Republic of the Philippines

ISABELA STATE UNIVERSITY

City of Ilagan Campus

COLLEGE OF NURSING

NUR 322 (Care of the Clients with Endocrine Disorders)

Subject Professor: CHARLES Z. ARIOLA JR., MSN, LPT, RN.

THE ADRENAL GLANDS

Anatomic and Physiologic Overview

- Each person has two adrenal glands, one attached to the upper portion of each kidney (Porth &
Matfin, 2009).
- Each adrenal gland is, in reality, two endocrine glands with separate, independent functions.
The adrenal medulla at the center of the gland secretes catecholamines, and the outer portion
of the gland, the adrenal cortex, secretes steroid hormones .
- The secretion of hormones from the adrenal cortex is regulated by the hypothalamic– pituitary–
adrenal axis.
- The hypothalamus secretes corticotropin-releasing hormone (CRH), which stimulates the
pituitary gland to secrete ACTH, which in turn stimulates the adrenal cortex to secrete
glucocorticoid hormone (cortisol).
- Increased levels of the adrenal hormone then inhibit the production or secretion of CRH and
ACTH. This system is an example of a negative feedback mechanism.
The Adrenal Medulla

- The adrenal medulla functions as part of the autonomic nervous system.

- Stimulation of preganglionic sympathetic nerve fibers, which travel directly to the cells of the
adrenal medulla, causes release of the catecholamine hormones epinephrine and
norepinephrine.
- About 90% of the secretion of the human adrenal medulla is epinephrine (also called
adrenaline). Catecholamines regulate metabolic pathways to promote catabolism of stored fuels
to meet caloric needs from endogenous sources.
- The major effects of epinephrine release are to prepare to meet a challenge (fight-or-flight
response).
- Secretion of epinephrine causes decreased blood flow to tissues that are not needed in
emergency situations, such as the GI tract, and increased blood flow to tissues that are
important for effective fight or flight, such as cardiac and skeletal muscle.
- Catecholamines also induce the release of free fatty acids, increase the basal metabolic rate,
and elevate the blood glucose level.

Adrenal Cortex

- Functioning adrenal cortex is necessary for life; adrenocortical secretions make it possible for
the body to adapt to stress of all kinds.
- The three types of steroid hormones produced by the adrenal cortex are:
Glucocorticoids - the prototype of which is hydrocortisone
Mineralocorticoids- mainly aldosterone
Sex hormones - mainly androgens (male sex hormones).
- Without the adrenal cortex, severe stress would cause peripheral circulatory failure, circulatory
shock, and prostration. Survival in the absence of a functioning adrenal cortex is possible only
with nutritional, electrolyte, and fluid replacement and appropriate replacement with
exogenous adrenocortical hormones.

Glucocorticoids

- The glucocorticoids are so named because they have an important influence on glucose
metabolism:
- Increased hydrocortisone secretion results in elevated blood glucose levels. However, the
glucocorticoids have major effects on the metabolism of almost all organs of the body.
- Glucocorticoids are secreted from the adrenal cortex in response to the release of ACTH from
the anterior lobe of the pituitary gland.
- This system represents an example of negative feedback. The presence of glucocorticoids in the
blood inhibits the release of CRH from the hypothalamus and also inhibits ACTH secretion from
the pituitary.
 - The resultant decrease in ACTH secretion causes diminished release of glucocorticoids from the
adrenal cortex. Glucocorticoids (in the form of corticosteroids) are administered frequently to
inhibit the inflammatory response to tissue injury and to suppress allergic manifestations.
- Their side effects include the development of diabetes mellitus, osteoporosis, peptic ulcer,
increased protein breakdown resulting in muscle wasting and poor wound healing, and
redistribution of body fat.
- Large amounts of exogenously administered glucocorticoids in the blood inhibit the release of
ACTH and endogenous glucocorticoids. Because of this, the adrenal cortex can atrophy. If
exogenous glucocorticoid administration is discontinued suddenly, adrenal insufficiency results
because of the inability of the atrophied cortex to respond adequately.

Mineralocorticoids

- Mineralocorticoids exert their major effects on electrolyte metabolism.

- They act principally on the renal tubular and GI epithelium to cause increased sodium ion
absorption in exchange for excretion of potassium or hydrogen ions.
- ACTH only minimally influences aldosterone secretion. It is primarily secreted in response to the
presence of angiotensin II in the bloodstream.
- Angiotensin II is a substance that elevates the blood pressure by constricting arterioles. Its
concentration is increased when renin is released from the kidney in response to decreased
perfusion pressure.
- The resultant increased aldosterone levels promote sodium reabsorption by the kidney and the
GI tract, which tends to restore blood pressure to normal.
- The release of aldosterone is also increased by hyperkalemia. Aldosterone is the primary
hormone for the long-term regulation of sodium balance.

Adrenal Sex Hormones (Androgens)

- Androgens, the third major type of steroid hormones produced by the adrenal cortex, exert
effects similar to those of male sex hormones.
- The adrenal gland may also secrete small amounts of some estrogens, or female sex hormones.
ACTH controls the secretion of adrenal androgens.
- When secreted in normal amounts, the adrenal androgens probably have little effect, but when
secreted in excess, as in certain inborn enzyme deficiencies, masculinization may result. This is
termed the adrenogenital syndrome.

