ADDISON’S DISEASE

Introduction
In 1855, Thomas Addison, an English
physician, provided the first detailed
description of primary adrenal insufficiency,
now called Addison’s disease. The use of this
term is reserved for primary adrenal
insufficiency in which adrenal cortical
hormones are deficient and ACTH levels are
elevated because of lack of feedback
inhibition.
 Addison’s disease is a relatively rare
disorder in which all the layers of the adrenal
cortex are destroyed.
Addison’s disease, like type 1 DM, is a
chronic metabolic disorder that requires
lifetime hormone replacement therapy. The
adrenal cortex has a large reserve capacity,
and the manifestations of adrenal
insufficiency usually do not become apparent
until approximately 90% of the gland has been
destroyed.
Anatomy and Physiology
The endocrine system is considered as one of the
body’s most complex systems. It affects functions of
the different organs or organ system. It is greatly
interrelated with the function of the nervous system,
to coordinate body processes.
Alterations in function of the endocrine system
result in a variety of physiologic changes, which may
be very serious and fatal, but at times may require
minimal hospital care.
The end result of most pathologic processes
affecting the endocrine system is hypersecretion or
hyposecretion of hormones.
Anatomy and Physiology

The endocrine system is composed of

an interrelated complex of glands
(pituitary, adrenals, thyroid,
parathyroids, islets of Langerhans of the
pancreas, thymus, pineal gland, ovaries,
and testes) that secrete a variety of
hormones directly into the bloodstream.
 Although the nervous and endocrine
systems act together to coordinate functions
of all body systems, their means of control are
different.
Exocrine glands (exo- outside) secrete
their products into ducts that carry the
secretions into body cavities, into lumen of an
organ, or to the outer surface of the body.
Endocrine glands, by contrast, secrete
their products (hormones) into the interstitial
fluid surrounding the secretory cells, rather
than into ducts.
Functions:
• Maintenance and regulation of vital functions
• Response to stress and injury
• Growth and development
• Energy metabolism
• Reproduction
• Fluid, electrolyte, and acid-base balance
Adrenal Gland
• Two small glands, one above each kidney
• Regulates sodium and electrolyte balance
• Affects carbohydrate, fat, and protein
metabolism
• Influences the development of sexual
characteristics
• Sustains the “flight or fight” response
Consist of two sections:
 Adrenal cortex (outer portion)
o The outer shell of the adrenal gland
o Produces mineralocorticoids
Aldosterone: regulates electrolyte balance by
promoting sodium retention and potassium
excretion.
o Produces Glucocorticoids
Cortisol, Cortisone, and Corticosterone
o Produces sex hormones govern development of
certain secondary sex characteristics. Secretion
of adrenal androgens is controlled by ACTH.
 Adrenal medulla (inner portion)
o The inner core of the adrenal gland
o Works as part of the sympathetic nervous system
o Produces two catecholamines: epinephrine (also
called as adrenalin), norepinephrine (sometimes
called noradrenalin).
 -Autoimmune disease
-Idiopathic atrophy of adrenal glands
(sudden cessation of glucocorticoids)
-Endocrine disorders
-Pituitary hypofunction

Mineralocorticoid Glucocorticoid Hyperpigmentation

deficiency deficiency

ADDISON’S
DISEASE
Clinical Manifestations:

Subjective: muscle weakness and lethargy, fatigue; anorexia,

nausea, abdominal pain, depression, thirst
 
Objective:
• Increased bronzelike pigmentation of skin and nipples,
scars, and buccal mucousa
• Personality changes
• Menstrual changes in women; impotence in men
• Vomiting; diarrhea (GI disturbance), weight loss,
dehydration
• Hypotension, weak pulse
• Decreased pubic hair and axillary hair
• Decrease capacity to deal with stress
Diagnostic Test:
• Physical examination –pariticularly for
hyperpigmentation of the skin
• Cortisol level blood test
• X-ray adrenal glands – detect calcium deposits
from TB-related Addison’s
• ACTH level blood test
Medications:
 Celestone (Bethamethasone)
 Cortone, Cortistan (Cortisone)
 Decadron (Dexamethasone)
 Florinef (Fludrocortisone)
 Cortef (Hydrocortisone)
 Medrol, Depo-Medrol, Solu-Medrol
(Methylprednisolone)
 Delta-Cortef, Prelone, Orapred, Pediapred
(Prednisone)
Surgery
Patients with Addison's disease who need
surgery with general anesthesia are treated
with injections of hydrocortisone and saline.
Injections begin on the evening before surgery
and continue until the patient is fully awake
and able to take medication by mouth. The
dosage is adjusted until the maintenance
dosage given before surgery is reached.
Nursing Diagnosis:

