Update

I Things are still going very well. Lu had a good night of sleep, and she is on her second "bolus" feeding which means a larger amount all at one time, like a meal. She has been tolerating everything very well, and I actually gave this last feed to her, so we are learning what we need to do. And then it took the nurse awhile to come when her feeding was over, so Chad and I just went ahead and finished it up all by ourselves and we did it right with any trouble at all. The nurse has been impressed I think.

I said to Chad this morning that even though it sounds crazy, when I'm looking at this contraption that is feeding my baby through a tube directly into her belly, it still looks easier to me than what we currently do. I think that I have said that Lu will still be able to eat whatever she wants by mouth, it just won't matter HOW much she eats by mouth because she will get the full amount of calories she needs through the feeding tube. So, anything she eats by mouth is just bonus! It will be good. She is napping now and has been such a big, brave girl throughout this whole process. I felt bad that I didn't add any pics of her and Daddy yesterday so here is one:

And here is the big girl relaxing in her crib after a nice lunch and a big poop:

And here are her adorable legs just being adorable:

Hopefully we will be home tomorrow sometime! 

Pre-op and post-op

Lu's upper GI was fine yesterday. She cried at the end and then cried for a half hour afterward, but she had a good nights sleep. Her IV went in easily which was a relief. The first time she had an IV at Danville they jabbed her for an hour before getting it in without it collapsing or whatever happens. 

Here's what the first 2/3 of our trip looked like yesterday morning:

Chad drove of course, and it was my job to just try and get as much food and water in Lu before 6:30, so I tried to not pay attention to the terror that was interstate 80. It eventually stopped completely, I guess we got ahead of the storm, and then we were fine.  Here is Lu being the big pile of awesome that she is, just enjoying the ride:

We had Lu ready to go down for surgery by 7 this morning and here we are waiting for "transport" (and  reading a great Berenstain Bears book about the importance of not being racist):

And here we are in the recovery room:

She had a few rough periods while waiting for the pain medication to start working, but other than that, she had a pretty decent day. Formula has been going into her g-tube for almost two hours now do it will slowly go in until 4:00am. They will work her up to her target amount to take at one time throughout the day tomorrow and as long as she tolerates it well, we will likely be able to go home on Sunday. We will have a nurse come to the house for awhile to help with it and to make sure everything is going ok also. I think the scariest parts are over and now we just have to wait for Lu to regain her plump and healthy body. I probably have a lot more thoughts to share, but I just want to lay down and rest and I just wanted any faithful Lucy fans to know that she is doing well and everything went smoothly. 

Thanks for caring! 

A mind of her own

This is Lucy's "new" dollie. I rescued her from Goodwill a few weeks ago and cleaned her all up, and even crocheted her up a few new frocks as she was scandalously nude, laying there dejectedly at the Goodwill. When Lu and I play babies, she often says, "brush hair" with her Tobii, but she didn't have a doll whose hair could be brushed very well and I had been looking for one for her. This little lady seems to have had an unfortunate experience with a "hairdresser" and has had a bit of a haircut, but it is easy for Lu to brush, and fortunately, always seems to need it. My favorite part about this new dollie, however, is her name. I, uncharacteristically, named her Babette without consulting Lucy at all. After Lu had Babette for about a week, I asked her if she liked her dollie's name to be Babette, and she said, "No". I said, mostly to myself, "How can we figure out how Lu can tell me what she wants this baby's name to be?" And then Lucy navigated to the first page of her PODD on her Tobii and said, "My name is Lucy" and then I said, "Do you want your baby's name to be Lucy too?" And she navigated to the yes/no page and said, "Yes!" So Babette became Baby Lucy. 

Both of those pictures above were done on Lu's Tobii, using her eyes! On the page I have made for her circle time at school there is a button that says, "Illustrator" because when they read books the teacher always asks questions about the parts of the book, including what is on the title page. Well, one day at lunch Lu went to this page and said "Illustrator" several times.  I asked her if she wanted to be an illustrator and she went to the first page of her PODD and said, "Me". Then a few minutes later she went back to illustrator again and said it a few more times and when I asked again if she wanted to be an illustrator she said, "I do"!!! It was so awesome. 

So, I went on a mission to figure out how Lu could do a little drawing on her Tobii. With the help of the Tobii rep for our area, Mary, that helped us get the Tobii in the first place, I figured out how to set up the Tobii so Lu can control the mouse. Then she sent me two programs through Dropbox and told me about a few websites. The first picture is from www.jacksonpollock.org. It is really easy; Lu just uses her eyes to make the lines, and then to change the color you just left click the mouse and a new random color is generated, which is fun because then Lu gets to see all different colors and shades with little effort.  Sometimes she can figure out how to change the color by herself with her eyes, but I don't know how she does it! And the second picture is a program Mary shared with me that has limited colors, but they are fun and easy and it can play music while she draws too.  I added a way for Lu to tell me when she wants to paint and which program she wants to use. It's all a great way for her to be learning how to control the mouse with her eyes so that she will continue to improve.  There are actual adult artists that use the Tobii to create very elaborate artwork, so there is no reason Lu couldn't do the same, and I told her so. 

We talk about what jobs she could or could probably not have when she is grown up, when they come up in conversation. Another occupation we have talked about is being an engineer because Lu seems to be fascinated with bridges. She often goes to a page in her PODD with the word/picture of a bridge and she will activate it over and over again. I asked her if she wanted to read some books about bridges  and she said "yes", and we did and she loved them! I told her that she could use a Tobii and her eyes to design bridges someday. I said she wouldn't be able to actually "build" the bridges herself, or be a bridge painter, but she could definitely design them and then other people could build them. 

