Adrenal Insufficiency

There are 2 ways the organ systems of the body communicate. Either the communication is electrical (nervous system) or it is chemical (endocrine system). Chemical communication in the body is important for many life functions including managing growth and reproductive cycles, dealing with stress and thermoregulation. Whereas the nervous system communicates in instantaneous, short-lived and direct fashion, the endocrine system sends signals more slowly and more generally over a sustained and sometimes cyclical time frame.

The adrenal glands are part of the hormonal regulatory system in the body. They are small glands that sit on top of each kidney. Each gland consists of a outer cortex and an inner medulla. The cortex produces steroid hormones like corticosteroids (cortisol) and mineralcorticoids (aldosterone) and small amount of male sex steroid hormones. The medulla produces hormones like adrenaline which help regulate aspects of the sympathetic nervous system.

In adrenal insufficiency, there is an abnormal production of these hormones. Primary adrenal insufficiency is a disorder that originates in the adrenal glands where there is an insufficient production of cortisol and sometimes aldosterone. Cortisol production is increased during times of stress. The function of cortisol is mobilize energy stores for use by the body so it increases blood glucose levels. If this hormone is not being produced, blood glucose levels can drop to dangerously low levels.

Enter my son. He was acting a little odd one day. We were out as a family at a park with food trucks and I had gotten my son a cheese pizza. It’s his favorite. Instead or eating, he laid on the ground and refused to get up. He stopped responding. He wanted to sleep. I had seen him decompensate before but this time I didn’t have a glucometer or ketone testing strips. I called an Uber and packed him in the back and went straight to the hospital. We was talking in the car, but quite nonsensically, almost sounding drunk. In the emergency at the hospital, he was taken back right away. Blood glucose testing showed sugar levels 10 mg/dL or 0.55 mmol/L. Normal blood sugar should be between 70-100 mg/dL or 4-7mmol/L. The doctors were surprised he was still conscious. They immediately tested his cortisol and ACTH levels among other things. Both his ACTH were undetectable.

At this juncture, doctors diagnosed him with Secondary Adrenal Insufficiency. What is SAI and how is it different from Primary Adrenal Insufficiency?

In Secondary Adrenal Insufficiency, the pituitary gland located deep in the center of the brain, does not produce sufficient levels of Adrenocorticotropin to stimulate cortisol production in the adrenal glands. There can be a variety of causes including a structural defect in the anterior pituitary or a tumor.

To treat SAI, it is necessary to dose glucocorticoids at regular intervals and increase such dosages during times of stress. Often aldosterone levels can be affected as well which may require supplementation with mineralocorticoids.

My son probably has a partial adrenal insufficiency, but I don’t think that has ever been his real issue. It is secondary to the metabolic disorder that we have yet to diagnose.