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PubMed This is a summary of 28 peer-reviewed journal articles Updated
Endocrinology

Daily Treatment and Stress Dosing for CAH

At a Glance

Congenital Adrenal Hyperplasia requires daily hydrocortisone to replace missing cortisol. During illness, fever, or injury, patients must "stress dose" by increasing their medication. An emergency injection is critical to treat severe vomiting or a life-threatening adrenal crisis.

Managing Classic Congenital Adrenal Hyperplasia (CAH) is a daily commitment to balancing your body’s chemistry. Because the adrenal glands cannot produce enough cortisol (the “stress hormone”) and often cannot produce aldosterone (the “salt-retaining hormone”), treatment focuses on replacing these missing pieces [1][2].

Daily Medication: The Maintenance Plan

For most children and adults with Classic CAH, the goal of daily treatment is threefold: to prevent an adrenal crisis, to keep male-type hormones (androgens) in a healthy range, and to support normal growth and development [3][4].

  • Glucocorticoids (Hydrocortisone): This is the replacement for cortisol. In children, hydrocortisone is the preferred choice because it is short-acting and less likely to interfere with growth [3][5]. It is typically taken three times a day to try and mimic the body’s natural rhythm [5][6].
  • Mineralocorticoids (Fludrocortisone): For those with the “salt-wasting” form, this medication replaces aldosterone [7]. It helps the kidneys hold onto salt and maintain healthy blood pressure [4].
  • Salt Supplements: Infants, in particular, may need extra sodium (salt) added to their formula or breast milk because their kidneys are still learning how to manage salt balance [8].

The Balancing Act: Your medical team will monitor hormone levels (like 17-OHP and androstenedione) and growth charts closely [9][4]. Too little medication can lead to early puberty or “virilization,” while too much can lead to weight gain, high blood pressure, or slowed growth [10][11].

Stress Dosing: Preparing for “Extra” Needs

In a person without CAH, the body naturally floods itself with cortisol during times of physical stress—such as a high fever, a broken bone, or surgery [12]. Because a person with CAH cannot make this extra cortisol, they must provide it through stress dosing [13][14].

When to Stress Dose:
Generally, “stress dosing” means doubling or tripling the usual oral dose of hydrocortisone for a short period [13][15]. Guidelines often recommend stress dosing for:

  • Fevers (usually over 101°F or 38.5°C) [13].
  • Significant injury (like a broken bone) or surgery [12].
  • Severe illness where the person is “acting sick” or listless [15].
  • Note: Stress doses are typically not needed for minor “sniffles,” small scrapes, or emotional stress [13].

The Adrenal Crisis: A Medical Emergency

An adrenal crisis occurs when the body’s demand for cortisol far exceeds the supply. This is a life-threatening emergency that requires immediate action [16][17].

Signs of an Adrenal Crisis:

  • Persistent vomiting that prevents oral medication from being absorbed [18].
    (Sick day rule: If you or your child vomits within 30-45 minutes of taking a routine or stress dose, attempt to repeat the oral dose. If vomiting continues and the repeated dose cannot be kept down, this is considered persistent vomiting and requires immediate action [18][13].)
  • Extreme lethargy or difficulty waking up [19].
  • Confusion or altered mental state [16].
  • Signs of shock (pale skin, rapid heart rate, or very low blood pressure) [19][20].

Your Emergency Toolkit

Every person with Classic CAH must have an emergency plan in place to stop a crisis before it becomes fatal [21][22].

  1. Emergency Injection: If a person is persistently vomiting or unconscious, they cannot take oral pills. You must be trained to give an intramuscular injection of hydrocortisone (often called a Solu-Cortef Act-O-Vial) [13][23]. This injection buys valuable time while you head to the emergency room [24][25].
  2. Medical Alert ID: A bracelet or necklace that says “Adrenal Insufficiency: Requires Hydrocortisone” is essential for first responders [23][26].
  3. Emergency Letter: Carry a letter from your endocrinologist that explains CAH and specifies the emergency treatment needed (including IV fluids and high-dose steroids) [27][13].
  4. Travel Preparedness: When traveling, always keep your emergency injection and daily medications in a carry-on bag, not checked luggage. Bring a doctor’s note explicitly stating the medical necessity of carrying injectable medications and needles through airport security [27].

Education is the most powerful tool in preventing a crisis. Almost all complications from adrenal crises are avoidable with prompt stress dosing and emergency injections [21][28].

