- Amyotrophic lateral sclerosis (ALS) is a disease of unknown cause in which there is a loss of motor neurons (nerve cells controlling muscles) in the anterior horns of the spinal cord and the motor nuclei of the lower brain stem.
- It is often referred to as Lou Gehrig’s disease.
- Free radical damage
- Oxidative stress
- Cigarette smoking
- As motor neuron cells die, the muscle fibers that they supply undergo atrophic changes. Neuronal degeneration may occur in both the upper and lower motor neuron systems.
- The leading theory held by researchers is that over excitation of nerve cells by the neurotransmitter glutamate leads to cell injury and neuronal degeneration.
Assessment/Clinical Manifestations/Signs and Symptoms
The signs and symptoms presented depend on the location of the affected neuron. Generally, the following presentations are evident:
- Progressive muscle weakness
- Progressive weakness
- Muscle atrophy (arms, trunk, legs)
- Brisk or overreactive muscle reflexes
- Muscle weakness
- Difficulty talking
- Difficulty swallowing
- Difficulty breathing
- Soft palate and upper esophageal weakness
- Weakness on the posterior tongue
- Progressive difficulty in speaking
- Difficulty in swallowing
- Articulation and speech effects
- Compromised respiratory function
The following tests and assessment tools are used to verify the presence of Amyotrophic Lateral Sclerosis:
- EMG studies of affected muscles indicate reduction in the number of functioning motor units
- MRI may show high signal intensity in the corticospinal tracts
No specific therapy exists for ALS. The main focus of medical and nursing management is on interventions to maintain or improve function, well-being and quality of life.
- Symptomatic treatment and rehabilitative measures are employed to support the patient and improve the quality of life.
- Baclofen (Lioresal), dantrolene sodium (Dantrium), or diazepam (Valium) may be useful for patients troubled by spasticity, which causes pain and interferes with self-care.
- A patient experiencing problems with aspiration and swallowing may require enteral feeding.
- Mechanical ventilation (using negative-pressure ventilators) is an option if alveolar hypoventilation develops.
- Patients are encouraged to complete an advance directive or “living will” to preserve their autonomy in decision making.
- Impaired mobility related to muscle wasting, weakness, and spasticity
- Impaired communication related to impairment of the muscles of speech
- High risk for aspiration related to impaired muscles of swallowing
- Ineffective breathing pattern related to impaired muscles of breathing
- Provide intellectual stimulating activities, because the client typically experiences no cognitive deficits and retains mental abilities.
- Provide client and family teaching.
- Promote measures to enhance body image.
- Promote client and family coping as the client and his family deal with the poor prognosis and the grieving process
- Provide referrals.
- Maximize functional abilities
- Prevent complications of immobility
- Promote self-care
- Maximize effective communication
- Ensure adequate nutrition
- Prevent respiratory complications
- Promote measures to maintain adequate airway
- Promote measures to enhance gas exchange, such as oxygen therapy and ventilator assistance.
- Promote measures to prevent respiratory infection
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Nursing Care Plan
- Terminal disease process
- Threat to self-concept
- Threat to or change in health status, socioeconomic status, independence
- Increased vigilance
- Increased tension
- Scared, wide-eyed appearance
- Poor eye contact
- Increased perspiration
- Feelings of inadequacy or helplessness
- Expressed concern about changes in life events
- Patient verbalizes reduction in or control of anxiety.
- NOC Outcome: Anxiety Self-Control
- Patient demonstrates use of at least one positive coping strategy.
- NIC Intervention: Anxiety Reduction
- Assess the level of anxiety (mild, severe). Note signs and symptoms, including nonverbal communication.
- Rationale: Patients remain alert and are aware that this is a progressive disease with no cure. They are understandably anxious about what the future holds for them. Cognitive ability, orientation, and thought processes are unaffected by the disease process, even though the patient may be unable to communicate verbally.
- Assess prior coping patterns (by interview with the patient or significant others).
- Rationale: Prior methods may be inadequate to handle this life-threatening disease.
- Evaluate supportive resources available to the patient.
- Rationale: Individualized referrals will be based on the patient’s need for additional resources to support effective coping with anxiety.
- Display a confident, calm manner and a tolerant, understanding attitude.
- Rationale: The patient’s feeling of stability increases in a calm, nonthreatening environment.
- Establish rapport, especially through continuity of home care nurses.
- Rationale: Continuity promotes development of a therapeutic relationship.
