Central Adrenal Insufficiency Treatment

What is central adrenal insufficiency?

Central adrenal insufficiency (also known as secondary adrenal insufficiency or ACTH deficiency) is a condition where the pituitary gland does not make enough of a hormone known as adrenocorticotropic hormone (ACTH). This may be due to:

• A brain tumor near the pituitary gland
• Radiation treatment to the brain
• Pituitary gland disease
• Some medicines

When the pituitary gland releases ACTH, it causes the adrenal glands to make another hormone called cortisol. Cortisol helps the body respond to stress by:

• Keeping blood pressure normal
• Keeping blood sugar normal
• Helping the immune system function correctly

Patients with central adrenal insufficiency do not make enough cortisol, so their bodies do not function normally.

Symptoms of central adrenal insufficiency

The symptoms of central adrenal insufficiency may start slowly and develop over time, such as:

• Feeling tired and not able to keep up with others
• Abdominal (belly) pain in the morning
• Loss of appetite
• Nausea and vomiting
• Weight loss

More severe symptoms can be a life-threatening emergency. This is known as an adrenal crisis. Symptoms of an adrenal crisis include:

• Low blood pressure
• Low blood sugar (known as hypoglycemia)
• Feeling faint or passing out (unconscious)

Treatment of central adrenal insufficiency

Patients with central adrenal insufficiency do not make enough of the hormone cortisol. Care providers give the medicine hydrocortisone to replace the missing cortisol. Patients need to take hydrocortisone for the rest of their lives, as it does not cure the disease.

Your child will need to take the right amount of hydrocortisone. This amount can change over time. There are 3 different hydrocortisone dosing procedures, depending on what your child needs:

• Maintenance dosing is given when your child is otherwise healthy. The medicine is given 2–3 times per day and mimics what the body would produce naturally. These are low doses.
• Stress doses are higher and mimic the body’s response to stressors, such as illness or injury. Give stress dosing at the first signs of an illness or injury. The stress dose is usually about 3 times more than the maintenance dose, but always follow your care team’s instructions.
  
• Emergency stress dosing is given when your child is too ill to take medicine by mouth. Give your child the shot (injection) and seek medical help right away. Your child should wear a medical alert bracelet that states they have adrenal insufficiency and need steroids for an emergency. You should also prepare a kit in advance with medical supplies for your child. See Emergency Stress Dose for Adrenal Insufficiency for how to make this kit. 

More severe symptoms of an adrenal crisis can be a life-threatening emergency. Symptoms of an adrenal crisis include:

• Low blood pressure
• Low blood sugar (known as hypoglycemia)
• Feeling faint or passing out (unconscious)

If your child has the more severe symptoms of an adrenal crisis, see the information on how to give an Emergency Stress Dose for Adrenal Insufficiency and get medical help right away.

Tell your health care provider about your child’s use of hydrocortisone, especially before they get any anesthesia, sedation, procedure, or surgery. Your health care team should contact the St. Jude Endocrine Clinic to find out if your child needs a stress dose by IV.

How to give maintenance doses of hydrocortisone (Cortef^®)

Hydrocortisone (Cortef^®) is available as 5-mg, 10-mg, and 20-mg white tablets. Your child can take hydrocortisone with or without food. It is also available as a liquid you can inject into a muscle in an emergency. Always follow the instructions given by your care team.

Give the maintenance dose that your care provider prescribes.

The maintenance dose mimics the amount of cortisol normally made by the body. Give the prescribed strength of tablet(s) of the hydrocortisone maintenance dose as follows:

When to give stress doses of hydrocortisone (Cortef^®)

The body makes more cortisol during illness or injury. Your child needs higher doses of hydrocortisone to mimic what the body produces during these stressful times. Give stress dosing for these symptoms:

• Fever:
  • Mouth (oral) temperature of 101°F (38.3°C) or greater
  • Underarm temperature of 101.6°F (38.7°C) or greater
  • Rectal temperature of 100.4°F (38°C) or greater
• Illness such as strep throat, ear infection, bronchitis, pneumonia
• Appears ill with a possible viral illness, such as cold symptoms or runny nose
• Illness with vomiting and diarrhea
• Injuries such as sprains or strains

Give the prescribed stress dose of hydrocortisone every 8 hours.

Stress doses of hydrocortisone can hide a fever. After having a fever, your child needs a medical exam to check for an infection and get treatment. Do not give stress doses for more than 3 days without seeing your health care provider.

Reminders about dosing

• There are 3 types of dosing: maintenance, stress, and emergency stress dosing for adrenal crisis.
• Caregivers should:
  • Understand when and how to give maintenance doses and stress doses.
  • Know the more severe symptoms of an adrenal crisis, be able to give an emergency stress injection, and get medical help right away. See Emergency Stress Dose for Adrenal Insufficiency for more details.
• Have your child wear a medical alert bracelet or necklace stating that they have adrenal insufficiency (see Medical Alert Bracelets and Medical ID Products).

How to plan for medical procedures

For any medical procedure, especially those using sedation or anesthesia, remind your care provider that you take hydrocortisone. Your child may need stress dosing before, during, or after the procedure.

The care provider should contact the St. Jude endocrinologist on-call to discuss whether your child needs stress dosing and the correct dose.