Addison's Disease (Primary Adrenocortical Insufficiency)

Addison's disease is caused by a deficiency of cortical hormones. It results whe

n the adrenal cortex function is inadequate to meet the patient's need for corti
cal hormones. Autoimmune or idiopathic atrophy of the adrenal glands is responsi
ble for 80% to 90% of cases. Other causes include surgical removal of both adren
al glands or infection (tuberculosis or histoplasmosis) of the adrenal glands. I
nadequate secretion of adrenocorticotropic hormone (ACTH) from the primary pitui
tary gland results in adrenal insufficiency. Symptoms may also result from sudde
n cessation of exogenous adrenocortical hormonal therapy, which interferes with
normal feedback mechanisms.

Clinical Manifestations


Chief clinical manifestations include muscle weakness, anorexia, gastrointestina
l symptoms, fatigue, emaciation, dark pigmentation of the skin and mucous membra
nes, hypotension, low blood glucose, low serum sodium, and high serum potassium.
The onset usually occurs with nonspecific symptoms. Mental changes (depression,
emotional lability, apathy, and confusion) are present in 60% to 80% of patient
s. In severe cases, disturbance of sodium and potassium metabolism may be marked
by depletion of sodium and water and severe, chronic dehydration.

Addisonian Crisis


This medical emergency develops as the disease progresses. Signs and symptoms in
clude:
Cyanosis, fever, and classic signs of circulatory shock: pallor, apprehension, r
apid and weak pulse, rapid respirations, and low blood pressure
Headache, nausea, abdominal pain, diarrhea, confusion, and restlessness
Slight overexertion, exposure to cold, and acute infections decrease salt intake
and may lead to circulatory collapse, shock, and death.
Stress of surgery or dehydration from preparation for diagnostic tests or surger
y may precipitate addisonian or hypotensive crisis.
Decreased blood glucose and sodium levels, increased serum potassium (hyperkalem
ia), and leukocytosis

Assessment and Diagnostic Findings


Primary adrenocortical insufficiency; greatly increased plasma ACTH (>22.0 pmol/
L); serum cortisol low normal or lower than normal (<165 nmol/L); decreased bloo
d glucose (hypoglycemia) and sodium (hyponatremia) levels, increased serum potas
sium (hyperkalemia) level, and increased white blood cell count (leukocytosis).

Medical Management


Immediate treatment is directed toward combating shock.
Restore blood circulation, administer fluids, monitor vital signs, and place pat
ient in a recumbent position with legs elevated.
Administer intravenous hydrocortisone, followed by 5% dextrose in normal saline.
Vasopressor amines may be required if hypotension persists.
Antibiotics may be prescribed for infection.
Oral intake may be initiated as soon as tolerated.
If adrenal gland does not regain function, lifelong replacement of corticosteroi
ds and mineralocorticoids is required.
Dietary intake should be supplemented with salt during times of gastrointestinal
losses of fluids through vomiting and diarrhea.

NURSING PROCESS: The Patient with Addison's Disease


Assessment


Assessment focuses on fluid imbalance and stress.
Check blood pressure from a lying to standing position; check pulse rate.
Assess skin color and turgor.
Assess history of weight changes, muscle weakness, and fatigue.
Ask patient and family about onset of illness or increased stress that may have
precipitated crisis.

Diagnosis


NURSING DIAGNOSES

Deficient fluid volume related to inadequate fluid intake and fluid loss seconda
ry to inadequate adrenal hormone secretion
Deficient knowledge about need for hormone replacement and dietary modification

COLLABORATIVE PROBLEMS/POTENTIAL COMPLICATIONS


Addisonian crisis

Planning and Goals


Goals may include improving fluid balance, improving response to activity, decre
asing stress, increasing knowledge about need for hormone replacement and dietar
y modifications, and ensuring absence of complications.

