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Addisons Disease 1

Addisons Disease
Kelsey LeVan
Nursing 101
March 14, 2013
Mrs. Toothaker
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Introduction
In 1855, Thomas Addison described Addisons disease in his classic paper; On the
Constitutional and Local Effects of Disease of the Supra-Renal Capsules, 158 years later,
Addisons disease is still prevalent in the United States (Griffing, 2012). Historically, former US
president John F. Kennedy battled with this disease throughout his presidency. While on a visit
to London in the fall of 1947, Kennedy became so severely ill with weakness, nausea, vomiting,
and low blood pressure that he was given the last rites of the Roman Catholic Church (Griffing,
2012). The physician who examined him diagnosed Kennedy with Addisons disease and told
his friends that he hasnt got a year to live (Gilbert, 1992, pg. 24). This might explain
Kennedys illness during his first campaign for the House of Representatives, where he collapsed
during the final campaign event, a parade in Charlestown, sweating heavily and his skin
discolored (Gilbert, 1992).
Statistically, the incidence of Addisons disease is 40-60 cases per a 1 million population
(Griffing, 2012). This disease is more common in females and children, and the most common
age at presentation in adults is 30-50 years, however, the disease could present itself earlier in
patients with polyglandular autoimmune syndromes, congenital adrenal hyperplasia (CAH) or a
disorder of long-chain fatty acid metabolism. This disease indiscrimately targets all races.
Although the prognosis of Addisons disease is satisfactory and most can lead normal lives
with exogenous cortisol replacement therapy; however, an Addisonian crisis is a potentially life
threatening complication of this disease. With that being said, it is essential for health care
providers to understand Addisons disease and its clinical presentation. The etiology,
pathophysiology, clinical presentation, diagnostic criteria, treatment, and patient education will
be discussed.
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Assessment
Addisons disease is a disorder resulting from destruction or dysfunction of the adrenal
cortex (LeMone & Burke, 2008). The adrenal cortex is the outer portion of the adrenal gland
that sits on top of each kidney. The adrenal cortex produces glucocorticocids, which regulate
carbohydrate and fat metabolism, mineralocorticoids which regulate salt and water balance in the
body, and also androgens (Crawford & Harris, 2012). Due to the destruction or dysfunction of
the adrenal cortex, there is a deficiency or absence of the cortical hormones (MedicineNet,
2011). These adrenal insufficiencies are divided into two categories, primary or secondary
(LeMone & Burke, 2008).
Primary insufficiencies are due to adrenal gland destruction and a lack of adrenal
hormones. Addisons disease is the most common form of primary insufficiency, and is caused
by an autoimmune response that leads to the destruction of the adrenal cortex. Additionally,
these insufficiencies may be caused by a bilateral adrenalectomy, infections such as tuberculosis
and septicemia, neoplasms, trauma, or a hemorrhage of the adrenal glands (LeMone & Burke,
2008). Causes of secondary insufficiency in Addisons disease includes hypopituitarism
(decreased ACTH), surgical removal of ectopic ACTH-secreting tumors (oat cell carcinoma of
the lungs), and abrupt withdrawal of long-standing corticosteroid drug therapy (LeMone &
Burke, 2008).
The onset of Addisons disease is insidious in most cases; the patient experiences
manifestations after about 90% of the function of the gland is lost (Griffing, 2012). The primary
clinical manifestations arise from increased ACTH, and decreased aldosterone and cortisol
(LeMone, Burke, & Bauldoff, 2010). Aldosterone deficiency affects the ability of the distal
tubules of the nephron to conserve sodium and excrete potassium, therefore sodium is lost,
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potassium is retained, extracellular fluid is depleted, and the blood volume is decreased. Postural
hypotension and syncope are common and hypovolemic shock may occur. Hyponatremia causes
dizziness, confusion, and neuromuscular irritability, and hyperkalemia can cause cardiac
dysrhythmias. Cortisol insufficiency also causes decreased hepatic glycogenesis with
hypoglycemia. The patient tolerates stress poorly and experiences lethargy, weakness, anorexia,
nausea, vomiting, and diarrhea. The increased levels stimulate hyperpigmentation in more than
90% of patients with Addisons disease. In Caucasian patients, the skin looks deeply tanned or
bronzed in color due to hyperpigmentation from increased ACTH (LeMone, Burke, & Bauldoff,
2010).
There are many probable risk factors for getting Addisons disease. The risk factors
include other autoimmune diseases such as an autoimmune destruction of the adrenals, chronic
thyroiditis, dermatis herpetiformis, Graves disease, hypoparathyroidism, hypopituitraism,
myasthenia gravis, pernicious anemia, testicular dysfunction, type one diabetes and vitiligo.
Other risk factors for acquiring Addisons disease include taking anticoagulants, AIDS,
tuberculosis, adrenoleukodystrophy, sepsis, ACTH deficit, and an abrupt withdrawal from taking
long term high dose steroids (LeMone, Burke, & Bauldoff, 2010).
Clinical manifestations of Addisons disease include changes in the integumentary,
cardiovascular, musculoskeletal, gastrointestinal, reproductive, and central nervous systems. In
addition there are metabolic effects such as hyperkalemia, hyponatremia, and hypoglycemia.
Manifestations of the integumentary system include delayed wound healing and
hyperpigmentation. Cardiovascular issues include postural hypotension, arrthymias such as
tachycardia. In addition, central nervous system changes can include lethargy, tremors,
emotional liability and confusion. Manifestations of the musculoskeletal system includes
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weakness, muscle wasting, joint pain, and muscle pain. Gastrointestinal system issues include
anorexia, nausea, vomiting, and diarrhea. Reproductive effects include menstrual changes
(LeMone, Burke, & Bauldoff, 2010).
An Addisonian crisis is a serious, life- threatening response to an acute adrenal
insufficiency. The triggers include surgery, acute systemic illness, sepsis, trauma, or abrupt
withdrawal from long term corticosteroid and glucocorticoid therapy. The patient with
Addisonian crisis may have a fever, severe vomiting and diarrhea, pain in the abdomen, lower
back and legs, severe hypotension, circulatory collapse, shock, and even coma (LeMone &
Burke, 2008). In a patient with Addisons disease extra exogenous cortisol must be given during
times of extreme stress such as a traumatic injury to prevent the Addisonian crisis. During times
of stress, the body demands higher levels of cortisol to appropriately deal with the stressor.
Insufficient cortisol during times of extreme stress can be deadly (Griffing, 2012).
Diagnostic procedures for Addisons disease includes a history, physical examination,
serum hormone levels (cortisol, ACTH, and androgens) levels, serum glucose levels, CBC, blood
chemistry, adrenal and pituitary CT and MRI, and a biopsy (Story, 2011). 24 hour urine test and
an ACTH stimulation test may also be included to evaluate for decreased cortisol levels. Serum
hormone levels will show plasma cortisol and aldosterone levels that are decreased in primary or
secondary forms, and a serum ACTH level that is increased in primary and decreased in
secondary. Serum electrolytes (done by blood chemistry) will show decreased sodium and
glucose, an increased potassium level, and possibly increased calcium. Signs of dehydration
may show an increased hematocrit and BUN (LeMone, Burke, & Bauldoff, 2010).
The primary treatment of Addisons disease is replacement of corticosteroids and
mineralcorticoids accompanied by increased sodium in the diet. Hydrocortisone (Cortef) is
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given orally to replace cortisol, meanwhile, fludrocortisones (Florinef) is given orally to replace
mineralocorticoids (LeMone, Burke, & Bauldoff, 2010).
Patient Teaching
Important information that needs to be taught to the patient, spouse, family, or primary
caregiver includes how to administer steroids and the importance to not abruptly discontinue the
medication (LeMone, Burke, & Bauldoff, 2010). Additionally, the nurse should emphasize the
importance of wearing a Medic Alert bracelet and carrying at all times an emergency kit for
administering 100 mg of parenteral cortisone during times of extreme stress such as trauma. It is
also beneficial for a steroid therapy card containing personal information on a persons health
condition to be with caregivers as well as the client in an emergency situation (Crawford &
Harris, 2 (Story, 2011)012). Furthermore, the aspect of exercising regularly and maintaining a
generous fluid intake and a diet high in sodium and protein and low in potassium should be
stressed (LeMone, Burke, & Bauldoff, 2010).
It is also important to teach the client to weigh themselves each day at the same time and
to report any consistent weight gain which indicates fluid retention. If the client is a woman and
is of childbearing age, they should be taught that corticosteroids may impair the effectiveness of
oral contraceptives. Woman of child bearing age should also be advised that undiagnosed and
diagnosed Addisons disease both entail increased risks of unfavorable pregnancy outcomes.
Addisons disease also influences the number of childbirths (Bjornsdottir, Cnattingius, Brandt,
Nordenstrom, & Eckborn, 2010). Clinically undiagnosed and diagnosed AAD both entail
increased risks of unfavorable pregnancy outcomes. AAD also influences the number of
childbirths. (Bjornsdottir, et al., 2010).
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A nurse should also stress to the client and those involved in his or her care they must
report any feelings of dizziness upon sitting or standing, nausea, vomiting, pain, thirst,
infections, malaise, and feelings of anxiety to their physician. It is most essential for the nurse to
discuss the effect of emotional or physical stressors on physical health and medication
management and emphasize the importance of continuing healthcare such as referring to
community health services and support groups for information and follow-up healthcare as
appropriate (LeMone & Burke, 2008).
