I cannot, and will probably never be able to say enough about how essential I believe the Tobii is to improving Lu's quality of life. Sometimes a day goes by where she doesn't say much, and then she tells me that she wants to be an illustrator and that she wants her baby doll to be named after her! I wish every person with complex communication needs who cannot speak or use their hands was just automatically given a Tobii. And I know there are many other visual communication languages like the PODD, but it's the one we know and it's the one that first opened Lu's door to communicating with us, so I have a bit of a soft spot for it. 

I guess in the face of Lu's upcoming procedures, maybe it is important for me to try and just remember how the feeding tube will also greatly improve her quality of life. Who knows what we will do in a day when we don't have to spend it in frustration and tedium trying to get nutrients into her. I'll tell you what we will do, all kinds of super fun stuff! It's funny the stigmas that do and do not pertain to different things, ie: people shy away from medication for mental conditions such as anxiety and depression, but will unhesitatingly take a pill for their heart, thyroid, headache, etc. Lu's Tobii is much more of an obvious and cumbersome piece of equipment that we lug EVERYWHERE with us, than her tiny little tube will be hidden under her shirt, but the look of devastation on peoples faces when they hear she needs one is so obvious, in comparison to the look of interest and enthusiasm for the acquisition of her Tobii. I get it though. However, I look forward to seeing her gain weight back, get some color back, and not need as many little rests throughout the days, as much as I looked forward to communicating with her.  She is pale, and tiny, and takes several little naps a day. She caught a stomach bug on the last day of school before Christmas break and then shared it with me. That is the fifth time she has been sick since the beginning of the school year!  I am looking forward to being able to fill up her belly so we can go somewhere and do something fun, without worrying about how and where she will be able to get a snack in a little bit. Here is one of my all time favorite quotes:

"I wanted a perfect ending. Now I've learned, the hard way, that some poems don't rhyme, and some stories don't have a clear beginning, middle and end.  Life is about not knowing, having to change, taking the moment and making the best of it, without knowing what's going to happen next." 

 -Gilda Radner

I will have plenty of time to share how the procedures have gone, I am sure, while Lu recovers.  hope everyone has a happy holiday of their choosing! 

Just some little tidbits...

Lu can't just sit in a chair in the living room and watch her cartoons because there is no support and she easily falls over. She does sit in our regular chairs sometimes, but they are not ideal for her times of relaxing. We had been using a Big Joe chair for her since the summer but the beans in it flatten so quickly and after refilling it once (for $25) and it smoothing out again within a month, I sought out a more durable solution. There are now bean bag-type chairs that are filled with shredded furniture foam. We ordered one from a company called Comfy Sack and they have a huge variety of fabrics to choose from to match your regular furniture. There was also a chair called the Chillout Chair that is a "special needs" product that would also have been awesome, maybe moreso, but since it is "special needs" it was like $1200 compared to $270 for the sack. So here are some pics of Lu enjoying her new sweet chair:

Here is a picture I sent to Chad at work the other day:

Lu ate an entire jar of baby food peas. This made us so sad, but so glad at the same time. She hasn't finished a container of anything in months. She used to eat whole cups of pudding, yogurt, applesauce, etc. and she barely even eats them at all anymore. She used to love baby food peas and I guess she still does. 

I also sent this picture to Chad at work and asked where this big girl came from! Sheesh, how can she not be a baby anymore?

And finally, I took this while driving to pt yesterday...I know dangerous, stupid, yada...but she looked so beautiful I just had to:

December 26th

Once upon a time Lu couldn't drink my milk. I've mentioned this before and have talked about how I felt like it was just the worst thing that was ever going to happen to us. Well, this week I scheduled the surgery to have Lucy's feeding tube placed. And this is still not the worst thing that could ever happen to us, it is just overwhelming and sad. It makes me feel like no matter how hard we fight, and work, and adapt, Rett Syndrome still just kicks our asses. We have done everything the swallowing specialist recommended in her report last year, and her mouth just still doesn't work for her most of the time. 

The GI doctor we see in New York talked with me on the phone two Fridays ago and we went over everything and he agreed it was time for this to happen. I had kept a log for him of her weight and what she choked on and when, and based on that and talking with me, and being a part of the Rett Clinic, he felt comfortable making this decision. He said we needed to have an Upper GI series done beforehand. This is a routine measure just to make sure anatomically everything will be fine. He said we could have it done locally, but when I contacted the pediatrician to help me set it up, he scheduled an appointment with a local pediatric GI doctor for a second opinion...in January! I said, ummm, no. I wasn't asking for a second opinion and we are not going to that appointment. So Lu will have the upper GI on December 26th at 10:00am and then the tube placement the next day. We will stay for at least 24 hours so she can get lots of antibiotics and they can make sure everything is okay. 

In regard to the local doctor thinking we need a second opinion: I always feel like they, the doctors, think I am acting like a crazy loon and over-reacting about everything. Well hey, let's fart around a few more months and maybe Lu can lose a few more pounds, so that we have to work extra hard to get her weight back on, and her health can get a little worse, and then they can come to the same conclusion. But Geisinger's motto should be, "Hey, we like to fart around with your health." When I responded to the email, I said that I trust the doctor who sees girls with Rett Syndrome EVERYDAY, and HE trusts ME and my judgement. I am very seldom made to feel by any local doctors that my judgement and observations matter or are to be trusted. I'm sure there are still lots and lots of things that I don't know about Rett Syndrome...I'm not Dr. Sasha after all...but I can feel certain that I know a whole hell of a lot more about it than Lucy's pediatrician, or ortho doctor, or the GI we saw last year! From day one, I have immersed myself in learning what it is all about. I may have said this before, but I will say it again, I think of it like keeping my friends close, and my enemies closer. Rett Syndrome is my enemy and I have to know everything I possibly can about it to help me fight it. I have to know what could be coming down the pike. 