Common questions in this guide

When do I need to stress dose for CAH?
You generally need to double or triple your normal hydrocortisone dose during physical stress, such as a fever over 101 degrees Fahrenheit, a significant injury, surgery, or severe illness. You typically do not need to stress dose for minor sniffles or emotional stress.
What are the signs of an adrenal crisis?
Signs of a life-threatening adrenal crisis include persistent vomiting, extreme lethargy, confusion, and signs of shock like pale skin, rapid heart rate, or low blood pressure. If these occur, immediate emergency medical treatment is required.
How do I manage CAH daily?
Daily management involves taking glucocorticoids like hydrocortisone to replace missing cortisol. Those with the salt-wasting form also take fludrocortisone and sometimes salt supplements to help the body maintain healthy sodium levels and blood pressure.
What should be in my CAH emergency toolkit?
Your emergency toolkit must include an injectable emergency dose of hydrocortisone, often called Solu-Cortef. You should also carry a medical alert ID and an emergency letter from your endocrinologist detailing your required treatment protocols for first responders.
What happens if my child's daily CAH medication dose is incorrect?
If the daily hormone dose is too low, it can lead to early puberty or virilization from excess androgens. If the dose is too high, it may cause unnecessary weight gain, high blood pressure, or stunted growth.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Can you provide us with a written, step-by-step Adrenal Crisis Action Plan?
  2. 2.What is the specific 'stress dose' for my/my child's current weight, and at what temperature/symptom should we start it?
  3. 3.Can you walk me through a practice session for giving the emergency hydrocortisone injection?
  4. 4.How will we know if the daily dose of hydrocortisone is too high (causing growth issues) or too low (allowing androgen excess)?
  5. 5.Do we need to add salt supplements to my baby's diet in addition to fludrocortisone?

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 (28)
  1. 1

    Steroid 21-hydroxylase deficiency in congenital adrenal hyperplasia.

    Parsa AA, New MI

    The Journal of steroid biochemistry and molecular biology 2017; (165(Pt A)):2-11 doi:10.1016/j.jsbmb.2016.06.015.

    PMID: 27380651
  2. 2

    Congenital adrenal hyperplasia with salt-wasting crisis and arrhythmia: a case study.

    Canlas JF, Ponmani C

    BMJ case reports 2019; (12(1)) doi:10.1136/bcr-2018-227565.

    PMID: 30700462
  3. 3

    Hydrocortisone dosing in children with classic congenital adrenal hyperplasia: results of the German/Austrian registry.

    Hoyer-Kuhn H, Huebner A, Richter-Unruh A, et al.

    Endocrine connections 2021; (10(5)):561-569.

    PMID: 33909597
  4. 4

    Treatment and Follow-up of Congenital Adrenal Hyperplasia Due to 21-hydroxylase Deficiency in Childhood and Adolescence

    Peltek Kendirci HN, Ünal E, Dündar İ, et al.

    Journal of clinical research in pediatric endocrinology 2025; (17(Suppl 1)):12-22 doi:10.4274/jcrpe.galenos.2024.2024-6-26-S.

    PMID: 39713876
  5. 5

    Practice Variation among Pediatric Endocrinologists in the Dosing of Glucocorticoids in Young Children with Congenital Adrenal Hyperplasia.

    Al-Rayess H, Lahoti A, Simpson LL, et al.

    Children (Basel, Switzerland) 2023; (10(12)) doi:10.3390/children10121871.

    PMID: 38136073
  6. 6

    PERSPECTIVE: Treatment with hydrocortisone modified-release capsules in children and adolescents with congenital adrenal hyperplasia: an expert opinion.

    Neumann U, Blankenstein O, Claahsen-van der Grinten HL

    Endocrine connections 2025; (14(5)).

    PMID: 40094399
  7. 7

    Blood Pressure in a Large Cohort of Children and Adolescents With Classic Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency.

    Bonfig W, Roehl FW, Riedl S, et al.

    American journal of hypertension 2016; (29(2)):266-72 doi:10.1093/ajh/hpv087.

    PMID: 26071487
  8. 8

    Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose.

    Bonfig W, Roehl F, Riedl S, et al.

    Hormone research in paediatrics 2018; (89(1)):7-12 doi:10.1159/000481775.

    PMID: 29073619
  9. 9

    Analysis of therapy monitoring in the International Congenital Adrenal Hyperplasia Registry.

    Lawrence N, Bacila I, Dawson J, et al.

    Clinical endocrinology 2022; (97(5)):551-561 doi:10.1111/cen.14796.

    PMID: 35781728
  10. 10

    Epidemiology and Long-Term Adverse Outcomes in Korean Patients with Congenital Adrenal Hyperplasia: A Nationwide Study.

    Kim JH, Choi S, Lee YA, et al.

    Endocrinology and metabolism (Seoul, Korea) 2022; (37(1)):138-147 doi:10.3803/EnM.2021.1328.

    PMID: 35255606
  11. 11

    Cognitive impairment in adolescents and adults with congenital adrenal hyperplasia.

