2nd Annual Shaffer Halloween Bash

Last year we started a tradition of having a little Halloween party for Lu and her pals so she can enjoy Halloween without the usual trick or treating part. Being that we live in a very rural area, if we wanted to take Lu trick or treating it would be an agonizing process of getting her in and out of the car and either awkwardly carrying her to the doors or getting her wheelchair in and out over and over...plus she is in bed by 7:00. So, trick or treating just isn't a super feasible option for us. And of course she wouldn't be able to eat most of the "treats" that she would get anyway. So, we have a little party so she can dress up and see her friends dressed up and eat snacks and have fun.

Lucy's shaking episodes have been making her fall asleep afterward for the past week and unfortunately she had one about ten minutes before party time. She slept for a little bit, but we were able to wake her for the party. While we were eating she said on her Tobii, "I don't like it, it noisy". We talked about how parties are usually a little noisy and I reminded her of how excited she had been all morning for the party. She wasn't crying or upset, and was actually smiling, and eventually it got a little quieter so she seemed ok.

Lucy has recently become a big fan of Tinkerbell from watching the Tinkerbell movies we borrowed from the library, and when I asked her if she wanted to be Tink for Halloween she enthusiastically nodded yes!  I found her this adorable costume at Kid to Kid:

I made a homemade Peter Pan costume and Chad was a homemade pirate:

And all of her sweet friends came to be with her:

I had also decided the night before that we would play a version of charades where we would be playing fast and loose with the rules, like the only rule was just don't say what you are trying to be! Chad and I came up with a bunch of Halloween characters and things and then I put them all into Lu's Tobii. I included them in a blank page within "special events" in her PODD because that is where all of the holiday words are. So this way she could guess along with the other kids. And in the morning I was showing her the page and telling her what we were going to do and she kept choosing Frankenstein over and over again, so I asked her if she wanted to act out Frankenstein for everyone. She said yes, so I changed the Frankenstein button to say this:

So when she activated it, it wouldn't say Frankenstein, and then we tried to walk like Frankenstein. When it came time to do it in front of everyone, I gave her a little time to activate it, but then I did it for her. When she is in front of a group like that I always worry about her feeling too much pressure and it being even harder to do what she wants. So I activated it, she did her act and someone guessed! Then a few minutes later she activated it on her own! It was great! 

What was also great was just the whole party in general. I don't know if people realize how much it means to us that they come and participate in a Lucy friendly way. We just feel so grateful to have an amazing group of friends and family that support us and love us and join us when we need to do things differently. We take Lucy out into the world all the time, but sometimes we just like to bring the world to her. Instead of figuring out how she will fit into what we want to do, we make everything fit around her. She enjoyed baked beans, Mac and cheese, and Reese cups for lunch, some of her favorites. We played a game pretty much designed with her in mind so she could actively participate, and we brought everyone to her. Sometimes it just needs to be like that and we are lucky to have so many people in our lives that understand that. 

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.

Teaching Lu

I just wanted to share a link to a sister blog I have created called Teaching Lu. As the title suggests, it will be all about how I educate Lu. It will be about activities that we do, what works, what doesn't, and how I decide how to teach her. A LOT goes into teaching Lu. A LOT goes into helping Lu express anything, let alone information that she has learned. I am pretty excited to write about it as I think about it all the time.  It will be interesting to see if readers have ideas or tips to help me out, as well as my ideas, thoughts, successes, and failures being useful to others.

Here is the link, and thanks for reading!

Teaching Lu

"Healthy"

I read this article by a father of a son with special needs yesterday. I have read a similar one in the past. Take a moment to read it:

http://themighty.com/2014/08/why-i-stopped-saying-just-as-long-as-its-healthy/

It raises a good point that most people might never even think about, the statement, "as long as it's healthy", but then what if "it" isn't? Life will end? You won't love your baby the same? I realize that it's a statement that is just part of small talk. It's what people say when they are expecting a baby, but again I ask, "What if it isn't?" I think it's a stupid thing to say, and I'm sure I said it plenty of times, without a second thought. 

What I have been having trouble sorting out in my brain is the idea that Lucy is not healthy. Technically, by definition, I guess she's not. Lu can't talk with her mouth, she cannot walk independently, she cannot use her hands functionally, she has to have a feeding tube because her mouth isn't capable of efficiently eating enough food to nourish her body, she holds her breath, grinds her teeth, has low muscle tone, tremors, wears braces on her legs, has chronic constipation, gas, and reflux, and takes a pile of medicine daily. AND, now we are worried about seizures again because Lu has started having almost daily episodes of stiff arms, wide confused eyes with dilated pupils, pale splotchy face, and uncontrollable shaking. In the past ten days she has had 1-2 episodes 8 out of the 10 days. We are going in for yet another extended EEG on September 30th to determine what is happening and if seizure medication is needed. So, just one more point against "health" I guess. 