SPECIFIC DISORDERS OF ADRENAL GLANDS

Pheochromocytoma

- Pheochromocytoma is a tumor that is usually benign and originates from the chromaffin cells of
the adrenal medulla. In 90% of patients (Porth & Matfin, 2009), the tumor arises in the medulla;
 in the remaining patients, it occurs in the extra-adrenal chromaffin tissue located in or near the
aorta, ovaries, spleen, or other organs.
- Pheochromocytoma may occur at any age, but its peak incidence is between 40 and 50 years of
age affecting men and women equally. Ten percent of the tumors are bilateral, and 10% are
malignant.
- Because of the high incidence of pheochromocytoma in family members of affected people, the
patient’s family members should be alerted and screened for this tumor.
- Pheochromocytoma may occur in the familial form as part of multiple endocrine neoplasia type
2; therefore, it should be considered a possibility in patients who have medullary thyroid
carcinoma and parathyroid hyperplasia or tumor.
- Pheochromocytoma is the cause of high blood pressure in 0.1% of patients with hypertension.
- Although it is uncommon, it is one form of hypertension that is usually cured by surgery;
however, without detection and treatment, it is usually fatal.

Clinical Manifestations

- The nature and severity of symptoms of functioning tumors of the adrenal medulla depend on
the relative proportions of epinephrine and norepinephrine secretion.
- The typical triad of symptoms is headache, diaphoresis, and palpitations in the patient with
hypertension.
- Approximately 8% of patients are completely asymptomatic.
- Hypertension and other cardiovascular disturbances are common. The hypertension may be
intermittent or persistent. However, only half of patients with pheochromocytoma have
sustained or persistent hypertension. If the hypertension is sustained, it may be difficult to
distinguish from other causes of hypertension.
- Other symptoms may include tremor, headache, flushing, and anxiety.
- Hyperglycemia may result from conversion of liver and muscle glycogen to glucose due to
epinephrine secretion; insulin may be required to maintain normal blood glucose levels. The
clinical picture in the paroxysmal form of pheochromocytoma is usually characterized by acute,
unpredictable attacks lasting seconds or several hours.
- Symptoms usually begin abruptly and subside slowly. During these attacks, the patient is
extremely anxious, tremulous, and weak.
- The patient may experience headache, vertigo, blurring of vision, tinnitus, air hunger, and
dyspnea.
- Other symptoms include polyuria, nausea, vomiting, diarrhea, abdominal pain, and a feeling
of impending doom.
- Palpitations and tachycardia are common (Porth & Matfin, 2009). Blood pressures exceeding
250/150 mm Hg have been recorded.
- Such blood pressure elevations are life-threatening and can cause severe complications, such
as cardiac dysrhythmias, dissecting aneurysm, stroke, and acute renal failure.
- Postural hypotension (decrease in systolic blood pressure, lightheadedness, dizziness on
standing) occurs in 70% of patients with untreated pheochromocytoma.
Assessment and Diagnostic Findings

- Pheochromocytoma is suspected if signs of sympathetic nervous system overactivity occur in

association with marked elevation of blood pressure.
- These signs can be associated with the “five H’s”: hypertension, headache, hyperhidrosis
(excessive sweating), hypermetabolism, and hyperglycemia. The presence of these signs is
highly predictive of pheochromocytoma.
- Paroxysmal symptoms of pheochromocytoma commonly develop in the fifth decade of life.
- Measurements of urine and plasma levels of catecholamines and metanephrine (MN), a
catecholamine metabolite, are the most direct and conclusive tests for overactivity of the
adrenal medulla. A new test for detecting pheochromocytoma has recently been developed that
measures free MN in plasma by high-pressure liquid chromatography and electrochemical
detection. A negative test result virtually excludes pheochromocytoma. However, increased
levels of at least one catecholamine or MN can occur in 10% of patients with essential
hypertension. Measurements of catecholamine metabolites (MN and vanillylmandelic acid
[VMA]) or free catecholamines have been extensively used in the clinical setting.
- In most cases, pheochromocytoma can be diagnosed or confirmed based on a properly collected
24-hour urine sample. Levels can be as high as two times the normal limit.
- A 24-hour specimen of urine is collected for determination of free catecholamines, MN, and
VMA; the use of combined tests increases the diagnostic accuracy of testing.
- A number of medications and foods, such as coffee and tea (including decaffeinated varieties),
bananas, chocolate, vanilla, and aspirin, may alter the results of these tests; therefore, careful
instructions to avoid restricted items must be given to the patient.
- Urine collected over a 2- or 3-hour period after an attack of hypertension can be assayed for
catecholamine content. The total plasma catecholamine (epinephrine and norepinephrine)
concentration is measured with the patient supine and at rest for 30 minutes.
- To prevent elevation of catecholamine levels resulting from the stress of venipuncture, a
butterfly needle, scalp vein needle, or venous catheter may be inserted 30 minutes before the
blood specimen is obtained.
- Factors that may elevate catecholamine concentrations must be controlled to obtain valid
results; these factors include consumption of coffee or tea (including decaffeinated varieties),
use of tobacco, emotional and physical stress, and use of many prescription and over-the-
counter medications (eg, amphetamines, nose drops or sprays, decongestant agents,
bronchodilators). Normal plasma values of epinephrine are 100 pg/mL (590 pmol/L); normal
values of norepinephrine are generally less than 100 to 550 pg/mL (590 to 3240 pmol/L).
- Values of epinephrine greater than 400 pg/mL (2180 pmol/L) or norepinephrine values greater
than 2000 pg/mL (11,800 pmol/L) are considered diagnostic of pheochromocytoma.
- Values that fall between normal levels and those diagnostic of pheochromocytoma indicate the
need for further testing. A clonidine suppression test may be performed if the results of plasma
and urine tests of catecholamines are inconclusive.
- Clonidine (Catapres) is a centrally acting anti- adrenergic medication that suppresses the release
of neurogenically mediated catecholamines. The suppression test is based on the principle that
catecholamine levels are normally increased through the activity of the sympathetic nervous
system. In pheochromocytoma, increased catecholamine levels result from the diffusion of
excess catecholamines into the circulation, bypassing normal storage and release mechanisms.
 - Therefore, in patients with pheochromocytoma, clonidine does not suppress the release of
catecholamines.
- Imaging studies, such as CT, MRI, and ultrasonography, may also be carried out to localize the
pheochromocytoma and to determine whether more than one tumor is present.
- Use of 131I-metaiodobenzylguanidine (MIBG) scintigraphy may be required to determine the
location of the pheochromocytoma and to detect metastatic sites outside the adrenal gland.
MIBG is a specific isotope for catecholamine-producing tissue.
- It has been helpful in identifying tumors not detected by other tests or procedures. MIBG
scintigraphy is a noninvasive, safe procedure that has increased the accuracy of diagnosis of
adrenal tumors. Other diagnostic studies may focus on evaluating the function of other
endocrine glands because of the association of pheochromocytoma in some patients with other
endocrine tumors.