• Deficient fluid volume related to excess

sodium loss
• Decrease cardiac output related to electrolyte
imbalances
 Implementation
• Monitor vital signs four times a day; be alert for the
elevation in temperature (infection, dehydration),
alterations in pulse rate and rhythm (hyperkalemia),
and alterations in blood pressure.
• Observe for signs of sodium and potassium imbalance
• Monitor intake and output and weigh daily to assess
for deficient fluid volume and nutritional status
• Administer hormone replacement therapy as ordered
to maintain or improve the client’s condition
• Glucocoticoids (cortisone, hydrocortisone): to
stimulate diurnal rhythm of cortisol release, give 2/3 of
dose in early morning and 1/3 of dose in afternoon.
• Mineralocorticoid: fludrocortisone acetate (Florinef)
• Give with milk or an antacid to limit ulcerogenic
factor of the drug
• Put the client in a private room to prevent contact
with clients having infectious diseases
• Limit the number of visitors; decrease stress in the
environment
• Don’t allow client to sit up or stand quickly to avoid
orthostatic hypotension
• Prevent exposure to infection
• Assess fluid balance (and tell the client to increase fluid
intake in hot weather) to prevent Addisonian crisis, which
may be precipitated by salt or fluid loss in hot weather and
during exercise
• Provide rest periods; prevent fatigue; advise the client to
avoid physical and emotional stress
• Review signs of adrenal hypofunction or hyperfunction so
client can recognize need for adjustment of steroid dose
• Instruct client to wear medical alert band
• Encourage the consistent goals with emphasis on diet high
in nutriten-dense foods (high in carbohydrates and
proteins) and adequate sodium to prevent hypoglycemia
and hyponatremia
• Administer antiemetics to prevent fluid and electrolyte loss
by vomiting
Nursing interventions for Steroid Therapy (Hormonal
Replacement Therapy – HRT)

Adrenocorticotropic Hormones
Corticosteroids/Glucocorticoids
Mineralocorticoids

a. Monitor VS. Retention of sodium and water may

cause elevation of BP
b. Monitor weight, I and O for edema.
c. Avoid exposure to infection. Steroids may mask signs
of infection and immunosuppresants.
d. Monitor potassium and calcium levels.
Steroids may cause hyperkalemia and
hypocalcemia.
e. Administer steroids after meals or with milk.
Steroids stimulate gastric acid secretions and
may cause gastric irritation and peptic ulcer
disease.
f. Diet of the client should be high protein, high
carbohydrate, high potassium, and low
sodium diet.
Provide client teaching and discharge planning concerning:

• Disease process; signs of adrenal insufficiency

• Use prescribed medications for lifelong replacement
therapy; never omit medications
• Need to avoid stress, trauma, and infections, and to notify
physician if these occurr ad medications dosage may need
to be adjusted
• Stress management techniques
• Diet modification (high in protein, carbohydrates, and
sodium) to promote nutritional balance
• Use of table salt tablets (if prescribed) or ingestions of salty
foods (potato chips) if experiencing increased sweating
• Importance of alternating regular exercise with rest periods
• Avoidance of strenous exercise especially in hot weather to
conserve energy and decrease metabolic demands.
Prognosis

With proper medication, patients can

expect to live a healthy and normal life.
 Adrenal Function After Living
Kidney Donation
Due the lack of suitable donor organs a large proportion of
kidney transplantations is now performed from living donors.
In the year 2002 the number of living donors surpassed the
number of cadaveric donors in Switzerland (Swisstransplant,
Swiss Transplant Society 2005). Kidney grafts from living
donors have superior long-term outcome (Hariharan S, N Engl
J Med 2000). At the University Hospital Zurich 28 of the 84
kidney transplantations performed in the year 2004 were from
living donors.
During living donor nephrectomy the renal artery and renal
vein are transected as far away from the kidney as possible, to
ensure an adequate vessel length for the subsequent
transplantation procedure.
 The arterial supply of the adrenal glands is accomplished
by several small vessels (ca. 5-15), while the venous drainage
is usually dependent on a single vein. While the right-sided
adrenal vein drains directly into the inferior vena cava (IVC),
the left-sided adrenal vein drains into the renal vein. Variants
are relatively rare and occur in 5% of cases (Sebe P, Surg
Radiol Anat 2002). Therefore, during donor nephrectomy of
the left kidney the adrenal vein is transected, while this is not
necessary on the right side.