Tonsils, Adenoids, and a Code Blue

This is kind of a delayed update, but it's been hard for me to write about it, and I wanted to wait a while before writing so I could look at it a little more objectively.

About 5 months ago Becca's geneticist Dr. Rope recommended we do a sleep study for Becca. She gets pretty tired during the day, and most days she wakes up between 4 and 6am, so there's definitely room for improvement in her sleep routine. We scheduled the sleep study (that's probably worthy of its own post. We'll never do another sleep study anywhere other than at Primary's. The sleep tech was pretty sure he wouldn't need to wrap the EEG nodes, but Becca went at them most of the night, and fought the nasal cannula as well... it was a blast...) and the big takeaways were that her oxygen levels are a bit low when she sleeps and that her brainwaves are all over the place (no surprise there, pretty typical for Rett girls). We decided to give her blow-by oxygen at the night, and see a ENT about possibly getting her tonsils removed.

The ENT, Dr. Grimmer, thought it might be helpful to remove Becca's tonsils and adenoids.  Her tonsils were enlarged, and while it wasn't a major concern, it might help her breathe a little more easily.  Becca does cough sometimes in the night, and it gets pretty bad when she's sick, so it seems like it'll be a good thing in the long run.

But first we had to make it through the surgery.  June 6th we went in to Primary Children's Hospital for her T&A.  We were really nervous before we went in because Becca had coughed a couple times that morning and had a low-grade fever, but she does just cough randomly sometimes, and the hospital told us to go ahead, so we brought her in for the surgery.  We won't ever take that kind of risk again.

After the surgery they took Becca to the floor for recovery (it was technically an outpatient surgery, but they wanted to keep her overnight to make sure everything was all right).  I wasn't there, but right away Paula knew something was wrong.  Becca wasn't breathing well and was obviously pretty miserable whenever she woke up from getting her tonsils and adenoids removed.

After a couple hours she had what Paula thought might have been a seizure.  For the previous few weeks sometimes Becca's arms and legs had started twitching rhythmically for a few minutes at a time. She was very in there when it would happen, but we weren't sure if they were seizures or not.  Paula took the opportunity to ask the staff if our neurologist could come see what was going on and confirm whether or not it was seizure activity.  Dr. Benedict wasn't available, but another neurologist named Dr. Lloyd checked in and called it a seizure (incidentally, after some follow-up EEGs we now know they weren't seizures, and it sounds like this kind of thing may be common for Rett girls, there just wasn't anybody around with any experience in Rett cases).  They gave her a huge dose of versed (or ativan, I can't remember which) and the twitching stopped but she got all messed up.  Her eyes weren't lined up anymore and she would either sleep or stare off at nothing -- and she still was not breathing well.

The breathing was the biggest concern for us.  Becca's always had a hard time with breathing, and when she gets sick at all or coughs a lot she stops swallowing and stuff builds up in the back of her throat and exacerbates the problem.  She had blow-by oxygen but her breaths were just really short and quick, and her oxygen saturation levels were all over the place (anywhere from 80% to 50%).  We asked a couple times about it, but none of the nurses were concerned with the situation and said it wasn't a big deal after a throat surgery, so we assumed it was fine.  They gave us a suction tool to help clear her out, but told us we shouldn't suction much or it would make things worse -- even though it was pretty clear she was collecting a lot of gunk back there.

This, by the way, was pretty much the end of our implicit trust in nursing staff.  We assume now that nurses won't know the right things to do for Becca, and then they can sometimes pleasantly prove us wrong and earn our trust.  If we'd known then what we know now we probably wouldn't have ended up in the PICU.  Hm... Actually, I guess if we'd known then what we know now we might have pushed them to take Becca directly to the PICU for recovery instead of just going to the floor.  We're a lot more confident now than we used to be that we know what's best for Becca, so I guess at least there's that.  It seems like maybe that's something a Rett parent just needs to learn eventually.

Anyway. I sent Paula home to get some sleep (she was 5 months pregnant, and Becca wasn't coughing quite as much anymore) and settled in for the night. At about 10:30pm Becca's breathing got a lot worse.  She started coughing pretty bad, and then she started coughing worse (this is when the nurse came in), and then she stopped inhaling, and then her coughs got really weak, almost non-existant.  Her oxygen saturation dropped to 20% and the nurse hit the emergency button.  The PICU staff got there within 30 seconds and right away started working on opening Becca's airways. Becca's lips were blue and her skin had lost its color almost completely.  She looked dead other than the faint spasms in her chest. Her oxygen got down to 12% before starting to slowly climb up again.  About half an hour later the respiratory technician finally had her up to 85% oxygen, and we decided that was enough to move her down to the PICU.

