Chapter 5 nursing study notes medical surgical nursing notes

Amyotrophic Lateral Sclerosis (ALS)
Nursing Care Plan & Management
Definition


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.
Risk Factors




Autoimmune
Free radical damage
Oxidative stress
Cigarette smoking
Pathophysiology


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:
▪ Fatigue
▪ Progressive muscle weakness
▪ Cramps
▪ Twitching
▪ Incoordination
Anterior horns
Progressive weakness
▪ Muscle atrophy (arms, trunk, legs)
▪ Spasticity
▪ Brisk or overreactive muscle reflexes
Cranial nerves
▪ Muscle weakness
▪ Difficulty talking
▪ Difficulty swallowing
▪ Difficulty breathing
▪ Soft palate and upper esophageal weakness
▪ Weakness on the posterior tongue
Bulbar muscles
▪ Progressive difficulty in speaking
▪ Difficulty in swallowing
▪ Articulation and speech effects
▪ Compromised respiratory function

Diagnostic Tests
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
Medical Management
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.
Nursing Diagnosis




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
Nursing Management
1. Provide intellectual stimulating activities, because the client
typically experiences no cognitive deficits and retains mental
abilities.
2. Provide client and family teaching.
3. Promote measures to enhance body image.
4. Promote client and family coping as the client and his family deal
with the poor prognosis and the grieving process
5. Provide referrals.
6. Maximize functional abilities
▪ Prevent complications of immobility
▪ Promote self-care
▪ Maximize effective communication
7. Ensure adequate nutrition
8. 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
Nursing Diagnosis

Anxiety
Related Factors



Terminal disease process
Threat to self-concept
Threat to or change in health status, socioeconomic status,
independence
Defining Characteristics















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
Expected Outcomes


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
Nursing Interventions
Ongoing Assessment
▪ 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.
Therapeutic Interventions
▪ 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.


Nursing Diagnosis

Ineffective Airway Clearance
Related Factors




Dysarthria
Aspiration
Progressive bulbar palsy
Respiratory muscle weakness
Defining Characteristics



Patient reports breathing difficulty
Abnormal lung sounds: rales (crackles), rhonchi, wheezes
Periods of apnea
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
Nursing Interventions
Ongoing Assessment
▪ 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.
Therapeutic Interventions
▪ 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.
Nursing Diagnosis

Impaired Physical Mobility
Related Factors






Increasing motor weakness caused by paralysis
Spasticity of extremities
Limited range of motion (ROM)
Fatigue
Neuromuscular impairment
Imposed restrictions of movement
Defining Characteristics



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
Expected Outcome

Patient maintains optimal physical mobility within limits of disease.
▪ NOC Outcomes: Mobility; Ambulation; Immobility
Consequences: Physiological
▪ NIC Intervention: Exercise Therapy: Muscle Control
Nursing Interventions
Ongoing Assessment
▪ 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.
Therapeutic Interventions
▪ 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.





Nursing Diagnosis

Imbalanced Nutrition: Less Than Body Requirements
Related Factors





Progressive bulbar palsy
Tongue atrophy or weakness
Dysphagia
Decreased salivation
Choking during meals
Defining Characteristics


Loss of appetite
Loss of weight
Expected Outcome

Patient maintains weight or does not continue to lose weight.
▪ NOC Outcome: Nutritional Status: Nutrient Intake

NIC Interventions: Nutrition Management; Swallowing
Therapy
Nursing Interventions
Ongoing Assessment
▪ 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.
Therapeutic Interventions
▪ 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.






Nursing Diagnosis

Impaired Verbal Communication
Related Factors



Dysarthria
Tongue weakness
Nasal tone to speech
Defining Characteristics


Difficulty in articulating words
Inability to express self clearly
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
Nursing Interventions
Ongoing Assessment
▪ 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 “hightech” communication support.
Therapeutic Interventions
▪ 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.
Nursing Diagnosis

Deficient Knowledge
Related Factor

Unfamiliarity with disease process and management
Defining Characteristics



Lack of questions
Multiple questions
Misconceptions
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
Nursing Interventions
Ongoing Assessment
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.
Therapeutic Interventions
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.

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