What You Don't See

Last week Lu and I went to Lowes to buy some perrenial flowers for a little space we are creating in the yard to attract tiny critters. The day we went to Lowes was not an incredibly sunny day, so a little overcast, and the temperature was mild, probably not 70. A perfect day for outside plant shopping. But, in spite of those comfortable conditions, Lu gradually wilted over the half hour of time that we spent browsing around the garden section. When I say "wilt", I mean she gets sleepy looking, her head droops, her face gets pink. She just isn't very engaged in what's going on around her. So we went to check out and the cashier made a comment about how cool her "iPad" was. I politely told her it wasn't an iPad, but a communication device that enables Lu to talk with us. The cashier then very respectfully looked at Lu and asked her name. Lu didn't answer because she was wilting, and frankly because she isn't quite to the level yet where she easily or quickly responds to a direct question like that, and definitely not if it's being asked by a stranger. And so when she didn't answer, and I answered for her, from that point on, the cashier spoke no more to her. We checked out, and she helped us out to the car with our bags, which was nice.

Then, when I turned to Lu to unbuckle her from her wheelchair and put her in her car seat, I suddenly saw what that cashier saw: a severely disabled little girl who couldn't even tell her her name. She saw a little girl drooling on her bib, struggling to hold her head up, flushed from the mild spring temperature, and half asleep...and suddenly I laughed! I was so surprised by my reaction that I had to stop and think for a moment as to why I found that assessment so funny.  It's really not funny. It's not funny to be aware that sometimes when people look at my daughter, they think they see an empty vessel. I had this same feeling a few months ago while I was feeding Lu her lunch at Valley Dairy, and I could feel eyes on us at times. However, I thought to myself how ironic it is that people might just see me feeding her and think she is helpless, when they are not aware that she had ordered her meal all by herself with her Tobii, when the waitress asked her what she wanted. And, actually, we were at Valley Dairy because she had asked if we could go there. 

But back to the laughter. It is not funny that in split seconds Lucy, and other people who may have similar physical characteristics, are sometimes disregarded, based simply on their outside appearance. It isn't funny, but I guess maybe I was having a realization that made me feel so proud; an understanding that we know how much, much more is inside of Lucy than just what people see on the outside. And because we know that, and because we work to learn more and more each day, and we never stop helping Lu learn how to express herself, I know without a doubt that as she grows, she will learn how to let the world know what she's all about. She will make sure that even if she has saliva running down her chin, or her mom is feeding her, or her body is misbehaving, that people will know she is in there, and that she is not to be overlooked. It just takes a lot of work on everybody's part to make continuous progress in her communication abilities so she can let he world know who she is.

And here's two great stories to illustrate her current level of progress:

Last weekend we were having lunch with my in-laws and Lu said with her Tobii: "Can I please, straw?" Then, "I, straw". She hasn't used a straw in a long time, so we immediately began working again on her using one. We rigged up a straw with a one-way valve that we had from a few years ago and put a soft silicone straw from a sippy cup on it so it wouldn't hurt her mouth:

The next day, at supper, we were eating taco salads, and Lu ate about a quarter of her puréed salad, and then started making a face like something was wrong. I was trying to gather to tell me if she didn't like it, and she wasn't really responding, but kept looking distressed. So as Chad and I were discussing what we should do next, and if she had eaten enough, or if she needed some chocolate milk, I suddenly heard her say "Feet!" with her Tobii. We immediately looked down at her feet to discover that her right foot was stuck behind her footrest! We fixed it, and she happily finished her supper! The way Lucy uses her PODD on her Tobii is she has to select the word she wants to use, it doesn't speak it right away, it just goes up into her message bar, and then she has to choose the "speak message" button to say it out loud. She went through that whole process to tell us her foot was stuck. I don't know if it was her discomfort, or the possibility of not getting to finish her supper, or a combination of both, but she was able to get her point across right when she needed to. It was fabulous! 

I've said this before, and I'll say it again, and again, and again: I don't believe Lucy is an anomaly. I believe all girls with Rett, and all nonverbal people in general, can learn some form of communication. The key is how it is presented to them, and the beliefs of the people who are responsible for supporting them and their efforts. If their support system does not believe they have something to say, then they might believe that too. I know Lucy always has something to say. I know her head is full of thoughts, and hopes, and dreams, and probably a complaint or a gripe here and there. I want to hear them all! I get so upset when I hear another parent comment that they wish they new what their nonverbal child was thinking...teach them how to tell you! It is their right as human beings to be taught an alternative means of communication! 