Nursing Interventions


RESTORING FLUID BALANCE

Record weight changes daily.
Assess skin turgor and mucous membranes.
Instruct patient to report increased thirst.
Monitor lying, sitting, and standing blood pressures frequently.
Assist patient in selecting, and encourage patient to consume, food and fluids t
hat assist in restoring and maintaining fluid and electrolyte balance (eg, foods
high in sodium during gastrointestinal disturbances and very hot weather). Incl
ude a dietitian for added guidance.
Assist patient and family in learning to administer hormone replacement and to m
odify dosage during illness and stress.
Provide written and verbal instructions about mineralocorticoid and glucocortico
id therapy.

IMPROVING ACTIVITY TOLERANCE

Avoid unnecessary activities and stress that might precipitate a hypotensive epi
sode.
Detect signs of infection or presence of stressors that may have triggered the c
risis.
Provide a quiet, nonstressful environment during acute crises; carry out all act
ivities for patient.
Explain all procedures to reduce fear and anxiety.
Explain rationale for minimizing stress during acute crisis.

MONITORING AND MANAGING COMPLICATIONS (ADDISONIAN CRISIS)

Assess for signs and symptoms of crisis: circulatory collapse and shock.
Avoid physical and psychological stress, including exposure to cold, overexertio
n, infection, and emotional distress.
Initiate immediate treatment with intravenous fluid, glucose, and electrolytes,
especially sodium; corticosteroid supplements; and vasopressors.
Avoid patient exertion; anticipate and take measures to meet patient's needs.
Monitor symptoms, vital signs, weight, and fluid and electrolyte balance to eval
uate return to precrisis state.
Identify factors that led to crisis.

Promoting Home and Community-Based Care


TEACHING PATIENTS SELF-CARE

Give patient and family explicit verbal and written instructions about the ratio
nale for replacement therapy and proper dosage.
Teach patient and family how to modify drug dosage and increase salt in times of
illness, very hot weather, and stressful situations.
Instruct patient to modify diet and fluid intake to maintain fluid and electroly
te balance.
Provide patient and family with a syringe and vial of injectable steroid (Solu-C
ortef) for emergency use and instruct when and how to use.
Advise patient to inform health care providers (eg, dentists) of steroid use. Ur
ge patient to wear a medical alert bracelet.
Teach patient and family signs of excessive or insufficient hormone replacement.
Instruct patient regarding modification of diet (sodium) during illness and hot
weather to maintain fluid and electrolyte balance.

CONTINUING CARE

Encourage patients to weigh themselves daily to detect significant changes in we
ight that indicate fluid loss (due to too little hormone) or retention (due to t
oo much hormone).
If patient cannot return to work and family responsibilities after hospital disc
harge, refer to home health care nurse.
Assess recovery, monitor hormone replacement, and assess stress in the home.
Assess patient's plans for follow-up visits to clinic or physician's office.




For more information, see Chapter 42 in Smeltzer and Bare: Brunner and Suddarth'
s Textbook of Medical-Surgical Nursing, 11th edition. Philadelphia: Lippincott W
illiams & Wilkins, 2008.

Alzheimer's Disease


Alzheimer's disease (AD) is one of the most common, irreversible, degenerative n
eurologic dementias. Dementia is an acquired syndrome of progressive deteriorati
on in global intellectual abilities. The deterioration interferes with the perso
n's customary occupational and social performance. In AD, specific neuropatholog
ic and biochemical changes are thought to result in decreased brain size and dec
reased acetylcholine production. The disease begins insidiously and is character
ized by gradual loss of cognitive and functional abilities and disturbances in b
ehavior and affect.
Combined factors are thought to cause AD, including genes, neurotransmitter chan
ges, vascular abnormalities, stress hormones, circadian changes, head trauma, an
d seizures.
The role of inflammation and oxidative stress and the contribution of brain infa
rctions are being explored as contributory factors to AD. Death occurs as a resu
lt of a complicating condition such as pneumonia, malnutrition, or dehydration.

