CHARGE Syndrome: Behavioral Issues

Chapters:

1. Behavioral Issues in CHARGE Syndrome
2. Sensory Deficits and Behavior
3. Behavior as Communication
4. Common Behavioral Diagnoses
5. Resources

CHAPTER 1: Behavioral Issues in CHARGE Syndrome

HARTSHORNE: I think when we first discovered that children with CHARGE syndrome were engaging in very unusual, sometimes very challenging behaviors, it took me and I think it took some other people by surprise. And I started being asked to be parts of seminars and conferences and addressing behavioral issues, which I didn't really know what to say, because I didn't understand the behaviors myself.

I think the behaviors are a puzzle to many people, and often I think the behaviors get attributed to something wrong with the child as opposed to something that's almost inevitable coming out of a child with a syndrome like CHARGE syndrome.

And so I want people to understand that it's not just that this kid has been raised wrong or has been spoiled or whatever it is, that this kid has a serious condition related to the syndrome, which helps to produce the kinds of behaviors that we see, and that the behaviors, though, are still understandable and still treatable, but we have to understand that it's not just we have a bad kid here, we've got a kid with a syndrome.

CHAPTER 2: Sensory Deficits and Behavior

HARTSHORNE: That dual sensory impairment multiplies the difficulties that the person has understanding their world and what's happening to them tremendously.

Potentially, every single one of the senses is impacted with CHARGE, so if you just think vision and hearing is a multiplicative kind of problem, imagine all these others on top of it.

So you have, really, a sensory syndrome, you know, people who have to learn how to understand a world through senses which are not working properly, not processing properly, and are not working together in a way that gives them the kind of feedback that we all use, because I can hear and see something at the same time. I have more confidence in it.

NARRATOR: In a photograph, a young boy with CHARGE is playing the game Connect Four. The boy is wearing glasses and sits close to the table to better see the game. He has also removed the transmitter of his cochlear implant, and it dangles on his shirt, visible below is trach tube.

HARTSHORNE: So I think a lot of the behaviors that you see are a kid who's trying to figure out, "How do I move and function in a world "that doesn't always make sense to me "because I'm constantly working so hard to try to function in that world?"

NARRATOR: In a photograph, a young girl with CHARGE is shown lying on a carpeted floor with a jump rope nearby. It appears she is taking a break from the activity going on around her.

HARTSHORNE: Learning to regulate all of our psychological physical systems is a task that everybody has to do. Problem managing arousal levels or how much stimulation is a real problem for kids with CHARGE, because to regulate the appropriate level of arousal takes all of us a certain amount of work, and so if I'm finding I'm kind of falling down, getting kind of lazy, I've got ways to get myself back up, but if I'm finding that I'm getting really worked up, I can kind of pull myself off a little bit, even in my head, and calm myself back down.

Sometimes, you'll see kids with CHARGE, they're in a room with lots of other people, they'll go find a quiet corner where they can just kind of be by themselves or crawl underneath a table where they can feel kind of protected, bring things down.

Other times, they're running like crazy around the room, bumping into everybody because they're one side or the other and it's hard to get to an even kind of level. So that's a particular challenge for them, when they're trying to manage that level of arousal.

NARRATOR: In a series of two photos, a young boy with CHARGE appears to be running and jumping excitedly after tossing a handful of objects up into the air above his head.

HARTSHORNE: You know, someday I hope we can understand better how to help kids with CHARGE to self-regulate, because I think this is really critical. I think, because of a lack of feedback from their environment as well as, you know, not quite understanding the way their body's functioning, it makes it difficult for them to get there.

CHAPTER 3: Behavior as Communication

HARTSHORNE: So if you think about behavior as communication, it doesn't necessarily mean that the child is thinking about, "How can I say this to my parents?"

They're engaging in behaviors, but it's our job to see whether that behavior leads us to understand something that's going on with our child. And so for example, one year, between Christmas and New Year, my son's... and he always has trouble going to sleep, but he was taking hours to go to sleep, and that's of course a problem because then, mostly my wife, doesn't get any sleep either.

And so she took him to the doctor, and the doctor's very good, he looks him all over, can't find anything, looks in his ears, little bit of wax but didn't see anything dramatic, sent us back home. It doesn't get any worse... or any better, it gets worse. I actually called the doctor, and the doctor said, "Well, maybe we should try some medication."

NARRATOR: In a photograph, a number of prescription bottles and pills are shown on a counter.

HARTSHORNE: But I said, "No, this is not a case of a kid who just can't settle himself. "There's something going on, "and I don't want it medicated away "so we never find out what the cause is. "There's something going on. "I know my son. This is not typical." So I went back to the doctor.

The doctor looked him over again, looked in the ear again, looked at that wax in his ear, it's a little different... played with it. "Oh, that's something stuck on the inside of his ear. Seems to be rubbing against his eardrum." It did take two nurses and my wife and I and the surgeon to hold him down and pull the thing out, but then he was okay again.

So I really think, for me, I want to look at pain first, and not think that... Especially when you get a new behavior, okay, "What's going on? Why is there a new behavior?

"Because he wasn't like this yesterday. "Something happened. "What is it that might have happened? "Is it something new in his environment? "Is he experiencing something internally that, you know... what's going on?"

But until I find that out, I'm very reluctant to run to a psychiatrist and say, "Well, let's diagnose him "as having some psychiatric condition that needs medication."

