Seizures and not seizures

Rett Syndrome is so maddeningly stupid that I just can't get over it sometimes. Things had been going really well with Lu since getting her feeding tube, so I suppose we were due for something to change, and change it did. About a month or so ago Lu started having some episodes that really looked like seizures, for the most part. I have some excellent videos, but have decided to just explain them and not show Lu in such a vulnerable state to the whole world. 

The most severe form of these episodes includes her arms and legs suddenly stiffening, her arms slowly going out to the sides and raising into the air, and then being followed by uncontrollable shaking. During the shaking her eyes are huge, her pupils are dilated, her face is pale and splotchy, and she is not breathing. They last much less than a minute, more between 30 and 45 seconds. These shaking spells are happening approximately once a day. Sometimes there's no shaking in a day, and sometimes it happens more than once in a day. Then, all throughout the day, Lu is also having very brief spells with just the stiffening and arm raising, but no shaking.

So, off we went to the third extended EEG if Lucy's lifetime. This time was relatively painless since these are occurring so often, we only had to spend one night in the hospital, and found out they are not seizures. Yes, it is great news. We don't have to give her a medication that might make her groggy, and we don't have to worry that they won't respond to medication and damage will be caused to her brain. However, 80% of all people with Rett will experience at least one seizure in their lifetime. Yes, maybe Lucy will be in that lucky 20%, but I'm just not holding my breath. We haven't missed out on any of the other big symptoms of Rett, and I just don't feel it's realistic to assume that we will miss out on seizures. I hope we do...more than anything else, oh I hope we do. They terrify me. People die from them. 

Plus, since they are not seizures, there is nothing we can do, that we know of right now, to help these spells. When she just raises her arms and goes stiff for a few seconds, I don't get too worked up, and it doesn't really disrupt her life. But, the "shakers" as we have started to refer to them, are utterly heartbreaking. Lu looks scared and confused, and I can't help her. We can just hold her hand, and smile and tell her it's okay, it'll be over soon. We will see Dr. Sasha at the end of the month, and she may or may not have any other thoughts on what they could be. But, chances are that since they are not seizures they are just another thing that Rett Syndrome is doing to Lucy's body, and we just have to let it happen and get used to it, like tooth grinding, breath holding, and hand flapping. It just is what it is I guess. 

Rett Syndrome is the pits...

Here is Lu covered in electrodes...and still smiling.

And then look what the electrodes did to her skin. She has/had big scabs all over her head. It makes my stomach sick that she has to go through these things.

Longing For Winter

Only when we are in Nanny and Pappy's pool or eating good food at a picinic am I in favor of summer this year.  I am feeling intolerable about any heat, and I find myself almost counting down to fall.  This has all been pretty baffling to me as I have generally loved summer and hated winter for most of my life. So, when I began seeing red and yellow leaves in the yard last week, I instantly felt relieved and then confused by my relief.   Why do I dread summer this year? I have been thinking and thinking about it and gradually came to the realization that winter is easy on me and Lu. I know, for those of you that must leave the house for work, school, etc. it is a nightmare at times, but if the roads are bad, or it's too cold, we don't have to go anywhere.  We are obliged to remain warm and safe in our sanctuary.  But in summer...we are expected to go places.  

Also about summer, kids are out of school, schedules are forgotten and unnecessary, and there is a constant feeling of freedom...unless you are us. We still have feedings every four hours.  It is still extremely difficult to get around to places. It's too hot for Lu a lot of the time. And regardless of the season, Lu still demands to be in bed at 7. So maybe I like winter better because everyone else has more of a structured life too, and then we don't feel so left behind. 

Right now we are waiting on some volunteers to build us a ramp.  We have the supplies sitting in the driveway and we are just waiting for the carpenters to have time.  Having a ramp I think (hope) will make getting outside a ton easier for us as I can then keep Lucy's wheelchair in the house and load her up and just zip outside to the swingset, to the yard, for a walk or whatever we want to do.  As of right now, I have to go out to the car, unload her wheelchair, load her into it after carrying all 40 pounds and 41 inches of her outside, and then reverse the whole process when we are done.  So, all of the things I imagine us doing, like catching bugs, swinging, picking flowers, gardening, gathering supplies for nature art...all seem so close, but so far away when I look out the window.  Everything is right there, but this whole awkward and laborious process stands in our way.  It's like all of the fun we could be having is mocking me every day, and if summer would just go away, then I wouldn't feel guilty for not doing more outside.  Let me tell you one thing though: when I do get her outside to ride her bike, or swing, or anything else I have mentioned, I am so damn proud of myself.  In spite of the fact that a typical child could do five fun things in the time it takes us to do one, I am just happy that we got that one thing done, and Lu had fun. 

