Everyone is capable of being lazy on occasion, whether able or disabled. For people with a Rett Syndrome, they too are capable of having a lazy day but we are not lazy by nature.
No two people with Rett Syndrome will be the same but many will share features in common. The exact same genetic error will not determine the same outcome.
Rett folk generally do not know what is wrong. Observing Rett folk generally will not provide that answer either. Some can walk while others cannot. In the group that walk, (which includes Sean and myself) we can see, firstly, an unusual gait. The gait will include a number of factors, for example; issues with feet/ankles, with knees and spasticity and these all affect gait individually. The wide base gait occurs for balance and security while upright. So when we see the wide base we can know that balance and security, while being on foot, is affected for that person. This is the reason for my wide base, which was far more pronounced when I was younger. At high school age I was told I walked like John Wayne, along with my arms out, for balance. I stood and walked in this fashion. I know this because I saw an old photo of myself, taken when I was visiting a little girl friend. No one had told me to put arms or feet in, when taking the photo.
As with many other children with Rett Syndrome, I didn’t begin walking till age 2.
So many things are affected in Rett Syndrome, much more than is currently understood.
When able people walk, they are all utilising memory, vast and varied amounts of memory. Little energy is being used because able people have something very much like a cache system that is drawn on, constantly and continuously, around the clock. Even while sleeping we have a memory system that is constantly at work.
Rett folk experience numerous things. Many walking Rett people are working in a similar way as I am. When I walk I am constantly drawing on energy. When I stand up I am physically lifting myself each and every time. I’m not drawing on memory to do so. When I have stood up enough times, my knees begin to struggle with the effort. It’s quite exhausting. When we watch weight lifters, we see the same after enough heavy lifts. Rett folk that can eat, will eat more than one would expect and this essentially is the reason. They require more energy and can spend energy much faster than able people. Because of this way of working, energy can be used to dangerously low levels and parents likely see their child at times commence breathing difficulties and/or tremor and other such difficulties when energy becomes severely compromised.
When left to their own, they will rest and let energy store, that’s what I do, so I wouldn’t think that is unique to me. Mostly, there really isn’t a choice in having to do this. As walking Rett folk get older, they get better at managing things. They figure ways to maximise their energy and move in a way that will conserve their energy. Even while they don’t understand why, time and experience with this condition teaches us slowly and surely. Like the weight lifter who can lift more with time and practice, Rett folk do similar. When we get a fright, or anxious, the response system suddenly draws a large amount of energy and can often result in a shortage of energy to life support functions such as breathing for example. For some it will look something like seizure activity, which is not to say that Rett folk don’t have epilepsy. Of course they do, but there is an action that will mimic a seizure, but is essentially due to such limited energy it has caused the person to temporarily crash. Which of course is not something we want to happen and so care must be taken.
Energy systems can and need strengthening where possible. Keeping up energy with food that provides above average energy helps a lot. I didn’t know just how much, until I met a man named Stanley who is extremely knowledgeable in foods (acid and alkaline) and for the years I’ve been eating his food, I’ve been able to improve function and energy stores.
Wouldn’t you know, eating requires energy for all people, but for Rett folk, because a good many things are working the very same way as I described when getting one’s self up and moving, eating can also be like this too. Every single movement, from breathing to blinking requires memory and energy. If a person watches their own movements, they will see that they do a movement in the very same way. Combing hair, brushing teeth, the way you wave to friend, these movements go into memory and are called upon each time you need them. If able people weren’t able to do so, they would be in the same boat as many Rett folk and only get so much done in a day. It’s an ingenious design and allows people to get a lot done in a day. And another really interesting aspect is those people who designed Windows were actually replicating part of the human condition.
All my life I fought with limited energy and in an emergency, a lolly will help, temporarily. I’ve eaten enough lollies to know that when necessary, they can help in an emergency or to help attend to an important task.
When energy is so compromised that one is not able to eat their energising food, a lolly a little before can assist. Possibly a lolly pop. I actually nearly lost my life one afternoon when a lolly went down my throat and I wasn’t ready. Because it was still full size, it became lodged and was incredibly painful at the same time as blocking my airway. So mums and dads know their child and how well or not their child can organise and manage a lolly. I found hard candy to be the most helpful, which is why I suggest a lolly pop, a) safety and b) its very tempting for those hands to make the effort to hold something.
I’ve never been able to spend time with another person with Rett except my son Sean. I’m pretty sure I’ve seen a few ladies, just a few, in passing, and I recall seeing an advertisement for a disability gathering via the post which pictured a young lady with Rett and her mother. It didn’t say her disability was Rett Syndrome, but I’m fairly certain that was her disability. And a very pretty girl too.
Having this feature of Rett, that to some looks like ‘laziness’ is really unfortunate because the effort is actually really, well I make it look easy, it’s not though.
Another way to gain an insight into how much effort, as well as difficulty is being experienced by the individual. I feel really heavy and my head feels really heavy and it takes a lot of energy to hold me and my head upright. If one really can look at me, you will see that I am balancing my head on my neck and my body on the tops of my legs. It saves energy to do this but if I don’t I will run out of energy quite quickly. So looking at your child, young or old, if you see this then you know they are dealing with this same difficulty. When someone holds you and takes some of your weight from you, it is one of the most comforting things, it is wonderful.
By Fiona MacLeod ©
