Easter and ERs: Learning to live with Adrenal Insuffieciency

Learning to live with adrenal insufficiency is a little daunting. Just before Easter break, Gabe got into a small accident on the playground that had us rushing to the emergency room and over the holiday he caught a cold. Both events underscored for us that we don't know what we're dealing with yet and we have a lot to learn about how to handle the normal kid stuff that arises like sniffles and black eyes.

Gabriel is a rough and tumble child and coming home with bruises and scrapes from playground adventures is a regular event. Last Wednesday, he was racing at recess with another boy when, in a passing maneuver, the other boy collided him and landed on Gabe's face. It happens. I got a phone call from the office secretary to alert me that Gabe would be coming home with scrapes on his face and an eye that is bloodshot and banged up pretty good. She said that Gabe had told her that his vision was briefly a little blurry in one eye following the head-landing-upon. Well, ordinarily (pre-ALD), I would have just said "thanks for the heads up" and added another mark to the mental checklist of Gabe's battle scars, but now I know that there's adrenal insufficiency involved and I have to take his playground injuries seriously! But, how seriously? Which injuries? I told the secretary that I was on my way and rushed out the door, dialing the number for Gabe's pediatrician. With an eye injury, did this necessitate a visit to the endocrinologist? The pediatrician? The ER? None of the above?

Now, ordinarily, your adrenal glands make cortisol when you are in a stressful situation like getting sick, getting injured, interviewing for a job, bungee jumping, training for underwater demolitions, or facing a fight-or-flight scenario. In ALD, the rogue very-long-chain-fatty-acids that accumulate in the body will damage the adrenal glands, impeding the body's ability to make cortisol and other hormones. Cortisol has stuff to do with your blood sugar, salt and water balance, lowering inflammation, metabolism... The production is stimulated by the pituitary gland, which tells the adrenal glands that it's time to produce their hormones. When a situation occurs in which the body SHOULD be producing cortisol and can't, the pituitary gland starts screaming at the adrenals by releasing lots of Adrenocorticotropic Hormone (ACTH). In Gabe's case, the adrenals respond just a little, but not enough, and the pituitary keeps yelling, hoping that they'll get the adrenal's attention. Gabe's ACTH has been extremely high lately, anyway, without injury or illness.

I got up with the pediatric nurse and explained the situation: that Gabe has adrenal insufficiency secondary to Adrenoleukodystrophy and had gotten an eye injury when another boy fell on his head on the playground. I hadn't seen it yet, but the school said it was bruised, bloodshot, and blurry. She said that I should give him a stress dose of hydrocortisone and go ahead and bring him to the ER since, with the adrenal issues and it involving his eye, they'd probably want to look him over. When I picked him up, I saw that the injury wasn't nearly as bad as it had sounded on the phone. I gave him twice his afternoon dose (10 mg) and brought him to the ER. They saw him quickly, deemed him okay, and confirmed the stress dose of double his hydrocortisone. The doctor we saw said that he probably wouldn't need the evening dose to be doubled, but it couldn't hurt, so we gave him a double dose in the evening. I was a little embarrassed to be in the ER over such a small injury, but we're new to this whole adrenal thing.

Two days after Gabe's injury, he woke up feeling bad. He had a terrible headache, felt nauseous  and had a
low temperature. I gave him some ibuprofen, but it didn't seem to help a bit. Two hours later, he was still lying on the couch moaning about his head and feeling sick. It took some time to get up with an Endocrinologist, even using the on-call number, and I was starting to freak out a little by the time I finally did.  She said to triple his hydrocortisone dose and to give him the 50mg shot if he threw up. It was surprising to see how fast he bounced back. Pretty soon, he was up playing video games, then he was feeling back to himself and we went for a walk. The following day, though, he was feeling sick again and had developed a cough. By the middle of the day on Easter, he was running a fever of 102.6. We were all on pins and needles, unsure if this was a true illness, if it was (at least in part) an adrenal crisis, if we were doing things right, if he needed to go back into the ER... I'm just glad that he didn't start throwing up! On Tuesday, just 4 days after it started, he was feeling a deal better and the cough had almost disappeared, so we lowered his hydrocortisone to twice the usual amount. Wednesday he seemed back to normal, so we returned to the regular dose. He's back on the usual dose now and seems okay, but we're still a little nervous. I guess that we'll get the hang of this eventually, but right now we're still learning the stress to hydrocortisone ratio and the appropriate scoring of emergencies.

To conclude on a high note, though, we did get spectacular news Tuesday! It would appear that our twelve year old daughter, Cai, is NOT a carrier for ALD! The very-long-chain-fatty-acid blood test has a 85% accuracy in being able to tell if a female is a carrier, so there's still a little wiggle room for error, but there is a really great chance (an 85% chance) that she is ALD gene free! That would mean that she also has a really great chance of being Gabe's donor should he need a bone marrow transplant.

Our family had a wonderful Easter and a fantastic Holy Week. We dyed eggs, were surprised by a live, fun Stations of the Cross event at church on Holy Thursday (we were expecting just regular subdued stations, not the traditional Via Crucis with actors and guitar playing), celebrated a beautiful Easter vigil service by candle light in which the whole church seemed filled with twinkling stars, watched The Ten Commandments like we do every year (no finer example of 50s cinema will one find than this!), had an Easter egg hunt, and of course, ate far too much candy. Many years ago I started an Easter Pinata tradition. I don't know why, it just seemed like a fun thing to do at the time, and we continued the tradition every year since. This year they had a lot of fun trying to bust it open with a golf club. Cai was the winner; she knocked that poor pinata right in half.  We hope that, whatever you celebrate you had a great holiday, too!