We are so proud of Lucy all of the time for her endless patience with herself and others. I don't think she worries about how the world views her when they only see what's on the outside, because she knows her Daddy and I don't give a damn what they think either. We know what she is made of and we know she will let the world know too, by and by, as she can. 

The moving target

Dr. Sasha always reminds us that Rett Syndrome is a moving target, meaning it changes all of the time, without warning. For example one day Lucy did not have seizures, and then the next day, without warning, she did. Once upon a time she had floppy muscle tone, and then one day it started getting tighter and her body started arching backward all...the...time. One day she could turn the pages of books, and then she couldn't. Unfortunately, the changes that occur always seem to be taking from her...until recently!

The other day I was folding clothes behind Lu while she had a feed and watched a movie. On her lap I had sat her singing Elsa doll and suddenly, it was singing! Lucy had managed to position her hands so she could press the button on the front of Elsa to make her sing! And then when she realized what she was doing, she did it over and over again and was so clearly excited and tickled with herself! I don't honestly know how long it has been since she was able to activate a toy like that all by herself.

When I mentioned above that Lucy's body started arching back, this pretty drastically effected her ability to sit unassisted. That was almost three years ago and in that time she has only been able to sit up on her own for seconds at a time, because her body just wouldn't allow it. But then a few weeks ago she was sitting for her PT, and doing a really excellent job! So, her super rad Dad started a sitting practice session each day while they wait for supper to be ready. And now, after three years of hardly any unassisted sitting, Lucy May has been sitting for minutes at a time, all by herself! 

And a couple of months ago Lu started being able to sometimes stand up from sitting, with little to no assistance, just holding onto our hands! This is super hard work for her and her body. Again, it was Daddy who first got this out of her. I think he is the best PT she will ever have, and he's not even a PT! 

The only downside to all of these wonderful new/old skills that Lu has acquired is the "moving target" part. For the past few years a lot of symptoms have either presented themselves for the first time, or have taken skills away from Lu, and our biggest consolation, our hopeful mantra is that "Rett is a moving target. It's always changing." And so we are often waiting for something that is making Lu's life harder to change, or go away, because eventually, it might. Not all things of course, and we know that. But now here we are with some changes in her body, and how Rett is presenting itself, that are wonderful! She seems stronger, and like she has gained a little control of her body back, and now instead of waiting anxiously for something to change so she will feel better, I am anxiously hoping that if anything does change it will only continue to be more positive changes. Unfortunately, upon being introduced to Rett Syndrome, it quickly establishes itself as cruel, relentless, and not something that tends to get easier to live with as time goes on. So, I guess I want to feel optimistic about these changes, but at the same time I know how quickly Rett can take from Lu, and that is so hard to swallow. HOWEVER, while I feel anxious, we will still keep working hard on what she has been excelling at, and looking for new things that her body might be capable of doing that she hasn't been able to do for awhile. Day by day is how we live here, and the days lately have been treating us pretty well.

Four Years Gone

This was taken right around the day we received the phone call that Lu had Rett Syndrome. The anniversary of the date of the call just passed, it was February 27th. And this was the first year since we received the diagnosis that the day we met with Dr. Coffman has existed, because it was February 29th. These days were more prominent to me in the first year or two. The 29th will always be a day where I bristle with a combination of rage and pride; rage at what Dr. Coffman told us Lucy was NOT going to do, and pride at all that she has learned to do so far, instead. This was the first year that I really had a talk with her about Dr. Coffman and how wrong he was, and how wrong many people are not only about children with Rett, but people with complex diagnoses in general. 

And then last night I dreamt that Lu was talking, with her mouth, and everything was just coming out like she had always been able to. In the dream it was just like, "oh, ok Lucy can talk now! Of course she can!" I wonder if in the dream we were so nonchalant about it either because she already does talk to us all day, just not with her mouth, and/or because I believe it is a given that someday she will be able to speak, when there is a treatment or a cure. 