Medical Management

- During an episode or attack of hypertension, tachycardia, anxiety, and the other symptoms of
pheochromocytoma, bed rest with the head of the bed elevated is prescribed to promote an
orthostatic decrease in blood pressure.

Pharmacologic Therapy

- The patient may be moved to the intensive care unit for close monitoring of ECG changes and
careful administration of alpha-adrenergic blocking agents (eg, phentolamine [Regitine]) or
smooth muscle relaxants (eg, sodium nitroprusside [Nipride]) to lower the blood pressure
quickly.
- Phenoxybenzamine (Dibenzyline), a long-acting alpha-blocker, may be used after the blood
pressure is stable to prepare the patient for surgery.
- Calcium channel blockers such as nifedipine (Procardia) are usually well tolerated by patients
and have reduced perioperative fluid requirements. They are also useful for prevention of
cardiovascular complications, because they prevent catecholamine-induced coronary vasospasm
and myocarditis.
- Beta-adrenergic blocking agents such as propranolol (Inderal) may be used in patients with
cardiac dysrhythmias and in those not responsive to alpha-blockers.
- Alpha-adrenergic and beta-adrenergic blocking agents must be used with caution, because
patients with pheochromocytoma may have increased sensitivity to them.
- Still other medications that may be used preoperatively are catecholamine synthesis inhibitors,
such as alpha-methyl-p-tyrosine (metyrosine [Demser]). These are occasionally used if
adrenergic blocking agents do not reduce the effects of catecholamines.

Surgical Management
- The definitive treatment of pheochromocytoma is surgical removal of the tumor, usually with
adrenalectomy.
- Bilateral adrenalectomy may be necessary if tumors are present in both adrenal glands.
- Patient preparation includes:
1. control of blood pressure and blood volumes; usually this is carried out over 4 to 7 days.
2. Nifedipine (Procardia) and nicardipine (Cardene) may be used safely without causing undue
hypotension. For episodes of severe hypertension, nifedipine is a fast and effective
treatment, because the capsules can be pierced and chewed.
3. The patient needs to be well hydrated before, during, and after surgery to prevent
hypotension.
- Manipulation of the tumor during surgical excision may cause release of stored epinephrine and
norepinephrine, with marked increases in blood pressure and changes in heart rate. Therefore,
use of sodium nitroprusside (Nipride) and alpha-adrenergic blocking agents may be required
during and after surgery.
- Exploration of other possible tumor sites is frequently undertaken to ensure removal of all
tumor tissue.
- As a result, the patient is subject to the stress and effects of a long surgical procedure, which
may increase the risk of hypertension postoperatively.
- Corticosteroid replacement is required if bilateral adrenalectomy has been necessary.
- Corticosteroids may also be required for the first few days or weeks after removal of a single
adrenal gland. IV administration of corticosteroids (methylprednisolone sodium succinate [Solu-
Medrol]) may begin on the evening before surgery and continue during the early postoperative
period to prevent adrenal insufficiency.
- Oral preparations of corticosteroids (prednisone) are prescribed after the acute stress of
surgery diminishes.
- Hypotension and hypoglycemia may occur in the post-operative period because of the sudden
withdrawal of excessive amounts of catecholamines. Therefore, careful attention is directed
toward monitoring and treating these changes.
- Blood pressure is expected to return to normal with treatment; however, one third of patients
continue to be hypertensive after surgery. This may result if not all pheochromocytoma tissue
was removed, if pheochromocytoma recurs, or if the blood vessels were damaged by severe and
prolonged hypertension. Several days after surgery, urine and plasma levels of catecholamines
and their metabolites are measured to determine whether the surgery was successful.