For example, we did get a little "gardening" done the other day.  It was on the deck, and in containers, but we still had a great time doing it! We planted basil, Venus Flytrap seeds, peas, and a Brussels sprout plant that we thought had died, but it came back to life.  I helped Lu get her hands right in the dirt and she even had it all over her lap!  Yes, Lucy actually getting dirty was one of my favorite parts of the whole thing!  And, she also was able to hold the pea seeds in her hand, and then drop them in the trench we made for them while I just supported her arm to keep it steady. 

I guess maybe what I'm saying is that there is just so much pressure to have a big freaking blast in the summer, and I've had enough of it for now. We go to the pool every single chance we get, which is most days, and not only does Lu LOVE it, but it is good for her, so that is a thing we can do and I have gotten pretty adept at the process of getting her there and into the water, even if no one is there to help me. The pool is the only thing I will lament about the end of summer. (And getting to go for walks with my Mom in the evening.) I miss it desperately all winter for both Lu and myself because I too love to swim.  Anyway, we are at least lucky to have that so easily accessible and lucky that Lu enjoys it and it makes her happy.  But, I will still not be sad when the pressure to go outside and have the time of our lives doing super fun and cool summer activities has passed.  I guess this year I am a Grinch about summer which probably means I should only allow myself to be half as Grinchy about Christmas this year. Thanks for listening to my whining!

Summer Freedom

It has crossed my mind many times already this summer what was going on with Lu last summer...a lot and not much of it was good. We were afraid she was having seizures, she started hyperventilating, falling over from sitting, and she lost her remaining hand functioning.  She couldn't chew on her chewlery anymore or even get it up to her mouth, she was losing weight, and coughing and choking when she would agree to open her mouth to try and eat.  She was also falling asleep all of the time, especially at the table and I had to always have the iPad with us to play her music to wake her up and keep her awake, specifically, "I'm Yours" by Jason Mraz would instantly perk her up and put a smile on her face.  I keep thinking about it because she is just doing so, so much better now! We have been trying to figure out the culprit of some daily crying, but that's common and comes and goes and we will figure it out...it's nothing compared to what we were worrying about last year at this time.

We just had three weeks off from therapy and while I always do miss our therapists because luckily they are all great ladies, I didn't miss having appointments all of the time and every day was all ours, we could do whatever we wanted, and/or whatever Lu's mood allowed. I discovered an amazing website, www.artfulparent.com that is just full of super fun ideas for doing all kinds of art with children. Besides the wealth of information, I especially love that the general mood of the site is one that supports very open-ended art, so there is no cookie-cutter end result that generally is boring, and often not all that possible for Lu to complete.  Most of the projects I have looked at are all things that Lu could enjoy with minimal adaptations.  Here is the first activity we tried:

These beautiful works of art were made on watercolor paper with shaving cream and food coloring! It was so much fun, and messy and creative, and Lu did it while in her stander so we were getting some pt in for the day too! Here is a link to this project:http://artfulparent.com/2014/06/shaving-cream-marbling-with-liquid-watercolors.html

We've been doing some swinging of course! 

And playing Mr. Potato Heads! I helped Lu take this picture with my phone and she was tickled with herself.  Her creation is the doctor! She has gotten more interested in choosing the parts using the pages I made for her Tobii. 

And, I found this little book at Ollie's for .99! In the back there was flash cards to cut out to practice matching the words with the pictures in the book because it is a "picture reader". We have read it a few times and so far Lu has correctly matched the words: tree, sun, house, stairs, and door with the pictures in the book!  We've been doing this activity while cuddling on the couch so it is a difficult position for her to make her usual head movements for yes and no, so I ask her to look at me when she sees which word she thinks matches.  I show her three choices, one at a time, until she looks at me.  When it is not her choice, she has even started closing her eyes so as not to look at me, but when it is her choice, she looks directly into my eyes.  So, we've been getting a little school work done here and there too.

And this last picture is of a travel-sized Hungry Hungry Hippos that I got at Kid to Kid over the weekend.  We haven't played it yet, but it have high hopes for the simple adaptation I added to it so she can easily hit the lever and gulped up the marbles! 

