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Endocrinology · Congenital Adrenal Hyperplasia

How is CAH stress dosing managed for fever in children?

At a Glance

For a child with classic congenital adrenal hyperplasia (CAH), a fever around 101°F (38.5°C) usually calls for stress-dose hydrocortisone under the written sick-day plan. If vomiting prevents medicine from staying down, give the prescribed emergency injection and call 911.

For children with classic congenital adrenal hyperplasia (CAH), standard Endocrine Society guidelines state that a fever typically over 101°F (38.5°C) requires stress dosing [1]. When a child has a significant fever, their body is under physical stress and needs extra cortisol to prevent a life-threatening adrenal crisis (a severe, sudden lack of cortisol that can cause shock and be fatal) [2]. The standard recommendation is usually doubling the oral dose of hydrocortisone, but you must always follow the exact instructions in your child’s written sick-day plan [2].

Managing a Fever (Sick Day Rules)

When your child’s temperature reaches the threshold—typically 101°F (38.5°C)—infections are placing stress on their body [3]. Here is how to manage it:

  • Increase the Dose: Follow your child’s written sick-day plan. Usually, this means doubling (and sometimes tripling) their normal daily hydrocortisone dose [2].
  • Give Every 8 Hours: Because the body burns through cortisol faster when sick, the stress dose is usually divided into three doses given every 8 hours [2].
  • Use Fever Reducers Wisely: Treat the underlying illness to make your child comfortable. Acetaminophen is standard. Ibuprofen can also be used, but avoid ibuprofen if your child is dehydrated or vomiting. Remember: reducing the fever does not stop the body’s stress. Do not stop stress dosing just because the fever medicine temporarily brought the temperature down.
  • Hydrate Constantly: Keep your child well-hydrated. Offer fluids frequently. Dehydration happens fast with a fever and increases the risk of a crisis.
  • Continue Other Meds: If your child takes fludrocortisone, continue it exactly as prescribed unless your endocrinologist tells you otherwise.

Note for Infants: A rectal temperature of 100.4°F (38°C) or higher in an infant under 3 months old requires immediate emergency medical evaluation, not just stress dosing [4].

When to Stop Stress Dosing

How long do you continue the doubled dose? You should follow your written plan, but generally, stress dosing continues until the fever has completely broken (without the help of fever reducers) and your child is clinically recovered, eating, and drinking normally.

Medical Emergencies: Vomiting and Adrenal Crisis

If your child is vomiting or cannot keep their oral hydrocortisone down, pills will not work [5]. This is a medical emergency.

Immediate Action for Vomiting or Severe Illness:

  1. Give the Emergency Injection: Immediately administer your child’s prescribed emergency intramuscular (IM) injection (a shot given deep into the muscle, often called Solu-Cortef) [2][6]. Do not wait for symptoms of a crisis to appear.
  2. Call Emergency Services (911): After giving the injection, seek emergency medical care immediately [2].

Signs of an Adrenal Crisis:
An adrenal crisis can happen quickly if stress dosing is missed or not absorbed. Watch for:

  • Unusual or severe weakness, fatigue, or extreme sleepiness [7]
  • Repeated vomiting or severe abdominal pain [2][8]
  • Pallor (pale skin) or fainting
  • Signs of hypoglycemia (low blood sugar), such as jitteriness or seizures [7][3]

Always keep an unexpired emergency injection kit at home, at school, and while traveling. Ensure all caregivers know how and when to use it.

Common questions in this guide

When should a child with CAH start stress dosing for a fever?
For many children with classic CAH, a fever over about 101°F (38.5°C) is the point at which stress dosing is started. Use the exact temperature threshold in the child's written sick-day plan because recommendations may be individualized.
How much hydrocortisone should a child take during a CAH fever?
Sick-day plans commonly call for doubling, and sometimes tripling, the child's usual oral hydrocortisone dose, divided into doses about every 8 hours. Use the prescribed plan rather than calculating a dose independently.
Should stress dosing continue if acetaminophen lowers the fever?
Yes. Fever reducers can lower the temperature but do not remove the body's stress from an infection, so stress dosing should not be stopped just because the fever temporarily improves. It generally continues until the fever has resolved without fever medicine and the child is recovered and eating and drinking normally, unless the written plan says otherwise.
What should I do if my child with CAH vomits hydrocortisone?
If a child is vomiting or cannot keep oral hydrocortisone down, give the prescribed emergency intramuscular hydrocortisone injection, often called Solu-Cortef, immediately. Then call 911 or seek emergency medical care without waiting for signs of adrenal crisis.
What are the warning signs of adrenal crisis in a child with CAH?
Warning signs include unusual or severe weakness, extreme sleepiness, repeated vomiting, severe abdominal pain, pale skin, fainting, or low blood sugar signs such as jitteriness or seizures. Adrenal crisis can worsen quickly and requires emergency medical care.
When can my child return to the regular hydrocortisone dose after a fever?
Stress dosing generally continues until the fever has completely broken without fever reducers and the child is clinically recovered, eating, and drinking normally. Follow the child's written plan or confirm the timing with the endocrinology team.
Is a fever in an infant under 3 months with CAH an emergency?
Yes. A rectal temperature of 100.4°F (38°C) or higher in an infant younger than 3 months requires immediate emergency medical evaluation, not just home stress dosing.

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References

References (8)
  1. 1

    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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    Management of Acute Adrenal Insufficiency-Related Adverse Events in Children with Congenital Adrenal Hyperplasia: Results of an International Survey of Specialist Centres.

    Ali SR, Bryce J, Krone NP, et al.

    Hormone research in paediatrics 2022; (95(4)):363-373 doi:10.1159/000525075.

    PMID: 35569445
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    Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life.

    Odenwald B, Nennstiel-Ratzel U, Dörr HG, et al.

    European journal of endocrinology 2016; (174(2)):177-86 doi:10.1530/EJE-15-0775.

    PMID: 26563979
  4. 4

    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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    Autoimmune Addison's disease.

    Saverino S, Falorni A

    Best practice & research. Clinical endocrinology & metabolism 2020; (34(1)):101379 doi:10.1016/j.beem.2020.101379.

    PMID: 32063488
  6. 6

    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
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    Hypoglycaemia in adrenal insufficiency.

    Lee SC, Baranowski ES, Sakremath R, et al.

    Frontiers in endocrinology 2023; (14()):1198519 doi:10.3389/fendo.2023.1198519.

    PMID: 38053731
  8. 8

    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

This page is for informational purposes only and does not constitute medical advice about your child's CAH. Follow the written sick-day plan from your child's endocrinology team, and seek emergency care for vomiting or severe illness.