Addison's Disease, also known as primary hypoadrenalism, is an endocrine disorder affecting the adrenal glands where the adrenal glands fail to produce cortisol and sometimes mineralcorticol steroids. The adrenal glands are located directly above the kidneys (one above each kidney). They consist of the outer portion, called the cortex, and the inner portion, called the medulla. The cortex produces three types of hormones, all of which are called corticosteroids. The medulla produces epinephrine and norepinephrine.
Addison's Disease is a rare disorder, occurring in an estimated 1 in 10,000 persons.
Symptoms are generally nonspecific, which can make Addison's very difficult to diagnose. Symptoms include fatigue, weakness, unintentional weight loss, nausea, abdominal pain, diarrhea, adult onset allergies, hyperpigmentation, and generalized anxiety. Most diagnoses occur when a patient experiences an adrenal crisis, characterized by lightheadedness or dizziness, weakness, sweating, tremors, abdominal pain, nausea and vomiting, extreme low blood pressure, and even loss of consciousness. An adrenal crisis is a medical emergency that can result in death.
In fact, a rare presentation of Addison's disease can be hyperkalaemic periodic paralysis as evidenced from studies of patients presenting periodic hyperkalaemic flaccid quadriplagia as their manifestation of Addison's.
Testing for Addison's Disease is done through a simple blood test measuring the blood's level of cortisol. Cortisol is a glucocorticoid, a corticosteroid that maintains glucose (blood sugar) regulation, suppresses the immune response, and is released as part of the body's response to stress. Cortisol is believed to have over 100 functions in the body and is essential for life.
A second test, called a cortisol stimulation test, may be performed and can indicate whether the cortisol deficiency is primary (a problem with the adrenal glands alone) or secondary (a problem with the pituitary and ACTH production). A normal stim test response means the adrenal glands produced cortisol at expected levels. An abnormal response means the adrenal glands failed to produce cortisol with stimulation, or even produced less cortisol when stimulated.
A CT scan of the adrenal glands is generally performed to determine whether or not there is any destruction of the adrenal glands. If so, antibody tests may be performed to determine whether or not destruction of the adrenals is due to an abnormal autoimmune response (where the body's immune system mistakenly attacks the adrenal glands).
Treatment of Addison's Disease is hormone replacement with either prednisone or hydrocortisone, and sometimes fludrocortisone. Patients on hydrocortisone are frequently told to maintain or increase their sodium intake and avoid potassium. This is not necessary with prednisone.
Regardless of treatment, patients diagnosed with Addison's Disease should have regular appointments with an endocrinologist and a bone density scan (generally every 2 years). Addison's patients should also wear a medic alert stating their condition, and carry an emergency shot of hydrocortisone. Patients should increase their medication intake whenever sick or injured (generally triple the normal daily dose) and should seek immediate medical attention when experiencing vomiting, as this can render taking oral medication impossible.
With treatment, patients with Addison's Disease can expect an average lifespan.
One famous sufferer from Addison's Disease was former United States president John F. Kennedy, who managed the illness throughout his life. He even hid the problem from doctors in the navy so he could enlist as an officer in World War II.
They Got It Wrong
While the television show House listed one of hypoadrenalism's main symptoms as intolerance to cold, may people experience problems with both intolerance to heat and cold. At times, when the patient is experiencing an Addisonian Crisis, the patient will experience hot and cold "flashes."
Contrary to the episode Sports Medicine, treatment of Addison's disease with steroids does not damage the kidneys because the treatment is bringing the hormone levels up to normal. In fact, treatment of Addison's with steroids often helps kidney function resolving electrolyte imbalances that Addison's can cause, including decreasing the frequency of kidney stones that some hypoadrenal patients experience.