Unfortunately, because I make myself learn and know so much, and because I am the person who interacts the most with the outside world regarding Lucy, I am CONSTANTLY feeling like I have to defend what I know to be true, because what is true is just too much for people to want to accept sometimes. And then all over again I feel like people think I am over-reacting, jumping to conclusions, being a loon.  And I have felt like this since we first started seeing concerns with Lucy's development, back before she was even a year old, because as I have said, I knew in my gut that the hypotonia was not just going to go away, and that their was an underlying cause for its existence.  At that time, it was early in and everybody, including the doctors it seemed, wanted to kind of act like it was nothing and that I was being a worry wart. Now that Lucy's in school I have a whole new group of people to educate and promote awareness with, and I just always feel like I am telling somebody how I want them to do something. 

Out of all of this grief, worry, strain, stress, and frustration, I still have been able to find a silver lining when it comes to the specific topic of advocating for Lucy. I would say that throughout life I have been somewhat of a "people pleaser" in that I always wanted to be liked and to make people happy. I was not a pleaser in the form of changing myself to please others, however. In the past year and a half, I have found the strength and confidence, and inner calm, to not give a crap about what others think when it comes to fighting for Lu. I still always care about people's feelings, I still try my very best to be polite, calm, and reasonable, but I know how to be the squeaky wheel now. I know how to gather data, take my time, collect my thoughts, and make my case heard loud and clear. I know how to speak up over and over again to whoever I need to, regardless of how annoying I feel I am being. I hate doing it. It is exhausting to be that squeak. But while I hate it, it is also liberating. If it's for Lu, I am like a quiet little bulldozer, with blinders on, focused only on what she needs, seldom worrying about what others might be thinking about me. 

I just read an article about behaviors that mentally strong people avoid. Number five here helped me come to the realization of what I said above. People never know how strong they can be until they are made to be, and then in the face of some situations, you can become even stronger. 

"5. Worry About Pleasing Others. Know any people pleasers? Or, conversely, people who go out of their way to dis-please others as a way of reinforcing an image of strength? Neither position is a good one. A mentally strong person strives to be kind and fair and to please others where appropriate, but is unafraid to speak up. They are able to withstand the possibility that someone will get upset and will navigate the situation, wherever possible, with grace."

It takes us a good hour each morning to "eat breakfast". Lu has a bottle of Pediasure when she first wakes up, then usually takes a little nap, and then we go to the table for medicine and something to eat. Breakfast is pretty much her worst meal of the day. In this picture is a jar of baby food, some flan from Cozy Shack, and Lucy's favorite tiny graham cracker squares. She barely ate any of anything and I have to give her the medicines little by little throughout the meal so that she doesn't gag on them and throw up anything I have managed to get into her. 

So...the thought of a feeding tube makes everyone sad, makes them want to find little slivers of reason to not get it, and makes them want to look for minuscule rays of hope. And I have to say I don't care about how sad it makes others. I don't care if family, friends, strangers, or local doctors don't think it's the right decision, because I know it is. I am the one who sits for hours and hours each day trying to coax food into my daughter's mouth. I only have enough strength to worry about how me and Chad, and Lucy feel. I only have enough strength to do what is best for Lu and stand by our decision. 

The Essence of Human Life

This is a reading list Lu made up using her Tobii the other day. I have been working at making pages on the Tobii of the covers of all of the books she owns (which is many) so she could choose what she wants me to read. We then read the paper version of the books because they are not in her Tobii (yet!). I have dreamed of this being a possibility for all of her life. Right now I have 7 pages of books with 20 books on each page and I asked Lu to go through and choose two from each page that she wants to read at some point. These were her choices. She almost always chooses the Avengers, and the C Book, and How The Grinch Stole Christmas. I never knew those were some of her favorite books, but I was always just dying to know! 

"It is the position of the American Speech-Language-Hearing Association (ASHA) that 'communication is the essence of human life and that all people have the right to communicate to the fullest extent possible. No individuals should be denied this right, irrespective of the type and/or severity of communication, linguistic, social, cognitive, motor, sensory, perceptual, and/or other disability(ies) they may present.' "

Linda Burkhart used the above quote at the end of the PODD training we went to last December. It has stayed in my brain ever since, but most especially the little part that says, "Communication is the essence of human life." I even used the quote in one of the appeal letters I sent to the insurance company as I pleaded for them to approve the Tobii. When we learned the PODD a year ago, that was the beginning of our glimpse into what Lu was thinking and feeling. When we got the Tobii a few months ago, it made me realize that with the ability to relay her thoughts so quickly and efficiently, people will see her as a real person. Because, being able to communicate truly is the essence of human life.  I believe it is a sad, but true fact that without the ability to communicate, people are seen as "less than", even if they have all kinds of thoughts and feelings swirling around inside of them. 