CHAPTER 4: Common Behavioral Diagnoses

HARTSHORNE: Well, kids with CHARGE will often engage in behaviors that seem obsessive-compulsive. If they don't get what they want or to do what they want to do, then they have the meltdowns, and the meltdowns are the things, I think, that the families really don't... worry about, don't quite know what to do with, because the kid... And sometimes they're just doing just fine and then all of a sudden, out of nowhere, it seems like they're having this big meltdown. So some of it's the obsessive-compulsive, some of it's just self-stimulatory. The kids may rock, kids may flap their arms, may do things that are tick-like. Their social skills often aren't very good.

GIRL: Andrea, I need a hug!

WOMAN: Oh, wait, some space. How about a side hug?

GIRL: Sure!

NARRATOR: In a video clip, a young girl with CHARGE enters a classroom to speak with her teacher. The teacher models the appropriate behavior.

HARTSHORNE: They kind of look through people, or even if they're looking at people, they may do it in kind of an awkward way. When they talk to somebody, um, it may seem kind of stilted and not natural, and that makes people think, "Oh, they must be autistic."

Um, they often have problems with attention and so they get diagnosed sometimes with Attention Deficit Disorder or even Hyperactivity Disorder, but, you know, if you're sitting there with sensory impairments, it's quite likely that your attention's going to wander as well, and so I think many of the behaviors that they engage in are understandable given the nature of the syndrome, but they take it to the next level, and they get engaged in such a way that parents can't control them, schools can't control them, and I've seen kids removed from not just the regular classroom or even the special education classroom, but sent to buildings apart from the school district because they're not controllable where they are.

And it's those kinds of things that make it very peculiar and hard for parents and teachers and others to make sense out of, and so we move to the labels. We say, "Oh, they have OCD, they have ADHD, they've got Tourette's, they've got autism," but I don't think those labels help very much.

NARRATOR: Text appears on the screen revealing: "Obsessive-Compulsive Disorder," "Attention Deficit Hyperactivity Disorder," "Tourette's Syndrome" and "Autism."

HARTSHORNE:I think what we see that we call autism, probably has lots of different causes. And so, yeah, okay, if you want to say that this kid is showing behaviors which we tend to describe as autistic-like, I'm partly there, but what my goal in understanding CHARGE behavior has been from the beginning is describing the unique features of the behavior and trying to understand that.

As soon as you call it autism, you lump a bunch of stuff together and people think you know what you're talking about and they stop focusing on what's unique about the way children with CHARGE behave because, "Oh, well, it's just autism."

Plus, I don't think all the treatments that are used for autism are necessarily very appropriate for a sensory disability kind of syndrome like CHARGE.

I think people with autism have difficulty dealing with their sensory systems, but not the same way. They're not sensory impaired. So they're coming at it from a different angle where they may have difficulty processing some of the sensory feedback, but they don't have the deficits.

It just worries me that parents are being misled to think that, "If we give you this diagnosis, "if the psychiatrist gives you this medication, the problem's going to go away," and it's not going to go away.

And I've seen kids who are on three or four different psychotropic medications who are basically zombie-like, and if we believe that behavior is communication, and then you start prescribing all these drugs to stop the behavior, what are you doing? You're taking away their form of communication. So now what are they going to do to show you what's going on with them? Because you've drugged that away from them.

So there are responsible professionals out there who disagree with me on that issue, and I think they're wrong, but, you know, I have to recognize I could be wrong too, but I don't think so in this case. I don't think we're getting better at working with CHARGE by putting psychiatric labels on the kids. Let's understand these behaviors come because of the syndrome, and let's better understand the syndrome.

If we understand that better and how it manifests itself and how each different part of it interacts with all the other parts of it, then, I think, we're going to have a better understanding of the behavior.

CHAPTER 5: Resources

HARTSHORNE: You know, there are a lot of groups out there for parents of children with CHARGE. There's a LISTSERV that's on the Internet, on Facebook there's a thousand people, I think, on the Facebook CHARGE page.

There's the foundation that provides many avenues of support. There are many of us that do consultation by e-mail or over the phone. So I encourage parents to reach out, even if it's not to someone who's a parent of a child with CHARGE.

There are other parents of children with other disabilities. Reach out and make those kinds of connections. Get to know the network of people who are involved in disability in your community.

NARRATOR: The home page of the CHARGE Syndrome Foundation appears on the screen, and then clicks through to the resource page, which displays a number of options for information.

HARTSHORNE: CHARGE isn't the only syndrome with behavioral issues. I didn't know that at first because I was too naive and not well educated about genetic syndromes, but most every syndrome has behavioral features, some worse than CHARGE.

CHARGE does have self-injurious behaviors, but so do other syndromes, some of which are actually even more self-injurious than we typically see with CHARGE.

So having the chance to reach out and get that connection with people who get it, who kind of understand, helps you, and when you feel more encouraged and more energized, you're probably going to do better in terms of coping with the behaviors that you see.

You know, I think as a parent of a kid with CHARGE, I kept hoping that the person who would manage our case, who would understand everything, would show up one day. That person never showed up, and one day my wife and I looked at each other and said, you know, "We're going to have to do this ourselves, aren't we?"

And so that's really what families have to do. They've got to take charge, knowing that it's going to be tough, but also, hopefully, discovering that there are people out there who will applaud them, support them, talk to them in the middle of the night when they need that talk, and then they can cope better.

NARRATOR: The resource page of the CHARGE Foundation website is shown. On it is a listing called "Parent to Parent" that offers assistance connecting families of children with CHARGE with one another.

HARTSHORNE: So it's an up and down kind of thing, but parents should not waste lots of time with guilt-- that's easy to say-- because you are doing the very best you can with a situation that none of those professionals would sign up for, you know. They don't want what you have to go through and probably appreciate what you're doing much more than you recognize.