*Adrenal Failure and the Average Person*
What You Need to Know
By: ali_marea
30 Sept 2008


(With a long background, so reader beware! *grin*)

First let me preface this by saying that unless you suffer from an adrenal insufficiency, you probably don't need to think about their function very often. In fact, before my son was born, I never thought about them or worried about them at all. Now that I am pregnant with my second child, who also has an adrenal disorder, I realize just how important a role our adrenals play in our life.

The background to this is that, from the time I found out I was pregnant, I have been on a high dosage of steroids. Dexamethasone, to be specific. It is the only corticosteroid that crosses the placenta and does the job that needs to be done. The job that needs to be done is to provide the corticosteroids that my daughter's adrenal glands are unable to provide for her. She has a disorder that my 2 and a half year old son also has, called Congenital Adrenal Hyperplasia (CAH).

Some more background (Please feel free to skip to the end if you just want the facts! I'm giving the background so my credibility is not in question.) My son's first couple of weeks of life were rough, to say the least. He was born with a pneumothorax (he basically busted a lung when he started crying as he came out via c-section) so he went directly to the NICU. After spending about 5 days in there, he was sent home. While he was in the NICU, my son experienced some excessive difficulty in eating and had a high bilirubin count. Of course high bilirubin is not unheard of in infants. But knowing what I know nowÉI wish someone had paid attention to all of his symptoms. We may have known sooner that there was something serious going on. When my son would feed, he'd barely eat, then he would vomit half of what he had just ingested, violently. The nurses assured me that it was just him spitting up and he'd be fine. They sent him home.

After 5 more days at home, the pediatrician who saw him for well-visits said that this was not 'fine' and he needed to be checked out. By that point he had not regained any of his lost weight and was still losing. He wasn't eating well, and he slept almost all the time. His body temperature was low too. I got a phone call from my husband, while I anxiously waited for him to return home with our son, saying he was in an ambulance on the way to the ER. Hours later, my son was given a preliminary diagnosis of an adrenal disorder of some sort. He was given glucose, sodium, and cortisol. Within one day there was a dramatic improvement. By the time he returned home a few days later, he was a completely different child. He went from a sleepy, eyes-closed, quiet baby who never ate, to the inquisitive, wide-eyed, energy-filled boy I know today. He started eating better (although his appetite has never been great) and finally after a while he started to gain weight. We found out, through many blood tests, that he has CAH and would have it for life. Of course we were stunned and heart-broken. Our lovely child was going to suffer. I asked if it was my fault. Had I done something to cause the CAH? The doctors assured me it was not my fault. This is a disorder that requires a recessive gene from both parents. The chances of my husband and I both being carriers was small, but there we were. The chances of my son having the disorder was 25%. But there he was. The good thing? The doctors words to me were this. "If I could choose a disorder for my child to have, this would be the one. That is how treatable it is." My son will live a full, normal life, for the most part. He will be on medication for life, though. Every day my son will take steroids to produce what his adrenal glands do not.

We were also told that if/when I got pregnant again, I would have to start taking steroids immediately so we could combat the effects of the disorder if the child happened to be a girl. As it turns out, girls who have CAH account for most cases of inter-gender disorders. Along with not producing enough stress hormones (such as cortisol), CAH causes the adrenals to overproduce aldosterone (male hormones). With boys, this is not an issue. You can't have a child who looks 'too much like a boy'. With little girls, however, they can be born with the internal reproductive organs being 'not quite right' and their external genitalia looking decidedly male. Treatment in-utero prevents this, for the most part, and gives the girl a fighting chance from the beginning.

The past two and a half years have been spent keeping my son healthy. He wears a medic alert bracelet, instructing emergency workers to administer cortisol in the event of an emergency. If my son breaks a limb, has a high fever, vomits frequently (or otherwise begins to lose fluids), he needs to be 'stress-dosed' with steroids. He needs it as soon as possible. If oral doses to not stay down, he needs a shot. Since he has been born there were two times where we almost lost him. The first time was before he was diagnosed. The second time was when the ER people would not let him in ASAP like he needed. I finally started getting loud and in he went. Good thing. He was starting to go into a hypo-glycemic coma. The doctors were even worried. (not something you want to hear from them, by the way) He recovered quickly, with only some glucose and sodium via IV. He was home within a few days. Still, we could have lost him within a few minutes, had the technician not been able to get the IV into him quickly. I watched my son lose consciousness and vowed to never let that happen again.

