UNIVERSITY OF THE ASSUMPTION City of San Fernando, Pampanga College of Nursing

Submitted by: Group C-7 Calma, Jeanne Margot Canlas, Mylene Daag, Claire Dionisio, Sarah Kay Mallari, Emmy Manlapat, Jim Bryan Santos, Junben Submitted to: Mrs. Gloria Vergara, R.N., M.A.N.

09 October 2006 INTRODUCTION

The endocrine system is a major controlling system of the body. It is often considered one of the most complex systems in the human body. Through hormones, it stimulates such long term processes as growth and development, metabolism, reproduction and body defense. Endocrine disorders can alter a patients health and self-image. These disorders may affect the patients growth and development, reproductive system, energy level, metabolic rate or ability to adapt to stress. Nurses have a major role in the care of clients with endocrine disorder. Therefore having sufficient knowledge and familiarity with Addisons disease and other endocrine disorders, one can have a lasting positive effect on patients with these disorders by understanding the pathophysiology of the condition, assessing patients accurately and selecting and implementing appropriate nursing dianoses. After doing a comprehensive study of the disorder the group aims to:

be familiar with the pathophysiology of Addisons disease be able to assess the clients with Adrenal Hypofunction correctly be competent in identifying appropriate nursing diagnoses be able to perform planning and implementation of appropriate be able to comprehend proper medical management and treatment of

nursing management

the disoder

ANATOMY AND PHYSIOLOGY Adrenal Glands The adrenal glands are small vital endocrine structures that rest upon the upper end of each kidney. Each adrenal gland is composed of an inner core, the medulla and an outer shell, the cortex.

The

inner

portion,

the

medulla

produces

the

catecholamines

epinephrine and norepinephrine. The much larger outer adrenal portion, the cortex has three zones. the middle and largest The outermost zone, the zona glomerulosa, The zona fasciculata, glucocorticoids cortisol zone, produces the produces mineralocorticoids, primarily aldosterone.

(hydrocortisone), cortisone, and corticosterone as well as small amounts of sex hormones androgen and estrogen. The inner zone, the zona reticularis, produces mainly glucocorticoids and some sex hormones.

The three (3) classes of steroid hormones produced by the adrenal cortex are all synthesized from cholesterol. cortex by the letter S, as salt, sugar, and sex. Mineralocorticoids (primarily aldosterone) regulate electrolyte balance by promoting sodium retention and potassium excretion. These physiologic activities, in turn, help sustain normal BP and cardiac output. Cortisol is the major glucocorticoid that has effects on glucose (increases blood glucose level) and protein (tissue wasting) metabolism, on fluid and electrolyte balance (increases sodium retention and potassium excretion), in inflammation and immunity (suppresses the inflammatory These could be easily remembered by representing the 3 functions of the hormones of the adrenal

response to tissue injury and the protective immune response to invasion by infectious agents), and on stress (decreases resistance to stress). Because cortisol is so vital to health, the amount of cortisol produced by the adrenals is precisely balanced. Like many other hormones, cortisol is regulated by the brain's hypothalamus and the pituitary gland. First, the hypothalamus sends "releasing hormones" to the pituitary gland. The pituitary responds by secreting other hormones that regulate growth, thyroid and adrenal function, and sex hormones such as estrogen and testosterone. One of the pituitary's main functions is to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals receive the pituitary's signal in the form of ACTH, they respond

by producing cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.

ADDISONS DISEASE Alternative names Adrenal Insufficiency Adrenocortical Hypofunction Hypocortisolism Primary Adrenocortical Insufficiency Chronic Adrenal Insufficiency Chronic Primary Adrenal Insufficiency Overview Adrenocortical insufficiency occurs when the adrenal cortex secretes inadequate amounts of adrenocortical hormones, primarily glucorticoids and mineralocorticoids. The disorder occurs in two (2) forms. Primary adrenocortical insufficiency (Addisons disease) results from destruction and subsequent hypofunction of the adrenal cortex, usually caused by an autoimmune process. Secondary adrenocortical insufficiency occurs because of adrenocorticotropic hormone (ACTH) deficiency from pituitary disease or suppression of the hypothalamic-pituitary axis by corticosteroids administered to treat non endocrine disorders, which causes the adrenal cortex to atrophy. Either primary or secondary Addisons disease can progress into adrenal crisis, also called addisonian crisis, wherein there is critical deficiency of mineralocorticoids and glucocorticoids.

