Handtevy Minute - Adrenal Insufficiency (CAH)

Hello, everyone. This is Dr. Peter Hentevi for another edition of the Hentavi Minute. Today I wanted to talk about something that's not so common, but I think it's easy to treat in the field. So let's talk about when children have an issue with their adrenal glands. So here's the kidney. Right above the kidney is the adrenal gland. The adrenal gland is important for two main reasons. It's because it produces these two hormones. These two hormones are actually important. Let's get into why that is.

When these hormones are created, they're actually created by an enzyme called. One of the enzymes is called 21 hydroxylase. If as a child you don't have that enzyme, that means you cannot create these two hormones. What happens is, for example, with aldosterone, if you can't create aldosterone, you end up having a low sodium and therefore you have a low blood pressure.

And that's very important. And you end up having an elevated potassium. But let's just leave the low sodium, low blood pressure. And then with cortisol, you end up having a low glucose because you can't make aldosterone or cortisol. It's important to understand that children who can't make those have to take it. They have to take pills or liquid on a regular basis. If those children get sick, they need to take a much higher dose while they're ill. So I want you to meet someone named Evan. And Evan is a little 8 year old boy who got really sick because he was not making aldosterone, not making cortisol. And when fire rescue comes to the house, they need to know how to give him the medication that actually he carries. So everyone meet Evan and we'll talk to his mom afterwards. And actually we'll show you how to give that medication via injection. Hi, everyone. So I'm here with Evan Gonder. Hey, Evan.

Hi.

How old are you?

I'm eight years old.

Eight years old. So can you tell us what you have?

I have adrenal insufficiency.

Adrenal insufficiency. And tell me, do you remember the very first time that you got sick with adrenal insufficiency? No, because how old were you at the time?

I was one.

And when your mom tells you the story, do you remember how you were acting when you were one and you got sick?

I didn't wake up.

So basically he was unconscious. Okay. And mom and dad weren't here because they were working and you had someone watching you. Right. Okay. And when you have adrenal insufficiency and you get sick, don't you need a certain medication?

Yes, you need.

You do. You do need a steroid. And so do you think that a lot of fire departments have this steroid with them?

No.

You think it's important that they have it?

Yes.

Do you want to teach them all about it? Want to do that? High five.

So I think it's important to know that all families with someone who have adrenal insufficiency should carry something at home just in case they get sick. Evan, do your parents carry anything at home just in case you get sick?

Yes. Always.

What is it?

It's this case right here.

Yeah.

So let's open it up and see what's inside.

Ah. What is that?

My steroids.

And what's the name of that steroids?

Solucor.

That's solucortive. How about we let your sister Lexi show us how easy it is to administer that medication? Good. Mm. All right. Okay. So here we have Lexi, Evan's sister, and she's 13, and she's going to show us exactly how to administer Solucortis. Lexi, take it away.

So first you get the needle out and put it together.

And then.

You push. You take the cap off of the medicine, and then push it down really hard.

And then the medicine will drop into the powder. And then you just shake it up and let it mix.

And then you take off the cap of your syringe and put it into the hole. And then make sure that the syringe isn't over the water. And then draw it up.

And then you just inject it.

So you saw how easy that was. That was like 30 seconds. And we have a 13 year old who got the medication mixed up. It's important to know how to use that vial and actually how to draw the medication and then administer it. This is an intramuscular injection. Lexi, great job.

Okay, so now we have Melanie, who is Evan's mom here. Melanie's an ER nurse, and she's the trauma systems line manager here at Memorial Herman. But I wanted Melanie to kind of talk about, from a parent's perspective, what do you think is important for other parents to know and other paramedics to know about Evan.

I think it's important to know that you should listen to the patient's family member, listen to their parent. Anybody that has a child with a circle special need of any sort is going to know what's best for their child. And if they're telling you what's wrong. You need to listen to them.

So I think it's important to understand that when our medics come to the scene and they have a child who has a known diagnosis, you have the kids all around the house.

Why do you think it's scary? Or maybe it's a little bit of an unknown for them? And why is it important for them to learn about this type of thing?

Well, adrenal insufficiency is not as common as some of the other diagnoses, such as diabetes or asthma. You don't really hear people say, I have adrenal insufficiency. So because it's rare, rarer, you really need to be informed. And if you're not informed, then you should listen to that person's caregiver and listen to what they're telling you to do.

So if a parent says, my child has adrenal deficiency and they need this steroid, and the instructions are right there, and the parent knows how to give it, we should let the parents do it, right?

Either let the parents do it or if they're too. I've talked to some parents who were too nervous and anxious to give it. If you can assist with that, then that obviously would be best. If you can. But if you can't.

Walk the parent through how to do it, show them how to do it.

And if they don't have the medication from you, the parent. We can carry some medications on the truck, right? Like Salumedrol as well, to give that in place of salucortif.

For sure. Every ambulance in our area, in the Houston area, carries Solu Medrol. We carry as parents, we carry Solucortac. But in an emergency situation, Solumedrol will help and will save that individual's life.

So what are some of the other symptoms? You know, Evan was not conscious, but what are some of the symptoms that you, as a parent, would stimulate you to actually administer the medication to Evan?

So if he was acting, altered or confused, severe previous vomiting where he can't hold down, his oral medications, broken bones, or sometimes something that will trigger an adrenal crisis. So any significant injury or illness, sometimes a fever, will set you into an adrenal crisis. So we really monitor how he's acting and behaving, and we monitor for those signs.

And there are some national foundations to help parents like you and you. I know it's a very big group. Can you talk about that for a moment?

There's actually several foundations that my husband and I are involved with. There's the CARES Foundation. They have EMS training videos that will help you for training your paramedics or your EMTs. There's the Adrenal Insufficiency United. They have a lot of paperwork and lots of great things that they provide to EMS that tell you the signs and symptoms. And then the Magic foundation also helps kids with adrenal insufficiency.

Awesome. And those entities have contacted people like me and other EMS medical directors across the country. So if you work for an EMS agency, look for the data. You can contact us and we'll put you in touch with that. But I think a child like Evan, it's pretty simple to do what's right for him at the home or in the back of your rescue. And so we wish that you all look into this and you speak to your medical directors about this. Evan and Melanie, thank you so much. I wanted to recap for everyone. The kidney is here, the adrenal glands above it. And there's an enzyme inside that adrenal gland that helps make these two hormones. When these two hormones are not made, you have low sodium, therefore low blood pressure, hypotension, and then you have a low blood sugar. So children are taking these medications. As you saw with Evan. We call this problem with an enzyme deficiency. The long name is congenital adrenal hyperplasia, or cah. Again, if you. You're at the scene and the parents have solucortec, give it. If not, let's give them solumedrol. Have a protocol. If you don't have one, we can help you with that. This has been Dr. Peter Antevi for another edition of Antevi Minute. Thank you.