Caring for patients is extremely rewarding… but it can also be rather Stressful. There are many complex math problems to solve and risk-benefit ratios to balance. Even the seemingly elementary problems can mentally taxing, especially in children who are prone to obscure their critical illness with “non-specific” symptoms (ex, Leukemia, Myocarditis). Additionally, you literally have to do arithmetic when ordering medications. One topic that combines both of these issues is Adrenal Insufficiency. Let us take a minute to digest a Morsel on Stress Dose Steroids:

Adrenal Insufficiency

• Adrenal crisis is a potentially life-threatening condition! [Miller, 2019; El-Maouche, 2018]
  • Adrenal crisis can be first presentation of conditions associated with adrenal insufficiency.
  • Adrenal insufficiency is associated with substantial morbidity and mortality in children (~5-10 cases per 100 patient-years). [Miller, 2019]

• Adrenal insufficiency is associated with numerous conditions.
  • Primary = defect in adrenal gland
    • Hyponatremia and Hyperkalemia can be seen.
    • Ex: Congenital Adrenal Hyperplasia, bilateral adrenal hemorrhage, and autoimmune adrenalitis
  • Secondary (Central) = defect in the hypothalamus and/or pituitary
    • Deficient ACTH eventually creates reduced glucocorticoids.
    • Mineralocorticoid production is preserved via Renin-Angiotensin system.
    • Ex.: most common cause is Iatrogenic
      • Cessation of high dose or chronic exogenous glucocorticoids
        • Prolonged and/or high doses of steroids are commonly used to treat conditions we encounter in the ED.
        • Ex.: Asthma, Leukemia, Inflammatory Bowel Disease, Rheumatologic conditions, Nephrotic Syndrome. [Rensen, 2017]
      • Exogenous steroid use preventing body’s ability to generate additional endogenous steroids in time of physiologic stress.

• Adrenal insufficiency can be exacerbated by acute physiologic stressors when endogenous steroids would normally increase to compensate:
  • Acute illness
    • Febrile Illness
    • Diarrhea
    • Vomiting
  • Physical injury / stress
  • Surgery
  • Anesthesia

• Early recognition is imperative! [Miller, 2019]
  • Clinical Signs & Symptoms:
    • Fatigue, Weakness
    • Nausea, Vomiting, Abdominal Pain
    • Hypotension
    • Dizziness
    • Diaphoresis
    • Seizures
    • Coma
  • Laboratory Findings:
    • Hyponatremia is most consistent finding.
    • Hyperkalemia is present in primary adrenal insufficiency
    • Hypercalcemia
    • Hypoglycemia is frequent in neonates and infants.
  • Hypotension and hypoglycemia can develop suddenly, even after an initial normal triage assessment.

Stress Dose Steroids

• Stress Dose Steroids = increased dose of exogenous steroids to account for physiologic stress requiring additional glucocorticoids.

• There is limited evidence on the optimal stress dose regimen for children. [Miller, 2019]

• Family and Patient Education is paramount! [Miller, 2019; El-Maouche, 2018; Dorr, 2018; Leblicq, 2011]
  • Since early recognition is important, families are empowered to help recognize possible exacerbating stressors and self-administer stress dose steroids.
  • There are Adrenal Insufficiency Action Plans that can help families and ED providers manage this difficult condition. [Miller, 2019]

• Currently recommendations include: [Miller, 2019]
  • Oral Stress Dose = Triple TOTAL daily dose of Hydrocortisone and divide that into 4 doses (q 6 hrs).
  • Emergent Intramuscular Dosing can be given if child is not tolerating oral medications.
    • IM hydrocortisone sodium succinate (Solu-Cortef)
      • 25 mg for child 3 years and younger
      • 50 mg for children >3 yrs – 12 years
      • 100 mg for children 12 years and older.
    • This is 5-10 times the physiologic cortisol secretory rate. [Miller, 2019]
    • An auto-injector is being developed.
  • Intravenous hydrocortisone is preferred once in the Emergency Department.
    • If unable to administer within 15 minutes, give IM hydrocortisone.
    • Dexamethasone is NOT suitable for primary adrenal insufficiency, because it has no mineralocorticoid action.
    • Methylprednisolone can be used, but HYDORCORTISONE is PREFERRED.
  • After initial stress dose of hydrocortisone sodium succinate is given, it should be followed by 50-100 mg/m^2/Day divided every 6 hours for 24 – 48 hours.

• Other Important Managements:
  • Volume resuscitation!
  • Monitor and supplement glucose as needed.
  • Evaluate and treat underlying exacerbating causes.

• Helpful labs:
  • Chemistry panel for glucose and acid-base assessment
  • Serum cortisol
  • Plasma ACTH level
  • Treatment is based on clinical presentation, not laboratory studies!
  • Ideally, collect blood samples prior to giving hydrocortisone, but do NOT DELAY giving the stress dosing! [Miller, 2019]

Moral of the Morsel

• Steroids don’t just make big muscles! Don’t overlook their importance!
• Parents often know best! Ask the family if their child has a Adrenal Insufficiency Action Plan! If they have it… USE IT!
• Fever, Vomiting, and Diarrhea… oh My! They are Stressors. If the child is on chronic steroids, help them to accommodate for the stress.
• Initial vital signs may deceive! Hypotension and hypoglycemia can develop abruptly.
• IV Hydrocortisone is preferred… but don’t wait! If you don’t have access after 15 minutes, give it IM!

References