Addison's disease is a rare endocrine, or hormonal disorder that affects about 1 in 100,000 people. It occurs in all age groups and afflicts men and women equally. The disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and nonexposed parts of the body. Also called adrenal insufficiency or hypocortisolism, Addison's disease can occur at any age, but is most common in people ages 30 to 50. Addison's disease occurs when the adrenal glands do not produce enough of the hormone cortisol and in some cases, the hormone aldosterone. For this reason, the disease is sometimes called chronic adrenal insufficiency, or hypocortisolism. Definition Addison's disease is a severe or total deficiency of the hormones made in the adrenal cortex, caused by a destruction of the adrenal cortex.

Etiology and Risk Factors Eighty to ninety percent of cases of Addison's disease are said to be due to autoantibodies directed against adrenal cells (autoimmune) containing 21-hydroxylase, an enzyme involved in the production of cortisol and aldosterone. Idiopathic atrophy of the adrenal glands is also said to be responsible for the cases of adrenocortical insufficiency. The remainder of cases are due to tuberculosis, HIV, sarcoidosis, amyloidosis, hemochromatosis, metastatic cancer to the adrenal glands, adrenal hemorrhage, Waterhouse-Friderichsen syndrome (massive, usually

bilateral,

hemorrhage

into

the

adrenal

glands

caused

by

fulminant

meningococcemia,), congenital adrenal hyperplasia and use of bloodthinning drugs (anticoagulants). Therapeutic use of corticosteroids is the most common cause of adrenocortical insufficiency. Symptoms may also result from the sudden cessation of exogenous adrenocortical hormonal therapy, which suppresses the bodys normal response to stress and interferes with normal feedback mechanisms. Risk factors for the autoimmune type of Addison's disease include other Chronic autoimmune thyroiditis, diseases: Type I diabetes, Hypoparathyroidism, Vitiligo and Hypopituitarism, Pernicious anemia, Testicular dysfunction, Graves' disease, Candidiasis, Dermatis herpetiformis, Myasthenia gravis. Clinical Manifestations

The onset of Addison's disease is usually insidious. The client experiences mild fatigue, languor, irritability, weight loss, nausea/vomiting, and postural hypotension weeks or months before diagnosis of the disease. As the disorder progresses, manifestations intensify. over 90% of both adrenal cortices. HORMONE Aldosterone FUNCTION ADDISONS DISEASE Promotes retention of More than 90% of sodium (and water) in adrenal gland is kidney destroyed before the clinical picture of adrenal insufficiency emerges. Increased secretion of sodium and water influences The development of clinical manifestations of adrenocortical insufficiency requires the loss of

Cortisol

Promotes gluconeogenesis Maintains glucose level plasma

Promotes appetite

Causes release of epinephrine from adrenal medulla

ACTH

Androgens

Assists in adaptation to stress by increased gluconeogenesis releasing an anti inflammatory response; augmenting release of catecholamines to increase BP regulates melanocyte Stimulates an increase stimulating hormone MSH: increases skin and mucous membrane pigmentation, especially fingers, toes and sun exposed body parts (skin appears bronzed) Female oligomenorrhea or amenorrhea, decrease in body hair Male No manifestations in males because testes produce adequate quantities of sex

dehydration, hyponatremia, orthostatic hypotension, decreased urine output, decreased cardiac output, weight loss, salt craving, acidosis, circulatory collapse and shock Decreased gluconeogenesis causes depleted liver glycogen stores manifested by hypoglycemia, weakness, fatigue anorexia, weight loss, vomiting, mental confusion, emotional disturbances (mild neurosis to depression) Inadequate release of epinephrine produces hypoglycemia and hypotension Lowers resistance to stress and produces a hyperresponse to stressors: hypoglycemia, hypotension, hyperthermia