Clinical Manifestations


Symptoms are highly variable; some include:
In early disease there is forgetfulness and subtle memory loss, although social
skills and behavior patterns remain intact. Forgetfulness is manifested in many
daily actions with progression of the disease (eg, the patient gets lost in a fa
miliar environment or repeats the same stories).
Ability to formulate concepts and think abstractly disappears.
Patient may exhibit inappropriate impulsive behavior.
Personality changes are evident; patient may become depressed, suspicious, paran
oid, hostile, and combative.
Speaking skills deteriorate to nonsense syllables; agitation and physical activi
ty increase.
Voracious appetite may develop from high activity level; dysphagia is noted with
disease progression.
Eventually patient requires help with all aspects of daily living, including toi
leting because incontinence occurs.
Terminal stage may last for months.

Assessment and Diagnostic Findings


The diagnosis, which is one of exclusion, is confirmed at autopsy.
Clinical symptoms are found through health history, including physical findings
and results from functional abilities assessments (eg, Mini-Mental Status Examin
ation)
Electroencephalography (EEG)
Computed tomography (CT) scan
Magnetic resonance imaging (MRI)
Laboratory tests, primarily blood and cerebrospinal fluid (CSF)

Medical Management


Without a cure or a way to slow progression of AD, treatment relies on managing
cognitive symptoms with cholinesterase inhibitors, such as donepezil (Aricept),
rivastigmine (Exelon), and galantamine (Reminyl). These drugs enhance acetylchol
ine uptake in the brain to maintain memory for a while. Memantine (Namenda) was
recently approved for use to reduce clinical deterioration in moderate to severe
AD.

NURSING PROCESS: The Patient with Alzheimer's Disease


Assessment


Obtain health history with mental status exam and physical examination, noting s
ymptoms indicating dementia. Report findings to physician. As indicated, assist
with diagnostic evaluation, promoting calm environment to maximize patient safet
y and cooperation.

Nursing Diagnoses

Impaired thought processes related to decline in cognitive function
Risk for injury related to decline in cognitive function
Anxiety related to confused thought processes
Imbalanced nutrition: less than body requirements related to cognitive decline
Activity intolerance related to imbalance in activity/rest pattern
Deficient self-care, bathing/hygiene, feeding, toileting related to cognitive de
cline
Impaired social interaction related to cognitive decline
Deficient knowledge of family/caregiver related to care for patient as cognitive
function declines
Ineffective family processes related to decline in patient's cognitive function

Planning and Goals


Goals for the patient may include supporting cognitive function, physical safety
, reduced anxiety, adequate nutrition, improved, activity tolerance, self-care,
and socialization support, and education of caregivers.

Nursing Interventions


SUPPORTING COGNITIVE FUNCTION


Provide a calm, predictable environment to minimize confusion and disorientation
. Help patient feel a sense of security with a quiet, pleasant manner, clear, si
mple explanations, and use of memory aids and cues.

PROMOTING PHYSICAL SAFETY

Provide a safe environment to allow patient to move about as freely as possible
and relieve family's worry about safety.
Prevent falls and other accidents by removing obvious hazards and providing adeq
uate lighting.
Monitor intake of medications and food.
Allow smoking only with supervision.
Reduce wandering behavior with gentle persuasion and distraction. Supervise all
activities outside the home to protect patient. As needed, secure doors leading
from the house. Ensure that patient wears an identification bracelet or neck cha
in.
Avoid restraints, because they may increase agitation.

REDUCING ANXIETY AND AGITATION

Give emotional support to support a positive self-image.
When skill losses occur, adjust goals to fit patient's declining ability and str
ucture activities to help prevent agitation.
Keep the environment simple, familiar, and noise-free; limit changes.
Remain calm and unhurried, particularly if the patient is experiencing a combati
ve, agitated state known as catastrophic reaction (overreaction to excessive sti
mulation).
Use easy-to-understand sentences to convey messages.

PROMOTING ADEQUATE NUTRITION

Keep mealtimes simple and calm; avoid confrontations.
Cut food into small pieces to prevent choking, and convert liquids to gelatin to
ease swallowing. Offer one dish at a time.
Prevent burns by serving typically hot food and beverages warm.

