Why Adrenal Insufficiency Is Like a Game of 'Telephone'
When many of us were children, we played a game called “telephone,” which began with a group of friends sitting in a circle. One person would think of something clever to say, such as “Billy wishes Susie would call him,” and then whisper it into the ear of the person bedside them. They had to try to hear it correctly before the next person passed along what they heard. The final person in the circle said what they heard aloud for all to enjoy. Usually it became something like, “Susie said Billy has a fat head.”
The result was endless hilarity when the circle realized what the original statement was, and how badly the signal became confused down the line.
The three types of adrenal insufficiency are similar to this game, only minus the hilarity. Instead, it becomes a game of life and death, and the circle is the human body, fighting to stay alive!
There are three main parts to what is called the HPA axis, or hypothalamic-pituitary-adrenal axis. First, is the hypothalamus, which sits toward the base of the skull. Next, is the pituitary gland, which hangs out in a little pocket behind the eyes. Finally, we have the adrenal glands, which reside atop the kidneys, one on each side.
As our bodies encounter stressful situations, whether illness, injury, or emotional, the body responds. The hypothalamus sends CRH, or cosyntropin releasing hormone, to the pituitary gland as an alert that the pituitary has a job to do.
The pituitary gland, in turn, produces ACTH, or adrenocorticotropic hormone, which it shoots down to the adrenal glands.
When the adrenal glands receive this ACTH signal, they recognize that there is a stressful situation, and produce cortisol. According to WebMD, cortisol helps the body regulate glucose levels, metabolism, and manage stress. It’s a hormone the body cannot live without.
Ideally, the body releases cortisol to balance out the adrenaline surge in the body, and allow organs to continue to function in times of stress. When the communication is disrupted, however, the result is not some funny phrase, but the stoppage of cortisol production. This is called adrenal insufficiency (AI).
There are three main types, based on the HPA axis. First, is primary adrenal insufficiency, also called Addison’s disease. This occurs when the adrenal gland receives the message from the pituitary, but can’t respond. This can be due to autoimmune disease, adrenal damage from an illness such as tuberculosis, or any other adrenal malfunction. Some of the clinical signs that occur with this are a severe deficiency in cortisol, high levels of ACTH, low aldosterone (another adrenal hormone), hyper-pigmented skin, weight loss, fatigue, nausea, and general malaise. Addison’s is a rare disease, and often is not clearly diagnosed until a patient has an adrenal crisis and lands in the emergency room.
Adrenal crisis occurs when the levels of cortisol are so low in the body that organs begin to fail, and if left untreated, death can occur in as short a time as 12 hours.
The next type of adrenal insufficiency is called secondary adrenal insufficiency. This occurs when the pituitary skips sending the message along to the adrenal glands. Often, if the adrenals were given a synthetic version of ACTH, they will still produce adequate cortisol, though sometimes the message has been lost for so long, the adrenal glands no longer know what to do. The term “secondary” comes from being one level removed from the adrenal glands themselves. Clinical presentation shows low blood cortisol, low to normal ACTH levels, relatively normal aldosterone, fatigue, nausea, and overall poor health. Secondary adrenal insufficiency does not have hyper-pigmented skin, as it is the ACTH excess that causes the “Addison’s tan.” Secondary AI is more common, because it can be caused by pituitary damage or disorders, but also caused by use of high doses or long-term corticosteroids like hydrocortisone or prednisone to treat other disorders. The body receives enough corticosteroids synthetically, that it decides to stop natural production, as it only produces exactly what we need. The increased use of steroids to treat other illnesses has led to an increase in secondary adrenal insufficiency.
Tertiary adrenal insufficiency is the most rare, and occurs when the hypothalamus fails to release CRH. Symptoms are similar to secondary.
No matter which type of adrenal insufficiency a person has, the treatment is the same: lifelong corticosteroid replacement therapy. Some will use hydrocortisone, others prednisone, but once adrenal insufficiency has occurred, there is no cure — unless it was temporarily brought on by long-term steroid use, in which case there is a chance of improving once steroid use is stopped.
Like the game of telephone, we can see what the story should have been, but along the way, the message was lost. Instead of a funny story, we have bodies that want to shut down. If we don’t step in and “fix” the message by replacing cortisol, the body will shut down, typically in three to five days. Adrenal crisis is severe, and should be treated like a heart attack, anaphylaxis, or diabetic coma. Understanding how adrenal insufficiency works is important for patients, doctors, and caregivers. Without a strong team helping to ensure the message is received, the adrenal insufficient patient is at great risk – it’s much more than a joke.