Adrenal Crisis Information 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 NCAH 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 (printable form of letter) 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.
Solu-Cortef Shortage and Alternatives (from Newsletter April 2003)
Unfortunately, Pfizer-Pharmacia is again experiencing production and distribution problems with Solu-Cortef and the company expects the shortages to continue through the end of 2003. Many of our families have been unable to obtain this important medication. On April 16, 2003, the company announced that it would no longer provide emergency supplies of t his medication for families unable to obtain it locally. I wrote to Pfizer/Pharmacia and asked them to change this policy immediately and please make arrangements to provide CAH families with emergency supplies of Solu-Cortef. I am now informed that the policy is changed and emergency supplies of Solu-Cortef will be made available to families unable to obtain it through their pharmacies. Those in need should have their pharmacists call Pfizer/Pharmacia customer service at 1-800-821-7000. They will drop ship the medications to your pharmacy. Families seeking further information about supplies should call 1-800 323-4204. The company asked, however, that families do not attempt to stockpile the medication but only ask for the medications needed on an emergency basis.
Given the seriousness of this shortage and the implications for the health of our children, one of our medical advisors, Dr. Phyllis Speiser, is now giving all of her CAH patients prescriptions for hydrocortisone suppositories. They are much simpler to use than injections, and are rapidly absorbed to therapeutic levels within an hour, longer than the IM Solu-Cortef injection, but with longer lasting effects. Parents will be on their way to ER by the time drastic measures are required, and this is better than giving oral drug which is vomited. Suppositories cannot be used when the child has diarrhea. One manufacturer is Monarch Pharmaceuticals, and the product is called Anusol-HC. It comes in packages of 12 –25 mg suppositories. Higher doses can be compounded at specialized pharmacies. Some physicians may not be that familiar with the use of HC suppositories, although they are used regularly in lieu of IM injections in Europe. Please refer them to the medical journal article: Newrick PG, et al.; Self-Management of Adrenal Insufficiency by Rectal Hydrocortisone , Lancet , 335:212-213 (1/27/90). Please ask your doctors about this alternative/complimentary product and refer them to this article for more information. We must have the tools we need on hand to save our children's lives if necessary.
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.
What are the signs and symptoms of adrenal insufficiency? When your essential life functions are not being maintained because of a lack of adrenal hormones, you will not feel well. Your symptoms could include the following: • unusual tiredness and weakness • dizziness when standing up • nausea, vomiting, diarrhea • loss of appetite • stomach ache. Other symptoms you may experience over time include these: • weight loss • darkened skin • craving for salt. (from NIH booklet....)
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 also 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.
MORE RESOURCES 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 aMedicAlert or similar ID bracelet!
Good 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.