- Encourage ventilation of feelings and concerns about dependency. (1) Listen carefully; sit down if possible. (2) Give an unhurried, attentive appearance; be aware of defense mechanisms used (denial, regression).
- Rationale: Discussion will assist in determining whether fears are based in reality. Attentive, active listening will encourage the patient to share feelings and concerns.
- Suggest the use of supportive measures (e.g., medications, clergy, social services, support groups).
- Rationale: Depressive symptoms need referral to mental health professionals. The patient needs to have access to an array of services. Anxiety and emotional liability may impair social interactions.
- Provide accurate information about the disease, medications, tests or procedures, and self-care.
- Rationale: Patients need to make informed decisions about their care and future (i.e., whether to be placed on a ventilator).
- Allow expressions of frustrations about loss and eventual outcome.
- Rationale: Fear or depression is normal and expected in this setting.
- Understand that the patient may have inappropriate behaviors (e.g., outbursts of laughing or crying).
- Rationale: This is known as pseudobulbar affect. These behaviors are not consistent with the patient’s mood.
- Try to direct the patient to positive aspects of living to the maximum for the present. Encourage the use of home services.
- Rationale: These help maintain the quality of life for as long as possible.
- Reinforce the things the patient can do versus what he or she cannot.
- Rationale: Emphasis on positive aspects of the patient’s life can promote effective coping.
- Ineffective Airway Clearance
- Progressive bulbar palsy
- Respiratory muscle weakness
- Patient reports breathing difficulty
- Abnormal lung sounds: rales (crackles), rhonchi, wheezes
- Periods of apnea
- Patient maintains effective airway clearance as evidenced by clear lung sounds, productive coughing, and normal respiratory rate.
- NOC Outcome :Respiratory Status: Airway Patency
- NIC Intervention: Airway Management
- Assess lung sounds and respiratory movement as indicated.
- Rationale: Respiratory failure is common for most people with ALS. Pulmonary changes tend to reflect restrictive rather than obstructive problems.
- Observe for signs of respiratory distress (e.g., increased respiratory rate, restlessness, rales, rhonchi, decreased breath sounds).
- Rationale: If increased distress is noted, the patient may need hospitalization and artificial ventilation.
- During a home visit, check pulse oximetry as indicated.
- Rationale: If desaturation occurs, the patient will need supplemental oxygen, possible suctioning, and/or repositioning.
- Evaluate cough reflex.
- Rationale: This allows an estimate of the patient’s ability to protect the airway. Aspiration is a common problem.
- Observe for signs or symptoms of infection (change in sputum color, amount, character; increased white blood cell count).
- Rationale: Signs of infection indicate increased expiratory resistance. These changes may indicate acute respiratory failure.
- Refer for pulmonary function tests.
- Rationale: In patients with ALS, pulmonary function tests are a more reliable measure of changes in respiratory function than are arterial blood gases changes.
- Arrange for home oxygen therapy if needed.
- Rationale: Supplemental oxygen may reduce the work of breathing.
- Implement transcutaneous nerve stimulation of the diaphragm as indicated.
- Rationale: Noninvasive techniques can be used successfully to support respiratory function. These approaches may delay the need for artificial airway support and mechanical ventilation.
- Anticipate hospitalization and/or home mechanical ventilation if signs of distress are noted.
- Rationale: Mechanical ventilation is a choice made by many ALS patients to support respiratory effort.
Instruct caregivers to use the following measures:
- Elevate head of bed and change the patient’s position every 2 hours and as needed.
- Rationale: Position changes promote postural drainage.
- Encourage deep breathing exercises and use of incentive spirometry.
- Rationale: These procedures prevent atelectasis.
- Encourage fluid intake to 2000 mL daily within the level of cardiac reserve. Encourage warm liquids.
- Rationale: Hydration keeps secretions thin; warm liquids loosen secretions.
- Assist the patient to suction himself or herself if possible. Teach caregivers how to suction the patient.
- Rationale: Frequent suctioning helps control excessive drooling and dysphagia. In the absence of a respiratory infection, most ALS patients need assistance in removing oral-pharyngeal secretions using a mushroom-tipped or Yankauer suction catheter.
- Impaired Physical Mobility
- Increasing motor weakness caused by paralysis
- Spasticity of extremities
- Limited range of motion (ROM)
- Neuromuscular impairment
- Imposed restrictions of movement
- Intolerance to activity, decreased strength and endurance
- Inability to move purposefully within the physical environment (including bed mobility, transfer, and ambulation)
- Impaired coordination, limited ROM, decreased muscle strength control, and/or muscle mass
- Patient maintains optimal physical mobility within limits of disease.