In my mind, I have such a clear separation of who Lucy is and what Rett Syndrome is, that it guess I don't consider her to be "unhealthy". And when I hear people talking about their relief at how healthy their baby or child is, how they feel like they have won a grand prize, I immediately feel indignant. Like, just because Lucy is not "healthy", I didn't win a prize? Like I don't think Lucy is the most amazing, beautiful, incredible child we ever could've created because she was born "unhealthy"? Once, when we were at the training to learn the PODD, another mother asked me in the restroom if I was a mother of a child with Rett and I said yes, and she said, "Oh I'm sorry." I get why she said it, I'm sorry too that Lucy was born with this horrible, debilitating condition, but I have never, ever once been sorry that I am Lucy's mother and as soon as she said it, I felt like she also socked me in the gut.  I am sad all of the time that Lucy's life has to be so hard for her and, that there is so much that just isn't possible for us as a family, but I never feel like I didn't win just as big as every other mother I meet. 

Lucy is Lucy. She is a whole person whose mind, and heart are 100% healthy. Rett Syndrome is a condition that makes her body not work, and in essence makes her "unhealthy". I've said this before, but I have been thinking about it a lot, because Rett Syndrome is not all she is, it is not who she is, she just has it.  It's an obstacle that we have to overcome each day. But in spite of it, her person, her essence, all that she is comes shining through all the time. That's why I guess I don't think of her as "unhealthy" on a regular basis, I just think of her as Lucy; my daughter who loves to cuddle, and play babies and Mr. Potato Heads, and read all kinds of books. My daughter who loves to swing, and ride her bike, and who especially loves to swim. My daughter who loves cheese, and Mulan, and the Magic Treehouse books that she and her Daddy have been reading.  My daughter, the artist:

(Fingerpainting)

The author:

(Lu used the alphabet on her Tobii to "write" this.)

The beauty:

It just so happens that she also has Rett Syndrome. 

The Hard Way

Last Sunday morning began like any other morning. Lu had her feed, watched cartoons, and was in a fine mood. When we brought her out to the table for breakfast (chocolate chip pancakes!) she got very upset. Tears, quivering lip, scrunched up face, heart breaking cries...the whole works. I persevered for a few bites of pancake, which she ate, but her sadness continued to increase in intensity. I asked her to use her PODD on her Tobii to try and tell us what was wrong. When it opened she immediately said, "I want, cuddle".  I took her over to the couch and after about 10 minutes of cuddling this is what happened:

And she slept for over a half hour, which is a long nap for Lu. We then returned to the table, Lucy full of smiles, and ate our reheated pancakes. At this point, it seems like a simple enough thing that happened: I asked why she was crying, she used her Tobii to tell me, and then I fixed it. But it isn't simple, and the process to getting here, to this point, which I still view as a very beginning point, has been anything but easy. 

The PODD system itself is quite a process to learn.  It is an awkward feeling at first when you begin to carry the book around everywhere with you, especially because you feel like everybody notices. I felt nervous and self-conscious.  It is hard to "Be Zen", like Gayle Porter, the creator of PODD encourages you to be, when you are a nervous wreck about making sure you model enough to teach your child how to use the system. It can take a long time for a child with severe apraxia, such as children with Rett experience, to have a reliable yes and no head movement. 

Then...we decided to begin the process of acquiring her Tobii. I contacted our local sales rep. We did a trial of both the Tobii and the Dynavox eye gaze system. Our early intervention speech therapist wrote out a long, and in depth report on how well Lucy used the Tobii. We submitted everything. Our primary insurance approved it right away, but Lu's secondary insurance, her state insurance, stubbornly denied it over and over. We got it anyway by stating we would pay any part that the primary wouldn't. Luckily we didn't have to pay anything. And that was all just to get it! I taught myself to program it, to make pages, games, and books for Lucy. With the help of Linda Burkhart, our PODD trainer, I figured out how to get the best version of the PODD on the Tobii, and it was a whole different piece of software that I then had to learn, and figure out how to program those pages.

 And those are all parts where Lu wasn't even involved. None of that included actually teaching her how to use these systems. We have been so fortunate to have wonderful speech therapists throughout this journey, first my cousin Danielle who worked with Lucy three days a week for two years, and now our therapist, Roxann, through the intermediate unit.  With Danielle we started from nothing and Lucy learned to make choices, to use her head to indicate yes and no, and then began to learn to use the PODD. And now with Roxann, Lucy has the basics down and is learning how to actually use language.  

I model language to her all day with both her paper PODD book, and on her Tobii, and of course just by talking to her all day long. We always make sure she has one or both with her no matter where she is. Our old pt gave us a mount for on her wheelchair, so her Tobii can always be with her when we are out and about. In her Tobii, there are many places where she can say she wants to use her book, and in her book she can say she wants to use her Tobii. And, I can get almost any information from her with simple yes and no questions as well.  