Nursing Management

- The patient who has undergone surgery to treat pheochromocytoma has experienced a stressful
preoperative and postoperative course and may remain fearful of repeated attacks.
- Although it is usually expected that all pheochromocytoma tissue has been removed, there is a
possibility that other sites were undetected and that attacks may recur.
- The patient is monitored for several days in the intensive care unit with special attention given
to ECG changes, arterial pressures, fluid and electrolyte balance, and blood glucose levels.
- Several IV lines are inserted for administration of fluids and medications.
Home and Community Based care

Teaching Patients Self-Care

- During the preoperative and postoperative phases of care, the nurse informs the patient about
the importance of follow-up monitoring to ensure that pheochromocytoma does not recur
undetected.
- After adrenalectomy, use of corticosteroids may be needed. Therefore, the nurse instructs the
patient about their purpose, the medication schedule, and the risks of skipping doses or
stopping their administration abruptly.
- It is important to teach the patient and family how to measure the patient’s blood pressure and
when to notify the physician about changes in blood pressure.
- In addition, the nurse provides verbal and written instructions about the procedure for
collecting 24-hour urine specimens to monitor urine catecholamine levels.

Continuing Care

- A follow-up visit from a home care nurse may be indicated to assess the patient’s postoperative
recovery, surgical incision, and compliance with the medication schedule. This may help
reinforce previous teaching about management and monitoring.
- The home care nurse also obtains blood pressure measurements and assists the patient in
preventing or dealing with problems that may result from long-term use of corticosteroids.
- Because of the risk of recurrence of hypertension, periodic checkups are required, especially in
young patients and in those whose families have a history of pheochromocytoma.
- The patient is scheduled for periodic follow-up appointments to observe for return of normal
blood pressure and plasma and urine levels of catecholamines.

ADRENAL INSUFFICIENCY (ADDISON’S DISEASE)

- Addison’s disease, or adrenocortical insufficiency, occurs when adrenal cortex function is

inadequate to meet the patient’s need for cortical hormones.
- Autoimmune or idiopathic atrophy of the adrenal glands is responsible for the vast majority of
cases.
- Other causes include surgical removal of both adrenal glands and infection of the adrenal
glands.
- Tuberculosis and histoplasmosis are the most common infections that destroy adrenal gland
tissue.
- Although autoimmune destruction has replaced tuberculosis as the principal cause of Addison’s
disease, tuberculosis should be considered in the diagnostic workup because of its increasing
incidence.
 - Inadequate secretion of ACTH from the pituitary gland also results in adrenal insufficiency
because of decreased stimulation of the adrenal cortex.
- Therapeutic use of corticosteroids is the most common cause of adrenocortical insufficiency
(Porth & Matfin, 2009).
- Symptoms of adrenocortical insufficiency may also result from the sudden cessation of
exogenous adrenocortical hormonal therapy, which suppresses the body’s normal response to
stress and interferes with normal feedback mechanisms.
- Treatment with daily administration of corticosteroids for 2 to 4 weeks may suppress function of
the adrenal cortex; therefore, adrenal insufficiency should be considered in any patient who has
been treated with corticosteroids.

Clinical Manifestations

- Addison’s disease is characterized by:

 muscle weakness;
 anorexia;
 GI symptoms;
 fatigue;
 emaciation;
 dark pigmentation of the mucous membranes and the skin, especially of the knuckles,
knees, and elbows;
 hypotension;
 low blood glucose, low serum sodium, and high serum potassium levels.
 Mental status changes such as depression, emotional lability, apathy, and confusion are
present in 60% to 80% of patients.
 In severe cases, the disturbance of sodium and potassium metabolism may be marked
by depletion of sodium and water and severe, chronic dehydration.
 With disease progression and acute hypotension, addisonian crisis develops.
 This condition is characterized by cyanosis and the classic signs of circulatory
shock: pallor, apprehension, rapid and weak pulse, rapid respirations, and low
blood pressure. In addition, the patient may complain of headache, nausea,
abdominal pain, and diarrhea and may show signs of confusion and restlessness.
Even slight overexertion, exposure to cold, acute infection, or a decrease in salt
intake may lead to circulatory collapse, shock, and death if untreated. The stress
of surgery or dehydration resulting from preparation for diagnostic tests or
surgery may precipitate an addisonian or hypotensive crisis

Assessment and Diagnostic Findings

- Although the clinical manifestations presented appear specific, the onset of Addison’s disease
usually occurs with nonspecific symptoms.
 - The diagnosis is confirmed by laboratory test results. Combined measurements of early-morning
serum cortisol and plasma ACTH are performed to differentiate primary adrenal insufficiency
from secondary adrenal insufficiency and from normal adrenal function.
- Patients with primary insufficiency have a greatly increased plasma ACTH level (more than 22.0
pmol/L) and a serum cortisol concentration lower than the normal range (less than 165 nmol/L)
or in the low-normal range.
- Other laboratory findings include decreased levels of blood glucose (hypoglycemia) and sodium
(hyponatremia), an increased serum potassium concentration (hyperkalemia), and an increased
white blood cell count (leukocytosis).
- The diagnosis is confirmed by low levels of adrenocortical hormones in the blood or urine and
decreased serum cortisol levels. If the adrenal cortex is destroyed, baseline values are low, and
ACTH administration fails to cause the normal increase in plasma cortisol and urinary 17-
hydroxycorticosteroids.
- If the adrenal gland is normal but not stimulated properly by the pituitary, a normal response to
repeated doses of exogenous ACTH is seen, but no response occurs after the administration of
metyrapone (Metopirone), which stimulates endogenous ACTH.