The thing is, when I am awake and not dreaming, I spend very little time thinking about treatments and cures, and I've talked about why before. However, every single day I hope for Lucy to stay healthy, to get stronger, to learn new things, for her spine to not curve more, and on and on. Chad and I don't live in the shadow of the possibility of a cure because that hasn't happened yet. And while there are so many exciting things going on in the scientific world in regard to treatments and a cure, there is no guarantee that it is going to happen, so we just focus on how to keep Lucy as healthy, and happy as possible, and how to prepare her for life and the world, even if she always has Rett Syndrome. 

We believe this:

-Dr. Seuss

And this:

She is who she is and we worship her just how she is. We would take away all of her difficulties in a white hot instant, immediately, without a second thought, if that option was ever presented to us. But, Chad and I just naturally focus on who she is, not who we thought she was going to be. Lately, more people than usual have asked me if there are any new developments in the trials for treatments and medications. I know with 100% certainty that these are well-meaning inquiries. I know that our family and friends just want Lucy's life to be easier for her. They want to see her "get better", and get to live a more "normal" life. I know they just love her, and us. But...I instantly, and I suppose irrationally, become defensive inside. I answer politely with what they want to know, but on the inside my heart says, " Why? Isn't she good enough for you the way she is?" And I know full well that's not what they are saying, but it's just my gut reaction. 

In regard to Rett Syndrome, the Shaffers have been kicking it's ass and taking its name since 2012.  I hate it, I fear it, and I wish we had never had to learn what it was.  But, in the event that Lucy never receives any reprieve from it, we are prepared to keep up the fight and help her to become the person that she wants to be, in spite of Rett Syndrome. 

"I want to watch..."

This is what Lu looked like yesterday after she told me with her Tobii that she wanted to watch Despicable Me 2. She was so tickled to be watching the movie of her choice. I have been modeling in her Tobii the sentence, "I want to watch..." And then she fills in the blank. Because I know that eventually she will create that sentence on her own, instead of just essentially shouting out the names of movies and tv shows that she wants to watch. 

The other day she told me that what was happening (which was that she was waiting for me to give her a snack and was watching Peter Rabbit) was "boring".  Being bored must be a feeling she is exploring lately because she also told Chad a month or so ago that she was "bored" when he was reading to her one night before supper. 

A week or two ago Lu and I were planning a small shopping trip. I read the list of things I needed and then I asked her if there was anything she wanted me to add. This is what she said:

That night for supper I made grilled cheese sandwiches with salami in them, and she was in love! We had blueberry pancakes the following Sunday, and we haven't had sloppy joes or lasagna yet, but we will.

Lucy's buddies Miles, Chase, and Isaac stopped by for a quick surprise visit after Christmas to meet Olive, her new kitty. She loves those boys, but look what she said when they left:

She often asks where her Dad is during the day when he's at work or makes this suggestion:

I asked her if she missed him and she nodded "yes". She followed up in her book by saying, "not see him". 

And during speech the other day, she and her therapist, Roxann, we're making a story about a picture of Lucy dressed up for Halloween. They had started the story the day before, so Roxann brought a picture of herself dressed up like Tinkerbell a few years ago to show it to Lu and talk a little bit about it too. In Lucy's picture she is with her dad and her Poppa, so as part of the story Roxann asked her what her Poppa thought about her costume, and Lucy said, "everyone, silly, yours" obviously referring to Roxann dressed like Tink, since she was a grown up! It was very funny!

Just like any other five year old, Lucy makes requests, shares her feelings, makes observations, expresses her opinions, and likes to watch the same movie over and over again, and can tell me she wants to do so. I have a pretty strong belief that because she can do these things, and has control over it, is why she is generally a happy little girl. She certainly can have bad days as we all do, and sometimes we go through periods where certain parts of the day incite an inexplicable unhappiness in her, but in general, I feel like in spite of all of the parts of her life that she has no control over, and in spite of her physical limitations, she is content, happy, and thriving.

I'm just so grateful for the technology of eye gaze, and the creation of the PODD. I know this isn't new information, and that I'm like a broken record about it. But I often wonder how different Lucy would be without her ever-increasing ability to communicate with us. Fortunately, we will never know. 

