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PubMed This is a summary of 18 peer-reviewed journal articles Updated
Endocrinology · Congenital Adrenal Hyperplasia

Emergency Preparedness and Sick Day Rules

At a Glance

People with Congenital Adrenal Hyperplasia (CAH) must manually increase their hydrocortisone medication (stress dosing) during illnesses to prevent a life-threatening adrenal crisis. If vomiting, severe trauma, or unconsciousness occurs, an emergency intramuscular injection is required immediately.

In a healthy person, the body naturally produces extra cortisol during times of illness, injury, or extreme stress to help maintain blood pressure and energy levels. Because people with Congenital Adrenal Hyperplasia (CAH) cannot make their own cortisol, they must manually provide this “stress response” using stress dosing [1][2].

Adrenal crises are serious medical emergencies, but they are highly preventable with proper stress dosing education and preparation [2].

Recognizing an Adrenal Crisis

An adrenal crisis occurs when cortisol levels are too low to support the body’s vital functions. This is a “911” emergency. Symptoms include:

  • Severe weakness or lethargy [3]
  • Vomiting and diarrhea [4][5]
  • Confusion or dizziness [3]
  • Low blood pressure (hypotension) and shock [6][7]
  • Low blood sugar (hypoglycemia), which is especially dangerous in infants and children [6][7]

Oral Stress Dosing (Sick Day Rules)

For moderate physical stress, doctors typically recommend increasing the oral dose of hydrocortisone. These are often called Sick Day Rules [8].

  • When to Stress Dose: Common triggers include a high fever (usually over 101°F or 102°F), a “flu-like” illness with significant fatigue, or major dental work [4][9].
  • Gastroenteritis (Stomach Flu): Illnesses involving diarrhea require particularly close monitoring. Diarrhea can rapidly impair the absorption of oral stress doses even before severe vomiting occurs [4][9].
  • How Much to Give: Guidelines often suggest doubling or tripling the daily dose of hydrocortisone, divided into three or four doses throughout the day [10][9].
  • Minor Stress: A minor cold, a scrape, or emotional stress typically does not require a stress dose, but you should always monitor for signs of worsening illness [5][9].

When to Use the Emergency Injection

Sometimes, oral medication is not enough. The emergency kit (such as Solu-Cortef) is not an auto-injector like an EpiPen. It typically comes as a vial that requires mixing a powder and liquid, drawing the medicine into a syringe, and administering an intramuscular (IM) shot [11][12]. This is why practical training is so absolutely necessary.

An emergency injection is required in the following situations [11][13]:

  1. Vomiting: If a routine or stress dose is vomited within 30-60 minutes, first attempt to give a replacement oral dose. The emergency injection must be used if the replacement dose is also vomited, or if persistent vomiting prevents oral intake entirely. [4][14]
  2. Major Trauma: A serious injury, such as a broken bone or a deep wound [2][9].
  3. Unconsciousness: If the person is passed out or having a seizure [3].

Every person with classic CAH must have a current, unexpired emergency injection kit at all times. After giving an injection, you must seek emergency medical care immediately [15][16].

Preparation is Key

To prevent a crisis, parents and patients should take the following steps:

  • Medical Alert ID: Wear a bracelet or necklace that says “Adrenal Insufficiency - Needs Stress Dose Hydrocortisone” [17].
  • Emergency Plan: Keep a written “Emergency Action Plan” signed by your endocrinologist to show ER staff, who may not be familiar with CAH [18].
  • Injection Training: Ensure all caregivers (including school nurses and relatives) are practically trained on how to draw up and give the emergency injection [15][12].