 Around the same time that we got the Tobii, maybe even a little bit before, I looked at Lucy one day and had a startling, but exciting realization. I looked at her and thought, "How could someone not fall in love with her some day? Why couldn't she get married and have a life with someone?" Then I read a little about Stephen Hawking as a comparison for how I think that could be a possibility... When he married his first wife, the ALS was just beginning to be an issue for him. He had recently received the diagnosis and she still married him. She took care of him a lot throughout their marriage, but they also always had nurses, and assistants that did a lot of the physical caring for him. It was just their way of life to have extra people with them to tend to Stephen's needs. Him and his first wife eventually divorced after many years together and after having three children. He remarried one of his nurses. He remarried even though at this point ALS had completely taken over his body and had done so years ago. BUT, just like Lu, his mind remains in tact. A woman fell in love with HIM and married him, in spite of the fact that he has no control over his body, in spite of the fact that he communicates using a speech generating device, and in spite of the fact that all of his very serious medical conditions require round the clock care by her and others.  I think Lu is so incredibly awesome and amazing that I believe she too will meet someone someday who also will be able to look past Rett Syndrome and fall in love with HER. 

While out to eat at Red Lobster on Saturday night with Chad's dad and stepmom, Lucy kept saying, "panties" with her Tobii. It was funny, but it also gave us the opportunity to talk about when and where it is and is not ok to talk about bathroom things. That's what you do with children; you teach them manners and about topics that are better left for discussion at home, in private. And now Chad and I get to do that too.  I wish every person on earth that cannot speak on their own was just automatically given a Tobii. There are many families of girls with Rett Syndrome that have no communication system for their daughters, let alone a Tobii or some other kind of speech generating device.  While I am so grateful that Lu is able to have her Tobii, it also breaks my heart that there are so many people that do not have one and have not been given the opportunity to communicate. 

This morning Lu and I talked a little bit about what she might be when she grows up...because she will be SOMETHING! With or without a cure, she will do something wonderful, and maybe be someone's wonderful wife. And contribute to society just like others do. I just know it.

"Obviously, because of my disability, I need assistance. But I have always tried to overcome the limitations of my condition and lead as full a life as possible. I have traveled the world, from the Antarctic to zero gravity."

-Stephen Hawking

"All my adult life people have been helping me."

-Stephen Hawking

"Among physicists, I'm respected I hope."

-Stephen Hawking

29 pounds

This is the fourth blog post I have started since November 5th and I just can't seem to get one finished. I'll try to finish this one. Last year at this time Lu weighed 34 pounds. In April she had dropped down to 31-32 pounds. In August, when we went to New York for the EEG, she weighed 30 pounds. In the past month she has lost yet another pound. Her mouth, and tongue, and throat are just not working very well for her anymore. It took her an hour and 15 minutes to eat a very small slice of banana nut bread the other day. But at least she ate it that day. There are many days that she just eats very little solid food and is getting a lot of her calories through liquids in her bottle. She drinks 2-3 Pediasures a day and I have been experimenting with a new blender we got called a Blendtec to make her juices and smoothies high in calories. I load things up with butter, peanut butter, cream cheese, finely chopped nuts, coconut oil, olive oil...whatever I read about that adds extra, but relatively nutritious calories.  Many things Lu eats these days such as PB&J sandwiches, banana bread, and cereal bars all need to be dipped in milk and yesterday I bought a container of heavy cream to use instead of whole milk. But nothing is working. She is not maintaining her weight.

Since school started at the beginning of September she has had three colds. Luckily they don't get worse than a cold, but when Lucy has a cold, this means that she gags and coughs on snot all day and night and often throws up when it gets to be too much, so it is exhausting for all of us. I have started giving her vitamin c and zinc supplements, but she still got another cold. And when she is sick she eats even less. I know kids get sick a lot when they first start school, but I also think that Lucy's lack of adequate calories and nutrition is making it worse.

Yes, she has gotten taller in the past year, but when people get bigger, they should gain weight, not lose it. Children should not lose weight. Dr. Sasha said in New York when we were there that it wasn't going to get better. She is an expert and knows that as a fact. Unfortunately, none of the well-meaning "Well it could be X, Y, or Z" statements that people come up with to try and ease my fears or explain it away are just not accurate. The progression of this particular problem in the realm of Rett Syndrome is that it just won't get better. It is the apraxia that arrests her entire body that causes the problem. Her brain cannot tell her mouth and tongue, and throat what to do. This causes her to choke and gag often throughout meals and when she is drinking. This causes concern for aspirating which could then lead to aspiration pneumonia. The International Rett Syndrome Foundation sends out a newsletter quarterly and at the end is always a memorial section that Chad and I stupidly, but compulsively read. 90% of the cause of those girls' deaths tends to be from complications with pneumonia and respiratory issues. 

After keeping a weight and eating log for two weeks, I determined that the majority of the time, Lucy weighed 29 pounds. The log took place in between colds where she was eating more than when she is sick. She coughs, chokes, or gags on something daily.  She is not even able to eat things like applesauce, pudding, and yogurt consistently anymore as she often gags as soon as I put a bite in her mouth. The smooth texture seems to really bother her all of the sudden.  We struggle every day to figure out what to feed her.  Somehow she still very successfully eats string cheese. I think it quickly gets nice and mushy in her mouth. She loves tuna sandwiches and last night she was able to eat about a quarter of a cheeseburger from McDonalds which she requests with her Tobii all of the time. 

So, last weekend I emailed Dr. Sasha and the GI doctor we see in New York and shared my log with them as well as a ton more information and said Chad and I think it is time for the feeding tube. They agreed. I am going to talk to Dr. Loizides, the GI doctor on the phone some time this coming week to go over whatever we need to discuss with each other and we will see what the next step is. 