As you can imagine, I was pretty much a wreck at this point. I actually think I did really well until somebody said something about "life support" and for some reason that was the thing that threw me. I managed to get a call out to Paula and speak enough that she knew to get there right away. They wheeled Becca down to the PICU and got her all checked in.  She couldn't breathe at all on her own, but at least she was stable.  We found out later one of her lungs had completely collapsed, and the other was significantly obstructed.  The event was classified as "acute respiratory arrest".

I think this is enough for one post. Maybe I'll do the PICU experience in another post.

The Life That Might Have Been

I often wonder if I will ever stop thinking about what Becca’s life would be like if she didn’t have Rett Syndrome.  Sometimes I look in her eyes and see so much personality locked up in her little body and it truly breaks my heart.  I know she has a wonderful sense of humor just like her Dad.  And I know she’s smart, just like her dad.  She seems to understand more about life than any of us do. 

Anyway, I was looking through old pictures and videos earlier today and was, once again, surprised at how Becca has changed through time.  It’s always a bittersweet experience for me.  I’m not sure why I do this when I just end up crying…I guess I can’t explain it, it’s just something I like to do.  Though I had that familiar ache in my heart and a lump in my throat as I looked through our many pictures, I came to a realization.  When Becca was little I was always waiting.  Waiting for her to master the next skill, waiting for her to get stronger muscles, waiting for her to catch up to other kids, and waiting to figure out what was wrong.  I was always wishing time would fly by so things would get better (if that makes any sense).  I didn’t stop to enjoy what a sweet baby she was.  I wish I could go back and do it over.  If only I knew that she had mastered skills that she would later lose. 

But I also came to the realization that I don’t do that anymore.  With Becca’s diagnosis came sadness but a whole lot of peace.  I just enjoy her now.  I’m not wishing time away now that I know.  And I try to focus on reality rather than the life the she might have had.  I am just so grateful for her and that she chose to come to earth even though she knew it would be hard.  I’m so grateful that she came to our family because we truly do need each other.  I am so grateful for the sweet spirit she brings into our family for all she has taught us and will continue to teach us in the years to come.  I think I might always feel inadequate when it comes to raising her, but I know I will be given the strength necessary to do so.  Becca was blessed with a Daddy that understand her better than any other person.  He knows how to cheer her up or provide for her at times when I don’t.  God definitely knew what He was doing when He sent sweet Becca to us.

Wow, I came to update on Becca’s life and then out came all of that. I guess I don’t feel like I have too much to report.  Becca has been sick a lot this winter already.  Each time she’s sick I find myself wishing that she could tell me what’s bothering her.  It’s sure hard to take care of a sick kid that can’t communicate.  But she’s been blessed to overcome the bugs that came her way.  She thinks the holidays are exhausting…

But she has also had a lot of joy this holiday season

New House and Preschool

With moving to a new house I was worried that it would take Becca a while to adjust, but she has done great! The whole time we were looking for a house we kept telling Becca we were looking for a house for her. She seemed to say, we finally moved to my house! She has a bigger living room to roll around in and her very own bedroom.

Becca started preschool last month and it has been an adjustment for all of us. We went in the week before she started and met her teacher. We absolutely love her. She is so good with Becca. I was excited to hear that she had a girl with rett in the past. It has sure been nice that I haven’t had to explain all about Rett. She seems to understand Becca and her needs. We’ve started with her going Mon/Wed from 9-11 and we’ll see how she does with that.
So this year we decided to send Becca on the bus. She went on a field trip last year and they took them on the bus. I was told she smiled and giggled the whole way. So, I thought this would be something she would enjoy and would give her a little more independence and lengthen her preschool time a little bit. Anyway, the first day came and I hadn’t heard anything from the bus. So I assumed they hadn’t got Becca’s information yet but I was totally h
appy to take her. As I went out to pack up the bus pulled up. I was like…oh ok so I pull her wheelchair out and assemble it (I’m getting faster at this but I’m still sort of slow). Then I ran in and got Becca, brought her out and strapped her in. I totally expected the bus driver to introduce himself and ask about Becca. But no…he actually didn’t really say anything. He quickly strapped her in while I’m chattering away about how I didn’t know if the bus was coming and about Becca. Nothing…and then he drove off. With my precious child. And then I cried. Like a lot. I had just sent my sweet daughter who has special needs with a stranger who didn’t seem to even look at her. I had no idea if Becca was scared. I hoped he took her to the right school! I truly felt like a horrible mom. My sister was in town so I could thankfu
lly leave Katie with her. I waited a little while to give Becca time to get to school and get settled and then I drove to the school to check on her. It turned out that it was good I went. Her teacher had a few questions about her wheelchair and it was good for me to be able to see Becca happy and in one piece. I also found out that the strange bus driver was actually a sub. Whew. Here’s Becca on her first day of school with her backpack that is actually my old backpack. How embarrassing! Sorry Becca :).