Medical Management

- Immediate treatment is directed toward combating circulatory shock:

o restoring blood circulation, administering fluids and corticosteroids, monitoring vital
signs, and placing the patient in a recumbent position with the legs elevated.
o Hydrocortisone (Solu-Cortef) is administered by IV, followed by 5% dextrose in normal
saline.
o Vasopressor amines may be required if hypotension persists.
o Antibiotics may be administered if infection has precipitated adrenal crisis in a patient
with chronic adrenal insufficiency. In addition, the patient is assessed closely to identify
other factors, stressors, or illnesses that led to the acute episode. Oral intake may be
initiated as soon as tolerated.
o IV fluids are gradually decreased after oral fluid intake is adequate to prevent
hypovolemia.
- If the adrenal gland does not regain function, the patient needs lifelong replacement of
corticosteroids and mineralocorticoids to prevent recurrence of adrenal insufficiency.
- During stressful procedures or significant illnesses, additional supplementary therapy with
glucocorticoids is required to prevent addisonian crisis.
- In addition, the patient may need to supplement dietary intake with added salt during
gastrointestinal losses of fluids through vomiting and diarrhea

Nursing Management

Assisting the Patient

- The health history and examination focus on the presence of symptoms of fluid imbalance and
on the patient’s level of stress.
 - The nurse should monitor the blood pressure and pulse rate as the patient moves from a lying,
sitting, and standing position to assess for inadequate fluid volume.
- A decrease in systolic pressure (20 mm Hg or more) may indicate depletion of fluid volume,
especially if accompanied by symptoms.
- The skin should be assessed for changes in color and turgor, which could indicate chronic
adrenal insufficiency and hypovolemia.
- The patient is assessed for change in weight, muscle weakness, fatigue, and any illness or stress
that may have precipitated the acute crisis.

Monitoring and Managing Addisonian Crisis

- The patient at risk is monitored for signs and symptoms indicative of addisonian crisis, which can
include:
 shock;
 hypotension;
 rapid, weak pulse;
 rapid respiratory rate;
 pallor;
 extreme weakness
- Physical and psychological stressors such as cold exposure, overexertion, infection, and
emotional distress should be avoided.
- The patient with addisonian crisis requires immediate treatment with IV administration of fluid,
glucose, and electrolytes, especially sodium; replacement of missing steroid hormones; and
vasopressors.
- The nurse anticipates and meets the patient’s needs to promote return to a pre-crisis state.

Restoring Fluid Balance

- The nurse encourages the patient to consume foods and fluids that assist in restoring and
maintaining fluid and electrolyte balance.
- Along with the dietitian, the nurse helps the patient select foods high in sodium during GI
disturbances and in very hot weather.
- The nurse instructs the patient and family to administer hormone replacement as prescribed
and to modify the dosage during illness and other stressful situations.
- Written and verbal instructions are provided about the administration of mineralocorticoid
(Florinef) or corticosteroid (prednisone) as prescribed.

Improving Activity Tolerance

- Until the patient’s condition is stabilized, the nurse takes precautions to avoid unnecessary
activity and stress that could precipitate another hypotensive episode.
- Efforts are made to detect signs of infection or the presence of other stressors.
- Explaining the rationale for minimizing stress during the acute crisis assists the patient to
increase activity gradually.

Promoting Home and Community-based Care

Teaching Patients Self-Care

 - Because of the need for lifelong replacement of adrenal cortex hormones to prevent addisonian
crises, the patient and family members receive explicit verbal and written instructions about the
rationale for replacement therapy and proper dosage.
- In addition, they are instructed about how to modify the medication dosage and increase salt
intake in times of illness, very hot weather, and other stressful situations.
- The patient also learns how to modify diet and fluid intake to help maintain fluid and electrolyte
balance.
- The patient and family are frequently prescribed preloaded, single-injection syringes of
corticosteroid for use in emergencies.
- Specific instructions about how and when to use the injection are also provided. It is important
to instruct the patient to inform other health care providers, such as dentists, about the use of
corticosteroids; to wear a medical alert bracelet; and to carry information at all times about the
need for corticosteroids.
- If the patient with Addison’s disease requires surgery, careful administration of fluids and
corticosteroids is necessary before, during, and after surgery to prevent addisonian crisis.
- The patient and family need to know the signs of excessive or insufficient hormone
replacement.
- The development of edema or weight gain may signify too high a dose of hormone; postural
hypotension and weight loss frequently signify too low a dose

Continuing Care

- Although most patients can return to their job and family responsibilities soon after hospital
discharge, others cannot do so because of concurrent illnesses or incomplete recovery from the
episode of adrenal insufficiency.
- In these circumstances, a referral for home care enables the home care nurse to assess the
patient’s recovery, monitor hormone replacement, and evaluate stress in the home. The nurse
assesses the patient’s and family’s knowledge about medication therapy and dietary
modifications.
- A home visit also allows the nurse to assess the patient’s plans for follow-up visits to the clinic
or physician’s office.
- The nurse reminds the patient and family about the importance of participating in health
promotion activities and health screening.

