Acute adrenal insufficiency crisis usually occurs after a prolonged period of nonspecific complaints due to a loss of both glucocorticoids and mineralocorticoids; by the time overt symptoms occur, 90% of the adrenal gland may be destroyed.3 Patients (such as ours) may present with symptoms such as abdominal pain, weakness, vomiting, fever, and decreased responsiveness. Hyponatremia and hyperkalemia are commonly seen at initial diagnosis. BP can be compromised in some patients due to loss of vascular tone; our patient did not present with this finding.
Treatment includes hydrocortisone and fludrocortisone for life
Initial management focuses on rehydration, maintenance of BP, cardiac monitoring, and electrolyte monitoring with a focus on slow normalization of electrolyte abnormalities. Patients should be treated with hydrocortisone (approximately 10 mg/m2/d) and fludrocortisone (usually 0.1 mg/d), and they will be maintained on this regimen for life.1,3
During acute illness, the doses of hydrocortisone are usually tripled and given 3 times per day to address the increased cortisol needs of the stress response. Lack of stress dose steroids in the setting of illness can lead to repeat adrenal crisis events.
Patients should be taught about intramuscular (IM) hydrocortisone use (100 mg IM) for emergencies and should have medical identification. In many states, emergency medical technicians (EMTs) are now able to administer the patient’s own supply of hydrocortisone. EMTs have even begun carrying hydrocortisone in some states in response to a campaign by the CARES Foundation, a nonprofit organization dedicated to helping families and individuals affected by congenital adrenal hyperplasia.
We started our patient on 100 mg/m2/d hydrocortisone 24 hours after he was admitted to the PICU. (At that time, his sodium level was 110 mEq/L.) Forty-eight hours after admission, we started the patient on fludrocortisone for mineralocorticoid effect at 0.1 mg/d. (The patient’s sodium level was 122 mEq/L). At 72 hours after admission, the patient’s sodium level was 137 mEq/L and his mental status was normal. Normal saline was discontinued when sodium normalized. He was discharged 2 days later. He was informed he should continue these medications for life, though doses might be adjusted slightly with time.
Two weeks later, our patient’s sodium level had reached 141 mEq/L and his weight loss, depression, vomiting, and fatigue had resolved. He stopped taking his SSRI. He was still craving extra salt, but not as much, and his urine was no longer a very dark yellow.
In retrospect, starting this patient on steroids earlier may not have resulted in any more of a rapid sodium rise than that which occurred otherwise, but we believe that our concern for CPM at that time justified the delay in steroid use. We felt it was safe to delay steroids because the patient’s BP was stable and his clinical picture was rapidly improving. In most cases, however, delaying steroids is not advisable.
THE TAKEAWAY
Adrenal insufficiency can be clearly diagnosed via labs and clinical presentation, and is potentially lethal if unrecognized. The predominant manifestations of adrenal crisis are hypotension and shock, usually with hyponatremia and hyperkalemia. During stressful events or illness, patients should increase their glucocorticoid dose. If they are on hydrocortisone, instructions are usually to triple the dose, and give the medication 3 times a day. Patients require instruction beforehand on how and when to increase doses for illness so that they can handle this on their own. Patients should carry a medical identification card so that their condition is evident to anyone caring for them in the ED.