So, we have been doing some fun stuff.  Besides these things, there has been some bike riding, lots of picinic so and a TON of swimming! It's been a good summer so far! 

Going Places

Going places... It just seems to keep getting harder and harder, both physically and emotionally. Here are the basics we might need IF we don't need to do a feed while we are out: wheelchair, Tobii, wheelchair mount for Tobii, PODD book, backpack full of diapers, extra clothes, snack, straw cup, chin dabbers for dribbling and drooling. If we are going out to eat we also need Lu's silverware that she's used to using, bibs, after-meal meds, and sometimes a tiny food chopper. And if we are going to need to do a feed while we are out then we also need her little backpack that holds her feeding pump, the bag with formula and other accessories.  It is exhausting. My goal is always to do my best to scope out situations and places before we go there to eliminate as much of the unexpected as possible and to hopefully avoid getting all prepared for something and then arriving and finding out a place is not accessible to us. It doesn't always work out for us though, and I understand that is bound to happen, but it still is hard to handle, depending on the day and my emotional stability on that particular day.

I joke sometimes about being a little "hermitty", but then sometimes I find myself out in the world and my tendencies to just want to stay home don't seem all that funny as I can get very overwhelmed by the world and I just want to go home. I know it's not a good thing. I am okay when we are going to a place that I know well, like the library. I know how we can get in and out there. I know the librarians, and they are so sweet and kind. We are just about pros at going out to eat, so that is seldom stressful.  There are a few other places that work well for us.  But mostly, home is what is most comfortable and where we can be most successful.

The thing is, we can't really keep up with busy gatherings, like birthday parties for others, and big picnics and things like that. We are just always lagging behind and so not really getting to participate like everyone else. Not getting to do things like everyone else isn't a new thing, but lagging behind everyone is something that has increased.  It just makes us feel like we're not really part of anything sometimes. In addition to that, the heartbreaking realization that the world is just not designed for people in wheelchairs has been becoming more and more obvious as Lu gets older, and heavier, and we want to take her more places. For example, we thought it would be fun to take her to Penns Cave and learn about caves beforehand...but it is not one bit wheelchair accessible.  There are TWO, let me repeat that TWO ADA (which stands for Americans with Disabilities Act) certified trails in state parks here in PA. Luckily for us, one is in Black Moshannon which is local, but the other one is in Warren. Lu loves being outside and enjoying nature, but her ability to do so is severely limited. 

It is my opinion that by not bothering to make a place wheelchair accessible, the owners are basically saying, "People in wheelchairs are not welcome." I don't mean personal homes of course, but public places that are meant for everyone to be able to enjoy. In the year 2014, would it be okay to leave any other group of people out of a place? Would it be okay to put up a sign at Penns Cave that says, "No homosexuals allowed" or "No children" or "No African Americans"?  Absolutely not! And even though there is obviously not a sign that says "No people in wheelchairs", by it not being accessible, it is certainly being implied. 

So, we nearly need a U-Haul (aka our minivan) to go anywhere. And then when we get there we may or may not be able to enjoy ourselves depending on the level of accessibility. And even if it is accessible, we will most likely be lagging behind everyone and just trying to help Lu participate in any way she can.  Most of the time I think we are real troopers about it and we just do the best we can. However, sometimes, like everything else, I just let it get me down. We went to a family picinic at Parker Dam over the weekend. That's what prompted this post actually. It wasn't an easy time and it wasn't anyone's fault, it's just how it goes sometimes. I cried the whole way home. It just was hard, and it is really hard to watch Lucy seeing what she can't do. However, she very seldom seems upset by it. And if she was, she would say so.  I guess probably because this has always been her life and she just accepts it as it is. I know I need to remind myself to take a page from her book sometimes. It is what it is. We can't go everywhere, we can't do everything. We can't keep up with groups of rambunctious children. We are slow and have to take mounds of crap with us everywhere we go. I know. 

Lately we have experienced some social situations that just make Lucy cry and cry.  It seems like maybe she is overwhelmed and she can't handle it and so she cries.  It is confusing though because situations that I think are going to be too busy and crazy for her are fine sometimes, and then other times when I don't feel worried at all she has a tough time.  So, lately we have been worrying every time we go somewhere where there will a group of people that Lu might get upset and we will have to leave. It just seems like sometimes her senses give her a hard time and she struggles with processing what is happening around her. 

