The excerpt below is from our webinar Challenging Behaviors of Children with DMD. Thank you to psychologist Dr. Natalie Truba of Nationwide Children’s Hospital for being our guest speaker. Click here to listen to the podcast episode.
The Duchenne Spectrum of Behaviors
I have worked with these boys long enough that I don’t think that they’re the most unlucky kids in the world. I don’t think they have Duchenne and then four or five stand alone mental health conditions.
I think when you look at these lists of our most common diagnoses, a lot of these have one thing in common. That is that they’re disorders of emotional overwhelm or overflow in a lot of ways. So they’re not those more psychiatric presentations. These are very often related to brain dysfunction and/or physiological dysfunction.
So we should be stepping back and saying, “What is it about Duchenne maybe that makes our boys more vulnerable to being classified with these diagnoses? Is this simply due to Duchenne or do they all have stand alone separate mental health conditions that they would have if they did not have Duchenne?” And I think often they would not have these conditions without the Duchenne.
So then we’re saying, “Is this a developmental disease, along with a muscular disease?” I advocate and argue that it is. If we look at it that way, then we can say, “Oh yeah, they’re all very similar – kind of on a spectrum, right?” So the Duchenne spectrum of behavior is how I think we need to be thinking about this because we are seeing some very real shared experiences, as you all know.
We see a lot of executive function deficits, particularly with attention, attentional control, and that ability to naturally suppress background information.
For example, if any of you – until right now – have not noticed that you can hear cars in the background of your apartment or your home, or if there’s a fan going that you weren’t paying attention to until I just asked you…. that’s because your brain is doing its job and it’s saying, “We’re in this setting. You’re focused on this thing and we’re safe, so you don’t need to know these other things.” It kind of suppresses the background distractions for you.
We see a lot of low tolerance for distressing events or adverse stimulus – like clean socks or harsh noises (toilets, hair dryers, etc.), certain lights and tactile things. So these boys don’t focus and suppress distractions like you do and therefore environments are often overwhelming for them. As a result, information processing and memory tend to be impacted.
So imagine the school setting for these boys. There’s a lot of difficulty transitioning from one task to a next. We see those – and I talk about them affectionately – rage-like meltdowns that seem to come out of the blue or related to something others would consider small.
You also see a higher rate of selective mutism than you would anticipate or would be predicted based on how prevalent selective mutism is in the normative population.
So those affect things like learning difficulties, right? So generalizing learning across environments or contexts, as well as specific learning problems or applying things that they know really well in one context to a novel situation to solve a problem, right? So that learning does not generalize as concepts outside of that context.
They also have really unique information processing abilities.
They really do struggle from a direct learning standpoint. They might need a lot of learning opportunities to learn a math concept, for example, multiple visual. spatial and tactile opportunities.
On the other hand, they actually have really, really, really good memory and recall abilities for certain things when those things are learned in the right context, when they are not being negatively impacted as they are, by the world and how Duchenne interacts with the world.
For example, you might be driving down the road and they’ll be like, “Hey, remember that time on Tuesday on January 16th and you said that thing and like, Grandma did this?” And you’re like, “What? That was like 7 years ago. How do you remember that?”
And then we see OCD-like behavior, and this usually presents as ordering things and aligning things in a lot of ways. They like things very particular in their immediate environment or in things that are preferred.
I also see a lot of hyper-focus, especially during preferred activities. For example, they’ll make lists – like they’ll list all of their favorite monster trucks, and then all of the secondary monster trucks. And so they kind of go through these rows or patterns of listing.
Those are the most common areas I see it play out.
Stuck in Thought Loops
We also see that perseveration or getting stuck in thought loops.
So the example I use all time – because it’s super common for some reason – is that parents say, “Hey, we’re going to Grammas on Tuesday.” And then for the foreseeable future, until Tuesday, that kid is like, “When are we going to Grammas? When are we going to Grammas? When are we going?” Even though you just told them 50 times, they’re going to keep coming back and ask “When are we going to grandma’s?” And so it’s getting stuck in that thought loop about things in the future.
Not only from a breathing standpoint – like the impact of breathing on sleep as they age – but even as little kids, you can see some really problematic sleep dysfunction.
We see it experienced so commonly that we would be amiss not to study that under the lens of Duchenne to a degree and when we do that what we see is that there’s something going on here that could be very, very concerning and very, very important, right? But we don’t really have a good treatment for it yet.
So remembering how important sleep is for all of us as humans – and for these boys in particular – that even if these other things weren’t true, then just having sleep dysfunction would.