It is heartbreaking. However, I think that once we get past the initial adjustment period, it will improve Lu's quality of life greatly. With more calories and nutrients she will be healthier, have more energy, and have a full belly. We will have huge chunks of the day freed up to do other things besides try to get calories into her. I spend a great deal of time each day feeding her and giving her bottles. And, she will still be able to eat things that she enjoys, by mouth, whenever she feels like it, without the stress of trying to get all of the calories she needs by mouth. 

So, that's where we are with that. Sad, but true.

This is a picture from yesterday. 

This is from last winter. She is still wearing those clothes she has on in the picture, and they just hang on her. 

Busy girls

Both Lucy and I have been busy girls lately, which is my excuse for neglecting my blog. As I have said, Lu uses a version of the PODD on her Tobii. It is incredibly helpful as a visual language that she already knows, but I have been feeling that she needs a more advanced version of the PODD, like the paper version we had made for her in the spring.  The version she uses on her Tobii right now has 12 buttons per page, but she was using 20 per page in the paper version.  Unfortunately, there isn't a Tobii version of the PODD available as of now with 29 buttons per page. Dr. Sasha's speech therapist in New York said another mother is apparently working on this version, but she doesn't know when it will be done. I have decided to start working on the new version on my own instead of waiting for someone else. To get it done. And, that is why I have neglected my blog. Usually in the morning between Lucy's bottle of Pediasure and her breakfast I have a cup of coffee and that's when I can get a blog post in, but I have been using that time to work on the PODD. So, here is a condensed, pictorial synopsis of all of the amazing things Lu has done recently:

Lu has gotten her first chore. She pulls her dirty laundry behind her walker in a clothes basket that is velcroed to a scooter board. She is so proud to be doing an actual important job that helps out the household! And she gets practice walking!

Lu got two new fish, Little Bluie, and Scooby Dooby Doo!

Using her hand cuff, water colors, and masking tape, we made this beautiful painting of the letter E. (That's the letter of the week at preschool) Lu chose all of the colors using her Tobii. It was a lot of fun.

Also using her Tobii, Lu designed her first jack-o-lantern and she and her Daddy carved it. I have to add two pictures of this because they are so cute!

Lucy was Elmo for Halloween! We had a party in lieu of trick-or-treating

Lu had a ton of fun with her cousins and friends! It was a good time.

And the grand finale: several times this week Lu has said with her Tobii, "I have to pee" and then I take her to the potty and and she pees! And today at breakfast she said, "panties, me" so we sat on the potty and then [Lu] put panties on. The picture above is of Lucy coloring a Halloween picture, while wearing her panties which she didn't pee in and waited until I took her to the potty to pee, after about 45 minutes of wearing them! She was so proud of herself and of course we were so proud of her too, but the look on her face brought tears to my eyes. 

So, that's what we gave been up to. I apologize to our faithful readers for my slacking. I might not improve on it for a bit though, so bear with me.

The wide open air

Here is a beautiful picture Chad made for Lucy at my request:

It is a page from "Oh The Places You'll Go!" By Dr. Seuss. Chad has been getting more and more into art in the past couple of years so I asked him to make this picture for Lu to hang on her bedroom wall and he did an amazing job! Here's a little story about why I wanted this page in particular. 

This was the first book I read to Lucy and I started reading it in the first week we were home from the hospital after she was born. In that first week I was of course emotional as new moms tend to be with all of the hormonal upheaval, and then the added distress of Lucy not being able to nurse really had my tears standing at the ready for any little thing to set them off. The page before this page says:

"You'll look up and down streets, look'em over with care, and about some you will say, 'I do not choose to go there.' And you might not find any that you'll want to go down, and in that case of course, you'll head straight out of town." 

And then comes this page stating, "It's opener there, in the wide open air." (Sheesh, I'm tearing up as I write this!) As I read that to teeny, Utiny, less than a week old Lucy, I cried each time at the mere thought that my brand new baby, fresh from the oven, would leave me some day.  I simply could not bear it! But, as I read it to her, I also assured her that it most definitely is opener out there in the wide open air and I would not ever keep her from it.

Fast forward 21 months and we receive Lu's diagnosis. We had read that book to the point of near memorization, but after we received the diagnosis of Rett, I couldn't bear to even pick the book up, let alone read it to Lu.  After some months I tried, and I couldn't. I felt like the book had somehow betrayed me. I believed (at that time) that Lu was never going to get the opportunity to choose where she was going to go in life. I believed that she would never know how opener it was out there. That she would never experience the wide open air. So, I had gone from lamenting the day she would leave me, to despairing over her future of never getting to be free of me. 

 I'll tell you what I did...I sold that book on half.com. The moment it was sold, I instantly regretted it. I immediately felt like now I had betrayed Lu. So, I bought her a brand new copy and I wrote her a note in the front of it. I told her about all of these feelings of betrayal, but then I explained that I had bought her a fresh copy for the new dreams we would have for her. But more important than any dreams I have for her, are the dreams she will have for herself. 

So, I am writing this today because it was the first day that I didn't stay with Lucy at preschool, at all!  Her personal care aide fed her lunch and snack without me being there. I was terrified to leave her there. Not because she isn't surrounded by wonderful people, because she is, but because I do everything. I am the one who takes care of her the majority of the time. I suppose I'm a control-freak about her care and about being the one to do it. But...this is her first breath of the wide open air. If I wanted to be selfish and do everything the way I want to do it, I'd never send her to school or away from me at all.  But she likes it and she deserves the openness, just like everyone who wants it does. 

