Emergency Preparedness and Adrenal Crisis

While daily management of Familial Glucocorticoid Deficiency (FGD) becomes a routine part of life, there are times when your child’s body needs more support. Because children with FGD cannot produce their own “stress hormone,” they rely entirely on you to provide extra medication during illness or injury. Being prepared for these moments is the most important way you can prevent a serious complication called an adrenal crisis ^[1][2].

Managing Illness: The ‘Sick Day Rules’

When your child is healthy, they take a maintenance dose of hydrocortisone. However, when the body is stressed by an infection or a significant injury, it normally produces 2 to 3 times more cortisol than usual ^[3][1]. Since your child’s adrenal glands cannot do this, you must step in using an individualized written plan from your endocrinologist.

• When to Increase: Most pediatric endocrinologists recommend “stress dosing”—usually doubling or tripling the oral dose according to your specific written plan—when your child has a fever or significant illness ^[4][3].
• The Danger of Vomiting: If your child vomits a dose of medication, or has severe diarrhea, they cannot absorb the hydrocortisone they need. Follow your written plan for when to repeat a single vomited dose. If they cannot keep medications down or have repeated vomiting, move immediately to the emergency injection protocol ^[4][5].

Recognizing an Adrenal Crisis

An adrenal crisis occurs when the body’s demand for cortisol far exceeds the supply. It is a life-threatening emergency that requires immediate action ^[6][7].

Red Flags to Watch For:

• Severe Weakness or Lethargy: Your child is unusually sleepy, limp, or difficult to wake up ^[6][7].
• Persistent Vomiting: They cannot keep any fluids or medications down ^[4].
• Signs of Hypoglycemia (Low Blood Sugar): Shaking, sweating, confusion, irritability, or, in severe cases, seizures ^[8][5]. (Give fast-acting carbohydrate only if the child is alert and can safely swallow; never put anything in the mouth during a seizure or reduced consciousness).
• Altered Mental Status: Confusion, disorientation, or acting “out of it” ^[6].
• Shock-like Symptoms: Pale skin, cold hands/feet, or fainting/collapse ^[6][9].

The Emergency Injection (Solu-Cortef)

Every family with a child with FGD must have an emergency injection kit (usually Solu-Cortef or an Act-O-Vial) and know how to use it. This injection delivers a high dose of hydrocortisone directly into the muscle, bypassing the stomach ^[10][5].

1. Give the Injection First: If your child is vomiting repeatedly, has had a seizure, or has collapsed, give the prescribed dose of injectable hydrocortisone immediately ^[10][1]. Emergency doses are prescribed based on your child’s age, weight, or body surface area, and you must follow the specific dose your doctor has written for your child ^[11].
2. Call 911 (or local emergency services): Tell the dispatcher: “My child has Adrenal Insufficiency and is having an Adrenal Crisis. We have given an emergency injection, and they need immediate transport and IV fluids” ^[1][12].
3. Do Not Delay: It is much safer to give the injection and find out later it wasn’t strictly necessary than to wait and allow a crisis to progress ^[5][13].

Building a Safety Net

Preparation is the best defense against a crisis. Ensure these three things are always in place:

• Medical Alert Jewelry: Your child should wear a bracelet or necklace at all times stating “Adrenal Insufficiency - Needs Hydrocortisone” ^[14][15].
• The School/Daycare Plan: Provide a written Adrenal Action Plan signed by your doctor. This plan should give staff permission to give the emergency injection and call 911 immediately ^[1][2].
• The Emergency Letter: Keep a “To the Emergency Room Physician” letter from your endocrinologist in your car and diaper bag. This letter explains FGD and the need for immediate parenteral hydrocortisone and appropriate IV fluids (with dextrose checked and treated if low), which helps speed up care in a busy ER ^[1][2].