BALANCING ACTIVITY AND REST

Help patient to relax to sleep with music, warm milk, or a back rub.
To enhance nighttime sleep, provide sufficient opportunities for daytime exercis
e. Discourage long periods of daytime sleeping.
Assess and address any unmet underlying physical or psychological needs that may
prompt wandering or other inappropriate behavior.

PROMOTING INDEPENDENCE IN SELF-CARE ACTIVITIES

Simplify daily activities into short achievable steps so that patient feels a se
nse of accomplishment.
Maintain patient's personal dignity and autonomy.
Encourage patient to make choices when appropriate and to participate in self-ca
re activities as much as possible.

MEETING SOCIALIZATION NEEDS

Encourage visits, letters, and phone calls (visits should be brief and nonstress
ful, with one or two visitors at a time).
Advise that the nonjudgmental friendliness of a pet can provide satisfying activ
ity and an outlet for energy.
Encourage spouse to talk about any sexual concerns, and suggest sexual counselin
g if necessary.

PROMOTING HOME AND COMMUNITY-BASED CARE


Be sensitive to the highly emotional issues that the family is confronting. Enco
urage early visits to explore long-term care facilities to minimize "crisis relo
cation" if and when it becomes necessary. Refer family to the Alzheimer's Associ
ation for assistance with family support groups, respite care, and adult day car
e services.

Evaluation


EXPECTED PATIENT OUTCOMES

Patient maintains cognitive, functional, and social interaction abilities for as
long as possible.
Patient remains free of injury.
Patient demonstrates minimal anxiety and agitation.
Patient receives adequate nutrition, activity and rest.
Patient's socialization needs are met.
Patient and family caregivers are knowledgeable about condition and treatment an
d care regimens.




For more information, see Chapter 12 in Smeltzer and Bare: Brunner and Suddarth'
s Textbook of Medical-Surgical Nursing, 11th edition. Philadelphia: Lippincott W
illiams & Wilkins, 2008.

Amyotrophic Lateral Sclerosis


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 ho
rns of the spinal cord and the motor nuclei of the lower brain stem. As these ce
lls die, the muscle fibers that they supply undergo atrophic changes. The degene
ration of the neurons may occur in both the upper and lower motor neuron systems
. ALS affects more men than women, with onset occurring usually in the fifth or
sixth decade of life. In the United States, it is often referred to as Lou Gehri
g's disease. Death occurs from infection, respiratory failure, or aspiration. Th
e average time from onset to death is about 3 years.

Clinical Manifestations


Clinical features of ALS depend on the location of the affected motor neurons. I
n most patients, the chief symptoms are progressive muscle weakness, cramps, inc
oordination, atrophy, and fasciculations (twitching).

Loss of Motor Neurons in Anterior Horns of Spinal Cord

Progressive weakness and atrophy of the arms, trunk, or leg muscles
Spasticity; deep tendon stretch reflexes are brisk and overactive.
Anal and bladder sphincters not affected

Weakness in Muscles Supplied by Cranial Nerves (25% of Patients in Early Stage)

Difficulty talking, swallowing, and ultimately breathing
Soft palate and upper esophageal weakness, causing liquids to be regurgitated th
rough nose
Impaired ability to laugh, cough, or blow the nose

Bulbar Muscle Impairment

Progressive difficulty in speaking and swallowing, and aspiration
Nasal voice and unintelligible speech
Emotional lability, but intellectual function unimpaired
Eventually, compromised respiratory function

Assessment and Diagnostic Methods


Diagnosis is based on signs and symptoms because no clinical or laboratory tests
are specific for this disease. Electromyographic (EMG) studies and magnetic res
onance imaging may be helpful.

Medical Management


No specific treatment for ALS is available. See below for symptomatic treatment.

Supportive and Rehabilitative Measures

Baclofen, dantrolene sodium, or diazepam for spasticity
Quinine for muscle cramps
Riluzole (a glutamate antagonist)
Enteral feedings (percutaneous endoscopic gastrostomy [PEG]) for patients with a
spiration or swallowing difficulties

Mechanical Ventilation

Decision is based on patient and family's understanding of the disease, prognosi
s, and implications of initiating such therapy.
Encourage patient to complete an advance directive or "living will" to preserve
autonomy.

