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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Endocrinology · Classic Salt-Wasting Congenital Adrenal Hyperplasia

Emergency Management: Sick Day Rules and Adrenal Crises

At a Glance

Emergency management is critical for children with classic salt-wasting CAH. Caregivers must use sick day rules to increase oral hydrocortisone during illnesses, and administer a Solu-Cortef emergency injection for severe vomiting, lethargy, or major trauma to prevent a fatal adrenal crisis.

In a healthy body, the adrenal glands automatically pump out extra cortisol during times of physical stress, like a high fever or a broken bone. Because a child with Classic Salt-Wasting CAH cannot make their own cortisol, you must act as their “external adrenal gland” during these times [1][2].

Failure to provide extra medication during stress is the leading cause of death in children with CAH [3]. Emergency management is the most critical skill you will learn as a CAH parent.

What is an Adrenal Crisis?

An adrenal crisis is a life-threatening emergency where the body’s systems (like blood pressure and heart rate) begin to shut down because there isn’t enough cortisol to keep them running [1][4].

Common Triggers include:

  • Illness: Especially stomach bugs (vomiting/diarrhea) and high fevers [5][6].
  • Trauma: Major injuries like a broken bone or a deep wound [7].
  • Surgery: Any procedure requiring anesthesia [7][8].

Warning Signs of a Crisis:
Early signs can be subtle, like extreme fatigue, nausea, or “not acting like themselves” [6]. As the crisis worsens, it can lead to vomiting, severe dehydration, low blood pressure (shock), and loss of consciousness [9][10].

The “Sick Day Rules” for Oral Medication

When your child is ill but not vomiting, you will follow “Sick Day Rules” to prevent a crisis from starting. This involves stress dosing, which means giving more than the usual daily amount of oral hydrocortisone [7][11].

  • Mild Stress (Fever >101°F, minor illness): Typically, you will double or triple the normal oral dose of hydrocortisone for the duration of the illness [7][12].
  • Duration: Continue the stress doses until your child is back to their normal self. You do not need to “taper” or slowly lower the dose back down for short-term sick days [13].
  • Salt: If your child is sweating heavily or has diarrhea, they may also need extra salt or electrolyte drinks to prevent salt-wasting [14][15].

When to Use the Emergency Injection

An intramuscular (IM) injection of hydrocortisone (often called Solu-Cortef) is required when your child cannot absorb oral medication or when their body needs cortisol instantly [7][8]. The injection is typically given in the thick muscle of the outer thigh (vastus lateralis) [16].

You must give the injection if your child:

  1. Vomits Repeatedly: If your child spits up or vomits a dose, you typically wait 15-30 minutes and try to give the oral dose one more time. If they vomit a second time, their body is not absorbing the medication, and you must give the injection [7].
  2. Is Unconscious or Severely Lethargic: If they cannot be woken up or are too weak to swallow [9][16].
  3. Experiences Major Trauma: Such as a car accident or a serious fall with potential internal injuries [7].

After giving the injection, you must call 911 or go to the nearest Emergency Room immediately. The injection buys you time, but the child still needs medical evaluation and likely IV fluids [8][17].

Your Safety Toolkit

You should never be more than a few minutes away from your child’s life-saving supplies.

  • Emergency Injection Kit: This should contain a vial of injectable hydrocortisone, syringes, needles, and alcohol wipes [16][18].
  • Medical Alert ID: Your child should wear a bracelet or necklace at all times stating: “Adrenal Insufficiency: Needs Stress Doses/Emergency Injection” [16][8].
  • Emergency Letter: Carry a letter from your endocrinologist that explains CAH and provides specific instructions for ER doctors, who may not be familiar with the condition [16][19].
  • Education: Ensure all caregivers—including teachers and grandparents—know how to recognize a crisis and give the injection [16][18].

Return to Home Page

Common questions in this guide

What is an adrenal crisis in a child with CAH?
An adrenal crisis is a life-threatening emergency that occurs when the body lacks enough cortisol to function properly during physical stress or illness. Without enough cortisol, a child can experience severe dehydration, dangerously low blood pressure, and loss of consciousness.
When should I use the Solu-Cortef emergency injection?
You must give the emergency hydrocortisone injection if your child vomits a medication dose twice, becomes unconscious or severely lethargic, or experiences major trauma. After giving the injection, immediately call 911 or go to the nearest emergency room.
How do sick day rules work for CAH?
Sick day rules involve giving 'stress doses' of oral hydrocortisone—usually doubling or tripling the normal amount—when your child has a fever over 101°F or a minor illness. You continue these higher doses until your child is acting normally again.
What should I keep in my child's CAH emergency kit?
Your safety toolkit should always be nearby and include a vial of injectable hydrocortisone, syringes, needles, alcohol wipes, and an emergency letter from your endocrinologist. Additionally, your child should always wear a medical alert ID bracelet or necklace.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the exact dose of injectable hydrocortisone (Solu-Cortef) my child needs right now based on their current weight?
  2. 2.Can you or a nurse demonstrate exactly how to give an emergency hydrocortisone injection in the thigh and let me practice it today?
  3. 3.At what exact temperature should I automatically start stress dosing my child?
  4. 4.If my child has a minor injury like a scraped knee, do they need a stress dose, or is that only for major trauma?
  5. 5.Can you review my child's emergency kit with me to ensure I have all the necessary supplies and that nothing is expired?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (19)
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    Assessment of medication adherence in children and adults with congenital adrenal hyperplasia and the impact of knowledge and self-management.

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    Clinical endocrinology 2021; (94(5)):753-764 doi:10.1111/cen.14398.

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    Mortality in children with classic congenital adrenal hyperplasia and 21-hydroxylase deficiency (CAH) in Germany.

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    BMC endocrine disorders 2018; (18(1)):37 doi:10.1186/s12902-018-0263-1.

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    Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia.

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    PMID: 34283908
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    Longitudinal Assessment of Illnesses, Stress Dosing, and Illness Sequelae in Patients With Congenital Adrenal Hyperplasia.

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    Acute Gastroenteritis Induced Adrenal Crisis in a patient with Congenital Adrenal Hyperplasia: A Case Report.

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This page provides educational information on managing CAH emergencies and adrenal crises. Always follow your endocrinologist's specific sick day rules, stress dosing protocols, and emergency injection instructions for your child.

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