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. 

Not Just for Some

http://www.janefarrall.com/aac-dont-demand-prerequisite-skills/

I just finished reading this article by Jane Farrall, an AAC professional that provides a great deal of very helpful information on her website not just about AAC and communicators with complex communication needs, but also about education and literacy. She incorporates how to use the PODD or whatever communication system a learner uses into lessons, and provides a point of view that is very close to my heart. The point of view that every person has the right as a human to communicate, and to communicate fully using a robust communication system, not simply pressing a button, or answering yes and no questions all day long.

I'd like to preface my thoughts today with a few facts that I know are true. 

I know when our children are sick, nothing else matters but getting them well. I know that some children are sick often, and that maybe implementing a robust communication system might not seem like it should be a priority when you are in the hospital, or maybe just trying to keep yourself going after nights and nights of being up caring for you loved one. 

I know many people don't always have team members or speech therapists that possess forward thinking views, or ones that will presume our children are competent. I know it is hard to convince the system to believe you that your child deserves to be able to communicate, learn, and socialize. 

I know there are millions of different life circumstances that can make obtaining a Tobii, or a PODD book, or any type of communication system a difficult task. My intention is not to place judgement or blame on anyone for what they can or cannot do. I want to talk about our views as parents in regard to the importance of overcoming any obstacles that are in the way of letting our children communicate. 

Here's something that not only makes my blood boil, but also breaks my heart: in the past three years of attending conferences, parent gatherings, strollathons, and awareness events, I have seen one other girl, one time, with a means of communication available to her right then at the event. I have met many  girls. I have met girls who I know had Tobiis and PODD books at home, but they weren't there with them.  To me, this sends a message that they can only communicate at certain times. Not only that there are only certain times that they are allowed to communicate, but that there are evidently times when nobody cares if they have something to say. That may sound harsh, but what other message could it be sending?  In the groups on Facebook for parents with Rett Syndrome, and even communication groups, I have heard people comment that the Tobii is too bulky to bring along, the book is too big, there's already so much to bring with them, they can't remember it, etc., etc. Whatever the excuse may be, all the AAC user sees and hears is that it's not important. 

In addition to the occurrence of making excuses for not always having a means of communication present for AAC users, I feel that many people still believe, or are told by professionals and then taught to believe, that their child actually is "too something" like Jane's article discusses. Too physically impaired, too cognitively impaired, too behaviorally challenging, too old, too young, the list could go on and on. I was actually surprised when I read this article that the idea of nobody being too anything to communicate has been in practice for so long, because I consistently hear other parents talking about how their therapists and/or teachers have decided their child is "too something" after all. Often times, I think when a professional says something, then a parent believes it. That "professional" might be more educated, so why shouldn't we believe them? 

However, if there is one thing I have learned since beginning this journey as Lu's mother it is that professionals are not always right, and if I don't think they are right, it is my job to make them see why they are wrong. This can be intimidating because they are the "professionals", and I'm just the parent. I have heard umpteen times that I am the expert on my child, but on very few occasions have I actually felt that the person making that statement really believed it. But I believe it, with every fiber of my being, and because of that, I calmly, patiently, but relentlessly make that fact known to anyone who needs to know it. As the expert on my child, I don't let people second guess what I know to be true: that Lucy is brilliant, strong, funny, kind, and capable. She experiences many things all day that could probably be considered as severe impediments to her ability to communicate, but I have never let anyone say that any of those things were too anything for her to be able to talk to us. She has very little control over her body, but has learned to use her head to indicate yes and no. She holds her breath and grinds her teeth constantly, she knows we will wait until she is breathing again so she can continue with what she was saying. She can't use her hands to sign or point to what she wants to say, so she uses eye gaze and partner-assisted scanning. There are ways to work through anything. 

When I see and hear so many reasons why people do not have a way to communicate, I feel frustrated with the adults who should be advocating and problem-solving for them. However, mostly my heart breaks for these children and adults who have so much to say, and are not being provided with a way to say it. Seriously, can you imagine having the brain you have, and all of the thoughts and feelings you have, but not being able to express yourself? Now, if you can imagine that, imagine that nobody around you believes that you have anything to say, so they don't try to figure out how to help you communicate. Or imagine that you have a magical machine that can let you talk just by using your eyes and choosing what you want to say, but it's only available to you once in awhile. Or imagine there is a book full of symbols that you understand and could communicate with, but nobody ever seems to bring it along. Imagine what it would be like if people discounted your abilities based solely on your disabilities. 

Parents need to believe in their child's ability to communicate, and professionals need to presume competence ALWAYS, so that they at least can help parents change their view points if necessary. It needs to become a more widely spread belief that nobody is too anything to communicate. Every person should simply be seen as too human to not be allowed to have their thoughts heard.