Being Brave

There are a few things that Brian and I have had to do for Becca that we never could have guessed we would ever have to do.  One thing that I hoped I would never have to do is to send my young child in to a procedure where she would be put under.  We have now done that five times and it has only gotten harder (especially since last summer).  Going right along with last summer we never thought we would ever have to watch our child be on life support (hope that one never happens again).  There are definitely more (suctioning, constipation remedies, placing feeding tube down her nose), but this post is about how I will have to be brave tonight.

Ok, that sounds dramatic…but I actually am a pretty dramatic person so there you go.  The time has come to replace Becca’s G-tube.  Yikes!  Her current button (her g-tube is called a  Mic-key button) was placed by her GI doctor and as he placed it he said, now you will replace future buttons in your home.  I’ll admit I thought, well gross.  But, after placing a NG tube down Becca’s nose (Brian is the brave one who would do this for the most part but I had to try it so that I would know how to do it if she pulled it out when he was out of town), this should be a piece of cake.  Last night I told Brian I wanted to be brave and replace it.  I am a little nervous though.  I hope it doesn’t hurt her and I hope it’s not too messy.  And well, I guess the idea of having an open hole to her tummy for a brief moment grosses me out.  Wish me luck!  I think we’ll do it before dinner ;).

It is time!

That is it’s time to revive this blog.  Last night my husband accused me of always sugar coating everything.  I started to defend myself and attempted to prove him wrong only to realize that it’s true.  I tend to preface or conclude difficult aspects to my life with super positive statements.  For example, “Becca is having a hard time breathing at night.  But she has been so healthy lately and I’m so grateful for her!”  I guess I’m not good at telling things how they are without feeling like I’m complaining.  I really truly do love my life and adore Becca and so I guess I figure that if I talk about hard things, I also have to prove that I really am happy. 

The beauty of this blog is I can just tell it how it is.  This is meant to be a record of our life with Becca.  I really need this right now.  I have been struggling with the fact that I don’t really talk to other people about Becca.  I don’t know how to without making other people feel awkward.  It seems like people feel bad that they can’t really relate to that aspect of my life and it just gets awkward (and remember how I don’t know how to talk about life with Becca without coming across as complaining?) and I just don’t like making people sad.

So here we are!  I have a list of topics I want to write about that cover different aspects of Becca’s life and hope to post sort of regularly (whatever that is) about our life with Becca.

 
I took this picture of Becca today.  It is sure hard to take non blurry pictures with her constant shaking and busy hands.  But she’s looking older huh?

Becca's Schedule - July 2012

5-6am: wake up.
Paula usually gets up, puts a toy in her bed and turns off her oxygen. Becca usually plays pretty quietly by herself for a few hours.

8am: get Becca out of bed & have breakfast
We carry Becca into the living room and lay her on the floor or the couch. We change her diaper and give her her morning dose of Trileptal (anti-seizure medication) along with a cupful of water mixed with Miralax through her feeding tube.

Then we give her breakfast. Becca was having a hard time with the oatmeal, so we've recently changed to just yogurt in the morning. She's all smiles while eating her yogurt, and she goes through it in probably ten to fifteen minutes.

next: morning therapy
We've hired a teenage boy in the neighborhood to do some physical therapy with Becca a few times a week. Basically this consists of stretching her limbs, some time sitting up, time on her stomach, and time in her stander. We started doing this at 10:00am, but she was falling asleep about then so we tried moving it to the afternoon for a bit, but now we're going to try a little earlier in the morning.

next: formula and a show
Becca usually gets a little more formula in the morning before lunchtime. By the way, when I say "formula" I'm talking about this stuff. Becca sometimes gets upset in the late mornings, and shows definitely help. Even when she's not upset it's a nice change of pace for her. Becca's favorite show right now is Mickey Mouse Clubhouse. We also discovered recently that she really likes the movie Wall-E.

12:30pm: lunch & nap
For lunch Becca has just under half a can of Spaghettios and some more formula.  Sometimes we blend up the Spaghettios, sometimes we don't, just depending on what she seems able to handle. We'll also sometimes switch the formula for some squished cheese cubes.

After lunch Becca goes down for a nap. We make sure to change her diaper before naps or it'll be too full by the time she wakes up. We put her in her bed and turn on her blow-by oxygen (she has a PVC frame with sheets on top that serves as an oxygen tent).

