One in a Million: The Energy You Don’t See (ATP1A3 Explained Simply)
As I write and share more about Paige—g-tube formula, parenting a medically complex child, and training a service dog—I’m realizing that many of you have very little idea what Paige’s life is actually like day to day.
Paige’s diagnosis is complicated. It’s the kind where you might see a picture of her and think, “Wow, she looks pretty ‘normal.’” Then, another day, she looks like a completely different person.
Alternating Hemiplegia of Childhood (disorder of the ATP1A3 gene), in and of itself, is complicated. Every child is different. And when I say that, I don’t mean small differences—I mean huge differences. No two children are medicated the same way, experience the same symptoms, or have the same delays. This disorder stumps world-renowned doctors, not just you and me.
With this information, patients don’t just need sympathy—they need understanding. In this post, I’m going to talk about “ATP for dummies.” The gene ATP is everywhere in our body, and does more than we even know. This post is NOT medical in any way – it is trying to express what ATP “sort of” does in order to help others understand it, or at least wrap their minds around a small part. Sometimes analogies help us to understand big concepts.
In the next post, I’ll explain what that looks like in my daughter’s everyday life.
When Do You Use Energy?
Imagine you have a big bucket. Every time you use energy in some way—emotional, physical, social, you name it—a bead drops into that bucket.
Now imagine that not all beads are the same size.
Smiling might be a teeny, tiny bead.
Getting stressed or triggered? Big bead.
Even being around a stressful person can make your heart rate increase, which uses more energy than having a calm conversation.
Now picture all those beads together—different sizes, different shapes. Smiling is the smallest. Stress is massive. What falls in between? A brisk walk. Doing the dishes. Using mental energy to complete a task on the computer.
How fast would your bucket fill up?
Little beads plus big beads—my bucket would be full pretty quickly.
What Is ATP?
ATP is where we get that energy.
ATP is what produces energy in our brains. It’s one of the systems that helps neurons communicate so you can move, think, speak, and function. And I’m not talking about the kind of “energy” we usually mean—like feeling hyped or tired. I’m talking about the broader kind of energy your body uses to exist.
The energy it takes for your brain to:
- Form a sentence
- Spell it correctly
- Use your oral muscles to speak it
- Understand what you just said and why
Guess what? That’s a bead in your bucket, too.
ATP controls how your brain communicates with your body—your muscles, your breathing, your cognitive function. All of it.
When the Pump Works… and When It Doesn’t
So let’s go back to our bucket.
For you and me, our buckets fill up fast because we do a lot in a single day. But when our ATP “pump” is working well, our cells do their jobs. They recharge. They recover. The bucket fills, empties, and life goes on.
But what if that pump doesn’t work right?
What do you imagine that would feel like?
We’d probably feel weak. If the brain isn’t sending signals correctly to the body, we won’t have the same kind of energy we would if everything were functioning properly.
Now let’s add something simple—like smiling.
That smile suddenly takes way more energy than it should. That bead just got bigger.
What about eating?
You have to use your jaw, tongue, teeth. The nerves have to fire correctly. You have to chew, swallow, digest. Whew—I’m getting tired just thinking about it.
The list goes on.
Add even a little stress, and suddenly the energy needed to handle it just isn’t there. So the body has to pull that energy from somewhere else.
I can almost imagine the little energy bubbles in Paige’s brain saying,
“Hey, we can’t handle this stress today. Where should we take energy from? Her arm? Her respiratory system?”
It might sound funny when I say it like that—but it’s not.
When Paige is around stress, that energy has to show up somewhere. Whether it’s in that moment or two days later, it often shows up as paralysis.
Too Little… or Too Much
How much energy do you use when you’re excited?
Excitement gives me energy. I get things done with it. For Paige, excitement is still energy—but her body has to use it very carefully. If it doesn’t, her brain may steal energy from somewhere else.
And sometimes, the pump doesn’t just produce too little energy—it produces too much.
That can look like:
- Muscles contracting too strongly
- Brain signals firing too rapidly
- Insomnia
ATP — But for Dummies
When you look at Paige’s symptoms through this lens, it starts to make sense:
- Dystonia: too much energy towards contracting muscles; no energy towards relaxing muscles
- Paralysis: too little energy from brain to limbs resulting in no neurological signals
- Respiratory failure: a breakdown of energy from brain to respiratory system
- Dysphagia: too little energy to swallow
- Hypotonia: a baseline low energy simply because the gene is misspelled
- Seizures: too much energy in the wrong places
- Cognitive delay: too little energy
Energy. Energy. Energy.
I bet you’ve never thought about all the little beads you collect in a single day—the energy it takes to draw one breath, or give one loving smile.
It all takes ATP.
Next time you see Paige – in person, or in a smiley little picture – try and think of this analogy. The energy her little body is using, trying to preserve, and then try to see how easy it is for her brain to stop working – simply because it doesn’t have energy. One day will look different than the next. How can you help? We’ll explore more of that in the next post.
Don’t take your genes for granted 😉
