Not Just a Muscle Disease

Click here to read the first-hand account of how Duchenne is not just a muscle disease for Wyatt Rownd, a 12-year-old with Duchenne.

Not Just a Muscle Disease

Duchenne Muscular Dystrophy is largely known for the weakness it creates in the muscles of the body. Unfortunately, the effects of the disease don’t stop there. The lack of dystrophin also affects the brain and emotions. These, in turn, affect behaviors and relationships.

It’s typical that our kids with Duchenne cannot meet society’s expectations, especially those rules about “good behavior” and “following directions.” And so, they are often reprimanded or punished for their behaviors… for behaviors that are rooted in their disability and far beyond their control.

The Duchenne Spectrum of Behaviors

Due to the way the lack of dystrophin affects the brain (more about that below), there are very specific patterns of behaviors that are quite common with a Duchenne diagnosis.

Please educate yourself about The Duchenne Spectrum of Behaviors, recognize them for what they are, and create a plan in advance for ways to support the children when these challenging behaviors arise.

Higher Rate of Mental Health Concerns

Our loved ones with Duchenne have a much higher incidence of mental health diagnoses (and their associated behaviors) than the general population. In this 3 minute clip, Dr. Natalie Truba explains the common mental health diagnoses associated with Duchenne, how the diagnoses manifest themselves in “Duchenne Behaviors” and what dystrophin has to do with it.

Duchenne Induced

It’s ADHD, but it isn’t.

It’s autism, but it isn’t.

It’s OCD, but it isn’t.

Rarely does an individual with Duchenne fit neatly into a mental health diagnosis and it’s important to clarify this whenever possible. That’s one of the reasons Dr. Truba recommends calling these diagnoses “Duchenne Induced…”

This phrase emphasizes that these behaviors are rooted in the Duchenne diagnosis and are not purposeful. Hopefully, the term will create more compassion for our children who struggle to fit in socially, despite these involuntary outbursts.

It’s a reframing our community desperately needs.

Small Window of Tolerance

The child with Duchenne experiences chronic physiological stress that significantly shrinks their Window of Tolerance. That physiological stress also primes the child to interpret neutral and even positive interactions (or input) as dangerous. Understandably, they respond explosively.

In this clip, Dr. Truba explains what a typical Window of Tolerance is like. From 3 minutes on, she explains what the Duchenne Window of Tolerance looks like.

4 Things Fueling Meltdowns

As Dr. Truba explained above, that Window of Tolerance can get rather small for the child with Duchenne. But why?

So far, science has identified at least four contributing factors: the dystrophin missing in the brain, the weak respiratory muscles, the chronic stress and steroids. Those four Duchenne factors shrink the Window of Tolerance and set the child up for socially unexpected behavior.

Sensory Processing Disorder

Adding to the already multifaceted sources of chronic stress, our children with Duchenne frequently experience Sensory Processing Disorder as well.

While few clinical trials have conclusive findings, parents of children with Duchenne report incidences of increased stress and anxiety levels for their child when experiencing typical sounds, touch, smells, tastes and even images.

No Physical Way to Relieve Stress

Clearly, our children carry a very heavy load of Duchenne induced stress. Typically, humans diffuse stress of that magnitude with intense physical play or sports. And yet, children with Duchenne just don’t have that option.

If they did try that method, not only would their bodies likely fatigue before diffusing the stress, but the risk of bone fracture and also rhabdomyolysis is quite high in our children with Duchenne.

Unfortunately, the stress stays trapped in their bodies until it escapes in an emotional explosion or a meltdown.

Understanding, Compassion and Help

When those unavoidable meltdowns and explosions occur, all that our children with Duchenne can hope for is an adult to understand what’s going on, to show them compassion, and to help guide them back to a manageable level of stress.

More to Consider…