CUSHING’S SYNDROME

- Cushing’s syndrome results from excessive, rather than deficient, adrenocortical activity (Porth
& Matfin, 2009).
- Cushing’s syndrome is commonly caused by use of corticosteroid medications and is
infrequently the result of excessive corticosteroid production secondary to hyperplasia of the
adrenal cortex. However, overproduction of endogenous corticosteroids may be caused by
several mechanisms, including a tumor of the pituitary gland that produces ACTH and stimulates
the adrenal cortex to increase its hormone secretion despite production of adequate amounts.
 - Primary hyperplasia of the adrenal glands in the absence of a pituitary tumor is less common.
- Another less common cause of Cushing’s syndrome is the ectopic production of ACTH by
malignancies; bronchogenic carcinoma is the most common type.
- Regardless of the cause, the normal feedback mechanisms that control the function of the
adrenal cortex become ineffective, and the usual diurnal pattern of cortisol is lost.
- The signs and symptoms of Cushing’s syndrome are primarily a result of oversecretion of
glucocorticoids and androgens (sex hormones), although mineralocorticoid secretion also may
be affected (Porth & Matfin, 2009).

Clinical Manifestations

- When overproduction of the adrenal cortical hormone occurs, arrest of growth, obesity, and
musculoskeletal changes occur along with glucose intolerance.
- The classic picture of Cushing’s syndrome in the adult is that of central-type obesity, with a fatty
“buffalo hump” in the neck and supraclavicular areas, a heavy trunk, and relatively thin
extremities.
- The skin is thin, fragile, and easily traumatized; ecchymoses (bruises) and striae develop. The
patient complains of weakness and lassitude. Sleep is disturbed because of altered diurnal
secretion of cortisol.
- Excessive protein catabolism occurs, producing muscle wasting and osteoporosis.
- Kyphosis, backache, and compression fractures of the vertebrae may result.
- Retention of sodium and water occurs as a result of increased mineralocorticoid activity,
producing hypertension and heart failure.
- The patient develops a “moon-faced” appearance and may experience increased oiliness of the
skin and acne. There is increased susceptibility to infection.
- Hyperglycemia or overt diabetes may develop. The patient may also report weight gain, slow
healing of minor cuts, and bruises.
- Women between the ages of 20 and 40 years are five times more likely than men to develop
Cushing’s syndrome.
- In females of all ages, virilization may occur as a result of excess androgens. Virilization is
characterized by the appearance of masculine traits and the recession of feminine traits.
- There is an excessive growth of hair on the face (hirsutism), the breasts atrophy, menses cease,
the clitoris enlarges, and the voice deepens. Libido is lost in men and women.
- Changes occur in mood and mental activity, and psychosis may develop.
- Distress and depression are common and are increased by the severity of the physical changes
that occur with this syndrome.
- If Cushing’s syndrome is a consequence of pituitary tumor, visual disturbances may occur
because of pressure of the growing tumor on the optic chiasm.

Assessment and Diagnostic Findings

- An overnight dexamethasone suppression test is the most widely used and most sensitive
screening test for diagnosis of pituitary and adrenal causes of Cushing’s syndrome. It can be
performed on an outpatient basis. Dexamethasone (1 mg) is administered orally at 11 PM, and a
plasma cortisol level is obtained at 8 AM the next morning. Suppression of cortisol to less than 5
mg/dL indicates that the hypothalamic–pituitary–adrenal axis is functioning properly. Stress,
 obesity, depression, and medications such as antiseizure agents, estrogen (during pregnancy or
as oral medications), and rifampin (Rifadin) can falsely elevate cortisol levels. Nighttime salivary
cortisol levels show promise in screening for Cushing’s syndrome (Gross, Mindea, Pick, et al.,
2007).
- Indicators of Cushing’s syndrome include an increase in serum sodium and blood glucose levels
and a decrease in serum potassium, a reduction in the number of blood eosinophils, and
disappearance of lymphoid tissue.
- Measurements of plasma and urinary cortisol levels are obtained. Several blood samples may be
collected to determine whether the normal diurnal variation in plasma levels is present; this
variation is frequently absent in adrenal dysfunction. If several blood samples are required, they
must be collected at the times specified, and the time of collection must be noted on the
requisition slip.
- Other diagnostic studies include a 24-hour urinary free cortisol level and a low-dose
dexamethasone suppression test. Low-dose suppression tests are similar to the overnight test
but vary in dosage and timing.
- Measurement of plasma ACTH by radioimmunoassay is used in conjunction with the high-dose
suppression test to distinguish pituitary tumors from ectopic sites of ACTH production as the
cause of Cushing’s syndrome.
- Elevation of both ACTH and cortisol indicates pituitary or hypothalamic disease.
- A low ACTH with a high cortisol level indicates adrenal disease. CT, ultrasound, or MRI may be
performed to localize adrenal tissue and detect tumors of the adrenal gland.