One of the most important things I took away from our PODD training was that the PODD is not work, it is Lucy's voice. I have said this over, and over, and over again to anyone who has needed to hear it, and I believe that keeping that attitude always in the front of my mind is what has made Lucy love it so much, and what makes her excel at using it. It is hers. She can use it or not. We don't "practice" using it, or ever make her use it. Look at her face in this picture below. We were adding items to lists in her new PODD book which has 64 pictures per page.

She just cares so much. She knows it is all for her, all so we can know her and know what she has to say.

But, it is hard. I will never deny that there are days when I drop the ball and maybe don't model much. I will also never deny that there are days, and times of the day, where it all just exhausts me. On top of all that goes into Lucy having meaningful communication, then we add her wide array of equipment that she needs just to have her body positioned safely and comfortably. The next time you witness a child just standing and talking, or even yourself standing and talking, think of this picture:

You can see extra wide shoes to go over special braces that are needed for buckling into a less than tiny standing frame. Then, her Tobii, mounted on the rolling floor mount that is so weird and awkward I've almost made a habit out of banging my toes off of it. All of this dragged back the hall so Lu could stand and talk to me while I put laundry away. It totally sucks, but I do it because she needs it. She needs to stand, she needs to stay strong, but even more importantly, it is her right as a human to be able to communicate with me whenever she wants to, and so that includes her communication system always being available to her. 

I just feel so strongly that all humans, regardless of their abilities, have the absolute right to be able to communicate with the world. "They are nonverbal" should not be the end of the sentence. That's just not all their is to it. Yes, I can generally tell when Lu is hungry, tired, needs to poop, is bored, etc., but that is not exactly rocket science, that's just being a mother. I would never even dream of saying that's all I need to know about my daughter, and I don't think any other parents should have to settle for that either. In the short time we have been in this world of a Rett Syndrome and learning all their is to know about it, what I have noticed is that there might be a lack of aggression on the part of speech therapists to obtain actual, functional, robust communication systems for children who are nonverbal. Not in our personal experience of course, but in other parents I have met. I did a training on Rett at our school district's paraprofessional conference last month, and in the two sessions I had, there were a handful of aides who worked with children who were nonverbal, and not a single one of them had a communication system of any kind! NOTHING! 

In my opinion, it is unacceptable for a human being to not have a means of communication. That is just my opinion of course, which I can share because this is my blog. It is also my opinion that communication systems are lacking in children's lives because it is hard work. Lucy's speech therapists and Chad and I have busted our butts, as I described above, for the past two years to get her to where she is now.  And I say all the time that this is just the beginning for her, and I imagine that in not too many years, she will be able to use her Tobii as fluently as if she were using her mouth. I believe that with all of my heart. I believe it is our right (and obligation) as parents of children who cannot communicate in the typical fashion to not just request that they be provided with alternative means of communicating, but that we demand it. It is their right. "Communication is the essence of human life..."says ASHA, the American Speech-Language-Hearing Association. I've shared the entire quote before, but that is the part that always pops into my head. Lucy deserves everything in life that all other humans get to enjoy automatically, even if it requires huge amounts of effort on the part of everyone around her. She just deserves it. 

Strolling for a Cure

On September 6th, we will be joining other families in a strollathon, the signature fundraiser of Rettsyndrome.org (formerly known as the International Rett Syndrome Foundation) to raise much needed funds for the search for a cure. I have spoken recently about my feelings about a cure and how it simply terrifies me to think about it too long and too hard because my hopes just start to soar. But regardless of my protective coping skills, progress is being made all of the time in the search for ultimately a cure, but also treatments for symptoms. 

In 2007, Rett Syndrome was reversed in mice in a lab. Click on the link below and watch the first two videos on the page. The first is before the reversal, and the second is after. That could be Lucy someday. But isn't everything always about money? Unfortunately it is, and we as a community of parents and loved ones of girls with this miserable condition are often the ones out beating the streets, asking (begging/pleading) for the crucial funds to continue research so that our sweet girls might someday get to live full lives. It costs money to safely translate the success in mice to humans and we just want to help move that along as much as we can. 

Reversal of Rett in mice

There are also several very promising clinical trials taking place throughout the world, testing medications that could change Lucy's life. They also cost a lot of money. I know that money doesn't grow on trees, and that everybody works hard for what they have.  We are just asking that if anybody who can spare anything, ANY amount, would be willing to sponsor us in the strollathon, we would be eternally grateful to you and thankful for your caring and generosity. We just want to see a cure within Lucy's lifetime, and from what we hear, this is a very real possibility, but not without the funds that keep the wheels turning. 

