Q: We’ve noticed in past few years that there seems to be a “seasonal” change in our 5-year-old son with SWCAH as the hotter weather arrives. He seems to go thru fits of rage/anger and extreme emotional volatility.  We then have blood tests done to determine levels, and he doesn’t get too far out of range. We have him drinking and staying hydrated all day but can’t figure out if he’s getting oversuppressed/undersuppressed within a given day but “ok” overall over a longer time period (so it doesn’t manifest itself during blood tests). Just wondering what we should be thinking/doing differently given it seems to be a pattern with hot weather coming into play? Thanks so much in advance for your guidance.

A: I have a few thoughts given the observation that it seems to be seasonal, and it doesn’t seem to show up in blood tests. It is certainly possible that the blood tests, which are after all just a snapshot in time, simply aren’t catching the fluctuations, as you mentioned.

The manifestation of emotional volatility could be related to higher androgen levels, or it could also be due to low sodium levels, which may be making him irritable. In extremely hot weather, salt-wasters are certainly at higher risk for dehydration, but they also at risk for low sodium. It is important to keep him well-hydrated, as you mentioned, but he also probably has increased sodium requirements (FYI, Gatorade has plenty of glucose, but very little sodium). Some salt-wasters find that they do better with extra Florinef on particularly hot days when they are going to be outside for extended periods of time (and/or doing strenuous activity).

You could ask your endocrinologist if a little extra Florinef on those occasions might benefit your son as well. The hot weather itself can raise the body’s core temperature slightly, which in turn can speed up the body’s metabolism, so his requirements for Cortef may also be slightly increased. If you do end up giving him little more Florinef, Florinef actually has some glucocorticoid (Cortef) activity as well, and that could be enough to suppress any extra androgens that may be occurring during the hot weather.

I would certainly talk with your endocrinologist before doing anything, but hopefully we can find a way to help your son during the hot weather.

originally printed in CARES Foundation Spring 2003 Newsletter

I have the solution for easy medication administration! It is called “The Medicator” by Munchkin. I found it at Wal-Mart, but I’ve seen them at other places too. My daughter is nearly 3 yrs. old and I still use it, just because it is so easy. (Unless, a child will chew or swallow the pills, this is the next best thing.) It is a small 10 ml. container connected to a screw- on nipple (like the nipple on a bottle, not a pacifier). Since the Cortef and Florinef dissolve so easily, I just put the pills in the little container and put a little clear juice in it. (I use clear liquid so I can see if all of the particles have dissolved.) If you leave the pills in the liquid long enough, they will dissolve completely, but if you don’t won’t to wait, then I have a solution for that too! I took the plunger out of a syringe (like an extra syringe you may have for the solu-cortef injectable) and the flat part of it fits perfectly down into that little medicator for crushing those pills! No medication is lost from crushing the pills in a crusher, then trying to put that powder into another container to give it to the child. And this can be used for very small infants, so you don’t have to worry about the medicine dribbling out of a baby’s mouth, like you do if you are squirting it in their mouth with a syringe. Here’s a tip though-Don’t put any other kind of medication in this dispenser!! You don’t want your child to associate something that tastes bad with this particular dispenser. So, find a different kind of dispenser for other meds such as Tylenol, Benadryl, etc. Hope this helps!

Gina Murray, Mississippi

originally printed in CARES Foundation Fall 2009 Newsletter

by Jessica Hall Upchurch

As the parent of a child with SWCAH, you know the day will come when you have to give your child his first injection. What you don’t know is where you will be, will you be able to calm down to do it and will you have someone there to help you. Here is how it happened for me.

I was a trainer at a workshop in Denver when my son, Clay, became ill with the stomach flu. And as we know, the stomach flu for a child with SWCAH can be serious—this time it was, as we ended up in the ER in an adrenal crisis. I got the call that he had thrown-up so I immediately left the workshop and headed to the hotel room. We followed the oral stress dose protocol but he continued to vomit—and that is when it hit and it took all of twenty minutes but it felt like a second. He was limp, eyes rolling into the back of his head, pale and cold—and I was scared to death!

I called 911 as my fear was coming alive in front of me, my son was in dire need and I had to act fast. The paramedics and hotel staff arrived in minutes—so quickly in fact, I had just pulled his injection and solu-cortef from the medical bag I carry with us. Oh help me, give me the strength to slow this down because I knew I had to give him a shot—and this was the first time.

At that point they put an oxygen mask on my angel, he was only three years old at the time, and started asking lots of questions. Of course, they told me they could not give him his injection. Imagine, here we were with trained personnel surrounding us and they couldn’t “legally” give my son a shot that would save his life. Reason being “the attending doctor at the hospital to which we were being transported didn’t prescribe it.” It was up to me!

Literally, I had to tell them to “stop” as they were trying to load he and I onto the gurney and I still hadn’t given him his shot. I got them to stop, and with a kind paramedic next to me, he coached me through it. The injection was completed and within 10 minutes, my little angel was blinking his big brown eyes, color was back in his skin and I was in tears of relief and gratitude. If it wasn’t for CARES, I wouldn’t have been as prepared and informed when it came to saving my own child’s life. And that is what I did that day, I saved his life.

Thank you CARES for providing me with the knowledge and skills to save my son. I am forever grateful for the guidance and support you provide our family!