This is why she rides

This winter, Sarah has had very few riding lessons. This is because of the snow storms that came each week right on her scheduled riding days. It was frustrating, but as it's an hour drive on the highway to get there, I couldn't take the risk.
So now we are getting back in the groove. Yesterday was Sarah's second lesson in a row.

Pause.

It was enlightening and a bit frightening. But she is OK. I'm still recovering.

You see, I generally take her riding quite regularly. We plop her on Casey's cute purple saddle and off we go. She rides in the ring or outside, depends on the weather. We generally do the same manuevers each time...turns, steps over poles, stop and start. And Sarah sits there, seemingly, mostly, effortlessly. She does not hold onto reins, she just balances herself with her feet in stirrups. It looks like she is really doing nothing at all as we go round and round.

But she is doing something. And now we know this for sure.

So, yesterday, she rode outside. Even though it was in the 30s, the sun was very warm. Usually, she rides for 45 minutes. Last week, she rode for only about 30 minutes in the ring because it was very cold. So we were outside, Casey was ambling along, god bless him, and we were about 20 minutes into the 'walk'. Casey took a slow turn to the left and I saw Sarah give a slight jerk to accomodate his turn and then she began to lean over to my side, way over. I tried to push her back up straight in the saddle but I couldn't. She started fussing because she knew she was in trouble. She didn't have the trunk strength to hold herself up any longer. I pushed and pushed but couldn't help her.

OK, so I told the instructor that she was coming down off the horse, we released her feet from the stirrups and she landed in my arms like an infant. Well, we were far from my car or the stables and there was no way I was going to be able to carry her more than 5 feet. She is heavy and dead weight. So the instructor ran off with Casey to get Sarah's wheelchair.

In the meantime, Sarah's chin was down on her chest due to the awkward position that I was holding her in, and I heard her struggling for air. A very deep snore. The strap under her chin from the riding helmet was cutting off her airway. So that was it. I had to lay her down. Sarah and I went slowly down to the pavement, her draped over my legs, and I undid the helmet strap and flung it off to the side.

So there we were. I was sitting on the cold pavement, with Sarah's body lying partway on me, partway on the asphalt, her head in my lap. She accepted the situation and began her ususal hand dance.

The instructor brought the chair and somehow, I DON'T KNOW HOW, we got her off me and the ground and into the chair.

So that was that.

This is what I have learned, because if I don't learn something, then, really, what was the point of this?

1. Carry my cellphone with me at all times. Even during a 1/2 hour lesson. Because I might need to call 911. You never know.

2. Stay close to Sarah's wheelchair after 15 minutes of riding. Or bring the chair to the area where we will be riding.

3. Inquire about another person to accompany us on Sarah's lessons.

4. And finally, Sarah loses ground when she does not ride regularly. It is clearly maintaining her trunk tone.

Imagine what would happen if she did not ride at all?

Today, we are back on track:

Only a few months left

John is graduating from college in May. College. All over. It seems to have snuck up on me so fast. Where he will live and what type of job he will get after he graduates is still up for grabs. And that's OK. One thing at a time.
It just doesn't seem possible that after May all 'formal' schooling will be complete in our little family. (Sarah has home based schooling) And the group will have officially moved into a new chapter. However, this chapter has no boundaries. It is a wide open book as the boys make their way into the work world and through their 20s.
It is certainly going to be interesting with two out there now floating on the open seas. Jimmy's been out there bobbing around quite happily for a few years and now John will take the jump. I'll hang around and throw the lifering if needed. But I have a feeling they are both going to do just fine.

And here are the boys when it all began, each at age 4!
John is in the pink, Jimmy in the red.

Why Rett?

I had mentioned a while back that I would put up a list of which of the qualities of Rett Syndrome Sarah displays and tonight I had a little time to write it all down, so here you go:

scoliosis and kyphosis
small hands and feet
breath holding
parkinsonian tremor
anxiety
compulsive hand movements
loss of motor control
poor gut motility
lack of puberty growth spurt
apraxia
loss of hand function
normal head size at birth
blue feet
vacant spells
reduced bone mineral density
nonverbal
innability to chew
dystonia
normal prenatal and perinatal history
teeth grinding
laughing spells
lower limb muscle atrophy
deceleration of head growth in first year
delayed from birth
eye pointing


Here are the ones she doesn't have(yet):

-seizures(not all have seizures)
-disrupted sleep pattern(she requires lots of rest/sleep, more than the average bear, but is that considered 'disrupted'? Not sure.)

Now, granted, kids with all sorts of different issues will show some of these things, but the key here is that Sarah shows all of them, except one or two.

So that's why I stick with Rett.

Sarah is turning out to be a teeny, tiny 14 1/2 year old. She holds at 4'10 inches and 69 pounds. She continues for the past 8 years to wear a size 12 toddler shoe. Her pants have remained a girls size 10 for the past 3 years. She is done growing and at this point I work to keep her weight up.