- NOC Outcomes: Mobility; Ambulation; Immobility Consequences: Physiological
- NIC Intervention: Exercise Therapy: Muscle Control
- Assess ROM, muscle strength, previous activity level, gait, coordination, and movement.
- Rationale: These assessments provide a baseline measurement of the patient’s mobility level.
- Assess the patient’s current level of independence: self-care ability, ability to transfer from bed or chair to bathroom or the need for help.
- Rationale: The patient may require assistance with only some activities.
- Assess the patient’s endurance in performing activities of daily living (ADLs) and in home maintenance.
- Rationale: Progressive muscle weakness and fatigue are major problems in ALS. As the disease progresses, the patient may need an assistive caregiver.
- Evaluate requirements for assistive devices.
- Rationale: Splints and braces may be indicated to support weakened muscles. Modified equipment for ambulating, eating, and performing other ADLs can promote independence with self-care and mobility as the disease progresses.
- Demonstrate how to position the patient for optimum comfort, facilitation of ventilation, and prevention of skin breakdown. Instruct the caregiver to reposition the patient regularly.
- Rationale: Nonpharmacological methods can promote quality of life.
- Instruct the patient and caregiver to do the following: Maintain exercise program: active or passive ROM.
- Rationale: Exercises prevent venous stasis, maintain joint mobility and good body alignment, and prevent footdrop and contractures.
- Instruct the patient and caregiver to do the following: Alternate periods of activity with adequate rest periods.
- Rationale: Energy must be conserved to prevent excessive fatigue.
- Coordinate physical therapy and occupational therapy as needed.
- Rationale: Coordinated home care services can promote quality of life and help the patient remain in the home setting as long as desired.
- Encourage the patient and significant others’ involvement in care; help them learn ways to manage problems of immobility (ROM, positioning, braces, splints).
- Rationale: These measures can promote functioning and delay a more costly and complicated pharmacological approach to symptom management.
- Instruct in provision of safety measures as indicated by the individual situation.
- Rationale: Modification of the home environment can promote mobility and prevent fall-related injuries.
- Encourage participation in activities and in occupational or recreational therapy.
- Rationale: Attention to social or “fun” activities can enhance both physical and emotional states.
- Instruct in provision of skin care: wash and dry skin well; use gentle massage and lotion.
- Rationale: Massage stimulates circulation.
- Administer muscle relaxants.
- Rationale: These medications decrease spasticity, which interferes with mobility.
- Imbalanced Nutrition: Less Than Body Requirements
- Progressive bulbar palsy
- Tongue atrophy or weakness
- Decreased salivation
- Choking during meals
- Loss of appetite
- Loss of weight
- Patient maintains weight or does not continue to lose weight.
- NOC Outcome: Nutritional Status: Nutrient Intake
- NIC Interventions: Nutrition Management; Swallowing Therapy
- Assess swallowing and presence or absence of gag reflex.
- Rationale: ALS progressively affects bulbar muscles, leading to swallowing difficulties.
- Assess nutritional status.
- Rationale: Total protein and serum albumin levels provide an index of the patient’s nutritional state.
- Inquire about food and fluid preferences.
- Rationale: This encourages intake of nutrients.
- Assess weight loss; inquire about weight gain or loss over the recent weeks or months.
- Rationale: Patients may estimate their weight and be unaware of their actual weight or weight loss. It is important to determine an accurate baseline.
- Assess tissue turgor, mucous membranes, muscle weakness, and tremors.
- Rationale: Progressive muscle weakness interferes with safe oral feeding. Decreased intake of nutrients can contribute to weakness and fatigue.
- Encourage family meals if possible.
- Rationale: This approach helps the patient and family maintain a sense of normalcy.
- Encourage intake of food that the patient can swallow; provide frequent small meals and supplements. Avoid sticky foods and milk products.
- Rationale: Sticky foods and milk products increase mucus thickness.
- Instruct the patient not to talk while eating.
- Rationale: Swallowing requires concentration to reduce the risk for aspiration. The muscles of the face fatigue more quickly when used for talking and chewing at the same time.
- Encourage the patient to chew thoroughly and eat slowly.
- Rationale: A large food bolus is more difficult to swallow. Rushing through a meal increases the patient’s risk for aspiration.
- Instruct the patient to use high Fowler’s position during and after meals.