Smiles

I'm not a big fan of Christmas, as I have mentioned before. I am a little Grinchey, mainly because of the materialistic aspect, which I try to minimize as much as possible by making gifts and just trying to not focus so much on all the "stuff". We don't do Santa either. That's not to say I don't love to give people gifts, because I do. But I love to give gifts that really make a person happy and that brings a genuine smile to their face, not just a gift to fill the "amount of stuff you give someone" quota. And really, the smiles that matter most to both Chad and I obviously belong to Lu. 

Lucy May isn't a gal that shows joy for just any old toy. I don't mean that there aren't many things that she likes and enjoys, but I really want to make her face light up with something that she is really going to have fun with. She also can't just play with any old toy that comes her way since she can't use her hands on her own, so it's a very tricky process thinking up things that she will love, and be able to enjoy. I have read a lot of parents' posts on Facebook and in blogs expressing very sad feelings about choosing gifts for their daughters because of the trickiness, but I seldom feel sad about it. I know what Lucy loves, and I don't mind the challenge of figuring out how she can use things. So, I wanted to share her smiles over the past few weeks that are a direct result of Chad and I always thinking outside the box and figuring out what will make joy shine out of her eyes.

I had been planning gifts for quite some time when Chad realized one day that we had not actually asked Lu what she wanted as a Christmas gift! I was mortified with ourselves! We ask her dozens of things everyday, but we had not asked her this question which certainly is mega important to any five year old. The next day as we talked about what she might want, she said on her Tobii, after I started her out with the first three words:

 Well, I said we wouldn't be doing any construction to build a fireplace, and Chad was adamant that we are currently done with fish, and she didn't want to elaborate on what she meant by "eye doctor", so I decided to work on the cat request. This was not a new request, and we had a sweetie of an outside cat who had mostly lived inside with us, but had spent the past couple of years outside, not for any reason other than she was afraid of one of our past dogs. But...she loves Finn and Finn loves her, so I just had to convince Chad. It took a few days, but he eventually relented. We got the kitty, Olive, all cleaned up at the groomer's, and she joined us inside:

This smile was from a race car track that just needs a push on a lever to make it go:

She had a lot of fun enjoying it with her cousins on New Year's Day.  I would say that the race track produced the biggest smiles, besides the little "Digibirds" that you can see hanging on her Tobii stand. I showed them to her MeeMee and Poppa after seeing them in the store, and they got her two and she loves them. I am going to make a button on her Tobii that will whistle when she activates it, and then she can make the birds sing on her own. This is how she felt when she first saw them:

She just smiled a lot in general over the weeks of hustle and bustle, and visiting, and eating, and I am glad she enjoyed herself so much. In preparation for the holiday, Lu used these fabulous, amazing new hand grippies her OT bought for her to try out to bake some chocolate cinnamon bread that we gave away as gifts:

 They are called "EaZyhold" and can be bought on their website. They can be used to hold so many, many different things! Like a spoon for stirring:

Look at that concentration!

Or holding different sized markers to color! Lu colored this which I then shrunk and made copies of to put on the front of our homemade cards, that I am still trying to get in the mail!

I think people make the holidays as stressful or not stressful as they want them to be. Each year I wrack my brain making a plan for how to eliminate more and more stress from all of the festivities. But with all of the going places we are always worrying about accessibility, and times things are happening in relation to Lucy's rigid daily schedule. Plus, just sometimes places and activities can be too much for her and she gets overwhelmed which can present itself in different ways. 

But, we do our best to pick and choose what is worth the effort, and what just isn't. This year one thing that I knew would be absolutely worth the effort was going to see Lucy's buddies, Miles, Chase, and Isaac in their Christmas show at their school. And I was right, she smiled the whole time, even though we were past her lunch time. After the show we had lunch at Burger King with Erin, the boys' mom and my old friend, and Lu enthusiastically ate almost an entire [pureed] cheeseburger. It was a great day. Full of smiles, and while it was exhausting, it was so totally worth it!

So anyway, I hope everyone had a very happy holiday season, whatever holiday you may celebrate. I've already started planning ways to continue my goal of simplifying the holidays for next year: minimal stress, maximum smiles on Lucy's face, that's my goal! I hope everyone also has a healthy and happy New Year! 