Common questions in this guide

What are the sick day rules for CAH?
Sick day rules involve doubling or tripling your daily oral hydrocortisone dose during moderate physical stress, such as a high fever or flu-like illness. This extra medication replaces the stress-response cortisol your body would naturally make.
When do I need to use the emergency hydrocortisone injection?
You must use the emergency injection if the patient has a major trauma, becomes unconscious, or cannot keep oral medication down due to persistent vomiting. Always seek emergency medical care immediately after giving the injection.
What are the signs of an adrenal crisis?
An adrenal crisis is a life-threatening medical emergency. Warning signs include severe weakness or lethargy, vomiting, diarrhea, confusion, low blood pressure, and low blood sugar.
Do minor illnesses require a stress dose of hydrocortisone?
Minor physical stress, like a simple cold, a small scrape, or emotional stress, typically does not require a stress dose. However, you should closely monitor the person for any signs of worsening illness.
How do I properly prepare for a CAH emergency?
Preparation includes wearing a medical alert ID, carrying an unexpired emergency injection kit at all times, keeping a written Emergency Action Plan, and ensuring all caregivers know how to administer the injection.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the specific temperature threshold (e.g., 101°F or 102°F) that should trigger a stress dose for my child?
  2. 2.Can you provide a written Emergency Action Plan that I can give to my child's school, daycare, or an ER doctor?
  3. 3.How often should we practice using the injectable hydrocortisone kit, and can you walk me through the injection technique again?
  4. 4.In what specific scenarios (e.g., a broken bone vs. a minor scrape) is an emergency injection required versus just an oral stress dose?
  5. 5.Are there specific ERs in our area that you recommend because they are familiar with CAH protocols?

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 (18)
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    Metabolic emergencies in the NICU.

    Gupta R, Joshi S, Asghar A, Gray MM

    Seminars in perinatology 2024; (48(8)):151987 doi:10.1016/j.semperi.2024.151987.

    PMID: 39428318
  2. 2

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

    Dörr HG, Wollmann HA, Hauffa BP, et al.

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

    PMID: 29884168
  3. 3

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

    Chapagain N, Adhikari A, Adhikari N, et al.

    JNMA; journal of the Nepal Medical Association 2023; (61(257)):84-86 doi:10.31729/jnma.7926.

    PMID: 37203925
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    Electrolyte abnormalities and stress dosing predict illness-related hospitalizations among infants and toddlers with congenital adrenal hyperplasia.

    Tseng T, Seagroves A, Tanawattanacharoen VK, et al.

    Clinical endocrinology 2023; (98(4)):536-542 doi:10.1111/cen.14876.

    PMID: 36593179
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    Longitudinal Assessment of Illnesses, Stress Dosing, and Illness Sequelae in Patients With Congenital Adrenal Hyperplasia.

    El-Maouche D, Hargreaves CJ, Sinaii N, et al.

    The Journal of clinical endocrinology and metabolism 2018; (103(6)):2336-2345 doi:10.1210/jc.2018-00208.

    PMID: 29584889
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    Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan.

    Ishii T, Adachi M, Takasawa K, et al.

    Hormone research in paediatrics 2018; (89(3)):166-171 doi:10.1159/000486393.

    PMID: 29455197
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    Case of Junctional Rhythm in the Setting of Acute Adrenal Insufficiency.

    Patel P, Kelschenbach K

    Cureus 2022; (14(8)):e27605 doi:10.7759/cureus.27605.

    PMID: 36059370
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    Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline.

    Speiser PW, Arlt W, Auchus RJ, et al.

    The Journal of clinical endocrinology and metabolism 2018; (103(11)):4043-4088 doi:10.1210/jc.2018-01865.

    PMID: 30272171
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    The effect of patient-managed stress dosing on electrolytes and blood pressure in acute illness in children with adrenal insufficiency.

    Chrisp GL, Torpy DJ, Maguire AM, et al.

    Clinical endocrinology 2020; (93(2)):97-103 doi:10.1111/cen.14196.

    PMID: 32301148
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    Diagnosis and management of secondary adrenal crisis.

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    The Key to Adrenal Insufficiency Education: Repetition, Repetition, Repetition.

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    PMID: 28647949
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    Corticotropic insufficiency in a monocentric prospective cohort of patients with lung cancer treated with nivolumab: Prevalence and etiology.

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This page provides general guidelines for CAH emergency preparedness and sick day rules. Always follow the specific Emergency Action Plan provided by your treating endocrinologist.

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