 One final, and major obstacle that we face when it comes to "going places" is that Lucy is very, VERY rigid about bed time. It is just like her mind and body start saying, "Okay, that's enough for today around 5ish and she is falling asleep in my lap on the couch immediately after supper, around 6ish, and this is before she has had her Trazadone that helps her stay asleep through the night. If this routine is messed with, Lu can sometimes deal, but will often-times have a total meltdown.  There is nothing that makes her feel better until she is in bed.  So, the Shaffers pretty much don't go anywhere at night....ever.  And that's just how it is. 

I guess maybe I wrote this to help all of our friends and family understand what all goes into us simply showing up somewhere, not mention what we do once we get somewhere.  

The facts are: 

-We will usually be a tad late.  

-We will most likely need to leave early.

-Lu might get overwhelmed and cry without warning.

-I get tense sometimes because I am out of my comfort zone.

-If an event is after 5:00pm we won't be coming.  It is nothing personal, it just isn't possible for us.

-We need time to prepare for outings so we unfortunately can't just go somewhere at the drop of a hat.

-Lu has feeds at 7am, 11am, 3pm, and 7pm, so we need to always be working around that schedule. 

-Some places aren't wheelchair accessible so we can't go to there.

-And the #1, most important fact of all is that Lucy's comfort, well-being, and happiness are my very top priority, and we just have rules in our life that we need to follow to maintain as smooth of a ride as possible.  If those rules hurt anyone's feelings I will not apologize,  but I will ask that you try to understand how difficult it is for us to go places, and understand that, like I said, it's not personal. It's just what we need to do.  

Here is a homemade haircut I gave Lu after trying repeatedly to figure out a time I could manage to get her a mile down the road for a haircut:

Here she is enthusiastically riding her bike in our own driveway:

And here she is comfortably relaxing with her Daddy on our back porch:

It's just easier at home. I hate to leave, but I will for Lu's sake at least! 

Waving the White Flag

The Germ

A mighty creature is the germ,

Though smaller than a pachyderm.

His customary dwelling place

Is deep within the human race.

His childish pride he often pleases

By giving people strange diseases.

Do you, my poppet, feel infirm?

You probably contain a germ.

       -Ogden Nash

Lu went back to school last week for the first time since Christmas break. Inevitably she got a little tiny cold that produced an indescribable amount of snot that has been making her throw her feeds up off and on since Sunday. She has no other symptoms, seems fine, but is so congested that it seems her belly is fully of mucous and makes her throw up when we put milk on top of it. I took the above picture on Monday. Her hair is all pulled back to avoid it getting soaked in puke, and in the picture you can see one of the two towels I had her swaddled in. She looks a little paler than she has been looking on her new diet of sufficient calories and nutrition. We have not left the house all week because A.) It's freezing outside, but B.) it is taking over two hours for each feed so she doesn't throw up and then time afterward to let her belly settle. 

Lucy has been sick more in the past 5 months than she has been in her whole life total.  It has felt like we spend most of the time just trying to get her well again from whatever germ she picked up at school. I know that kids get sick a lot when they first start school, but I believe since Lu has not been able to get all of the calories she needs, it has made her even more susceptible to every single stinking germ that comes her way. So, after much discussion and weighing of the pros and cons, Chad and I are throwing in the towel on school. We are waving the white flag, laying down our arms, and admitting defeat.  The germs have won. Our hope is that by gaining some weight, and having months of proper nutrition under her belt, Lu might be able to combat at least some of the germs she would encounter at school, and we can make another go of it in the fall.

When Lucy started throwing up last weekend, Chad commented it was obviously because she went to school that week and got a bug. I told him I was basically waiting for him to give the word that it was time to give up on school because honestly, I had decided in my heart, long ago, what I felt about school at this point. However, most of my reasons are far from the most logical. The main one being, I just like Lu to be home with me. I also wasn't 100% sure that she was ready for school, once we actually started. Other than the constant illness, my reasons for wanting her to stop going were more emotional, and not necessarily the most reasonable. So, as is often the case, I was just waiting for Chad to make a logical decision, because I would know that it really was time if he said so. Lu loved her friends at school and the kids loved Lu, and I hate to take that away from her. However, she is only three. She has her whole life to make friends and "socialize".  I'd rather she miss her friends and not get pneumonia than the other way around. 

So, on the heels of one big decision, the feeding tube, now we have made another one; no more school for Lu right now. It was hard. Her team of therapists and teachers have worked so diligently to try and make school work for her and we appreciate it so much. But, I am looking forward to keeping Lucy healthy, and happy, and whole, right here at home.