Girl Scouts

Some years I like to talk about all of the things I am thankful for in life, but this year, there is one new thing in our life, and in Lucy's life specifically that I am especially thankful for. She became a Girl Scout this fall. She is technically a "Daisy", but in our small rural town, all ages are in the same troop, and it is a small little troop. 

Lucy's older cousin, Emmie is in the troop, and her best friend Hannah, and that was a big help in Lucy being instantly welcomed and accepted. And I can tell when we are there at the meetings, of which of course I need to attend with her, she feels at ease. One of my favorite examples of knowing she feels comfortable was when the girls were talking about movies and they asked Lu what her favorite movie is. Lucy went to her "About Me" section in her PODD on her Tobii and found the button that says, "my favorite movie is Mulan". Plus, she participates in the crafts and activities by using her Tobii and any adaptive equipment I have brought along that day with no anxiety. 

We are lucky to have joined a troop led by two women who not only welcome Lu, but think about her needs when choosing snacks, crafts, and other activities. And we are so thankful to have been lucky enough to join a group of such kind, and patient girls. They ask questions, but not constantly, and always politely. And they don't make a big deal about all of the things Lu does differently. They don't stare at her when I am feeding her or wiping the saliva from her chin. When I pulled out Lu's adaptive scissors one day when there was cutting to do, the girl across from Lu said, "I am very interested to see how Lucy is going to use those scissors." It was just right. And that's why Lu is comfortable, because she is certainly aware of all of the things she does differently and this group of girls just accept those things and have welcomed her into their group. They treat her like a real friend.

For the meeting before Thanksgiving, the girls had a talent show and Emmie and two of her friends asked Lu if she would like to play her bongo drums while they played some of their band music. Lu's OT bought these silicone grips after I showed them to her and Lu has been using them for a variety of activities, including holding her drumstick during the talent show:

They are called "EaZyhold" silicone grips and they are wonderful. 

So Lucy was able to play with the girls by me just helping to support her elbow and keep her hand over her drum.

Then later the girls took Lu out to the floor to play tag with them...without me! Such a minute thing, but that has literally never happened in Lucy's life. I helped sort candy that the girls had sold while Lu played with her friends. Just to reiterate: she played without me... WITHOUT...ME. It was one of the most beautiful things I have ever seen in my whole entire life. The girls took turns pushing her and tagging her, and letting her tag them, and all the while a giant smile was spread across her face. 

They just include her as a matter of course.

This is just what I was hoping Girl Scouts would be: a valuable social group for Lucy. It is mostly quiet, low-key, slow moving, and low pressure. Just our kind of scene, and that's one of the things I am so very thankful for this year. Thank you Maria and Kristy for leading the troop and doing all that you do!

The past few weeks...

We've kind of had a time of it the past few weeks:

A few Monday's ago: 

 I took Lu to just a regular eye doctor for an exam. In the past we have taken Lu to a pediatric ophthalmologist in Danville, but when we saw him last January, I felt like his attitude was not what I am looking for in a doctor. He found a very slight near-sightedness in her left eye and said it wasn't bad enough yet for a prescription (which I thought was fine), but then he also said for us to come back in two years...two years to check on a pair of eyes that work so hard every day to use her Tobii and her PODD book, and that already are showing some near-sightedness? No, I didn't think we would wait two years. So when I was at my eye doctor, Dr. Danielle Trego, I asked if she would be willing to give Lu an exam when the time came and she was more than happy to. Fast forward a few more months and one night Lucy said with her paper PODD book, "not, see, me". I asked if she was having trouble seeing and she said yes. Since the doctor had noted the near-sightedness, Chad and I have talked with her often about letting us know if she starts having any trouble seeing, or if things begin to get blurry. I checked in with her a few days after she said she was having trouble seeing and asked her again, and she still said yes, so I made her an appointment and low and behold, she has some astigmatism. Since she had made a complaint about it, we decided to give some glasses a try and she has been so happy with them since the moment we put them on her face!

In other news, Lucille May has lost her first two teeth also! 