Anybody who is interested is more than welcome to also join our team and help to raise money by getting their own sponsors, and participating in the strollathon with us. The more the merrier!

For anyone else, please feel free to make a donation that is comfortable for you. Again, the Shaffer's and the Rett community will be forever thankful to you!

Please visit the link below to join Team Lucy OR to make your donation:

Team Lucy's Strollathon Page

Here's Lu helping me to prepare a container for some Brussels sprout seeds yesterday afternoon. She always asks to garden. What if someday she could do it without a walker, braces on her legs, and me needing to help her hold all of the tools, and guide her hands for planting the seeds? What if she could do that someday? 

Every Single Word

You know, I wonder if people who read my blog ever think, "Ok Julie, we get it, Lucy is awesome, and she talks to you all the time!"  But what I really want others to understand is that Lucy talking to us, communicating with us, and sharing herself and her personality with us NEVER, EVER gets old! Once a person [doctor] tells you there will be nothing your child can do, she will basically be a vegetable, and then all day every day she proves him wrong, you never forget what he said. I am never less amazed, or delighted, and I am always thankful every single time she talks to us. Even when she's just exploring and "babbling" by saying random words, she is talking! That might sound unrealistic, yeah, sure Julie, you cherish every word that your daughter utters all day long...but, yeah, I really, really do. 

In the past couple of weeks Lu has been developing a little of the "mine" attitude that children go through and we are loving it! We talk to her about sharing and not being greedy, but at the same time, it makes our hearts melt to have Lu doing anything "developmentally appropriate" such as claiming everything as hers. She loves chocolate no-bake cookies. I don't understand why they don't make her choke, but they don't and she wolfs them down.  I don't make them because my Mom makes them pretty often and sends some down to us. The other night before supper I brought some in and showed them to Lu and said Grammy sent some of her favorite cookies. Well, after supper, Chad opened the container to have one and Lu immediately said, "Mine! Mine!" with her Tobii. It was adorable! And so unexpected! We reminded her that Grammy sent them for everyone and we would share them. Then, a few nights later, also at supper, Lucy said, "I want, mine channel, mine, mine, channel" or some similar combination of those words. And she seemed to be looking at Chad. I asked if she wanted to watch her channel when she and Daddy watch cartoons, and she emphatically nodded "yes"! 

Chad and I tell Lu all the time if she thinks of anything in particular that she wants for supper to let us know and one morning she said, 

Needless to say spaghetti was served for supper that night and Lu and I made a homemade apple pie in the afternoon to have for dessert. One day last week I made a pageset up in her Tobii with some fabric choices from the armbraces.com site to see what Lucy might like for a pair of arm braces she has started to need. Immediately, a pattern caught her eye, and she repeated it over and over, even after I encouraged her to explore her other options, she still constantly came back to that one choice. After some time for Lu to look at all of her choices I asked her which was her favorite and not surprisingly she chose her first choice. She knows what she wants and is really starting to be able to tell us more and more, so she doesn't have to just settle for whatever I or anybody else chooses for her. 

Yesterday morning Lucy said:

Why, yes, yes we do, Lu. Perfect observation.

I cut my finger badly two week ago and for like a half hour it just wouldn't quit bleeding. When I finally got it under control Lu and I sat down to eat lunch and she said with her Tobii, "Are you alright?" That us all one button she can choose. She might want to hog all the cookies, but she still has compassion and cares about others which to me is one the most important character traits to instill in a child. 

And last night at supper, I told her she could have [one of Grammy's] cookies for dessert and then chad reminded me that we also have ice cream and hot fudge that she could have. I said to her, "Or you can have ice cream if you want." She said with her Tobii that she wanted to use her book. Right now Lucy is transitioning between a 20 per page book to a 64 per page and when she says she wants to use her book, I give her the choice of which she wants to use. She has mostly been choosing her new book, but last night she chose her old book and said, "I do, want it". I asked if she meant she wanted the ice cream and she very emphatically nodded "yes"! 

I said to Chad last night that I believe eventually Lucy will use her alternative communication system as naturally and fluently as we speak with our mouths. I think getting girls started as early as possible is critical to helping them understand that this is just how they do it, and it becomes the norm to them. It often occurs to me, kind of surprisingly at times,  that Lucy doesn't talk with her mouth. We talk to each other so much all day, that I seldom dwell on her being "nonverbal".  Even when we might be on the couch, in the middle of a crying episode where she is so distraught that she won't even look at her book or her Tobii, she will still often answer my yes and no questions to determine if she knows what is making her cry so I can possibly help. Does Lu use her mouth to speak to us, aside from the occasional, no, yeah, ma, da, nope she doesn't, but does she let the world know who Lucy Shaffer is anyway, uh yeah, all day, every day!