NCP Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS)

Lou Gehrig’s Disease; Motor Neuron Disease; Progressive Bulbar Palsy; Progressive Muscular Atrophy

Amyotrophic lateral sclerosis (ALS), commonly called Lou Gehrig’s disease, is a rare progressive disease that attacks motor neurons, which control the movement of muscles through the anterior horns of the spinal cord and the motor nuclei of the lower brainstem. Onset is usually between 40 and 70 years of age (2:1 ratio of male to female occurrence). Signs and symptoms of the disease include atrophic weakness of the hands and forearms (early), mild lower extremity spasticity, and diffuse hyperreflexia. Sensation and sphincter control are usually maintained. When the bulbar muscles are affected, dysarthria and dysphagia are seen with fasciculations in the tongue. There is usually progressive paralysis, with death occurring within 2 to 6 years. This care plan focuses on home care, where the patient usually remains until care needs are no longer manageable.

Nursing Diagnosis

Anxiety

Common Related Factors	Defining Characteristics
Terminal disease process Threat to self-concept Threat to or change in health status, socioeconomic status, independence	Restlessness Increased vigilance Insomnia Fearfulness Increased tension Scared, wide-eyed appearance Poor eye contact Jitteriness Distress Increased perspiration Apprehension Uncertainty Feelings of inadequacy or helplessness Expressed concern about changes in life events Trembling
Common Expected Outcomes Patient verbalizes reduction in or control of anxiety. Patient demonstrates use of at least one positive coping strategy.	NOC Outcome Anxiety Self-Control NIC Intervention Anxiety Reduction

Ongoing Assessment

Actions/Interventions	Rationale
Assess the level of anxiety (mild, severe). Note signs and symptoms, including nonverbal communication.	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).	Prior methods may be inadequate to handle this life-threatening disease.
Evaluate supportive resources available to the patient.	Individualized referrals will be based on the patient's need for additional resources to support effective coping with anxiety.

Therapeutic Interventions

Actions/Interventions	Rationale
Display a confident, calm manner and a tolerant, understanding attitude.	The patient’s feeling of stability increases in a calm, nonthreatening environment.
Establish rapport, especially through continuity of home care nurses.	Continuity promotes development of a therapeutic relationship.
Encourage ventilation of feelings and concerns about dependency. · Listen carefully; sit down if possible. · Give an unhurried, attentive appearance; be aware of defense mechanisms used (denial, regression).	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).	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.	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.	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).	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.	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.	Emphasis on positive aspects of the patient’s life can promote effective coping.

Nursing Diagnosis

Ineffective Airway Clearance

Common Related Factors	Defining Characteristics
Dysarthria Aspiration Progressive bulbar palsy Respiratory muscle weakness	Patient reports breathing difficulty Abnormal lung sounds: rales (crackles), rhonchi, wheezes Periods of apnea
Common Expected Outcome 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

Ongoing Assessment

Actions/Interventions	Rationale
Assess lung sounds and respiratory movement as indicated.	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).	If increased distress is noted, the patient may need hospitalization and artificial ventilation.
During a home visit, check pulse oximetry as indicated.	If desaturation occurs, the patient will need supplemental oxygen, possible suctioning, and/or repositioning.
Evaluate cough reflex.	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).	Signs of infection indicate increased expiratory resistance. These changes may indicate acute respiratory failure.
Refer for pulmonary function tests.	In patients with ALS, pulmonary function tests are a more reliable measure of changes in respiratory function than are arterial blood gases changes.

Therapeutic Interventions

Actions/Interventions	Rationale
Instruct caregivers to use the following measures:
Elevate head of bed and change the patient’s position every 2 hours and as needed.	Position changes promote postural drainage.
Encourage deep breathing exercises and use of incentive spirometry.	These procedures prevent atelectasis.
Encourage fluid intake to 2000 mL daily within the level of cardiac reserve. Encourage warm liquids.	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.	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.
Arrange for home oxygen therapy if needed.	Supplemental oxygen may reduce the work of breathing.
Implement transcutaneous nerve stimulation of the diaphragm as indicated.	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.	Mechanical ventilation is a choice made by many ALS patients to support respiratory effort.

