“Tone”

This 4 letter word has more power than I’d like to admit.  It rules our world!

William’s tone is relentless. It fluctuates from hour to hour despite medications and well-planned routines. Everything impacts his tone – excitement, pain, hunger or him being too full, boredom, frustration, fatigue or the water being too hot or too cold in the bath. More recently, William has been gaining weight and as he grows his medications are no longer effective and require a new dose. Puberty wreaks havoc on his tone too. He’s 11 years old and we’ve been warned about the struggles of puberty for children with dystonia and we’ve started seeing the effects first-hand.

When I say that tone rules our world, it’s no joke. The effects of tone are substantial and life altering for William. Imagine one muscle contracting and not being able to relax. Now imagine every muscle in your body contracting and not being able to relax – not just the skeletal muscles but the internal muscles that control digestion and breathing and every other internal process that are subconsciously allowing us to survive.

William’s autonomic nervous system is also impacted by tone and for most of us this system is automatic in a good way but for him, it’s often automatic in a scary and out-of-control way. When tone rears it’s ugly head, it means vomit, an inability to go to the bathroom, extreme fatigue, pain, difficulty clearing his airway and all of this can lead to illness. AND all of these things create MORE TONE. It’s a debilitating cycle.

Physiotherapy is our first line of defense against the negative impacts of tone.

For our sweet boy, stretching and pool therapy are the two things we have in our arsenal to combat the impacts of tone. Over time, contracting muscles become naturally shorter allowing for less range of motion which has long term impacts on bones – they can’t grow (lengthen) normally. Tight and short muscles make it more and more difficult to be in certain positions, like simply standing up, which is crucial for digestion and strong bones.

The standing frame is our next line of defense against the negative impacts of tone but it’s dependant on physiotherapy.

This piece of equipment allows William to be in a standing position but without lengthened muscles, it essentially becomes a torture device. Without some level of muscle relaxation, the time spent in this frame can be exhausting for Will. His tone will fight the frame for the duration of time spent in it which is exhausting, painful and can just cause more tone – debilitating cycle begins again. But when he is relaxed, this frame provides William an opportunity to stand up and participate. It allows him to feel his feet under the weight of his body and it allows his bones to stay strong.

Medications are next in line.

Early in William’s life, I was scared to start the journey of medications that were being recommended and still to this day I fear doses that are too high. These medications come with side effects like increased secretions which are hard for William to manage with weak swallowing and weak cough reflex, slowing his breathing and heart rate which impacts oxygen levels, and the obvious one … him being snowed in and unable to really function or have quality of life. However, the right medications with the right doses allow him to be comfortable, aware and involved.

I would give anything for the tone to relent. But instead, I manage the hell out of it – challenge accepted. I’m relentless in my advocacy with our medical team and I’ve become unapologetic about the hundreds of emails I send with questions and requests.

Luckily, over the years, my relentlessness has earned me respect, trust and fewer eye rolls lately. Of course I’m still questioned but it now comes from a place of confirmation rather than doubt.

Now, I feel heard and for that I’m grateful.

I still keep an open mind and want to know all the options to best support my kiddo, but I follow my gut with confidence – no one knows William the way I do.