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References
Bjornsdottir, S., Cnattingius, S., Brandt, L., Nordenstrom, A., & Eckborn, A. (2010).
Addison's Disease in Women is a Risk Factor for an Adverse Pregnancy Outcome.
Journal Clinical Endocrinol Metabolism: December, 2010; 95(12): 5249-5257.
Crawford, A., & Harris, H. (2012). Adrenal cortex disorders: Hormones out of kilter. Nursing,

42(10), 32-39.

Gilbert, R. E. (1992). The Mortal Presidency: Illness and Anguish in the White House . JFK and
Addison's Disease. 37(7), pgs. 22-24.
Griffing, G. T. (2012). Addison Disease. Retrieved March 11, 2013, from:
http://emedicine.medscape.com/article/116467-followup.
LeMone, P., & Burke, K. M. (2008). Clinical Handbook for Medical Surgical Nursing: Critical
Thinking in Client Care . Upper Saddle River: Pearson Education .
LeMone, P., Burke, K., & Bauldoff, G. (2010). Medical Surgical Nursing : Critical Thinking in
Patient Care . Upper Saddle River, NJ: Prentice Hall.
MedicineNet. (2011). Definition of Adrenal Cortex. Retrieved March 16, 2013, from
MedicineNet: http://www.medterms.com/script/main/art.asp?articlekey=9704.
Moloney, S., & Dowling, M. (2012). Early Intervention and management of adrenal
insufficiency in children. Nursing Children & Young People: 2012: 24 (7), 25-28.
Story, L. (2011). Pathophysiology: A Practical Approach. Burlington: Jones and Bartlett
Learning.




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