My big girl.

This kid's face

Look at this kid's face:

That giant smile is because of her Tobii. Here is the page I made for her that caused the smile:

Those are the movies we have that she can choose from and she chose to watch Despicable Me and was so happy. What a little thing, but sooooo huge. Choosing what movie Lu  would like to watch may not "technically" fall under the "medical necessity" umbrella, but I think it should in regard to how it makes her feel mentally and emotionally to make choices so easily and readily.  I made a page also with all of Lucy's classmates at preschool and she can look at the page and say hi to them. Also, that is a tiny thing that is actually huge for her. I've put some books on her Tobii too. Some I found that were already made for eye gaze, two so far I have made myself. Lu can now look at some books and turn the pages again...with her eyes! It has been almost a year since her hands quit being able to turn the pages of books, which used to be one of her favorite things to do. 

Luckily our primary insurance company covered all that needed to be covered in regard to the Tobii. The secondary insurance, Gateway Health, again denied it as not being medically necessary. Fortunately we don't need anything from them, but it is just sickening to me that they could say it is not medically necessary for a person to be able to independently communicate their wants and needs. Again I say, look at that kid's face! She is often smiling, but that smile is because she was able to tell me something; quickly, easily, and accurately. The Tobii is expensive, that's why it took so stinking long to get it here, but I know that Chad and I would pay any amount for the rest of our lives if we had to, to keep that smile on her face and to keep hearing what she has to say. 

A whole new world!

Last Friday Lu and I had a nice girls day out. First Lu saw Mari, for her outpatient pt. There was a wheelchair mount at Easter Seals that had been there for a long time and nobody was using. It worked with Lu's Tobii and they just gave it to us! And then Mari (because she is such a super smarty pants) figured out how to mount it to Lucy's Kimba Spring wheelchair.  So, after pt we went out to lunch at Wegmans and shopping at Target with Lu's Tobii mounted right in front of her to communicate at her will! It was so exciting and she said while we were eating lunch, "I think it's, great/awesome!" 

Lucy has been saving [hoarding/squirreling away] her money in her little purse for months! Each time anyone would give her a little bit I'd put it in her little owl purse and she just would not spend it! Even on vacation, she did not want to buy anything. But on Friday, she said with her Tobii that she wanted to go look at baby dolls. After A LOT of careful consideration, she chose a beautiful Merida doll (from Brave) and was tickled with herself! I made a special shopping page on her Tobii and one very, very special button said, "Stop, I see something I like!" I have always, always wanted to hear her say that! And she said it several times as we walked through the store. It was wonderful! 

Outside of Target.

Her choice!

I'm glad to be me

You don't realize how trivial a lot of life is until a situation arises in your life that puts things into perspective. I never mention it, but often people have worries, complaints, and gripes that really do not compare to the challenges that I watch Lucy face every day. They do not really compare (in my opinion) to what it is like to live with the fact that your daughter, the beautiful amazing child you had so many other plans for, cannot do most of the things her peers can do. For all of the petty things that people whine about I want to say, "Uh huh, but my daughter can barely eat on her own. Lucy can't walk or talk, or hold a crayon.  Suck it up."

And then, there are other people's lives that are so much worse than mine and I am reminded of how incredibly lucky we are that although Lucy's disabilities are severe, she is relatively healthy. She is not dying. She doesn't even have seizures at this point which is great! She is smart and alert and happy. I have a friend on Facebook from Massachusetts who I have never met. Her daughter was originally diagnosed with Rett Syndrome even though she did not have the genetic mutation on the MECP2 chromosome. Last year she found out that her daughter does not in fact have Rett Syndrome, she has Tay-Sachs Disease. The life expectancy of Tay-Sachs is 4 years and she is currently dying. She is not yet 3. And so when I read her posts I think about how lucky we are that even though Lu can't do all of the "typical" things a three year old can do, at least she isn't dying. 

You know what I often think when I see people look at Lucy and I when we are out in public? I can tell that sometimes people (especially mothers) are thinking, "I'm glad I'm not her." I get it. I understand why people would be glad to not be me. And I get bristly about it sometimes by thinking what a luxury it must be to get to feel that way. But I will confess that I selfishly think about how glad I am that I am not my friend from Massachusetts. It's terrible to think that, but I can't help it. And I bet that my friend completely understands that people are glad to not be her. My heart breaks for her and I don't even know her. I hope her daughter goes as peacefully as possible. I hope she and her son can keep themselves together enough to move on a little at a time. I hope I am never her.

The past week

So Lucy did get her amazing and wonderful Tobii last Thursday! And it's totally awesome and I have been wanting to share the sweet and funny things she has said, but she has also been sick for over a week and I am exhausted! She had a fever all last weekend with a lot of congestion and has basically not had a full night sleep since last Monday night. She just keeps waking up over and over again gagging on snot. And then on Sunday night she developed a hacking cough. Tuesday I took her back to the doctor (because we had already been there on Friday), just to make sure her lungs still sounded clear and she threw up all over herself in the car on the way there from coughing so hard. Last night was a little better. It's just a virus, nothing is infected, her lungs are clear sounding, so we just have to wait for it to clear up. Here are some sad little pictures of her in this past week:

She's been napping a lot!