    Karlsson L, Gezelius A, Nordenström A, et al.

    Clinical endocrinology 2017; (87(6)):651-659 doi:10.1111/cen.13441.

    PMID: 28771762
  12. 12

    Prevention of Adrenal Crisis: Cortisol Responses to Major Stress Compared to Stress Dose Hydrocortisone Delivery.

    Prete A, Taylor AE, Bancos I, et al.

    The Journal of clinical endocrinology and metabolism 2020; (105(7)) doi:10.1210/clinem/dgaa133.

    PMID: 32170323
  13. 13

    Treatment and Prevention of Adrenal Crisis and Family Education

    Çamtosun E, Sangün Ö

    Journal of clinical research in pediatric endocrinology 2025; (17(Suppl 1)):80-92 doi:10.4274/jcrpe.galenos.2024.2024-6-12-S.

    PMID: 39713905
  14. 14

    Adrenal crisis in infants and young children with adrenal insufficiency: Management and prevention.

    Bizzarri C, Capalbo D, Wasniewska MG, et al.

    Frontiers in endocrinology 2023; (14()):1133376 doi:10.3389/fendo.2023.1133376.

    PMID: 36860362
  15. 15

    Daily adjustment of glucocorticoids by patients with adrenal insufficiency.

    Schöfl C, Mayr B, Maison N, et al.

    Clinical endocrinology 2019; (91(2)):256-262 doi:10.1111/cen.14004.

    PMID: 31050815
  16. 16

    The progression of salt-wasting and the body weight change during the first 2 weeks of life in classical 21-hydroxylase deficiency patients.

    Gau M, Konishi K, Takasawa K, et al.

    Clinical endocrinology 2021; (94(2)):229-236 doi:10.1111/cen.14347.

    PMID: 33001476
  17. 17

    [Adrenal crisis in a child].

    Holmberg NH, Haagensen AL

    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 2023; (143(2)) doi:10.4045/tidsskr.22.0354.

    PMID: 36718904
  18. 18

    Variations in the management of acute illness in children with congenital adrenal hyperplasia: An audit of three paediatric hospitals.

    Chrisp GL, Maguire AM, Quartararo M, et al.

    Clinical endocrinology 2018; (89(5)):577-585 doi:10.1111/cen.13826.

    PMID: 30086199
  19. 19

    Conduct protocol in emergency: Acute adrenal insufficiency.

    Fares AB, Santos RA

    Revista da Associacao Medica Brasileira (1992) 2016; (62(8)):728-734 doi:10.1590/1806-9282.62.08.728.

    PMID: 27992012
  20. 20

    Adrenal crisis during pregnancy: Case report and obstetric perspective.

    Gardella B, Gritti A, Scatigno AL, et al.

    Frontiers in medicine 2022; (9()):891101 doi:10.3389/fmed.2022.891101.

    PMID: 36186806
  21. 21

    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
  22. 22

    Utilizing health information technology to improve the recognition and management of life-threatening adrenal crisis in the pediatric emergency department: medical alert identification in the 21st century.

    Halpin KL, Paprocki EL, McDonough RJ

    Journal of pediatric endocrinology & metabolism : JPEM 2019; (32(5)):513-518.

    PMID: 31042645
  23. 23

    Adrenal insufficiency.

    Hahner S, Ross RJ, Arlt W, et al.

    Nature reviews. Disease primers 2021; (7(1)):19 doi:10.1038/s41572-021-00252-7.

    PMID: 33707469
  24. 24

    Emergency treatment of adrenal crisis with prednisone suppositories: a bioequivalence study in female patients with Addison's disease.

    Burger-Stritt S, Bachmann L, Kurlbaum M, Hahner S

    Endocrine connections 2019; (8(4)):425-434.

    PMID: 30952111
  25. 25

    Adrenal Insufficiency in Adults: A Review.

    Vaidya A, Findling J, Bancos I

    JAMA 2025; (334(8)):714-725 doi:10.1001/jama.2025.5485.

    PMID: 40522647
  26. 26

    Guidance for the prevention and emergency management of adult patients with adrenal insufficiency.

    Simpson H, Tomlinson J, Wass J, et al.

    Clinical medicine (London, England) 2020; (20(4)):371-378 doi:10.7861/clinmed.2019-0324.

    PMID: 32675141
  27. 27

    Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings.

    Miller BS, Spencer SP, Geffner ME, et al.

    Journal of investigative medicine : the official publication of the American Federation for Clinical Research 2020; (68(1)):16-25 doi:10.1136/jim-2019-000999.

    PMID: 30819831
  28. 28

    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

This page explains daily management and emergency stress dosing for CAH for educational purposes. Always consult your endocrinologist for your specific medication dosages and a personalized adrenal crisis action plan.

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