3:30-4pm: wake up from nap
Becca usually wakes up in a good mood. If we go in and it looks like she barely woke up we give her some time, because she seems to do better if she has a bit to wake up before we get her out of bed. She usually needs a diaper change, and then we carry her back to the living room. She plays on the floor with some of her electronic toys. She really likes to watch her sister and brother play on the floor. Especially Christopher, he's crawling around a lot more now, and she seems very entertained by him.

6pm: dinner
For dinner we try as much as possible to give her whatever we're eating. She's been doing well with eating the same as us whenever possible. We keep some tubs of ravioli or mac & cheese in case we're eating something like sandwiches that don't blend well, but for the most part she eats with us. We've had to be careful lately to keep her feeding chair tight on her shoulders, or she ends up tipping her head back and choking and sometimes throwing up her food.

7:00pm: get ready for bed
After dinner and some hanging out, we start Becca's routine. We've been trying to give her melatonin and formula a little earlier in the morning because she's been throwing up in the night sometimes. It seems to be helping.

Becca's feeding tube has been good for the past little while, so we haven't needed to change the dressings anymore. She's also been pulling at the tube less which has helped as well. We haven't needed to wrap the tube or anything for at least a few months, which has been nice. We still clean out around the tube probably twice a week, but that's a lot less than before.

8pm: bedtime
After that it's family prayer and bedtime. We put Becca in her bed, brush her teeth, sing her a song, tuck her in and she goes to bed. We turn on her blow-by oxygen through the night and when she first goes to bed we lift a corner of her oxygen tent so she can see the snail night light/lamp Paula's uncle got Becca for Christmas a few years ago. Once she falls asleep we turn off the light and close the tent flap because if she does wake up in the night she'll be more likely to go back to sleep.

That's about it!

Becca's Schedule - January 2012

I've been meaning to do this for a while, but I keep not doing it. I want to start writing down Becca's schedule every six months or so so that we can get a better feel for how her routine is changing over time. Maybe somebody else will find it useful as well. I kept forgetting to post this, so this schedule is from the beginning of the year.

5-6am: wake up.
Paula usually gets up, puts a toy in her bed and turns off her oxygen. She usually plays pretty quietly by herself for a few hours.

8am: get Becca out of bed & have breakfast
We carry Becca into the living room and lay her on the floor or the couch. We change her diaper and give her her morning dose of Trileptal (anti-seizure medication) along with a cupful of water mixed with Miralax through her feeding tube.

Then we give her breakfast. We warm up a bowl of oatmeal, mix in a tablespoon of butter, and add whole milk to thin it down to about the consistency of yogurt. We sit her in her support chair at the table and feed her breakfast. She usually eats almost all of her oatmeal, and it usually takes her fifteen to twenty minutes. Every once in a while she won't eat well and only gets half of it down, sometimes less, before she starts coughing and trying to throw up -- that's our sign that she's done :-).

next: head off to school
A couple days a week Becca goes to preschool. The bus driver calls five minutes before she gets here so we can get Becca into her wheelchair and out to the curb. Then we help load her into the bus, they strap her wheelchair in, and she heads off to school. We always send her with a backpack with an extra diaper, an emergency pack for replacing her tube, and her school folder.We don't send her wheelchair tray to school with her because they try to keep her out of her chair while at school, so they never use it anyway.

next: come back from school
The bus drops Becca back off from school. She usually comes off pretty happy. She normally has some kind of craft in her backpack from school, and sometimes a note from her teacher. Her teacher uses email too, though, so that's how we usually communicate.

Right away we give Becca a little formula and (if we're feeling diligent) give her some time in her stander before naps. She watches a show while she naps, usually Signing Time.

12:30pm: lunch & nap
For lunch Becca has yogurt and either some cheddar cheese cubes or some more formula. If we give her cheese we cut it up into small pieces and squish them with our fingers before putting them into her mouth. If we're feeling diligent we'll push the cheese pieces onto her teeth and try to get her to bite down on it before rolling it with her tongue. Usually she holds the pieces against the roof of her mouth until they soften enough for her to squish them and swallow.

After lunch Becca goes down for a nap. We make sure to change her diaper before naps or it'll be too full by the time she wakes up. We put her in her bed and turn on her blow-by oxygen (she has a PVC frame with sheets on top that serves as an oxygen tent).

3:30-4pm: wake up from nap
Becca usually wakes up in a good mood. If we go in and it looks like she barely woke up we give her some time, because she seems to do better if she has a bit to wake up before we get her out of bed. She usually needs a diaper change, and then we carry her back to the living room. She'll play on the floor, we'll sit her up on the couch sometimes to watch a show, or try to get her more time in her stander.