The second day of school Becca came off the bus crying :(. I said, oh no! She’s crying! And the bus driver told me that she actually cried the whole way there and the whole way back. Well that broke my heart. I called her teacher right away and asked her how she was coming off the bus. Again, it was good I called because her teacher said she was really upset coming off the bus and it seemed like she was aspirating. Great. She had a swallow study done last year that she passed, but I know that she is at a high risk for this. When Becca gets sad and worked up, thick mucus builds up in the back of her throat. She feels like she is suffocating because she has a hard time swallowing. Pretty scary. Anyway, this whole bus thing wasn’t ok with me if it was upsetting her. So her teacher and I came to an agreement that I would bring her to school and then she would come home on the bus. I felt so good about that because then she would still get a chance to get used to the bus. The first day of this was wonderful. I hear she was happy at school and she came off the bus happy. Apparently she has a new boyfriend on the bus too.
Becca has been in a good place lately. Though we haven’t seen any fine or gross motor improvements she has been really happy. She seems to be trying to communicate through her eyes more which has been really wonderful too. She’s finally over her cold (we all passed a cold around a couple weeks ago and like always hers lasted like two weeks longer than ours because she can’t get rid of congestions)! For the most part she has been sleeping well (last night she was awake until like 11 just chatting away, but she doesn’t usually do that). She hasn’t had any seizure activity that we’ve seen lately as well. All wonderful things!

That’s Better

I just wanted to quickly mention that Becca has been doing a lot better the past couple days.  I’m not sure what was bugging her, but she seems a lot more content and happy.  Also, no seizures yesterday or today so far.  Things can sure go up and down quickly.  But I’m grateful to, once again, have a happy Becca.

An Off Week

Becca is having a bit of an off week. Brian and I had just mentioned last week that Becca has been in a really good place lately. She’s been so content. By content I mean happy 97% of the time. She hasn't been making any improvements in motor skills (gross or fine), but she hasn’t been losing skills lately either. We are so grateful that she has stayed healthy for the most part through the sick season and she has been gaining weight well. She also has seemed more with it lately. I don’t know what it is but she seems to be trying to communicate more through the way she looks at me. But, as is often the case, when you say things are going well, things take a turn for the worse.

Brian and I think that Becca may be having little seizures this week. She’s been doing this thing where her eyes roll back and her limbs shake a bit and then she’s fine. They only last a few seconds. We have no way of knowing if they’re actually seizures. She shakes quite a bit on a regular basis without having a seizure. This morning she had one of her moments where she zones out for a while and just lays there (I think it’s a little seizure but of course I can’t be sure). The only thing she would move was her poor little hands that never get rest (except for when she’s sleeping). She has been pretty unhappy these past couple days. I’m hoping these seizures or whatever may be bothering her get better soon. Brian and I both get really discouraged when we can’t figure out what to do for her. I scheduled an appointment with her neurologist. Maybe an adjustment in her medication will do the trick. I think that we’re just really worried that she may be starting to go downhill again. I don’t feel brave enough to see her lose more.

Also, I really feel like Becca is trying to tell me something lately. Sometimes she will look deep into my eyes with a look that seems to say help me. It truly breaks my heart every time. I know that she is going to learn how to better communicate with us, but until then we just have to try to be patient.

I can’t remember if we mentioned on here, but at the end March we got Becca’s wheelchair! We’re really liking being able to move her around more easily and it seems like a really nice chair. I don’t have a picture that shows all of it yet, but this will give you an idea…

A few weeks ago my family was in town for graduation. It’s always neat for me to see how much Becca loves people. She really seemed to enjoy having everyone in town. This video is from that weekend. She was having a fun time playing with her Daddy…

I guess this post is rather random. I have so much on my mind with Becca and getting a tiny bit out helps. But, in the spirit of randomness, here is a random picture of Becca that I absolutely love.

I could just cuddle with you all day dear Becca.

PS- Becca, you have really pretty teeth…will you stop grinding them? Thanks.

Hand Movements

One common symptom of Rett Syndrome is repetitive hand movements. Rett girls flap their hands constantly (it sure seems like they'd have some pretty buff hand muscles, no?) often at or near their mouths. Becca fits this symptom exactly. Coupled with her very poor motor skills, her hand movements makes it really hard for her to do anything useful with her hands. When she was younger we taught Becca some basic signs, including "eat" and "drink", and she's still able to sign both of these words, but that's about all she can do successfully. Becca also could pick up food with her hands, and even at one point started using pincer grasp to pick up food and small items, but she gradually lost that skill.

I wish we had some better videos to illustrate Becca's gradual loss of motor control, but at the time we were a little sensitive and only took very flattering pictures and videos of Becca, so I'll just have to re-cap with words.