But, even sick she has been doing awesome with her Tobii! We have a version of her PODD on the Tobii so that is helpful as it is an actual language that she knows well enough to make complete thoughts, but she just has to use her eyes! So, here are some of the amazing things she has said in the past week, hold onto your hats:

1.)"I'm hungry, tuna sandwich...so I made her a tuna sandwich!"

2.) "make something, cooking, yummy"

3.)" I want to do what the others are doing, something at school, outside/recess, slide, playground, swing, park" (this isn't as sad as it seems, at "recess" in preschool Cen-Clear doesn't allow the kids to play on the playground because they are too little, I think that's the reason)

4.) We found the I love you button and mom and I suggested to Lu that she tell her Daddy she loves him when he came in the door from work, and she did and he was so happy and then she said, "Just kidding" and just started cracking up! And we were laughing too so she laughed harder and it was awesome because she knew she made a joke!

5.) Yesterday I was getting some paint ready for a little project and she navigated to "I love you" all on her own and then when I told her I love her too she said "thank you"!

6.) Before we went to the doctor last Friday she told me she had a headache and that her back, teeth, and nose hurt.

7.) she has also been telling us to hurry up (which I said she has probably been waiting her whole life to tell me because I tend to be pretty slow), she tells us when she wants "more" at meals and when she is "finished/done", and when she is tired. 

8.) Chad will likely roll his eyes that I am including this next one, but she said it and it was a little startling and weird. She said, "I'm telling you something, it's going to happen, baby, boy, we/us/our"

We have NO plans of adding any babies to our household, however, so I don't know where that came from.

 

So, she's doing great with it and I am just getting started at figuring out the full capabilities of the device. I have started putting some of her board books in so she can look at them and hear the words and turn the pages by herself! It's just going to change our lives, and hers especially!

Bravery According to Lu

A few nights ago, Chad said he was proud of me and my blog and how long I have been writing it.  In November it will be two years since I started writing. I am proud of myself too. Something I am also proud of is in these past two years I have not needed to increase the dose of my anxiety medicine, I have not started smoking again, and I have not once drowned my sorrows in Clarion River Red (my favorite wine). All of those things have of course crossed my mind [often], but my medicine is fine, I don't have time to smoke (among a zillion other reasons to not smoke) and I cannot bring myself to even have one glass of wine in the event that Lu needs me. I must always be alert for her, I believe. So, I have been a person who leaned on vices in the past, but not now, and I am proud to say I have bravely traversed this whole life-changing situation sans crutches. 

On our way home from New York last week, as we crossed the Tappan Zee bridge and were finally leaving the wretched city, I said to Chad that I feel so proud of us for taking Lu to Dr. Sasha and going to the city to give her what she needs. It makes me feel brave and it makes me think of quotes about bravery and what is often said about being brave...I'm a real sucker for quotes:

“I learned that courage was not the absence of fear, but the triumph over it. The brave man is not he who does not feel afraid, but he who conquers that fear.” 

― Nelson Mandela

The first time I was told about Dr. Sasha and heard she was in the Bronx I immediately thought, "Uh, no. That's absurd, we are not going there, no way, no how." And now, it's barely an issue, we just get in the car and go, no big whoop. So, even though we were terrified, we did it. That's being brave the quotes say, and I agree.  And I often say that Lu is the bravest girl I know because how could she not be afraid of some things, but she still just carries on. I have been thinking over this past week about what is even braver than that about Lu. What I truly think makes her the bravest person I know is that she is happy, even though she has plenty of things she could be sad about. That is brave. 

“It is hard to be brave, when you're only a Very Small Animal.” 

― A.A. Milne, Winnie-the-Pooh

Lu IS just a Very Small Animal, but she acts braver than most Very Big Animals I know. She acts braver than me to be honest. And, quite frankly, I believe that a great deal of the bravery I muster up each day is inspired by...and demanded by...Lu. How can I wallow in grief when she sits beside me smiling?  I have known people in my life that have been sad about, what appeared to be, nothing. I have been sad about nothing.  But instead of being like, "Oh boo hoo, whoa is me, I can't do things just like everybody else! Wahhhh!" Lucy says, "This isn't how the other kids do it, but I'm still doing it! Hooray!" 

In these two pictures Lu is wearing a baby doll sling I got her on Etsy so she can hold her babies. This isn't how she would "typically" be able to hold them, but it's better than not holding them at all! 

Here Lu is coloring some thank you cards for generous contributors to the "Sometimes it's expensive to be Lucy's parents" fund (which isn't a real thing!). She's sitting in her special chair, using her cuff, and is still happy as pie. 

"I’m not perfect, no I’m not

I’m not perfect, but I’ve got what I’ve got

I do my very best, I do my very best

I do my very best each day

But I’m not perfect

And I hope you like me that way"

-Laurie Berkner

 And this is her usual morning face.

“A kind of light spread out from her. And everything changed color. And the world opened out. And a day was good to awaken to. And there were no limits to anything. And the people of the world were good and handsome. And I was not afraid any more.” 

― John Steinbeck, East of Eden

I often wonder how so much strength and optimism can be inside of such a little girl. Imagine for a moment how we might behave if we were dealt the hand Lucy has been given. What if we were trapped inside of our bodies like she is? I feel certain that I would not be likely to behave well. I realize that life has never been different for Lu and that she doesn't know any other way, but she can see what she cannot do, and she can hear what she cannot say. But, she still gets up each day with a smile and gets on with it. One of the greatest gifts I believe I can give her as her mother is to do the same. 

“What's the bravest thing you ever did?

He spat in the road a bloody phlegm. Getting up this morning, he said.” 

