The excerpt below is from our webinar “Challenging Behaviors of Children with DMD”. Thank you to our guest speaker psychologist Dr. Natalie Truba of Nationwide Children’s Hospital. Click here to listen to the full podcast episode.
The Duchenne Spectrum of Behaviors
I think it’s important that we start to talk about how we understand these topographies – those labeled as autism or ADHD, or OCD, or anxiety – as Duchenne.
If we look at the list that I showed you earlier and we start to think Well, what would we expect to see based on everything we know about the brain and the body? we would expect to see these:
- Executive Function Challenges
- Emotional and Behavioral Manifestations
- Maladaptive Coping
- Sleep Dysfunction
These things are what we’re talking about when we’re talking about Duchenne as a neurocognitive, neurodevelopmental disease process, as well as a physical. So it really is a quadruple whammy for them. Maybe all these boys experience it on a sort of spectrum – just some to a different degree than others.
Executive Function Challenges
- Learning difficulties
- Difficulties with attention control
- Difficulties with naturally suppressing extraneous background stimuli
- Information processing difficulties
- Memory/recall difficulties
You’re going to have a worse memory in that school setting than you are for events in a more amenable setting. Like that thing you did on Tuesday, January 16th, 1997 when you’re at grandma’s and you were not stressed. You’re going to be able to recall those things, maybe better.
Emotional and Behavioral Manifestations
- Low tolerance for distressing events, contact with aversive sensory stimuli & for particularly loud and/or harsh noises/sounds
- Difficulty transitioning from one task to another & tolerating/adjusting to changes in plans, schedules and routines
- Hyperactivity and general behavioral restlessness
- Rage-like meltdowns that seem sudden or related to something small
- Selective mutism
- Hyper-focus while using electronics and engaged in other preferred leisure
These examples would be emotional and behavioral manifestations of physiological over-arousal. When we are really aroused, we have a small window, so you don’t have as much tolerance for things. Things that are loud are going to be louder. Our pupils are more dilated and we’re taking in more auditory information. That is what happens when your brain is in constant fight or flight.
We see rage-like meltdowns. If you’ve ever had a panic attack, this is very similar to that. So, when these boys have these meltdowns – no matter what you do at that point – you’ll notice nothing’s working. If anything, you’re just making it worse, and that’s like panic attacks.
So imagine if you’ve ever had a panic attack, channel right now that experience. And then imagine if people are telling you just to calm down and you’re like, OK, well, if I could do that, then I wouldn’t be having a panic attack.
Panic attacks last until your body depletes itself and shuts down, which is why panic attacks tend to not last more than 40 minutes. That’s usually how long our bodies can sustain that before weird things start to happen..
So keep that in mind when they’re in that moment: less is always more. So, when they’re having a rage-like meltdown, just disengage. Let them be themselves and feel their feelings until their bodies can actually shut down.
Maladaptive Coping
- Hyper-focus while using electronics
- Hyper-focus while engaged in preferred leisure activities
- Perseveration related to future plans/events
- OCD-like behavior in regard to personal items in their immediate environment
Maladaptive coping strategies are things that we tend to do when we are very hyper-aroused or over-aroused. We all do this.
For example, I am somebody who likes a clean put together space and I have things set up the way I like it. When I have a lot going on – maybe at work, at home, my personal life – and somebody comes in and moves something, I will be like, “Who touched my stuff? I just did this. I need this this way. I feel better in this environment and you come in and move my cup two inches. Why are you doing that?”
And so, they really have that experience as well. You know, we all have our things. Like some people rock or they bounce their feet when they’re feeling more physiologically aroused. When I get anxious, I pick at my fingers. So we all have our things we do really to help prevent overflow and these boys have their things, too.
Sleep Dysfunction
- Sleep difficulties
- Difficulties winding down prior to bed
When you’re like this, we would expect to also see sleep dysfunction. 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. 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.
