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.
4 Factors Driving Duchenne Meltdowns
In the years I’ve been doing this, I have never encountered a carrier or an individual with Duchenne without this pattern. What I see is a very chronic, insidious build up of physiological arousal over time. And all sorts of things happen when we have this chronic physiological pattern that we’re seeing.
- We don’t learn as well.
- Our body is under a ton of stress.
- Our brain is impacted.
- Our health is impacted.
- Our ability to soothe ourselves is impacted.
- Our executive control is impacted.
- Our memory is impacted.
People who may have a similar pattern – but who don’t have Duchenne – are people who might live in a very stressful environment all the time, like in a war zone. You always need to be on edge. Your body always needs to be ready. You’re always very heightened and your brain is always keeping itself safe.
These physiological patterns can also be seen in trauma survivors. So, the DMD brain actually has that “trauma brain” because that is just how chronic stress impacts us. These are some of the reasons why I think this happens.
1. The DMD Brain
Executive function tends to be weak for an individual with Duchenne. Executive function is our frontal lobe abilities – like self-soothing, attention, problem-solving, self-management, awareness. Unfortunately, we need those things to function complexly as humans in very demanding environments.
The reason I think we see these executive function challenges is there is something going on in the brain. Something is driving this autonomic system to not function as it should. It’s not as balanced as it should be.
So, there’s this bombardment of sensory stimulus on their brain and they don’t habituate. Their brain is always taking stuff in. It’s always responding. It’s always releasing excitatory chemicals in the brain. All of this leads to this over-activation of the sympathetic system.
That means that the sympathetic system is in hyperdrive – like the brain is always pushing on the gas. And when there is a more activated sympathetic system than a parasympathetic system, it is automatically going to negatively impact executive function.
2. Respiratory Muscles
Let’s go to the the 2nd part, which is the muscles. How are the breathing muscles impacted? What happens?
When I have these individuals hooked up to my monitoring equipment, I have noticed that they have this very choppy style of breathing and chest breathing. It is much more noticeable as they age – starting at about 9 or 10 years old – but you can kind of see it in those younger kids, too.
It seems like that as their agitation builds, their bodies are trying to shut down. It’s like, “Well, we can’t do this anymore. We just can’t do this anymore…” and they start to shut down, but they don’t completely. When they start to shut down, it’s not because their body can’t shut down, it’s that for some reason that shutdown is interrupted.
Because of how their respiratory muscles are impacted by Duchenne, it looks like their shallow breathing kicks their sympathetic system back up and keeps their brain going. They try to shut down and it just gets interrupted.
I think that’s why we see this stress increase throughout the day, or even in a short period of time. The effectiveness of that parasympathetic system is interrupted or inhibited, and over time they develop this very chronic physiological pattern.
3. Chronic Stress
So, really, the bigger issue is that window of tolerance.
When you’re chronically physiologically aroused, that window of tolerance gets smaller. So things that make that window smaller are chronic physiological arousals – things like trauma, being in unsafe environments, being ill, having other stressful things happen to you and also poor sleep.
Now, the opposite things make the window of tolerance bigger. We want that window to be big because then you have more opportunity and time to move around in it physiologically. You’re going to be able to tolerate distress a lot better.
What happens when we’re chronically stressed is that those windows just get smaller and smaller. And then over time, when your window gets smaller, you start to feel a lot more threat. Then you have a lot more anticipatory anxiety. And when you’re chronically overly aroused, you start to misperceive a neutral stimulus – and potentially even a positive stimulus – as potential threats. So you’re overly reacting to things as threats.
Where you see this play out often with kids with small windows is when they’re upset and they’re melting down – even when they’re not on steroids. And you’re genuinely being very calm and you’re like, “Hey, buddy, I know you’re upset. You just gotta calm down.” You’re using a calm voice and they’re like, “Why are you yelling at me?” And you’re like, “Literally, nobody’s yelling at you.”
But when their stress level is that high, that’s how the world sounds to them. So imagine how loud it is for them if whispering feels like being yelled at right in that moment.
So that’s where their brains are at. Eventually they develop some maladaptive – but effective – coping strategies to keep themselves in that little window they have. Anytime we start to do things that are repetitive – like pacing, rocking, ordering, lining, cracking your fingers – we feel better. So they start to do things like, “I don’t feel OK, so I’m just gonna order things in a line.”
Humans cope with these types of things. So when you see those things, they are sometimes coping strategies. So we won’t always want to target and get rid of them if they’re things that help kids self-soothe. But, we also don’t want to cultivate maladaptive coping strategies. So, we don’t want to get rid of the coping strategies, especially when they are something that could potentially help.
And then we put them on steroids and that exacerbates this whole process, but they need those steroids.
So it’s just a really, really less than ideal situation for helping these boys navigate the cross that they’re already bearing – which is how Duchenne is already affecting their brain.