― Cormac McCarthy, The Road

School!

Tuesday was Lucy's first real day of preschool. It went well and she loved it! I will elaborate some other day!

Results

We got home from New York late Sunday night. There was mostly good news and I started a post two days ago and it didn't save so I have been dragging my feet to start another. Sometimes, I just need a break from talking about Rett Syndrome! Anyway, the good news is Lu is NOT having seizures. The staring spells are still just that, zoning out. Her hyperventilation is "frequent but not severe" Dr. Sasha said. This irregular breathing is not effecting her oxygen level. It just seems to maybe be distracting to her and effecting her ability to focus as well as she usually does. Her O2 level did drop to the low 80s a few times in her sleep, but it came right back up on its own. The solution to the hyperventilation is an increase in her Lexapro (which she already takes for crying spells she was having that are caused by Rett and just a chemical imbalance). Lexapro can help to regulate breathing, but if the breathing is caused somewhat by anxiety also, it will help with that. So we are trying that for a few weeks to see if it helps.

Dr. Sasha said motorically she has just changed a lot in a short time. Her muscle tone has started to increase which she said she doesn't usually expect to start happening until around age ten. She agreed that all of the other things we have been seeing are different, but thankfully (that's a weird way to say it I think) they are not out of the ordinary. Things will just always change, for better or for worse. And that's all I wanted; to go to New York, have Dr. Sasha look at her and tell me that these changes were common. To us, these changes were devastating, but I thought they were probably nothing out of the ordinary, but I didn't know for sure. So, we just have to, as usual, adapt and move on.

The not-so-great news, Dr. Sasha was concerned with the weight loss and trouble eating. She said that regulating her breathing could help her be able to focus on eating more, but her ability to chew and swallow,which has been declining, will not get better. She said she will likely need a feeding tube in the not-too-distant future. We will watch her weight as she has the Pediasure more regularly and see what happens, but she needs to at least maintain her weight and definitely not lose more. The downsides of a feeding tube are obvious and I know it sounds scary and terribly medical, however, there are many positives that go with it. Lu will still be able to eat by mouth, what she can, but she will be able to get the calories and nutrition that she needs, plus as much fluids as she needs. I will not feel an overwhelming panic/urgency during meals because I know she is only eating a fraction of what she previously could eat, and meals will not need to last an hour or more each time because we will be supplementing with the feeding tube. This is not written in stone and we are just going to monitor her weight and check back in with Dr. Sasha in three months to see what her weight is doing and go from there. 

EEG

Here I sit in the Bronx, watching sweet Lu sleep in a giant steel crib. She's having an extended EEG to monitor her irregular breathing and staring spells. I'm anxious to know what is going on and what, if anything, can be done about it, but I also hate being here. Especially since we just got home from vacation and had to leave again so soon, and because only one parent can stay in the room at night so Chad has to go stay in a little "apartment" that's 95 stupid dollars a night. But, when we took Lu to Geisinger earlier in the summer, they were just like, "Yeah so what she has an abnormal EEG." And, "Girls with Rett Syndrome have irregular breathing, that's all it is." And here they are like, "Oh my something is going on, let's see what it is. We care."

Just to recap, here is what hasn't been going well for Lu this summer:

-near constant hyperventilation episodes throughout the day, occasionally being preceded by breath holding

-staring spells that include slumping over, drooling, and unresponsiveness

- she has lost the ability to remain sitting upright reliably and consistently

-has lost the majority of her remaining hand functioning

-has been having so much trouble chewing and swallowing that she has lost 1.5 pounds in about a month, has started needing to have her food puréed, is drinking Pediasure a couple of times a day

-painful hiccups

-increased muscle tone

-Lu has barely been able to use her PODD due to all of these difficulties

So, the doctor we saw today called it a regression, even though there isn't really supposed to be more than one regression period with Rett Syndrome.  But, I have been saying all along that was what was happening. It took us awhile to get it in order and to actually get here, but here we are, so they will see what they can see. 

Always a trooper! The bravest girl I know.

 

"What are you going to tell me?"

So, the battle continues with the secondary insurance, Gateway Health, but we finally have decided to by pass them and have the Tobii ordered and billed only through our primary insurance, BCBS. I was not aware that all along Blue Cross had approved the device 100%! However, even though they say they approve it, this is not a guarantee that they will pay. In reality, they usually do what they say and pay, but they have to state that its not a guarantee. So, we were given the option to bypass Gateway since they are being so difficult and we then agreed to pay any difference if for some reason the primary doesn't cover it all. But, Gateway is still deciding on my second-level appeal, so if they decide to not be so evil and they finally approve it, then they will pick up the difference, again, if there even is any!

I think if we had known this was an option I would've done it a long time ago, but maybe they wait until insurance is being real stinkers to take this route. Either way, Lucy's very own Tobii was ordered on Monday morning! It will be here in 2-3 weeks! The reaction I had surprised me. I got so excited and so emotional that I was just crying and laughing. I was showing Lucy pictures of it online and reminding her of what it did (since it was so long ago that we had the loaner) and she just started laughing and smiling and getting excited. As I cried, I asked her, "What are you going to tell me?" And she went wild with laughter. So, I tried to capture her excitement on video and succeeded! Here it is:

The Rising Sun

Lu and I (thanks to Lu) were up to watch the sunrise yesterday morning:

And here is her artistic interpretation of it:

We used her eye gaze board (that has clear pockets) to choose what colors she remembered seeing and what she wanted to use to show what she saw.