Addison's Disease

Addison's disease, also known as primary adrenal insufficiency or hypoadrenalism, is a rare disorder of
the adrenal glands.

The adrenal glands are 2 small glands that sit on top of the kidneys. They produce 2 essential hormones:
cortisol and aldosterone.

The adrenal gland is damaged in Addison's disease, so it does not produce enough cortisol or
aldosterone.

It can affect people of any age, although it's most common between the ages of 30 and 50. It's also more
common in women than men.

Signs and Symptoms

Early-stage symptoms of Addison's disease are similar to other more common health conditions, such as
depression or flu.

 A slight temperature
 Difficulty concentrating
 Headaches
 Extreme fatigue
 Weight loss and decreased appetite
 Increased thirst
 Darkening of your skin (hyperpigmentation)
  Low blood pressure, even fainting
 Salt craving
 Low blood sugar (hypoglycemia)
 Nausea, diarrhea or vomiting (gastrointestinal symptoms)
 Abdominal pain
 Abnormal drowsiness or tiredness (lethargy)
 Muscle or joint pains
 Irritability
 Depression or other behavioral symptoms
 Body hair loss or sexual dysfunction in women

Over time, these problems may become more severe and you may experience further symptoms, such
as dizziness, fainting, cramps and exhaustion.

Cause
Addison's disease is caused by damage to your adrenal glands, resulting in not enough of the hormone
cortisol and, often, not enough aldosterone as well. Your adrenal glands are part of your endocrine
system. They produce hormones that give instructions to virtually every organ and tissue in your body.

Primary adrenal insufficiency

When the cortex is damaged and doesn't produce enough adrenocortical hormones, the condition is
called primary adrenal insufficiency. This is most commonly the result of the body attacking itself
(autoimmune disease). For unknown reasons, your immune system views the adrenal cortex as foreign,
something to attack and destroy. People with Addison's disease are more likely than others to have
another autoimmune disease as well.

Other causes of adrenal gland failure may include:

 Tuberculosis
 Other infections of the adrenal glands
 Spread of cancer to the adrenal glands
 Bleeding into the adrenal glands. In this case, you may have an addisonian crisis without any
previous symptoms.

Secondary adrenal insufficiency

The pituitary gland makes a hormone called adrenocorticotropic hormone (ACTH). ACTH in turn
stimulates the adrenal cortex to produce its hormones. Benign pituitary tumors, inflammation and prior
pituitary surgery are common causes of not producing enough pituitary hormone.

Too little ACTH can lead to too little of the glucocorticoids and androgens normally produced by your
adrenal glands, even though your adrenal glands themselves aren't damaged. This is called secondary
adrenal insufficiency. Mineralocorticoid production is not affected by too little ACTH.

Most symptoms of secondary adrenal insufficiency are similar to those of primary adrenal insufficiency.
However, people with secondary adrenal insufficiency don't have hyperpigmentation and are less likely
to have severe dehydration or low blood pressure. They're more likely to have low blood sugar.

A temporary cause of secondary adrenal insufficiency occurs when people who take corticosteroids (for
example, prednisone) to treat chronic conditions, such as asthma or arthritis, stop taking the
corticosteroids all at once rather than tapering off.

Risk factor
Common risk factors in the development of Addison's disease include other autoimmune diseases:[1]

 Chronic thyroiditis
 Dermatitis herpetiformis
 Graves' disease
 Hypoparathyroidism
 Hypopituitarism
 Myasthenia gravis
 Pernicious anemia
 Testicular dysfunction
 Type I diabetes
 Vitiligo

Diagnostics
Your doctor will talk to you first about your medical history and your signs and symptoms. You may
undergo some of the following tests:

Blood test. Tests can measure your blood levels of sodium, potassium, cortisol and adrenocorticotropic
hormone (ACTH), which stimulates the adrenal cortex to produce its hormones. A blood test can also
measure antibodies associated with autoimmune Addison's disease.
ACTH stimulation test. ACTH signals your adrenal glands to produce cortisol. This test measures the
level of cortisol in your blood before and after an injection of synthetic ACTH.