So then, the day after the eye exam she woke up and the cold she had been trying to get over seemed  to be trying to make a comeback, so I took her to the doctor, just to be sure her lungs were clear. Everything looked and sounded great, but they were having trouble getting a high enough pulse ox reading (the level of oxygen present in her blood) and the doctor decided to do a chest x-ray just to be sure. The next morning I got a message in my email that her x-ray results were available on the online system through her medical center. Much to my relief, it stated her lungs were clear. As I read the entire report, however, I noticed this sentence:

 

"There is dextroscoliosis of the mid thoracic spine." It was like that sentence leapt off the screen and just socked me right in the heart, because this was news to me. I messaged the office to point this out and say her orthopedic doctor should look at the x-rays. Not long after, the nurse called to tell me that Lu's lungs were clear. She had not read my message about the scoliosis yet, and made no mention of those "findings". Nobody noticed but me that this was new. By the end of the day, I made sure Lucy had an appointment to address this, and luckily it was within the next week. 

The x-ray was taken while Lu sat in her wheelchair, so it is obvious in the picture that she was sitting a little crooked, but the doctor said she also had a very mild curve beginning. He said that he wanted to see her in Danville in 6 months to do more in-depth x-rays and to determine the degree of the curve. Lucy's tone in her arms and legs has been increasing over the past year and he said the tightness of her legs is tilting her pelvis and pulling on her spine. Scoliosis can have a variety of causes, and with Rett Syndrome it is a neuromuscular problem. This means that just like the rest of her body, her brain does not send her muscles the correct signals and they pull on her spine. It's why she had low tone all her life, until the tone started increasing and now she has high tone, because her brain just can't tell her muscles to have regular tone like everybody else's. The doctor gave us two pages of leg stretches to do throughout the day, and I have seen a difference already in her ability to straighten out her legs more easily.

As I mentioned, Lucy's arms are also becoming a little tight and her wrists have started doing this:

They are constantly curled over like that, so her OT has also taught me arm stretches to help maintain her range of motion and we are in the process of ordering some wrist splints for while she sleeps to help prevent contractures from occurring. 

So basically, I feel like Rett Syndrome is crushing us, more than usual. The astigmatism is not likely related to Rett, as Chad has it too, but the high tone, scoliosis, and fight against contractures is all Rett. In the beginning, when we first learned about the diagnosis, I made a mental list of the biggest problems that could occur from the condition and I went over that list over and over in order to learn as much as we could to try to stop any of them from ever happening. Unfortunately, it seems like there is just so much out of our control, no matter how hard we work to defend Lu. 

Here's the list:

Feeding troubles resulting in feeding tube: defeated us December 2013

Seizures: first attacked in September of 2014. After more than a year, we finally seem to have a combination of medications that are keeping them under control.

Scoliosis: reared its miserable head November 2015, in spite of three years of agonizing over proper seating, standing, walking, and exercising to prevent it

Pneumonia: We are still holding this bugger at bay. When Lucy gets a cold, it tends to hang on FOREVER with never-ending mucous and coughing, but it has only once required an antibiotic, which was last Christmas. Pneumonia can become very serious, very quickly for girls with Rett who already have irregular breathing patterns, as Lucy does, and especially because she is not ambulatory. I am terrified of this one.

Long qt syndrome: This one has also left Lucy alone so far. This is a condition that causes An irregular heart beat and if left untreated can cause sudden cardiac arrest and death. She gets checked for this annually. 

Contractures: These are new to the list because Lucy has always had such low muscle tone. She is not experiencing any of these as of yet, and we can do our best to make sure she doesn't. 

The scoliosis doctor said that we have been doing everything right and to keep up the good work. In a sudden gush of frustration and sadness, I tearfully pointed out that even though we did our best to prevent this from happening, it still happened. He said he had another way for us to look at it, and that was that if we hadn't done everything we have done so far, it would be so much worse at this point. I instantly felt better when he said that. But, it is still so hard to know that so many important things that could drastically effect Lu's life are out of my control. It is agonizing at times because all I want to do is protect her and make her life good, and happy, and comfortable. 

That damn curve could progress over time to a degree that will require surgery to fix it and to keep Lucy's organs from being effected by it, or it could only get a little worse. There is no knowing what it will do, and there is very little to do to change its progression. We just need to keep her as straight as we can, and keep her stretched out, and walking and standing as much as she is able to, and hope for the best. Chad and I, as usual, are on duty and wielding our big sticks. Just sometimes Rett Syndrome has a much bigger stick than us.