Two years ago i found out i have Addison's disease.
and for the first time ever i'm willing to openly speak of my "Afliction" in hopes that i can give hope to others with it.
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.
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.
Cortisol is normally produced by the adrenal
glands, located just above the kidneys. It belongs to a class of hormones called glucocorticoids, which affect almost every
organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most
important job is to help the body respond to stress. Among its other vital tasks, cortisol:
- helps maintain blood pressure and cardiovascular
- helps slow the immune system's inflammatory
- helps balance the effects of insulin in breaking
down sugar for energy; and
- helps regulate the metabolism of proteins,
carbohydrates, and fats.
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, a bean-sized organ at the base of the brain. 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.
Aldosterone belongs to a class of hormones called
mineralocorticoids, also produced by the adrenal glands. It helps maintain blood pressure and water and salt balance in the
body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are
not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
|Failure to produce adequate
levels of cortisol, or adrenal insufficiency, can occur for different reasons. The problem may be due to a disorder of the
adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary
Primary Adrenal Insufficiency
Most cases of Addison's disease are caused by
the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About
70 percent of reported cases of Addison's disease are due to autoimmune disorders, in which the immune system makes antibodies
that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent
of the adrenal cortex has been destroyed. As a result, often both glucocorticoid and mineralocorticoid hormones are lacking.
Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected,
as in the polyendocrine deficiency syndrome.
The polyendocrine deficiency syndrome is classified
into two separate forms, referred to as type I and type II. Type I occurs in children, and adrenal insufficiency may be accompanied
by underactive parathyroid glands, slow sexual development, pernicious anemia, chronic candida infections, chronic active
hepatitis, and, in very rare cases, hair loss. Type II, often called Schmidt's syndrome, usually afflicts young adults. Features
of type II may include an underactive thyroid gland, slow sexual development, and diabetes mellitus. About 10 percent of patients
with type II have vitiligo, or loss of pigment, on areas of the skin. Scientists think that the polyendocrine deficiency syndrome
is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.
Tuberculosis (TB) accounts for about 20 percent
of cases of primary adrenal insufficiency in developed countries. When adrenal insufficiency was first identified by Dr. Thomas
Addison in 1849, TB was found at autopsy in 70 to 90 percent of cases. As the treatment for TB improved, however, the incidence
of adrenal insufficiency due to TB of the adrenal glands has greatly decreased.
Less common causes of primary adrenal insufficiency
are chronic infections, mainly fungal infections; cancer cells spreading from other parts of the body to the adrenal glands;
amyloidosis; and surgical removal of the adrenal glands. Each of these causes is discussed in more detail below.
Secondary Adrenal Insufficiency
This form of Addison's disease can be traced
to a lack of ACTH, which causes a drop in the adrenal glands' production of cortisol but not aldosterone. A temporary form
of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone
for a long time abruptly stops or interrupts taking the medication. Glucocorticoid hormones, which are often used to treat
inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both corticotropin-releasing
hormone (CRH) and ACTH. Normally, CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is
not stimulated to release ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.
Another cause of secondary adrenal insufficiency
is the surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushing's disease). In this
case, the source of ACTH is suddenly removed, and replacement hormone must be taken until normal ACTH and cortisol production
resumes. Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing
ACTH. This can result from tumors or infections of the area, loss of blood flow to the pituitary, radiation for the treatment
of pituitary tumors, or surgical removal of parts of the hypothalamus or the pituitary gland during neurosurgery of these
|The symptoms of adrenal
insufficiency usually begin gradually. Chronic, worsening fatigue and muscle weakness, loss of appetite, and weight loss are
characteristic of the disease. Nausea, vomiting, and diarrhea occur in about 50 percent of cases. Blood pressure is low and
falls further when standing, causing dizziness or fainting. Skin changes also are common in Addison's disease, with areas
of hyperpigmentation, or dark tanning, covering exposed and nonexposed parts of the body. This darkening of the skin is most
visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips; and mucous membranes.
Addison's disease can cause irritability and
depression. Because of salt loss, craving of salty foods also is common. Hypoglycemia, or low blood sugar, is more severe
in children than in adults. In women, menstrual periods may become irregular or stop.
Because the symptoms progress slowly, they are
usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an addisonian
crisis, or acute adrenal insufficiency. In most patients, symptoms are severe enough to seek medical treatment before a crisis
occurs. However, in about 25 percent of patients, symptoms first appear during an addisonian crisis.
Symptoms of an addisonian crisis include sudden
penetrating pain in the lower back, abdomen, or legs; severe vomiting and diarrhea, followed by dehydration; low blood pressure;
and loss of consciousness. Left untreated, an addisonian crisis can be fatal.