Insulin-induced hypoglycemia test. You may be given this test if doctors think you may have adrenal
insufficiency as a result of pituitary disease (secondary adrenal insufficiency). The test involves checking
your blood sugar (blood glucose) and cortisol levels after an injection of insulin. In healthy people,
glucose levels fall and cortisol levels increase.

In certain situations doctors may do alternative tests for secondary adrenal insufficiency, such as a low-
dose ACTH stimulation test, prolonged ACTH stimulation test or glucagon stimulation test.

Imaging tests. You may undergo a computerized tomography (CT) scan of your abdomen to check the
size of your adrenal glands and look for other abnormalities. You may also undergo an MRI scan of your
pituitary gland if testing indicates you might have secondary adrenal insufficiency.

Treatment
All treatment for Addison's disease involves medication. You will be given hormone replacement
therapy to correct the levels of steroid hormones your body isn't producing. Some options for treatment
include oral corticosteroids such as:

 Hydrocortisone (Cortef), prednisone or methylprednisolone to replace cortisol. These

hormones are given on a schedule to mimic the normal 24-hour fluctuation of cortisol levels.
 Fludrocortisone acetate to replace aldosterone.

You will need to get plenty of salt (sodium) in your diet, especially during heavy exercise, when the
weather is hot or if you have gastrointestinal upsets, such as diarrhea.

Your doctor will also suggest a temporary increase in your medication dosage if your body is stressed,
such as from an operation, an infection or a minor illness. If you're ill with vomiting and can't keep down
oral medications, you may need injections of corticosteroids.

Other treatment recommendations include:

 Carry a medical alert card and bracelet at all times. A steroid emergency card and medical alert
identification will let emergency medical personnel know what kind of care you need. Also have
a written action plan.
 Keep extra medication handy. Missing even one day of medication may be dangerous, so keep
a small supply of medication at work and with you whenever you travel.
 Carry a glucocorticoid injection kit. The kit contains a needle, syringe and injectable form of
corticosteroids to use in case of emergency.
  Stay in contact with your doctor. Keep an ongoing relationship with your doctor to make sure
that the doses of replacement hormones are adequate, but not excessive. If you're having
ongoing problems with your medications, you may need adjustments in the doses or timing of
the medications.
 Have annual checkups. See your doctor or an endocrinology specialist at least once a year. Your
doctor may recommend annual screening for a number of autoimmune diseases.

Treatment for an addisonian crisis, which is a medical emergency, typically includes intravenous
injections of:

 Corticosteroids
 Saline solution
 Sugar (dextrose)

Potential future treatments

Researchers are working to develop delayed-release corticosteroids, which act more like the human
body. They are also working on pumps implanted under the skin that can deliver steroids in more-
accurate doses.

Future treatment may eventually involve using adrenocortical stem cells combined with
immunomodulatory treatment — modifying the immune response or the immune system — as well as
gene therapy.

Nursing considerations
Achieving Normal Fluid and Electrolyte Imbalance

 Assess fluid intake and output and serial daily weights

 Monitor results of serum sodium and potassium
 Assess skin turgor and mucous membrane for dehydration

Protecting well-being

 Minimize stressful situation

 Protect patient from infection
a. Control patient’s contacts so that infectious organisms
b. Protect patients from drafts, dampness, exposure to cold.
c. Prevent overexertion
d. Use meticulous hand washing and asepsis
 Assess comfort and emotional status of the patient
a. Control a temperature of the room to avoid sharp deviations in patient’s temperature.
b. Maintain a quiet, peaceful environment, avoid loud talking and noisy radios
 Observe and report early signs of addisonians crisis (sudden drop in BP, nausea and vomiting,
fever)
Increasing Activity tolerance

 Assist the patient with ADLs

 Provide for periods of rest and activity to avoid overexertion
 Provide for high-calorie, high-protein diet