What Is Addison's Disease–and How Do You Know If You Have It?
The rare hormonal disorder could be making you tired.
Addison’s disease is when your adrenal glands can’t produce enough of certain hormones that keep all the various parts of your body working.
Addison’s–also called primary adrenal insufficiency–specifically affects the outer cortex of your two adrenal glands, which are located just above your kidneys. People with Addison's disease don't make enough of the hormones cortisol and aldosterone.
Adrenal insufficiency can also occur when the pituitary gland is damaged and isn’t releasing enough adrenocorticotropic hormone (ACTH). ACTH usually instructs the adrenal glands to do their job releasing cortisol and aldosterone. If pituitary gland issues mean someone doesn’t have enough ACTH, it’s called secondary adrenal insufficiency.
So how can you tell if Addison’s disease might be plaguing you? Here’s what you need to know.
Addison's disease symptoms
Because cortisol and aldosterone have such wide-ranging responsibilities in the body–the hormones are involved in everything from stabilizing blood pressure and metabolism to aiding in digestion and responding to stress–the symptoms of Addison’s can be equally far-ranging. They’re also often vague and easily confused with symptoms of other concerns.
Some of the common signs of Addison’s include fatigue, weight loss, not wanting to eat, abdominal pain, low blood sugar, low blood pressure (especially when standing up), nausea and vomiting, diarrhea, muscle and joint pain, and depression.
“There’s a long list of things that can cause [those symptoms],” says Deborah Merke, MD, chief of the pediatric service at the National Institutes of Health Clinical Center in Bethesda, Maryland.
There are also more specific signs. “Low blood sugar, low sodium levels, and high potassium levels are a big clue,” says Josefina Farra, MD, assistant professor of surgery at the University of Miami Miller School of Medicine. This indicates that your body doesn’t have enough aldosterone, which normally increases sodium while decreasing potassium.
Another telltale sign is hyperpigmentation, or darkening of the skin. “That’s because the pituitary is overworking to try to stimulate the adrenal gland,” explains Dr. Merke. “The hormone ACTH causes some hyperpigmentation.”
If the adrenal glands are failing totally, you may also crave salty foods.
Addison's disease diagnosis
Diagnosing Addison’s disease is difficult not just because many of the symptoms are vague, but also because they tend to come on gradually. Sometimes Addison’s will be suspected because of the constellation of symptoms. Other times, routine blood tests will show the red flag of high potassium levels coupled with low sodium.
A more definitive diagnosis can be made through a series of lab tests. During an ACTH stimulation test, for instance, doctors will stimulate production of the hormone. In a healthy body, this should also prompt cortisol production. If that doesn’t happen, something may be wrong with the adrenal glands.
Another tip-off would be if ACTH was unusually high and cortisol low in the morning. “Cortisol levels are normally high in the morning in a healthy person,” explains Dr. Merke.
Addison's disease causes
Almost all cases of Addison’s in adults in the developed world (where it affects just 110 to 144 of every 1 million people, according to the National Institute of Diabetes and Digestive and Kidney Diseases) are autoimmune-related: Your body’s immune system misfires and starts attacking itself. In the developing world, infections such as tuberculosis are more common causes of Addison’s disease. And in children the world over, Addison’s is usually caused by genetic factors.
Additional tests can help narrow down a cause of adrenal gland damage, including measuring antibody levels. Antibodies are proteins produced by the immune system after encountering foreign elements–or what it thinks are foreign elements.
“If those are high, then you suspect an autoimmune cause,” says Dr. Merke. “If not, you need to start looking for other things.”
A tuberculosis skin test could rule out or confirm this infection as a cause of Addison’s.
Addison's disease treatment
Addison’s disease is fairly easily treated by giving replacement hormones for the cortisol and aldosterone that are missing.
“Cortisol is typically replaced by hydrocortisone,” says Dr. Merke. Patients take hydrocortisone pills two to three times a day.
Aldosterone is replaced with fludrocortisone, a synthetic steroid. This is usually a once-a-day pill. Medications have to be taken for the rest of your life.
“For the most part, it’s a very manageable thing,” says Dr. Farra. “It’s easy to take the medication once you’re on the correct dose.”
You will need to make adjustments if there’s a major stress on your body, like giving birth, having surgery, or even being sick with a fever or nausea and vomiting.
“Normally your adrenal glands function at those moments of stress to increase production of all these steroids because your body needs it,” says Dr. Farra. “We must do that with medication.”
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What is Addisonian crisis?
If you’re not getting treatment or the treatment you’re getting isn’t sufficient, you could end up with acute adrenal failure, explains Dr. Merke. This is also called adrenal or Addisonian crisis.
Addisonian crisis can be triggered by a physical stressor like illness (especially if there’s a fever or vomiting and diarrhea) or surgery. “Those are the times your body normally increases [hormone] production, so you’re behind the eight ball,” says Dr. Farra.
Symptoms include low blood pressure, low blood sugar (especially in children), vomiting and diarrhea (leading to dehydration), and pain in your lower back, abdomen, and legs. “It can be very dangerous and life-threatening,” Dr. Farra adds.
Standard therapy for Addisonian crisis includes immediate IV administration of corticosteroids and fluids. Most people with Addison’s disease seek out treatment before they reach a crisis state. Those people usually carry a corticosteroid injection with them all the time. In rare instances, symptoms show up for the first time during an Addisonian crisis.