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Pediatrics · Classic Congenital Adrenal Hyperplasia

Adrenal Crisis: Preventing and Managing Emergencies

At a Glance

An adrenal crisis is a life-threatening emergency in children with CAH caused by severe physical stress. To prevent a crisis, caregivers must double or triple oral hydrocortisone during moderate illnesses and immediately administer a Solu-Cortef injection if the child vomits or shows signs of shock.

An adrenal crisis is a life-threatening emergency that occurs when the body’s demand for cortisol far exceeds its supply [1][2]. In children with classic CAH, the adrenal glands cannot produce the “burst” of cortisol needed to survive physical stress.

Acting quickly to give stress doses or emergency injections is the single most important thing you can do to protect your child against life-threatening complications [3][4].

Why Crises Happen

When the body is under significant stress—such as from a high fever, severe injury, or surgery—it naturally requires up to three times its normal amount of cortisol [1][5]. Because your child’s adrenal glands cannot meet this demand, their blood pressure can drop dangerously low, and their heart rhythm can become unstable [6][7].

Common triggers include:

  • Infections with high fever (usually over 101°F or 38.5°C) [8][9].
  • Gastrointestinal illnesses involving vomiting or diarrhea [10][11].
  • Major physical trauma or surgery [1][5].

Recognizing the Warning Signs

A crisis can move quickly. You must act if you see any of the following “red flags” [12][2]:

  • Vomiting: This is a critical sign because the child cannot keep down their life-saving oral medication [13][14].
  • Hypoglycemia (Low Blood Sugar): Because cortisol helps regulate blood sugar, a sudden drop is a rapid-onset and dangerous feature of an impending crisis, especially in infants and toddlers. Signs include shakiness, extreme paleness, and sweating [5].
  • Extreme Lethargy: The child is unusually weak, floppy, or difficult to wake up [5][15].
  • Abdominal Pain: Intense stomach pain is a common, often overlooked sign of early crisis [12].
  • Confusion or Grayish Skin: These indicate advanced shock and require immediate action [13][16].

The Emergency Protocol: Stress Dosing

To prevent a crisis, you must follow “sick-day rules” to increase your child’s steroid levels during stress [17][18]. Note: Only hydrocortisone is increased; fludrocortisone stays at its normal dose.

1. Oral Stress Dosing

If your child has a fever or a moderate illness but is not vomiting, you will typically double or triple their normal dose of hydrocortisone for 24 to 48 hours [19][1]. Always follow the specific dosing chart provided by your endocrinologist. It is also important to offer sugar-containing fluids (like Pedialyte or juice) during illnesses to prevent hypoglycemia.

2. The Emergency Injection (Solu-Cortef)

If your child is vomiting, loses consciousness, or shows signs of shock, you must give an intramuscular (IM) injection of hydrocortisone (Solu-Cortef) immediately [20][21].

  • The 30-Minute Rule: If your child vomits within 30 to 45 minutes of taking their oral hydrocortisone, assume the medication was not absorbed and give the injection [13][22].
  • Where to Inject: Administer the injection into the large muscle of the outer thigh [23].
  • Do Not Wait: It is safer to give the injection when it might not be needed than to wait until it is too late [22][24].
  • Go to the ER: After giving the injection, your child must be evaluated in an emergency room to receive IV fluids and monitoring [3][22].

Your Safety Checklist

Preparation is the best defense against a fatal crisis.

  • Emergency Kit: Always carry an unexpired vial of Solu-Cortef, syringes, and needles [17][25].
  • Medical Alert: Your child should wear a medical alert bracelet or necklace that says: “Adrenal Insufficiency: Needs Stress Doses” [17].
  • Emergency Letter: Keep a copy of an ER instruction letter from your doctor in your diaper bag and give one to your child’s school or daycare [26][27].
  • Practice: Periodically practice the injection technique (even on an orange) so you are confident during a real emergency [23][17].

Common questions in this guide

When should I give my child with CAH an emergency Solu-Cortef injection?
You should give the injection immediately if your child is vomiting, loses consciousness, shows signs of shock, or vomits within 45 minutes of taking their oral hydrocortisone. It is always safer to give the injection when it might not be needed than to wait until it is too late.
How do I adjust my child's CAH medication when they are sick?
For a fever or moderate illness without vomiting, you will typically double or triple your child's normal oral hydrocortisone dose for 24 to 48 hours. Fludrocortisone stays at its normal dose. Always follow the specific sick-day plan provided by your endocrinologist.
What are the early warning signs of an adrenal crisis?
Early warning signs include intense abdominal pain, unexplained vomiting, extreme lethargy, and signs of low blood sugar like shakiness and pale skin. If you notice these symptoms, you must act quickly to provide stress doses of cortisol.
What do I need to keep in my CAH emergency kit?
Your emergency kit should always include an unexpired vial of Solu-Cortef (injectable hydrocortisone), syringes, needles, and a formal emergency instruction letter from your pediatric endocrinologist for the ER staff.
Do I need to give a stress dose for a common cold?
Minor illnesses, such as a common cold without a fever, typically do not require a stress dose of hydrocortisone. However, you should monitor your child closely and consult your healthcare provider if you are ever unsure.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is my child's specific weight-based dose for an emergency hydrocortisone (Solu-Cortef) injection?
  2. 2.Under exactly what circumstances should I switch from doubling the oral dose to giving the emergency injection?
  3. 3.Can you provide a formal 'emergency letter' for the ER and my child's school that explains the need for immediate treatment?
  4. 4.How often should we review the injection technique with your nursing staff to ensure I am ready to act in a crisis?
  5. 5.Which illnesses (like a cold with no fever) do not require a stress dose, and which ones always do?

Questions For You

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References

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This page provides educational information on managing adrenal crises in children with CAH. Always consult your pediatric endocrinologist for your child's specific stress dosing and emergency protocol.

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