Children are treated with hydrocortisone, a medication that replaces the cortisol their bodies can’t make. The goal is to prevent an adrenal crisis. 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
* 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.
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 in adrenal crisis need to be treated with hydrocortisone right away and then need to be taken to the hospital emergency department immediately for more treatment.
Know the warning signs of adrenal crisis:
Children with AI should always carry:
For reference, the National Adrenal Diseases Foundation has developed Adrenal Crisis Care Medication Information.
- Video: How to Administer Hydrocortisone Injection (English; 2:19)
- Video: Como Administrar Inyección de Hidrocortisona (Spanish; 2:17)