Nursing Diagnosis

Impaired Physical Mobility

Common Related Factors	Defining Characteristics
Increasing motor weakness caused by paralysis Spasticity of extremities Limited range of motion (ROM) Fatigue 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
Common Expected Outcome Patient maintains optimal physical mobility within limits of disease.	NOC Outcomes Mobility; Ambulation; Immobility Consequences: Physiological NIC Intervention Exercise Therapy: Muscle Control

Ongoing Assessment

Actions/Interventions	Rationale
Assess ROM, muscle strength, previous activity level, gait, coordination, and movement.	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.	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.	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.	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.

Therapeutic Interventions

Actions/Interventions	Rationale
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.	Nonpharmacological methods can promote quality of life.
Instruct the patient and caregiver to do the following:
· Maintain exercise program: active or passive ROM.	Exercises prevent venous stasis, maintain joint mobility and good body alignment, and prevent footdrop and contractures.
· Alternate periods of activity with adequate rest periods.	Energy must be conserved to prevent excessive fatigue.
Coordinate physical therapy and occupational therapy as needed.	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).	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.	Modification of the home environment can promote mobility and prevent fall-related injuries.
Encourage participation in activities and in occupational or recreational therapy.	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.	Massage stimulates circulation.
Administer muscle relaxants.	These medications decrease spasticity, which interferes with mobility.

Nursing Diagnosis

Imbalanced Nutrition: Less Than Body Requirements

Common Related Factors	Defining Characteristics
Progressive bulbar palsy Tongue atrophy or weakness Dysphagia Decreased salivation Choking during meals	Loss of appetite Loss of weight
Common Expected Outcome Patient maintains weight or does not continue to lose weight.	NOC Outcome Nutritional Status: Nutrient Intake NIC Interventions Nutrition Management; Swallowing Therapy

Ongoing Assessment

Actions/Interventions	Rationale
Assess swallowing and presence or absence of gag reflex.	ALS progressively affects bulbar muscles, leading to swallowing difficulties.
Assess nutritional status.	Total protein and serum albumin levels provide an index of the patient’s nutritional state.
Inquire about food and fluid preferences.	This encourages intake of nutrients.
Assess weight loss; inquire about weight gain or loss over the recent weeks or months.	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.	Progressive muscle weakness interferes with safe oral feeding. Decreased intake of nutrients can contribute to weakness and fatigue.

Therapeutic Interventions

Actions/Interventions	Rationale
Encourage family meals if possible.	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.	Sticky foods and milk products increase mucus thickness.
Instruct the patient not to talk while eating.	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.	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.	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.	Decreased salivation makes swallowing of certain foods difficult.
Discuss the need to keep the dining environment well ventilated, uncluttered, cheerful, and free of distraction.	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.	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.	Supplemental measures may be required to maintain adequate nutritional state and weight.

Nursing Diagnosis

Impaired Verbal Communication

Common Related Factors	Defining Characteristics
Dysarthria Tongue weakness Nasal tone to speech	Difficulty in articulating words Inability to express self clearly
Common Expected Outcome 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

Ongoing Assessment

Actions/Interventions	Rationale
Determine the degree of speech difficulty by assessing the patient’s ability to speak spontaneously and endurance of ability to speak.	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).	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.

Therapeutic Interventions

Actions/Interventions	Rationale
Inform the patient and family about dysarthria and its effects on speech and language ability.	Alternatives can be developed in anticipation of need.
Use closed-ended questions requiring only a “yes” or “no” response.	This minimizes effort, conserves energy, and decreases anxiety.
Allow the patient time to respond and to organize responses. Avoid interrupting.	It is difficult to respond under pressure. As the disease progresses, muscle weakness may slow speech production.
Anticipate needs.	These decrease feelings of helplessness.
Encourage use of writing pad or a spelling board, as indicated.	These increase self-esteem and ability to communicate.
Praise accomplishments.	Positive feedback provides encouragement to continue communication efforts.
Consult a speech therapist for additional help.	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.

Nursing Diagnosis

Deficient Knowledge

Common Related Factor	Defining Characteristics
Unfamiliarity with disease process and management	Lack of questions Multiple questions Misconceptions
Common Expected Outcome 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

Ongoing Assessment

Actions/Interventions	Rationale
Assess knowledge of the disease process, diagnostic tests, and treatment outcome.	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.	Awareness of other resources may improve coping mechanisms.

Therapeutic Interventions

Actions/Interventions	Rationale
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)	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	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.	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	This organization provides patients and families with information, resources, and equipment loans to support quality of life.