independent repositioning.
- Repositioning in the wheelchair based on the patient’s skin tolerance. Use of a wheelchair cushion to prevent skin breakdown.
- Proper positioning when ambulating or in a wheelchair, i.e., use of a sling for a weak upper extremity.
- Promote adequate nutritional intake.
II. Urinary Function
- Progressive loss of mobility
III. Altered Bowel Function
I. Nutritional Needs
1. Anthropometric Measures:
b) Pre-morbid “usual” weight
c) Current Weight
d) Weight gain/loss pattern since onset of disease
e) “Ideal body weight”
2. Laboratory Test – indicated if patient has lost ten percent or more of body weight in the last two months or weighs fifteen percent or more below “ideal body weight”.
3. Hydration status – determined by careful recording of fluid intake and output and by test of urine specific gravity. The average person needs 35cc fluid/kg body weight for adequate renal function. This figure can be used to calculate fluid requirements.
B. Dysphagia (difficulty in swallowing)
a) Assess gag, cough and swallowing reflexes, and chewing.
b) Assess patient’s ability to swallow liquids and solids.
c) Assess weight serially.
2. Common Nursing Diagnoses
a) Potential for injury.
b) Impaired nutritional status.
c) Knowledge deficit regarding alternatives for diet, food preparation, and alternative procedures for supplemental feeding.
a) Discuss techniques for protecting airway, i.e., sitting up straight, putting chin on chest when swallowing, concentrating while eating. Avoid increased stimulation, i.e., talking at mealtime.
b) Refer to dietician for determination of adequate nutritional intake with continued weight loss.
C. Nutritional Management of the Dysphagic Patient
1. Promotion of optimum weight
a) Smaller, more frequent meals.
b) Foods that are “easy-to-eat”, i.e., adjusted mechanical soft diet.
c) Increased daily activity with non-fatiguing exercise.
d) Liquid supplement between meals.
1. History: subjective symptoms (starving for air, shortness of breath and relation to position changes, fatigue).
2. Assess: changes from baseline respiratory rate, depth, pattern, chest expansion; adequacy of respiratory exchange and effort; retraction of intercostals spaces; abdominal breathing; diaphragmatic breathing; nasal flaring and use of cervical accessory muscles of respiration; color (cyanosis); cough, gag, and swallow reflexes.
3. Auscultate: decreased breath sounds, presence of extra or adventitious sounds (i.e., sales, wheezes).
B. Common Nursing Diagnoses
1. Ineffective airway clearances related to:
a) Impaired/absent gag reflex.
b) Impaired/absent swallowing reflex.
c) Impaired/absent cough/sneeze reflex.
2. Impaired gas exchange related to:
a) Aspiration secondary to impaired/absent gag, swallowing and cough/sneeze reflexes.
b) Atelectasis secondary to impaired cough/sneeze reflexes and/or hypoventilation.
c) Ineffective breathing pattern.
3. Knowledge deficit regarding:
a) Airway clearance and gas exchange.
b) Considerations regarding mechanical life supports.
1. Maintain airway patency
a) Use of aspirator to suction secretions and prevent choking.
b) Use of medications (i.e., Elvail – 25 mg t.i.d. or 25-75 mg. at hs to reduce secretions).
c) If the patient has a tracheostomy, suctioning and “trach care” should be done prn to prevent buildup of secretions, possible obstruction and infection.
2. Maintain adequate ventilation
a) Deep breathing and coughing exercises and use of incentive spirometer to encourage lung expansion.
b) Chest physiotherapy: percussion, assisted cough and postural drainage when indicated.
c) Oxygen, when ordered.
d) Elevation of the head of the bed with a foam wedge or hospital bed to ease shortness of breath and provide comfort during sleep.
e) When air hunger is chronic and distressing, help monitor and titrate medications that will help provide comfort.
3. Provide information and opportunities to discuss options for mechanical ventilation. Generally, if there are early signs of dysphagia, dyspnea, or after the patient has had time to adjust to his diagnosis, the patient and family should be assisted in considering options regarding life support systems. The important thing to remember is the patient should be thinking about these decisions before he/she needs them.
4. Provide information and opportunity to discuss Physician’s Directive (also called Living Will or Durable Power of Attorney for Medical Care). The pros and cons of life support should be discussed in detail prior to the patient filling out a Physician’s Directive.
1. Assess volume and clarity of speech. 2. Assess ability to communicate needs to family/significant others.
B. Common Nursing Diagnoses
1. Impaired verbal communications related to:
a) Altered volume of speech.
b) Altered clarity of speech (dysarthia).
c) Loss of speech.
1. With dysarthia or with total loss of speech and no use of upper extremities.
2. There are a variety of alternative modes of communication.
The ALS Association, 27001 Agoura Road, Suite 250, Calabasas Hills, CA 91301-5104,
Phone: (800) 782-4747 / email@example.com / www.alsa.org
3. Refer to a speech pathologist for evaluation and intervention.
1. Evaluate the patient and family’s support systems and coping patterns with awareness that with ongoing loss of independence, there will be ongoing grieving by patient and family.
B. Common Nursing Diagnoses
1. Ineffective coping strategies
2. Knowledge deficit regarding alternatives for mobility and comfort.
3. Impaired support system.
1. Provide an accepting environment in which the patient and family can share concerns and fears with each other and members of the health care team.
2. Help the patient and family anticipate care needs and implications.
3. Anticipate and support emotional responses to loss as a normal reaction, thus enabling the family to take in information and cope more effectively with practical decision-making.
4. Provide encouragement and anticipate unasked questions.
5. Encourage realistic expectations of the disease whenever possible.
6. Refer, if needed, to a counselor, psychiatrist, psychologist or psychiatric clinical nurse specialist.
7. Refer to an ALS support group if available.
--Excepted from “Amyotrophic Lateral Sclerosis: A Teaching Manual for Health Professionals”, Nancy Konikow, R.N., M.N., ALS Health Support Services, Kirkland, Washington.
Nursing Care Plan of Amyotrophic Lateral Sclerosis – Motor Neuron Disease
Weakness to flaccid paralysis
Emotional response to diagnosis and prognosis (anxiety, fear, denial, anger).
Keep patient as active as possible
Prevent skin breakdown
Prevent vein thrombosis, pulmonary embolism
To assist patient through grief and grieving process
To assist family to deal with patient and grief process
Altered body image and self-esteem
(When bulbar symptoms occur)
Difficulty swallowing and chewing
To assist patient in accepting new body image and maintaining self-esteem
Maintain adequate 02 delivery to patient; prevent complication of pneumonia assisted cough
Make food easier for patient to eat
Ensure adequate nutrition and fluid balance
Urinary frequency or incontinence of urine and feces of impaction
Establish effective means of communication
To maintain normal bowel and bladder function (usually do remain normal until later stages of disease)
Signs/symptoms of illness, to notify M.D.:
Signs of infection – cold, flu, elevated temperature, chills
Sudden increase in original signs and symptoms of disease
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