6pm: dinner
For dinner we try as much as possible to give her whatever we're eating. We worry less about getting a specific amount of food into her, and more about getting her eating with us. For all Becca's meals we strap her support chair to a dining room chair and she sits at the table with us. For dinner we blend up whatever we're eating and add whole milk to thin it down. Sometimes our meals aren't Becca-able, so we keep some Chef Boyardee microwaveable tubs around in case. She's seemed a lot healthier since we've switched from mostly tubs for dinner to mostly our food for dinner.

Paula made a bunch of bibs for Becca, and she uses them for each meal. Becca always tries to yank them off, and she likes to put her hands to her mouth, so meals can sometimes be a messy occasion.

7:30pm: get ready for bed
Between dinner and bedtime we try to play with all our kids. We'll read books or play games or rough house, and Becca always likes being involved.

Around 7:30 we start getting ready for bed. Becca gets 2mg of melatonin via 2 pills in her mouth. She swallows them fine. Then we give her another dose of Trileptal and another 120cc of formula. After that we change the dressing on her feeding tube. We were told the tube would heal up on its own, but it still leaks a bit every day, and there's usually a little bit of granulation tissue somewhere around the edge of the hole. We have a bandage wrap and some gauze to hold the tube in place, but we think Becca still yanks on it all the time, which may be why it's not healing well.

8pm: bedtime
After that it's family prayer and bedtime. We put Becca in her bed, brush her teeth, sing her a song, tuck her in and she goes to bed. We turn on her blow-by oxygen through the night and when she first goes to bed we lift a corner of her oxygen tent so she can see the snail night light/lamp Paula's uncle got Becca for Christmas a few years ago. Once she falls asleep we turn off the light and close the tent flap because if she does wake up in the night she'll be more likely to go back to sleep.

That's about it!

Competing with the Pink Ribbon

Most people don't know this, but Breast Cancer Awareness Month coincides with Rett Syndrome Awareness Month. I don't know who thought going up against breast cancer in October was a good idea, but I guess you probably wouldn't see very many purple ribbons regardless of the month IRSF chose. It's just not that well-known.

Quick recap: Rett Syndrome is a neurodevelopmental disorder caused by a genetic mutation resulting in a malformed protein. There are I think around 20 different known mutations all resulting in the same malformed MECP2 protein and gene. The mutation occurs on the X chromosome, so males do not typically survive since they don't have any properly functioning X chromosomes if they have a mutation. The severity of the disorder is dependent on the percentage of well-formed X chromosomes, which apparently is not always 50% like I would have assumed. Rett is an autism-spectrum disorder, but the symptoms are more commonly compared to cerebral palsy. Rett girls typically do not talk and only half are able to walk. The life expectancy is stated to be around forty, but most deaths I hear about are for girls in their twenties. One in twelve thousand girls are born with Rett.

Our little girl Rebecca has Rett Syndrome. She was diagnosed in August of 2009. The geneticist, Dr. Rope, walked me through the biological details of her specific case, and also Rett in general. He then said, "I can tell you all about the biological effects, but I've never had to take one of these girls home with me." I didn't realize at the time how apt his comment really was.

We've tried some to interact with other Rett families. We subscribe to some blogs (thank you everyone for sharing your experiences), we've been to a couple events (these are kind of depressing to me, I already have one Rett girl I don't need to see twenty more), there's even a mailing list. But when it comes down to it, raising a girl with Rett is I think a lot like raising any other kid: you just have to figure it out as you go. It's been great to get some advice for what to expect and how to make life a little easier, and Becca's doctors have been super helpful in knowing what to worry and not worry about, what equipment we might need, etc. 

But Becca is undoubtedly her own person. She gets annoyed if you touch her face. She thinks it's funny when her siblings cry. She doesn't appreciate it if we leave her inside by herself. She's not a big fan of mashed potatoes, but she loves cole slaw. She needs some time and space when she first wakes up or she'll be super grumpy. She loves to wear cute outfits and likes to play the piano. We recently discovered that she thinks The Nightmare Before Christmas is hilarious. She doesn't get as many opportunities to express herself, or to make decisions for herself, but it's very clear to us that she has opinions on things and that she is fiercely devoted to her family.

We are so grateful for our little Becca. I know some people think we're crazy for having more kids after giving birth to a disabled child, but I don't think there will be much that's more valuable to our other children than having the experience of growing up with their sister in our home. It makes us all think a lot more about what it really means to live a "full" life, and what God intends for each of His children when we can't even be sure some of them will ever be able to walk or talk.

At least, not in this life.

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