Now that I'm on steroids, I realize, to an even greater extent, just how much these steroids can impact anyone's life. You take it for granted that your body will work the way it should. Since I've been on the steroids, however, my adrenals have done just what my son's, and daughter's, have done from the beginning. They shut down. In my son and daughter's cases, their bodies were overproducing some steroids and underproducing others. In my case, however, I was giving my body major doses of steroids it didn't need. (for my daughter's sake) I started to notice side effects within days.


If you had previously stopped reading to get to the facts, now is the time to start again!

Side effects from steroids can be severe and can happen very quickly. Here is what I, personally, experienced (and still experience, and will until I am off the steroids completely.):


Here are all the side effects listed from Medline Plus online:


My general practitioner has monitored me closely, along with my son's endocrinologist. I am now slowly weaning off the steroids, as most of the 'work' has already been done. The adrenals and internal reproductive organs are already formed and well established. Now, the worst that might happen to my daughter is slightly enlarged external genitalia, but nothing that requires more than minor surgery. In the meantime, we need to get me off the steroids to allow hers and my adrenals to recover and work on their own. (Hers will never work fully, but they will work to some degree.)

As soon as my daughter is born, she will be put on steroids. She will be put on a steroid to do what her own body cannot, and she will be given a steroid (called Florinef) to help her retain salt.

Another symptom of this particular version of CAH is called salt-wasting. Salt-wasting CAH patients can dehydrate very quickly. Because of this, we always have lots of Gatorade on hand. That is what our son's doctor has recommended because it has more sodium than something like Pedialyte. Sugar and salt are the keys here. That's what has to be replaced quickly if you are a CAH patient experiencing adrenal crisis.

In the event of adrenal crisis, you also need a large dose of stress-hormone. In most cases, it will be given via syringe. That particular injection is of the steroid solution Solu-Cortef. You can also administer oral stress doses. In the event of fever or vomiting, we generally increase the dosage of the corticosteroid (prednisolone, prednisone, cortisone) to 3x (three times) the normal amount. That is generally administered until the issue has been resolved for 24 hours. At that point, you wean down to 2x (two times) the dose for 24 hours, then back to the regular dose.

While all of these problems are most common with adrenal patients, any of these issues can happen if you've been on large doses of steroids for any length of time. Be sure to work closely with your doctor if you are put on steroids. Ask what the side effects are. If your doctor is unfamiliar, find a doctor who is and work with them as well. Steroids affect the body in many ways. The last thing you want is to be caught off-guard because you didn't know that steroids would affect your immune system or your adrenal function. When you are on steroids, your adrenals atrophy. That is why you have to slowly wean off of them. When on steroids for any significant length of time, consider getting an emergency alert bracelet to let emergency workers know you have a temporary adrenal insufficiency. In the event you are seriously injured, they will need to administer corticosteroids. Also, taken precautions to avoid situations where you will be exposed to illness. Steroids limit your body's ability to fight infection by compromising your immune system.

Please remember, you do not need to have an adrenal disorder to experience the problems an adrenal patient can face! Stay informed and stay in close communication with your doctors!


Some web sites with more information on adrenal disorder, as well as how to handle an adrenal crisis are as follows:

http://www.nlm.nih.gov/medlineplus/druginfo/medmaster/a682792.html

http://www.caresfoundation.org/productcart/pc/index.html

http://www.congenitaladrenalhyperplasia.org/sites.html

http://www.dshs.state.tx.us/newborn/Cahoot4.shtm

http://www.nlm.nih.gov/MEDLINEPLUS/ency/article/000357.htm

http://www.emedicine.com/emerg/topic16.htm

http://www.caresfoundation.org/productcart/pc/Adrenal_Crisis.html


I hope this has been informative and potentially helpful to anyone on high dose steroids or with adrenal insufficiency!
ali_marea


www.alpharubicon.com
All materials at this site not otherwise credited are Copyright © 1996 - 2008 Trip Williams. All rights reserved. May be reproduced for personal use only. Use of any material contained herein is subject to stated terms or written permission.