Medical Management

- If Cushing’s syndrome is caused by pituitary tumors rather than tumors of the adrenal cortex,
treatment is directed at the pituitary gland.
- Surgical removal of the tumor by transsphenoidal hypophysectomy (see Chapter 61) is the
treatment of choice and has an 80% success rate.
- Radiation of the pituitary gland also has been successful, although it may take several months
for control of symptoms.
- Adrenalectomy is the treatment of choice in patients with primary adrenal hypertrophy.
- Postoperatively, symptoms of adrenal insufficiency may begin to appear 12 to 48 hours after
surgery because of reduction of the high levels of circulating adrenal hormones.
- Temporary replacement therapy with hydrocortisone may be necessary for several months,
until the adrenal glands begin to respond normally to the body’s needs.
- If both adrenal glands have been removed (bilateral adrenalectomy), lifetime replacement of
adrenal cortex hormones is necessary.
- Adrenal enzyme inhibitors (eg, metyrapone [Metopirone], aminoglutethimide [Cytadren],
mitotane [Lysodren], and ketoconazole [Nizoral]) may be used to reduce hyperadrenalism if the
syndrome is caused by ectopic ACTH secretion by a tumor that cannot be eradicated.
- Close monitoring is necessary, because symptoms of inadequate adrenal function may result
and side effects of the medications may occur.
- If Cushing’s syndrome is a result of the administration of corticosteroids, an attempt is made to
reduce or taper the medication to the minimum dosage needed to treat the underlying disease
process (eg, autoimmune or allergic disease, rejection of a transplanted organ).
 - Frequently, alternate-day therapy decreases the symptoms of Cushing’s syndrome and allows
recovery of the adrenal glands’ responsiveness to ACTH.

THE NURSING PROCESS: PATIENT WITH CUSHING’S SYNDROME

Assessment

- The health history and examination focus on the effects on the body of high concentrations of
adrenal cortex hormones and on the inability of the adrenal cortex to respond to changes in
cortisol and aldosterone levels.
- The history includes information about the patient’s level of activity and ability to carry out
routine and self-care activities.
- The skin is observed and assessed for trauma, infection, breakdown, bruising, and edema.
Changes in physical appearance are noted, and the patient’s responses to these changes are
elicited.
- The nurse assesses the patient’s mental function, including mood, responses to questions,
awareness of environment, and level of depression.
- The family is often a good source of information about gradual changes in the patient’s physical
appearance as well as emotional status.

Diagnosis

Nursing Diagnosis

- Based on all the assessment data, the major nursing diagnoses of the patient with Cushing’s
syndrome include the following:

• Risk for injury related to weakness

• Risk for infection related to altered protein metabolism and inflammatory response

• Self-care deficit related to weakness, fatigue, muscle wasting, and altered sleep patterns

• Impaired skin integrity related to edema, impaired healing, and thin and fragile skin

• Disturbed body image related to altered physical appearance, impaired sexual functioning, and
decreased activity level

• Disturbed thought processes related to mood swings, irritability, and depression

Collaborative Problems/Potential Complications

- Potential complications may include the following:

• Addisonian crisis
• Adverse effects of adrenocortical activity

Planning Goals
- The major goals for the patient include decreased risk of injury, decreased risk of infection, increased
ability to carry out self-care activities, improved skin integrity, improved body image, improved mental
function, and absence of complications.

Nursing Interventions

Decreasing Risk of Injury

- Establishing a protective environment helps prevent falls, fractures, and other injuries to bones
and soft tissues.
- The patient who is very weak may require assistance from the nurse in ambulating to avoid
falling or bumping into sharp corners of furniture.
- Foods high in protein, calcium, and vitamin D are recommended to minimize muscle wasting
and osteoporosis.
- Referral to a dietitian may assist the patient in selecting appropriate foods that are also low in
sodium and calories.

Decreasing Risk of Infection

- The patient should avoid unnecessary exposure to others with infections.

- The nurse frequently assesses the patient for subtle signs of infection, because the anti-
inflammatory effects of corticosteroids may mask the common signs of inflammation and
infection.

Preparing the Patient for Surgery

- The patient is prepared for adrenalectomy, if indicated, and the postoperative course.
- If Cushing’s syndrome is a result of a pituitary tumor, a transsphenoidal hypophysectomy may
be performed.
- Diabetes mellitus and peptic ulcer are common in patients with Cushing’s syndrome. Therefore,
insulin therapy and medication to treat peptic ulcer are initiated if needed.
- Before, during, and after surgery, blood glucose monitoring and assessment of stools for blood
are carried out to monitor for these complications.
- If the patient has other symptoms of Cushing’s syndrome, these are considered in the
preoperative preparation.
- For example, if the patient has experienced weight gain, special instruction is given about
postoperative breathing exercises.

