Running head: ADDISON DISEASE 1

Addison Disease
Astrid Nicolau-Raducu
November 26, 2013
NU 300 Disease Report #2






















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One of the patients I was assigned too while in clinical suffered from sudden onset of
weakness, hypotension (more specifically orthostatic in nature), and irregular blood sugar levels
(while following same routine that perfectly controlled her diabetes for more then ten years). As
I was reading the doctors notes in her chart, I saw that they were playing around with the idea of
Addison Disease and I wanted to learn more about it.
Addison Disease
Fist discovered in 1855, by Dr. Thomas Addison, Addison disease, is an autoimmune
disease also known as adrenocortical hypofunction or primary adrenal insufficiency. At that time
tuberculosis (TB) was the most common cause. Today, with the TB effector removed, the
disease is most commonly caused by non-specific autoimmune mechanisms that destroy adrenal
cortical cells. Autoimmune disorders occur when the bodys immune system produces antibodies
against itself, destroying tissues and organs over time. About 1 in 100,000 people in the Unites
States are diagnosed with this disease, roughly 70 percent of all reported cases resulting from
autoimmune disorders. Addison disease mostly affects adults ages 30 to 60 (more common in
women), it may appear unexpectedly at any point in time. Persons suffering of other autoimmune
disorders like Hashimoto thyroiditis, pernicious anemia, and idiopathic hypoparathyroidism
can develop an organ-specific form of Addison disease, called Idiopathic Addison disease. In
such cases the disease may be inherited as an autosomal recessive trait; and it may occur in
childhood (Type 1) or adulthood (Type 2). Other risk factors for Addison disease include
Graves' disease, Myasthenia gravis, Type I diabetes, Vitiligo, infection, hemorrhage, metastatic
carcinoma, and medications.
Pathogenesis of Addison Disease
In Addison disease, autoantibodies attack the steroid-producing cells of the adrenal
cortex, causing inflammation and fibrosis of the cortex that prevents further hormone secretion.
Adrenocorticotropin hormone (ACTH) released from the pituitary, also regulated by negative
feedback system, stimulates synthesis of the adrenocortical hormones, aldosterone and cortisol,
in the adrenal cortex zones. Primary adrenal insufficiency is induced by deficiencies of these
adrenocortical hormones caused by the gradual destruction of the zones of synthesis. Initially
adrenocortical hormone secretions meet basal requirements, however as stressors persist,
insufficiency is clinically displayed. These signs are congruent with cellular destruction of the
cortex, resulting in decreased plasma cortisol response to ACTH, while cortisol secretions
decrease, plasma levels of ACTH increase due to decreased negative feedback inhibition of
ACTH secretion. Once 90 percent of the adrenal cortex is damaged, significant decrease in
plasma cortisol and aldosterone levels occurs and clinical symptoms are overtly displayed.
Clinical Presentation of Addison Disease
Signs and symptoms of Addison disease are nonspecific and slow to develop, but are
directly related to decreasing levels of aldosterone and cortisol hormones. Aldosterone maintains
salt and water balance in extracellular fluids, aiding kidneys to retain Na
+
/release K
+
and
regulating blood pressure/volume. Cortisol maintains proper arousal and sense of well being,
aiding the body to respond to stress and regulating protein, fat, and carbohydrates metabolism for
energy. An insufficiency of production of these hormones will result in muscle weakness,
fatigue, weight loss, decreased appetite, orthostatic hypotension, hypovolemia, hyperkalemia,
hypoglycemia, nausea, vomiting, diarrhea, irritability, depression, muscle and joint pain, and also
hyper pigmentation of the skin and mucus membranes. Severe exacerbation of Addison disease,
called Addisonian crisis, is caused by acute adrenal insufficiency due to extremely low levels of
adrenocortical hormones and leads to severe hypoglycemia and high serum potassium. This
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condition is life threatening and includes symptoms of extreme weakness, mental confusion,
extreme drowsiness, in advanced cases slipping towards a coma, pronounced dizziness, nausea
and/or vomiting, severe headache, abnormal heart rate, abnormally low blood pressure, feeling
extremely cold, possible fever and some abdominal tenderness. Complications of Addisonian
crisis include shock, coma, seizures and death.
Diagnostic Testing and Nursing Implications for Addison Disease
Due to the wide variety of symptoms experienced with Addison disease, a chemistry
profile and a blood count test is performed to rule out other common diseases. Individuals with
Addison disease often display elevated blood urea nitrogen (BUN)/creatinine caused by
dehydration, hypoglycemia due to severe insulin sensitivity, and lymphocytosis due to increased
production of lymphocytes, plasma cells and eosinophils.
Testing for adrenal insufficiency measures cortisol, aldosterone, sodium, potassium, and
ACTH levels both in the blood and urine. Before performing the diagnostic test procedure the
nurse should obtain a history of the patients clinical symptoms, most recent drug regimen,
obtain vitals, and assess/observe for signs and symptoms of complications caused by hormone
and electrolyte imbalances. The nurse is also responsible for instructing patients about factors
that may lead to false test results. This teaching may include that physical activity/stress or
standing prior to collection of the blood sample affects ACTH, cortisol, and aldosterone levels
respectively. Hyperkalemia, hyponatremia, increased ACTH plasma levels, low serum cortisol
and aldosterone levels correlate with Addison disease characteristics.
Primary adrenal insufficiency is confirmed by the ACTH stimulation test. The test
determines adrenal gland function by measuring serum cortisol levels before and after injection
of synthetic ACTH. The nurse should explain this procedure, its purpose, and meaning of results
to the client prior to initiating the test. Addison disease is confirmed when serum cortisol level
does not increase with injected ACTH. Imaging tests may also be done to check the size of the
adrenal glands and look for any other abnormalities that may give an insight to the cause of an
adrenal insufficiency.
Plan and Management of Addison Disease
Management of Addison disease is achieved through replacement therapy of the
hormones no longer released from adrenal glands with synthetic ones, following autoimmune
destruction of the adrenal cortex. This is a life long treatment, as a cure for the disease has not
yet been discovered. Cortisol is replaced by prednisone, hydrocortisone, or cortisone acetate
normally taken in two divided doses, while Aldosterone is substituted by fludrocortisone taken
only once a day. Replacement medications for Cortisol will require adjustments due to
fluctuating requirement of the hormone occurring with illness, stress level increases, or other
situations that may arise. In situation, such as vomiting, when oral medication is not retained
injections are prescribed ensuring treatment continuity. Electrolyte levels and clinical findings
help monitor Fludrocortisone replacement therapy. If under dosed results in weakness, low
diastolic blood pressure, and low serum sodium levels. Diet modifications that increase protein,
carbohydrate, and sodium intake promote electrolyte balance. In order to maintain this
electrolyte and hormone balance, people with Addison disease are required to consume high
amounts of sodium when fluid loss is increased.
The plan for patients suffering from Addison disease is to make the person feel as healthy
and normal as possible, and administering synthetic adrenal hormones in a way that
approximates a natural pattern of adrenal steroid secretion. This plan will be fulfilled with
regulation of replacement medication levels leading to prevention of an Addisonian crisis.
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Patient must recognize signs and symptoms of potential stress that may cause an acute adrenal
crisis such as sudden penetrating pain in the legs, lower back or abdomen, severe vomiting and
diarrhea, hypoglycemia, confusion, slurred speech, convulsions, and/or fever. Teaching
treatments of minor illnesses by extra intake of salts, fluids, and hydrocortisone, self-
administration of emergency injection of hydrocortisone, and increasing oral steroid intake in
times of stress. With these tools a person with Addison disease can live a normal life with a
normal life expectancy.