Lately, there are a few troubling trends. One is that she has more and more hand tremors which is starting to interfere with the one thing she does do purposefully with her right hand: turn pages of a book or magazine. I notice that sometimes she cannot get her hand to turn the page, it just tremors and seems stuck in space. At which point I help her and get it going again. Secondly, she is having trouble initiating a swallow. It only happens when I am giving her a drink. I hold the cup to her lips but it takes her 10 seconds or so to get the swallowing thing going while the liquid drips down her chin. She works hard to do it because I see her hands fist up and get tense. This has never been a problem before. And finally, she is not going to the bathroom unless I give her a suppository. TMI, I know, but it is a worrisome development.
The first problem I attribute to her increasing dystonia, the second to her increasing apraxia and the third? Not sure on that one. The first two make me sad because I watch her struggle to do something which just a few months ago was effortless.

Boo. Hoo.

On a good note, she is happy and healthy. She listens intently to what we are explaining to her and now, she listens to me on the phone when I call from work! This is awesome because before I leave I tell her I will call her on the phone. She understands what I am saying while she listens to me babble on to her about her evening. It's great to be able to connect with her when I am not there. Pretty soon we are going to have to start calling her brothers!!!! Ha! She does not speak, but she is listening.

Her cognition is growing, while her body gets stiffer.

Rett Syndrome is a cruel disorder.

Just saying.

Simply Sarah

Sarah has never had an MRI of her brain. As in never. Ever. It's not that it hasn't been suggested once or twice or three times in the past 14 years, because it has. And that's the operative word here...suggested.

And I guess, from the outside, looking in, that seems kind of crazy. Not to do an MRI. Because her constellation of issues suggests something neurological. I mean, she can't walk, talk, feed herself, put a piece of clothing on. Her left side is much more affected than her right.

When she was very young I was told many things. At age one, maybe a stroke in utero. Atypical cerebral palsy. She was diagnosed with left hemiplegia cerebral palsy at the age of 2 because everything seemed left-sided. At the age of 4 it went to spastic diplegia CP because it became clear both of her legs were affected and finally at 6, it changed to the worst case scenario-spastic quadriplegia CP because none of her extremeties worked right.

Sometime around the age of five I was told she had autistic features as well. But she would never receive an autism diagnosis because she did not have enough features of it.

The cerebral palsy diagnosis is not right either. We go to the orthopedic surgeon and wait in the waiting room with lots of children who have CP. Sarah does not fit in this group. She sticks out like a sore thumb. But we hang onto the diagnosis for insurance purposes.

Because there is nothing else to use. Nada. Zippo. She has no official diagnosis.

And so, wouldn't you think I would go running to an MRI to see if that will give us a piece of the puzzle that she is. Yes, one would think I would. I mean, I would think I would.

But I don't. My reason now is different than my reason then. And my reason then was because of this event:

When Sarah was about 2 a mom called me who had a son about the same age. She had read that I had piece of equipment for Sarah and she wanted to come see if it would be good for her little boy. I had never met her before. So one afternoon she drove over and brought her son with her. He was lovely and blond and engaging. She placed him on our carpet and he inched his way over to our front door to slowly move it back and forth. She told me that he loved doors. So sweet.

However, she had had an MRI done of his brain a few months prior and all she could focus on was what was wrong with his brain, what it was missing and what it was shaped like. It was like, when she looked at him, all she could see was his brain. It was all she talked about. It seemed that the MRI had taken away her enjoyment of her little boy. Colored it so completely. She couldn't get past it. And see him. I felt so sad for her.

When they left I knew that I did not want an MRI for Sarah. Because I did not want that to happen to me. And I knew it would, if something was found. I would lose my pure enjoyment of my girl. I knew I would and I wasn't willing to go there. Ignorance was bliss as far as I was concerned. Looking back, it was definately the right decision for me. And no one was insistent anyway.

Fast forward to these latest years and the reason becomes more about anesthesia. And how any sedation sends Sarah into an ileus. And at this point we would only be doing it for our curiosity. Which is not a good reason to put her through pain. Not to me anyway.

The Rett Syndrome specialist wants one. His reason? Because if she had a 'normal' MRI, he would then give her a Rett Syndrome diagnosis, even though they cannot locate it yet in her DNA. THAT gave me pause, for about 2 minutes. Is it worth putting her through it for the diagnosis? I decided no. Nope. We can live without it, even though that's exactly what she has, in my humble opinion. And I join support groups and treat her accordingly. She is followed at the RS Clinic at Boston Children's. She is given every advantage.

Sometimes I think it is a good thing to not have a diagnosis. Because then, there are no preconceived limitations. No one knows what her potential is, not the schools, not the doctors. She is just simply, Sarah. And the focus is on the child, not the diagnosis.

So why am I even posting about this? Because I read many blogs concerning special kids like Sarah and it seems that all of them have regular MRIs. So, I just wanted to share the flip side of a child who has never had one and probably never will, unless something drastic happens and it becomes imperative. At which point. I would suck it up and roll her in, anesthesia and all.

The end.