hormones In some cases, Addison's symptoms may present rapidly. This "acute adrenal failure" is known as an Addisonian crisis (an exaggerated state of adrenal cortical insufficiency). An illness or accident can aggravate the adrenal problems causing the Addisonian crisis, although the most common cause is abrupt discontinuation of corticosteroid therapy without tapering the dose. It may be provoked by physical stress, such as an injury, infection or illness. An addisonian crisis is a life-threatening situation that results in low blood pressure, low blood levels of sugar and high blood levels of potassium. This situation requires immediate medical care and can be fatal if not treated quickly. Diagnostic Tests Diagnosis of Addison's disease depends primarily on blood and urine hormonal assays. Primary adrenal insufficiency is characterized by a low cortisol production rate and a high plasma ACTH concentration. Secondary adrenal insufficiency is characterized by a low cortisol production rate and a low plasma ACTH concentration. Other diagnostic tests may be ordered to evaluate the effects of hypofunction of the adrenals on the body: serum electrolytes (especially hyponatremia and hyperkalemia in primary adrenal insufficiency; hyponatremia alone in secondary disease); blood glucose; complete blood count (to assess for anemia); x-rays or computed tomography (CT) or magnetic resonance imaging (MRI) of the adrenals and pituitary. TEST PURPOSE NURSING CONSIDERATON INTERPRETATIO N

S Cortisol plasma Evaluates Plasma level function of cortisol level adrenal cortex have diurnal effect: levels higher in AM than PM Fasting prephlebotomy 2 hours of supine activity are necessary before test because activity increases cortisol level 17Measures 24-hour urine Hydroxysteroids metabolites of collection to be (Porter Silber glucocorticoids kept on ice reaction) and aldosterone CT or MRI of Identifies and No preparation adrenals locates adrenal except client glands education Cosyntropin test Diagnoses adrenal insufficiency with ACTH stimulation Give ACTH IV then 45 min later obtain serum cortisol Give cosyntropin IV then obtain plasma cortisol and aldosterone level at baseline and after 30 mins 24-hour urine test Keep collection cold

= disease

Addison

= disease

Addison

Rapid ACTH stimulation

Small, atrophied glands = autoimmune adrenal insufficiency = dysfunction of hypothalamicpituitary axis, secondary adrenal insufficiency cortisol and aldosterone = primary adrenal insufficiency = hypofunction of adrenal, or in clients with removal of testes or ovaries Addison

17-ketosteroids

Aldosterone

Measures steroid metabolites from adrenal cortex and testes (does not indicate testosterone) Measures Client mineralocorticoid supine

to be = 2 hours disease

production Renin Measures renin (enzyme produced in response to decreased blood flow to kidneys)

Plasma ACTH or Tests anterior serum pituitary function corticotropin as it may cause Addisons disease

before phlebotomy Client in supine position Results high in AM Note sodium intake in diet Fasting sample Stress may artificially increase result

= HPN, upright position with phlebotomy = high sodium diet = Addisons disease caused by adrenal gland failure, surgical removal of adrenals, adrenal suppression with long term exogenous steroid supply = secondary adrenal insufficiency caused by hypopituitarism = primary adrenal insufficiency = secondary asrena insufficiencyt

Corticotropin releasing hormone (CRH) stimulation

Measures CRH form hypothalamus to pituitary necessary to stimulate ACTH and cortisol release

Give CRH IV then measure ACTH and cortisol at baseline and after 15, 30, and 60 min

Laboratory findings Laboratory test results show:

Decreased blood glucose (hypoglycemia) Decreased serum sodium levels (hyponatremia) Increased serum potassium levels (hyperkalemia) Increased WBC (leukocytosis) & Elevated hematocrit on CBC

 Increased blood nitrogen (BUN) levels Low aldosterone levels Decreased cortisol levels in plasma & high plasma ACTH concentration

(Primary)
Low

cortisol production rate and low plasma ACTH concentration

(Secondary)
24-hour urine studies shows decreased levels of 17-ketosteroids, 17-

hydoxycorticoids and 17-ketogenic steroids Medical Management and Treatment Immediate treatment is directed toward combating circulatory shock: restoring elevated. blood circulation, administering fluids and corticosteroids, monitoring vital signs and placing patient in a recumbent position with legs Hydrocortisone is administered intravenously, followed with 5% dextrose in normal saline. Oral intake may be initiated as soon as tolerated. prevent hypovolemia. Adrenal crisis requires prompt administration of dexamethasone, hydrocortisone, or both. Later doses of hydrocortisone are given I.V. until the patients condition stabilizes. With proper treatment, the crisis usually subsides quickly, blood pressure stabilizes, and fluid and sodium levels return to normal. Subsequent oral maintenance doses of hydrocortisone preserve stability. Antibiotics may be administered if infection has precipitated adrenal crisis in a patient with chronic adrenal insufficiency. Gradually,