- Rationale: Upright positioning facilitates the gravitational flow of food or fluid through the alimentary tract. If head of bed (HOB) cannot be elevated because of the patient’s condition, use a right side-lying position after feeding to facilitate passage of stomach contents into the duodenum.
- Discuss the need for sufficient fluids with meals.
- Rationale: Decreased salivation makes swallowing of certain foods difficult.
- Discuss the need to keep the dining environment well ventilated, uncluttered, cheerful, and free of distraction.
- Rationale: As swallowing problems progress, the patient with ALS may experience loss of enjoyment at meals. A pleasant, unhurried environment may help promote normalcy at mealtime.
- Coordinate speech therapy consultation as appropriate to evaluate swallowing.
- Rationale: Special techniques can be taught to facilitate muscle control.
- Anticipate the need for a nasogastric or gastrostomy tube. Discuss with the patient and caregiver before the need is evident.
- Rationale: Supplemental measures may be required to maintain adequate nutritional state and weight.
- Impaired Verbal Communication
- Tongue weakness
- Nasal tone to speech
- Difficulty in articulating words
- Inability to express self clearly
- Patient uses language or an alternative form of communication, as evidenced by effective ability to communicate needs.
- NOC Outcome: Communication: Expressive Ability
- NIC Intervention: Communication Enhancement: Speech Deficit
- Determine the degree of speech difficulty by assessing the patient’s ability to speak spontaneously and endurance of ability to speak.
- Rationale: The patient with ALS experiences progressive weakness and lack of coordination of the muscles that control speech.
- Assess the patient’s ability to use alternative methods of communication (i.e., spelling board, finger writing, eye blinks, signal system, word cards).
- Rationale: Communication support methods need to be consistent with the patient’s muscle strength. As the disease progresses, the patient may require more “high-tech” communication support.
- Inform the patient and family about dysarthria and its effects on speech and language ability.
- Rationale: Alternatives can be developed in anticipation of need.
- Use closed-ended questions requiring only a “yes” or “no” response.
- Rationale: This minimizes effort, conserves energy, and decreases anxiety.
- Allow the patient time to respond and to organize responses. Avoid interrupting.
- Rationale: It is difficult to respond under pressure. As the disease progresses, muscle weakness may slow speech production.
- Anticipate needs.
- Rationale: These decrease feelings of helplessness.
- Encourage use of writing pad or a spelling board, as indicated.
- Rationale: These increase self-esteem and ability to communicate.
- Praise accomplishments.
- Rationale: Positive feedback provides encouragement to continue communication efforts.
- Consult a speech therapist for additional help.
- Rationale: The speech therapist can help the patient select appropriate devices for communication. The patient can use a variety of “low-tech” and “high-tech” devices to facilitate communication. Low-tech devices include alphabet boards, magic slate boards, and buzzer alarms. Hand-held and laptop computers are examples of high-tech communication tools. Some of these devices will vocalize for the patient and can be activated by special switches that require minimal muscle strength.
- Deficient Knowledge
- Unfamiliarity with disease process and management
- Lack of questions
- Multiple questions
- Patient and family demonstrate knowledge of ALS, progressive course of disease, nutritional and respiratory needs, and available community resources.
- NOC Outcome: Knowledge: Disease Process
- NIC Intervention: Teaching: Disease Process
- Assess knowledge of the disease process, diagnostic tests, and treatment outcome.
- Rationale: Many patients have been exposed to media information that causes frustration, fear, and anxiety. Misconceptions may exist.
- Evaluate knowledge or awareness of community support groups.
- Rationale: Awareness of other resources may improve coping mechanisms.
Provide information about the following:
- Disease process: progressive degenerative motor disease of unknown cause that interferes with motor activities (may include lower cranial nerves: swallowing, speech, and respiration)
- Rationale: Patients and families need knowledge about the progression of the disease. This information can help them make decisions in the early stages of the illness about feeding tubes, long-term care, ventilatory support, power of attorney, and living wills. These decisions should be discussed while the patient can verbally participate in the process.
- Diagnostic testing: electromyography, muscle biopsy, pulmonary function
- Rationale: These tests are done to rule out other muscle diseases. At this time there is no definitive test for ALS.
- Home care issues: nutrition, communication aids, respite and caregiver support.
- Rationale: Patients have major self-care problems. Some patients use ventilators at home for respiratory support.
Provide information on ALS support group:
- ALS Association: 21021 Ventura Blvd., Suite 321 Woodland Hills, CA 91364 (800)-782-4747
- Rationale: This organization provides patients and families with information, resources, and equipment loans to support quality of life.