This is Becca at about 12 months old. You can see here that she was good at holding on to things, and could use both hands. She had enough control at this point to bring food up to her mouth, and to hold on to something as long as she wanted. She would hold and shake toys, and really liked to hold things in her mouth. We were actually bad parents apparently since we let her drink her sippy cup lying on her back, but she could hold the cup by herself that way and we didn't really want to take that skill away from her, so :-P.

About this time (around 12 months) we started feeding Becca cream of wheat (mixed with ice cream) to get some heavier, caloried food into her (and mostly because it had more fiber than other foods we could find for her). She really liked it a lot. She would sign "eat" between bites, and end up getting cream of wheat on her hands (and the couch, and her clothes... cream of wheat is a horrible food to try to clean off of things, if you were wondering). She liked the way it felt and she would rub her hands together whenever she had cream of wheat on them.

After a little while it became sort of a reflex for her, and she'd flap her hands against each other whenever she was eating, even if her hands were clean. That was one of our big worries, since it sure seemed like something was wrong. But for a while it was just when she was eating, which technically could have just been a tick or something.

That same clasping movement started happening more and more often. Like you can see in the next video, it was relatively subtle at first, but she would close her hands repeatedly, and then bring them to her mouth.



She liked to bring everything to her mouth. We bought her an electric toothbrush because the therapist said sometimes if kids don't get enough oral stimulation they'll put everything in their mouth, which didn't help much, but at least we were cleaning her teeth better :-). The hand movements got worse -- very gradually -- and she started dropping things. If she wanted something, she'd reach out and grab it, pull it to her mouth, and then drop it after a second or two. She also stopped holding her own sippy cup.

By twenty months Becca couldn't really hold things anymore. Her fine motor skills were pretty much lost, but her gross motor skills had improved a little bit -- this was her peak, probably, as far as more broad gestures. She had a few weeks where she was even clapping! I'm glad we got to see that, even if only for a little while. She seemed happy to be able to express herself like that.



By two years her hands moved constantly and she couldn't really do anything productive with them other than mash big buttons on electronic toys. You can see ere where she tries to eat her birthday cupcake. She really wanted the it, but couldn't figure out how to get it to her mouth.



That's about where she's stayed since then. Her hands open and close constantly, and she usually tries to have one of them in her mouth. Every once in a while she gets them red and sore from sucking on them too much, but she's been better about that lately. She does still sign eat and drink, so I'm really glad we taught her those early on.



It's been hard to watch Becca lose skills like this, especially as we see other kids her age (and now her little sister, too) learning so easily. I don't think people appreciate what a huge miracle it is for kids to learn new skills so quickly and so often. Becca's little sister is a constant miracle to us as she just decides to pull herself up to standing one day or to start feeding herself or to walk from one piece of furniture to another.

Next time you're worried because your kid won't say "mama" or doesn't seem interested in walking or whatever, take another look -- I guarantee there's amazing things to notice that they're learning all the time. And if nothing else, be grateful that they hold onto the skills they learned six months before. You don't know what a big deal that really is.

First Day of School

Well, I sure never thought my daughter would have her first day of school at the age of three! Yep, Becca started preschool a couple weeks ago and this it how she feels about it...

This is so not what I pictured her "first day of school" picture to be like

Yep, she loves it! She loves all the attention she gets and watching all the other kids. She is currently on spring break (I asked her what she wanted to do for spring break and she just sort of looked and me like, ummm, what?) but she'll be back to school next week. She starting out with just an hour and a half on Wednesdays and Thursdays (because it's a smaller class on those days). She'll work up to staying the whole 2 and 1/2 hours.
You ask how mom is doing with this new development? Well, I guess I must first confess that I haven't actually left her at school yet. I've stayed with her the entire time all the times she's gone. I guess that makes me a wimp. But, I must say that it has made me a lot more comfortable about leaving her in the future because I know exactly what she does when she's there and I'm getting to know her teacher. Her teacher has been wonderful so far. She's so willing to let me come as long as I want to. I'll probably stay at least another week or two.
Preschool consists of play time (for Becca this is usually speech therapy), gym time (sometimes physical therapy for Becca), circle time (singing songs, story time, learning a letter of the alphabet, etc.), activity time (painting, coloring, crafts, etc), and snack time...we leave at this point so who knows what happens next! For all I know they could have spanking time next! Yikes! Ok, so I know what happens next, potty time. Becca has potty time 24/7 so I guess that won't be all that eventful for her and then reading time and then, something else that's probably cool.
This whole preschool thing was really hard for me as the day approached that Becca would be starting, but it's really a lot easier now that I see how much she enjoys it. Any guesses as to how long I will be attending preschool with Becca? Your guess is as good as mine.