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Cortisol Replacement Therapy

What is Adrenal Insufficiency?

Adrenal insufficiency is a condition in which the adrenal glands (located on top of each kidney) are unable to produce the right amount of important hormones. These hormones include cortisol and aldosterone. They help keep blood pressure and blood sugar normal, as well as maintain salt and water balance. These hormones are especially important when the body needs to fight an infection or recover from a broken bone or other illness. Adrenal insufficiency can be life threatening if not properly managed. 

What is Hypopituitarism?

Hypopituitarism is the decrease or absence of one or more of the pituitary hormones. The lack of all anterior and posterior pituitary hormones is know as panhypopituitarism. The pituitary is a small gland located at the base of the brain and is controlled by the hypothalamus. It is referred to as the "master gland" because its chemical messengers or hormones signal other endocrine glands to produce their own hormones. The anterior or front, of the pituitary produces hormones that are needed for normal body functions. These hormones include:

  • Growth Hormone (GH)
  • Adrenocorticotropic Hormone (ACTH) – Normally this hormone stimulates the adrenal gland (located on top of the kidneys) to produce cortisol. If the pituitary gland does not produce ACTH then cortisol will not be produced. Cortisol keeps the body's blood sugar at a normal level and helps the body deal with physical stress such as fever or injury.
  • Thyroid Stimulating Hormone (TSH)
  • Gonadotropins or sex hormones (LH/FSH)

The posterior, or back, of the pituitary produces:

  • Andtidiuretic Hormone (ADH)
  • Oxytocin

What is CAH?

Congenital Adrenal Hyperplasia (CAH) is a group of inherited disorders that affects the adrenal glands. It is caused by a deficiency of an enzyme needed for the adrenal glands to function properly. There are two main forms of the disorder: Classical CAH and Non-Classical CAH.

Classical CAH is a potentially life-threatening disorder that requires lifelong medical management. Early diagnosis and proper medical treatment help individuals lead normal, healthy and productive lives. Classical CAH occurs in 1 in 15,000 individuals worldwide.

Non-Classical CAH (NCAH), or late-onset CAH, is a milder form of CAH. It is not life-threatening but may require lifelong medical management. In some people, this hormone imbalance can result in: premature puberty, rapid growth in childhood with ultimate short stature, hirsutism (excessive hair growth), oily hair and skin, severe cystic acne, polycystic ovary syndrome (unwanted body hair, irregular menstrual periods), and infertility in males and females. NCAH affects 1 in 100 to 1 in 1,000 in the general population.

Cortisol Replacemet Therapy Booklet (English)

Cortisol Replacement Therapy Booklet (Spanish) – Terapia De Reemplazo De Cortisol

Stress Dosing CARES Foundation – Stress Dosing – Hydrocortisone Injection

Your child's body does not make enough of certain essential hormones: cortisol, and in salt wasting CAH, aldosterone. Cortisol, which is produced by the adrenal glands, has many purposes in the body such as maintaining energy supply, maintaining fluid and electrolyte balance, maintaining blood pressure, maintaining normal blood sugars levels and controlling the body's reaction to physical stress. Aldosterone is used by the kidneys to maintain a normal blood sodium level and fluid balance (salt and water). When corisol and aldosterone are not produced by the body, they must be replaced by medication.

Extra hydrocortisone must be given during times of extreme physical stress such as fever, vomiting, and diarrhea, surgery, and traumatic injuries such as broken bones and concussions. The Florinef dose does not change. The extra hydrocortisone is called a "stress dose". Make sure you discuss stress dosing with your child's physician and you know how he/she would like you to proceed in the event of an illness or injury.

If your child becomes ill, call the child's physician to alert him/her of the child's condition. Typically, stress dosing would require these actions:

Fever greater than 100.5: DOUBLE the hydrocortisone dose for the entire day

Fever greater than 102: TRIPLE the hydrocortisone dose for the entire day

Vomiting or Diarrhea: if your child vomits, wait 30 minutes and repeat the dose (double or triple according to child's needs). If your child still vomits that oral dose, you need to give the injectable hydrocortisone and contact the child's physician. DO NOT DELAY in giving the injectable hydrocortisone. Offer small amounts of clear liquids that contain SUGAR frequently, at least 1 ounce every 15 minutes. Injection may also be needed in the event of diarrhea due to loss of fluids. Remember that children tend to get sicker at night, so if in doubt, stress dose before bedtime.

Watch for signs of acute adrenal crisis from cortisol deficiency

  • Headache
  • Nausea
  • Abdominal pain
  • Confusion
  • Pale Skin
  • Listlessness
  • Dehydration
  • Dizziness

If these occur and continue after stress dosing, call the child's physician and take child to the nearest emergency room immediately.

Again, DO NOT WAIT to give the injectable hydrocortisone. It should be given BEFORE a trip to the emergency room or activation 911 if those actions become necessary.

How to give an injection of hydrocortisone:

  1. Stay calm.  Wash your hands and gather equipment:  needle, syringe, alcohol pad, and vial of hydrocortisone (Solu-Cortef Mix-O-Vial).
  2. Mix the medication by pushing down on top of the vial to release the cork into the vial.
  3. Shake the vial to mix medication, take off the top of the vial, and wipe down the rubber stopper with alcohol.
  4. Take the cap off the syringe needle and insert into the vial through the rubber stopper.
  5. Draw up the medication and replace the needle cap.  Know how much you child's dose is in mg and how many ml it is of the hydorcortisone.
  6. Select the site for the intrmuscular injection typically the outer portion in the middle of the thigh.
  7. Use the alcohol to clean the skin at the injection site.
  8. Take the cap off the needle and hold the syringe like a dart.
  9. Using your thumb and first two fingers, spread the skin and push down lightly.
  10. Dart the needle into the thigh, going at a 90 degree angle.
  11. Hold the syringe in place and pull back the plunger to make sure you don't see blood, meaning you are in a blood vessel. If you do (which would be rare), withdraw syringe and discard. Prepare another syringe with medication and inject in a slightly different site. (However, if this is the only dose, continue with the same syringe, injecting in a slightly different site).
  12. After injecting the medication, place tissue or cotton ball near the needle and pull the needle out quickly.
  13. Place the needle and syringe in a hard, unbreakable container.
  14. Call Doctor / 911 or go to hospital if necessary.

 Cortisol Dependent School Instructions

MEDICAL ALERT CARD – Adrenal Insufficiency and Steroid Dependent