intravenous fluids are decreased when oral fluid intake is adequate to

Corticosteroid replacement, usually with cortisone or hydrocortisone (both also have mineralocorticoid effect), is the primary lifelong treatment for patients with primary or secondary adrenal hypofunction. Additionally, the patient may need to supplement dietary intake with added salt during times of gastrointesintinal losses of fluids through vomiting and diarrhea. Nursing Management The three major goals of intervention are the following:
Reversal of shock Restoration of blood circulation (the client usually suffers from a deficit of

at least 20% of extracellular fluid volume)

Replenishment with essential steroids

Nursing Interventions
Monitor vital signs carefully for hypotension, volume depletion, and other

signs of shock (decreased LOC and urine output).

Watch

for

hyperkalemia

before

treatment

and

hypokalemia

after

treatment from excessive mineralocorticoid effect.

If the patient also has diabetes, check blood glucose levels periodically

because steroid replacement may necessitate changing the insulin dosage.

Carefully record weight and intake and output, because the patient may

have volume depletion.

Force

fluids

to

replace

excessive

fluid

loss

until

the

onset

of

mineralocorticoid effects.

 Arrange for a diet that maintains sodium and potassium balance. If the

patient is anorectic, suggest six small meals per day to increase calorie intake. Ask the dietician to provide a diet high in protein and carbohydrates.
Observe the patient receiving steroids for cushingoid signs such as fluid

retention around the eyes and face.

Watch for fluid and electrolyte imbalance, especially if the patient

receives mineralocorticoid.
Monitor for manifestations of addisonian crisis:

Sudden profound weakness Severe abdominal, back, and leg pain Hyperpyrexia (although this may be suppressed by Steroids) followed by hypothermia Hypotension in association with high cardiac output, normal wedge pressure, and low systemic resistance Coma Renal shutdown and death Emergency Situation
Monitor blood pressure Administer IV infusion and medications. Monitor hourly urine output and report oliguria (a manifestation of shock). Minimize exposure to emotional and physical stress. Observe for manifestations of glucocorticoid overdose and overhydration,

such as generalized edema due to fluid retention, hypertension, flaccid paralysis resulting from hypokalemia, psychosis, and loss of consciousness.

 Evaluate electrolytes for hyperkalemia, hyponatremia, and hypoglycemia

and correlate these findings with client manifestations Teaching Tips

Explain the lifelong cortisone replacement therapy is necessary. Advise the patient of symptoms of overdose and underdose, and that hell

need to increase the dosage during times of stress. ( when he has a cold, for example)
Warn that infection, injury, or profuse sweating in hot weather precipitate

crisis.
Instruct the patient to always carry a medical identification card and wear

a bracelet stating the name and dosage of the steroids he takes.

Teach the patient how to give himself a hydrocortisone injection Tell patient to keep an emergency kit containing hydrocortisone in a

prepared syringe for use in times of stress.

Warn that he may need additional cortisone to prevent a crisis after any

type of stress. Note:

Adrenal insufficiency is a potentially life-threatening condition, but when

properly recognized and treated, it has little or no effect on life span.

There are no dietary or activity restrictions.

Evaluation As a result of successful therapy for adrenal hypofunction, the patient will:
maintain a proper diet maintain normal serum sodium, potassium, and plasma cortisol levels

 understand the need to take his medications routinely Make necessary adjustments in times of stress

REFERENCES Handbook of Diseases 2nd edition (Springhouse) Illustrated Manual of Nursing Practice 3rd edition (Springhouse) Lippincotts Pocket Manual of Nursing Practice (Nettina) Medical Surgical Nursing 10th edition (Smeltzer & Bare) Medical Surgical Nursing: Concept and Clinical Application Medical Surgical Nursing Made Incredibly Easy (Lippinocott Williams & Wilkins) Mosbys Diagnostic and Laboratory Test Reference 2nd edition (Pagana) Pathophysiology Made Incredibly Easy 3rd edition (Lippinocott Williams & Wilkins)