Encouraging Rest and Activity

- Although the patient with Cushing’s syndrome experiences insomnia, weakness, fatigue, and
muscle wasting, the nurse should encourage moderate activity to prevent complications of
immobility and promote increased self-esteem.
- It is important to help the patient plan and space rest periods throughout the day and promote
a relaxing, quiet environment for rest and sleep.
Promoting Skin Integrity

- Meticulous skin care is necessary to avoid traumatizing the patient’s fragile skin.
- Use of adhesive tape is avoided, because it can irritate the skin and tear the fragile tissue when
the tape is removed.
- The nurse frequently assesses the skin and bony prominences and encourages and assists the
patient to change positions frequently to prevent skin breakdown.

Improving Body Image

- If treated successfully, the major physical changes associated with Cushing’s syndrome
disappear in time.
- The patient may benefit from discussion of the effect the changes have had on his or her self-
concept and relationships with others.
- Weight gain and edema may be modified by a low-carbohydrate, low-sodium diet, and a high
protein intake may reduce some of the other bothersome symptoms.

Improving Thought Processes

- Explanations to the patient and family members about the cause of emotional instability are
important in helping them cope with the mood swings, irritability, and depression that may
occur.
- Psychotic behavior may occur in a few patients and should be reported. The nurse encourages
the patient and family members to verbalize their feelings and concerns.

Monitoring and Managing Potential Complications

- ADDISONIAN CRISIS. The patient with Cushing’s syndrome whose symptoms are treated by
withdrawal of corticosteroids, by adrenalectomy, or by removal of a pituitary tumor is at risk for
adrenal hypofunction and addisonian crisis.
- If high levels of circulating adrenal hormones have suppressed the function of the adrenal
cortex, atrophy of the adrenal cortex is likely.
- If the circulating hormone level is decreased rapidly because of surgery or abrupt cessation of
corticosteroid agents, manifestations of adrenal hypofunction and addisonian crisis may
develop. Therefore, the patient with Cushing’s syndrome should be assessed for signs and
symptoms of addisonian crisis as previously discussed. If addisonian crisis occurs, the patient is
treated for circulatory collapse and shock.
- ADVERSE EFFECTS OF ADRENOCORTICAL ACTIVITY. The nurse assesses fluid and electrolyte
status by monitoring laboratory values and daily weights.
- Because of the increased risk of glucose intolerance and hyperglycemia, blood glucose
monitoring is initiated.
- The nurse reports elevated blood glucose levels to the physician so that treatment can be
prescribed if indicated.

Promoting Home and Community-Based Care

 - TEACHING PATIENTS SELF-CARE. The patient and family should be informed that acute adrenal
insufficiency and underlying symptoms will recur if corticosteroid therapy is stopped abruptly
without medical supervision.
- The patient should be instructed to always have an adequate supply of the corticosteroid
medication to avoid running out
- The nurse stresses the need for dietary modifications to ensure adequate calcium intake
without increasing the risks for hypertension, hyperglycemia, and weight gain.
- The patient and family can be taught to monitor blood pressure, blood glucose levels, and
weight. Patients should be advised to wear a medical alert bracelet and to notify other health
care providers (eg, dentist) about their condition.
- CONTINUING CARE. The need for follow-up depends on the origin and duration of the disease
and its management.
- The patient who has been treated by adrenalectomy or removal of a pituitary tumor requires
close monitoring to ensure that adrenal function has returned to normal and to ensure
adequacy of circulating adrenal hormones.
- The patient who requires continued corticosteroid therapy is monitored to ensure
understanding of the medications and the need for a dosage that treats the underlying disorder
while minimizing the side effects.
- Home care referral may be indicated to ensure a safe environment that minimizes stress and
risk of falls and other side effects.
- The home care nurse assesses the patient’s physical and psychological status and reports
changes to the physician.
- The nurse also assesses the patient’s understanding of the medication regimen and his or her
compliance with the regimen and reinforces previous teaching about the medications and the
importance of taking them as prescribed.
- The nurse emphasizes the importance of regular medical follow-up, the side effects and toxic
effects of medications, and the need to wear medical identification with Addison’s and Cushing’s
disease.
- In addition, the nurse reminds the patient and family about the importance of health promotion
activities and recommended health screening, including bone mineral density testing.

Evaluation

Expected Patient Outcomes

- Expected patient outcomes may include the following:

1. Decreases risk of injury
a. Is free of fractures or soft tissue injuries
b. Is free of ecchymotic areas

2. Decreases risk of infection

a. Experiences no temperature elevation, redness, pain, or other signs of infection or
inflammation
b. Avoids contact with others who have infections

3. Increases participation in self-care activities

a. Plans activities and exercises to allow alternating periods of rest and activity
b. Reports improved well-being
c. Is free of complications of immobility

4. Attains/maintains skin integrity

a. Has intact skin, without evidence of breakdown or infection
b. Exhibits decreased edema in extremities and trunk
c. Changes position frequently and inspects bony prominences daily

5. Achieves improved body image

a. Verbalizes feelings about changes in appearance, sexual function, and activity level
b. States that physical changes are a result of excessive corticosteroids

6. Exhibits improved mental functioning

7. Exhibits absence of complications

a. Exhibits normal vital signs and weight and is free of symptoms of addisonian crisis
b. Identifies signs and symptoms of adrenocortical hypofunction that should be reported and
measures to take in case of severe illness and stress
c. Identifies strategies to minimize complications of Cushing’s syndrome
d. Complies with recommendations for follow-up appointments and health screening