Central Adrenal Insufficiency

What is central adrenal insufficiency?

Some cancer treatments and diseases change hormone levels in the body. Hormones act like chemical messages; they help glands “talk” to each other so the body can function.

If a child has central adrenal insufficiency, their body does not make enough of a hormone called cortisol. If cortisol levels go too low, the body will not function normally.

Cortisol helps the body respond to stressful events such as fever, illness, and severe injuries by:

• Keeping blood sugar normal
• Keeping blood pressure normal
• Helping the immune response

Symptoms of central adrenal insufficiency

Usually, there are no symptoms of central adrenal insufficiency. If symptoms occur, they are often mild and may include:

• Feeling tired
• Weakness
• Poor appetite
• Dizziness

Stresses to the body such as fever, infection, surgery, or injury cause more severe symptoms. They include:

• Low blood sugar
• Nausea and vomiting
• Diarrhea
• Dehydration
• Fainting

These symptoms can become life threatening and require emergency treatment right away.
 

Causes of central adrenal insufficiency

Central adrenal insufficiency can develop in 1 of 2 ways:

• The pituitary, a small gland near the brain, does not make enough adrenocorticotropic hormone (ACTH). When ACTH is too low, the adrenal glands do not make enough cortisol. This is also known as secondary adrenal insufficiency.
• A part of the brain called the hypothalamus does not make enough corticotropin-releasing hormone (CRH). When CRH is too low, the pituitary gland does not make enough ACTH. When ACTH is too low, the adrenal glands do not make enough cortisol. This is also known as tertiary adrenal insufficiency.

Central adrenal insufficiency may happen after cancer treatments that affect gland function or the brain. This is known as endocrine late effects. Some causes are:

• Radiation to the brain
• Brain injury
• Surgical removal of the pituitary gland
• Tumors near the pituitary gland, even without surgery
• Medicines such as glucocorticoids (prednisone)
• Pituitary gland disease

Central adrenal insufficiency may also develop from pituitary gland disease, or from a rare condition passed down from a child’s parents (inherited).

Another type of adrenal insufficiency is primary adrenal insufficiency or Addison disease. This rare disease is caused by damage to the adrenal glands, so they do not make enough hormones. This is not the same disease as central adrenal insufficiency.

Diagnosis of central adrenal insufficiency

Tests for central adrenal insufficiency are:

• Morning cortisol test from a blood sample to test cortisol levels
• Low-dose ACTH stimulation test to test levels of cortisol produced when ACTH is given through an IV in the arm

Treatment of central adrenal insufficiency

The treatment for central adrenal insufficiency is a medicine called hydrocortisone. It is taken daily by mouth.

The amount of medicine may change over time and depends on what your child needs. There are 3 types of doses:

• Maintenance dosing is given when your child is otherwise healthy. This dosing imitates the amount of cortisol the body makes naturally. These are low doses.
• Stress doses are higher and imitate what the body makes during stress, such as illness or injury.
• Emergency stress dosing is given when your child is too ill to take medicine by mouth. It is a shot (injection) your child needs if they have severe symptoms of an adrenal crisis. In an adrenal crisis, your child’s body does not make enough cortisol, their blood pressure drops very low, and they could have severe symptoms leading to a medical emergency. This is a life-threating emergency where your child needs their emergency stress dose of cortisol medication right away. Symptoms of adrenal crisis include:
  • Low blood pressure
  • Low blood sugar (known as hypoglycemia)
  • Feeling faint or passing out (unconscious)

Always follow your care provider’s instructions for treating central adrenal insufficiency.

Do not stop hydrocortisone suddenly. This can lead to an adrenal crisis.

People with central adrenal insufficiency should wear medical alert jewelry or carry medical alert identification. This will alert medical workers in an emergency.

Prognosis for central adrenal insufficiency

The disease may only last for a short time if caused by medicines such as hormone treatments (transient).

It can be permanent if there is injury or damage to glands or the brain. People with permanent central adrenal insufficiency will need medicine for the rest of their lives, as medicine does not cure the disease.

Overall, the prognosis for this condition is good. With proper treatment and preparation for adrenal crisis, people can live normal lives.

Tips for families

• Ask your care provider about your child’s risk for central adrenal insufficiency.
• Children who had intense radiation treatment to the central part of their brains should have morning cortisol blood levels checked yearly, or they should see a doctor who treats central adrenal insufficiency (endocrinologist).
• Tell your doctor about your child’s central adrenal insufficiency and treatment with hydrocortisone before your child gets any anesthesia, sedation, procedure, or surgery.
• Speak to your child’s primary care provider about their risks. Share a copy of your child’s survivorship care plan, which includes a treatment summary. The summary has information about their treatments and any health problems that may happen because of their treatment.
• Tell your child’s caregivers about their condition and what to do during an adrenal crisis.
• Make an emergency kit of medicine and supplies for an adrenal crisis and always keep it with your child.

Questions to ask your care team

• What is the risk that my child will get central adrenal insufficiency?
• What caused my child’s central adrenal insufficiency?
• How should I treat their central adrenal insufficiency?
• How long does my child need treatment?
• What tests does my child need to monitor their central adrenal insufficiency?
• What do I do if my child has an adrenal crisis?
• What information should I list on my child’s medical alert jewelry or identification?