Nursing Management


The nursing care of the patient with ALS is generally the same as the basic care
plan for patients with degenerative neurologic disorders (see Myasthenia Gravis
). Encourage patient and family to contact the ALS Association for information a
nd support.




For more information, see Chapter 65 in Smeltzer and Bare: Brunner and Suddarth'
s Textbook of Medical-Surgical Nursing, 11th edition. Philadelphia: Lippincott W
illiams & Wilkins, 2008.

Anaphylaxis


Anaphylaxis is a severe, life-threatening allergic response to an immunologic re
action (type I hypersensitivity) between a specific antigen and an immunoglobuli
n E (IgE) antibody. Type I hypersensitivity requires previous exposure to the sp
ecific antigen through inhalation, injection, ingestion, or skin contact. An ana
phylactoid (anaphylaxis-like) reaction is clinically similar to anaphylaxis. Ana
phylaxis may occur with medications, food, latex, insect stings, and cytotoxic a
ntibody transfusions. Reactions may be local or systemic. Local anaphylactic rea
ctions usually involve urticaria (hives) and angioedema (swelling) at the exposu
re site. The reaction can be severe but is rarely fatal. Systemic reactions occu
r in major organ systems within minutes of exposure.

Clinical Manifestations


Clinical symptoms are determined by the amount of the allergen, the amount of me
diator released, the sensitivity of the target organ, and the route of allergen
entry. Type I hypersensitivity reactions may include both local and systemic ana
phylaxis.

Mild


Symptoms include peripheral tingling; a warm sensation; fullness in the mouth an
d throat; nasal congestion; periorbital swelling, pruritus, sneezing, and tearin
g eyes. Symptoms begin within 2 hours of exposure.

Moderate


May include any of the mild symptoms plus anxiety, bronchospasm, and edema of th
e airways or larynx with dyspnea, cough, and wheezing. Symptom onset is the same
as that of a mild reaction.

Severe


An abrupt onset with the same signs and symptoms described above, progressing ra
pidly to bronchospasm, laryngeal edema, severe dyspnea, cyanosis, and hypotensio
n. Dysphagia, abdominal cramping, vomiting, diarrhea, and seizures are additiona
l symptoms, with cardiac arrest and coma occurring rarely.

Assessment and Diagnostic Methods


Diagnostic evaluation of the patient with allergic disorders commonly includes b
lood tests (CBC with differential, high total serum IgE levels), smears of body
secretions, skin tests, and the radioallergosorbent test (RAST).

Prevention


Prevention by avoidance of allergens is of utmost importance. Health care provid
ers should always obtain a careful history of any sensitivities before administe
ring medications. Venom immunotherapy may be given to people who are allergic to
insect venom. Insulin-allergic diabetic patients or penicillin-sensitive patien
ts may require desensitization as well.

Medical Management


Respiratory and cardiovascular functions are evaluated and cardiopulmonary resus
citation (CPR) is initiated in cases of cardiac arrest. Oxygen is administered i
n high concentrations during CPR or when the patient is cyanotic, dyspneic, or w
heezing. Patients with mild reactions need to be educated about the risk for rec
urrences. Patients with severe reactions need to be observed for 12 to 14 hours.

Pharmacologic Therapy

Epinephrine, antihistamines, and corticosteroids may be given to prevent recurre
nces of the reaction and to relieve urticaria and angioedema.
Aminophylline may be administered if indicated.
Volume expanders and vasopressor agents may be used to maintain blood pressure a
nd normal hemodynamic status; glucagon also may be used.

Nursing Management

Teach people who are sensitive to insect bites and stings, certain medications,
or foods, and people who have idiopathic or exercise-induced anaphylactic reacti
ons to avoid exposure to allergens.
Instruct patient always to carry an emergency kit that contains injectable epine
phrine.
Provide verbal and written instruction about the emergency kit.
Ensure that patient can perform correct self-injection.
Encourage patient always to wear or carry medical identification.