ADRENAL CRISIS and STRESS DOSING:
ADRENAL CRISIS
Adrenal Crisis is the nightmare of every CAH parent and CAH affected individual. It must be treated promptly and sufficiently. Anytime a CAH affected child or adult is ill, signs of adrenal crisis must be watched for. Salt-wasters must be particularly careful, but simple virilizers can suffer from adrenal crisis as well.
Nonclassicals who are being treated with glucocorticoids must also be watchful under times of extreme physical stress. While hypothetically treated nonclassicals can have adrenal crisis, it is extremely rare. However, it is better to be safe than sorry, so nonclassicals wear Medical ID bracelets and receive stress dosing as prescribed by their doctor when under serious physical stress or when ill with a high fever. Those with NCCAH who are not taking glucocorticoid therapy need not be concerned with adrenal crisis.
Attached below are two equally important documents for you to have on hand.
Adrenal Crisis Emergency Letter:
The first item is a form of an adrenal crisis emergency letter. Every parent and affected adult should have such a letter available. If you do not, please print this out and bring it to your pediatric endocrinologists or endocrinologists. They can follow the format set forth in the form. Ask them to write it out on their letterhead, sign it and give you 3 originals: one for home, one for the car and one for school/day care. Don’t leave the doctor’s office without it! And, ask them to show you how to give an injection so that when the time comes that you may need to give your child the solu-cortef, you will not feel uncomfortable giving the shot. Children can go from merely feeling somewhat unwell to adrenal crisis very quickly. Ask your doctor to carefully explain the warning signs to you.
When your child needs to be taken to the emergency room, bring the letter with you! One child memorialized on the CARES Foundation Memory page died in the emergency room because the doctors there would not administer the IV glucocorticoids without approval from the endocrinologist, and they could not reach the endocrinologist. A letter with the doctor’s signature should help prevent these kinds of tragedies.
CARES recommends laminating the letter to preserve it. Click here for printable form of letter
Adrenal Insufficiency Booklet from NIH:
The National Institutes of Health have published a terrific booklet on all aspects of adrenal insufficiency. CARES strongly recommends that all parents and affected adults print out this pdf file and review it carefully. It contains step by step instructions (with pictures) on how to deal with an adrenal crisis and give solu-cortef injections. It is an important document to keep on hand along with the adrenal crisis
emergency instruction letter. Click here to download NIH adrenal Insufficiency Booklet.
Also, CARES Foundation, Inc strongly recommends wearing a MedicAlert or similar ID bracelet!
Medic Alert: www.medicalert.org
Web site with several other medical ID companies listed:
http://www.childrenwithdiabetes.com/d_06_700.htm This is a nice one for sports activities because it has a water proof paper inside the band to write down the critical information·doctor’s name and amounts of meds, etc.
http://www.safetysportid.com/ Very pretty Medical Alert bracelets for girls with beads and crystals:
http://www.laurenshope.com
Another good booklet, which must be ordered by mail
"Cortisol Replacement Therapy" from the Pediatric Endocrinology Nursing Society (PENS), contains a good description of what to expect with cortisol replacement therapy, how to give an injection of the solu-cortef and what signs to look out for that indicate adrenal crisis. This booklet can be helpful for the parents of a CAH/NCCAH child and for professionals not intimately familiar with the disorder and its treatment.
download PENS order form Stress Dosing
From The 2002 CAH Consensus Statement:
“Patients with CAH should carry medical identification and information concerning therapy for stress. Caregivers should have an emergency supply of IM [intramuscular] HC or glucocorticoid suppositories.” Rectal hydrocortisone suppositories are equally effective compared with IM injection, and much less scary for parent or caregiver to administer in crisis (but shouldn’t be given if child has diarrhea)!!
Patients should be given stress doses of hydrocortisone during illness with fever over 101F, when vomiting or when unable to take food by mouth, after serious injury and before any surgery. While engaging in endurance sports may require extra medication, mental and emotional stress does not. The stress dose is 2-3 times the regular glucocorticoids dose.
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