AI Treatment

Group 8
The gold standard of treating AI is to give enough hydrocortisone to replace the missing cortisol in adrenal insufficiency and in addition fludrocortisone to replace aldosterone in some cases.

Children are treated with hydrocortisone, a medication that replaces the cortisol their bodies can’t make. The goal of replacing glucocorticoids is to help children achieve normal growth, appropriate bone health, healthy brain development, and reproductive maturation. The goal for children with CAH is also to suppress the androgen hormones that cause virilization, while allowing for normal growth and development. Children with nonclassic CAH who do not show symptoms do not need cortisol replacement therapy.

A 2015 survey* of healthcare providers found that 1 mg or 2 mg were the most common doses of hydrocortisone prescribed for infants and newborns.1 Many caregivers have to crush or split tablets to get the dose prescribed.

In a 2018 study, researchers concluded that there is risk of under- or over-dosing children due to imprecise dosing as a result of manipulating tablets.2

Pediatric Endocrine Society Guidelines on Primary Adrenal Insufficiency recommend that children are given hydrocortisone in two or three divided doses per day, with different doses at different times to mimic the body’s natural rhythm.

* Online, 11-question survey of 1200 members of the European Society for Pediatric Endocrinology. 67 respondents; response rate about 5.5%.
27 parents/caregivers recruited for observation from pediatric endocrinology clinics at Birmingham Children’s Hospital in the UK. 30 inexperienced participants recruited for observation from University of Birmingham in the UK.
48 Bornstein p. 374-375

Children with classic salt-wasting CAH do not make enough aldosterone. Fludrocortisone is prescribed to replace this missing hormone. In the first year of life infants also may need treatment with salt which later in life may not be needed as children can have salty foods. Fludrocortisone replaces the aldosterone they lack, which helps prevent the loss of salt and stabilizes blood pressure.

Stress Dosing (Sick Day Dosing)

When Children with AI experience physical stress for example infection, diarrhea, trauma, burns, or surgery, they require extra cortisol that they are unable to make. They need additional doses of hydrocortisone, called stress dosing, to recover from illness or stressful situations like surgery. Families need to have a good understanding of stress dosing to prevent the serious complications of adrenal crisis.

Talk to your doctor about a stress dosing plan tailored to your child’s needs. Each situation is different and there is no “one size fits all” approach. As a reference, the National Adrenal Diseases Foundation has developed an Adrenal Insufficiency Patient Guide to Management of Illness and Stress-Related Medical Events for Children.

Adrenal Crisis

Physical stress caused by illness, infection, surgery, or an accident can suddenly make symptoms of AI much worse. This emergency illness is called an adrenal crisis, acute adrenal insufficiency, or Addisonian crisis.

The severe lack of cortisol at these times can cause life-threatening low blood pressure, low blood glucose, low blood sodium, and high blood potassium. If left untreated, adrenal crisis can cause death.

Children with adrenal crisis need to be treated with an immediate injection of hydrocortisone and transported to the hospital emergency department for additional treatment.

Know the warning signs of adrenal crisis:

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    Severe weakness

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    Low blood pressure and fainting

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    Abdominal pain, nausea, vomiting

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    Back pain

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Children with adrenal crisis might also experience seizures due to low blood sugar (hypoglycemia). Talk with your doctor about how to prevent an adrenal crisis.

Children with AI should always carry:

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    Steroid emergency card

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    Medical alert identification

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    Injection kit

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