Becca the Blog is going to get noisy.

I promised an update on the school thing, this is not that update. I'd say it's still in progress.

As part of the ongoing-ness, though, I'm going to start posting some sessions that we're doing with Becca after school.

Basically we didn't know just how capable Becca was, and we've been surprised with some of the things she's been doing since the beginning of summer, and I wanted to see just what she could do. Then that sort of evolved into an experiment to see how close we could get to a first grader's curriculum with Becca.

I took the state core standards for first grade and started marking all the ones I thought Becca could learn but probably wouldn't learn unless someone spent some time with her. I was surprised, actually, with how few I wrote off as out of her reach. Turns out the hardest thing in first grade is math, the rest is very approachable for Becca with some straightforward adaptations.

So then I took those standards and organized them into categories that I thought we could work on at home. Things like "order & organize", "sun & moon", "creative piece of writing", "jobs & roles", etc. I was curious just what we were up against in scope. In the end there were about 36 categories of work, and with 35-ish weeks in the school year this was starting to sound not impossible.

Since then we've been working with Becca on some of these categories. Every time after we work with her we take detailed notes of what happened, trying to keep out our own interpretations and noting our biases as we find them. Mostly we want to have evidence of just how capable Becca really is. Because it turns out she can remember a lot of things, and she likes interacting and communicating with her eyes. She likes "school time", as we call it.

But that means this blog is going to get a lot noisier than it has been, for the foreseeable future. Feel free to unsubscribe, we won't get our feelings hurt :-). If you hang around, I'm going to put "school time" in all the titles of these logs so they'll be easier to skip over.

Becca Can!

Becca really wanted to go the pool a few weeks ago.

We're in the middle of a little "adventure" dealing with the school's perspective on Becca. I don't want to go into it in this blog post, because I don't want to jump to conclusions, but for first grade Becca was put into the "medically intensive" room. I've been all kinds of frustrated and worked up making assumptions about why the school did this and how it's going to be this huge uphill battle to get what I consider a problem to be fixed, but that's a post for another day.

The only reason I bring it up is because as part of prepping for our meetings I've been making a list of the things Becca *can* do. I was pleased by the list, and to me it makes it pretty clear Becca is "in there" and she just needs people who believe that's true in order for her to succeed. We have made so much progress even just over the summer, I'm honestly a little scared to lose it if I can't get the school on board.

Here's what Becca can do:

- reach out in front of her when something she wants is in front of her (i.e. toy boat in the pool, toy dog on the floor, candy crush on the tablet, communication switch)

- make eye contact to confirm a question ("yes", i.e. "is yellow your favorite color" "do you want to go to the pool")

- not make eye contact to say "no" to a question (i.e. "do you want to go outside", "do you want to stay home from school today")

- not make eye contact when she knows the answer is "yes", then laughing when we "catch" her (i.e. "did you poop", "did you go swimming at school today", "do you love your brother")

- reach out and hit a large communication switch she knows is for communication without prompting

- look directly at something she wants (i.e. crackers, tv, a different pillow, etc.)

- look back and forth between something she wants and the person who can get it for her (i.e. tv to grandma)

- reach out quickly and grab something she is interested/bothered by (i.e. nasal canula breathing tube, syringe full of formula, baby brother's face)

Becca's sister is such a good helper

- laugh when she is able to physically touch or hit something in a way that causes an upset or surprised reaction (i.e. messing up Paula when playing Candy Crush, knocking over the syringe full or formula while feeding, hitting Christopher in the face to make him cry)

- make verbal noises when something is mentioned she is excited about (i.e. "we're still planning to go to the pool tomorrow" resulted in a "gie-ood" noise and excited laugh, "tomorrow is Christmas!" was similar)

- have opinions about decisions (i.e. grandma really wanted to get her to watch Brave, she kept picking everything but Brave)

- have a preference for who to communicate with (didn't want to tell Paula she wanted to go to school, she wanted to tell me)

- not say yes or no when she's not in the mood (i.e. shopping at target she wouldn't pick, wouldn't "engage" to be able to say either yes or no)

- pick the option that we're pretty sure isn't her favorite, just because her sister wants the other one (i.e. what movie to watch)

- eye gaze at a communication system (on a tablet) with 6 items, with person vocalizing the items "selected", then confirming she wanted where we ended up on the communication system

- look directly at someone when she knows they can help her communicate a desire/interest (i.e. someone she knows can get her communication board)

- look directly to something that is conceptually related to another point or idea (i.e. I helped her cheer "Go BYU!" and she then looked directly at my BYU t-shirt, "do you know what time it is" and she looked directly at my watch)

I also realized lately that a lot of the time Becca spends at home isn't very challenging or even stimulating for her, it's watching shows and playing with toys that she's mastered a long time ago. I want to start working with her at least some at home now, so I started that today. Here's what happened:

9/1/13

We read Memoirs of a Goldfish (which is a really cute book, by the way). I asked Becca who the story was about, who was in it from the start, and she picked Cha-Cha (the story is about the goldfish, not Cha-Cha. I probably should have asked her something better, like who was telling the story). So we talked about it and how the goldfish is the one in the whole story, so that's who I would have picked. 

Then I asked her which character she liked better, Mr. Bubbles or Mervin. She picked Mervin, so I talked about what was silly about Mervin, then we talked about what was silly about Mr. Bubbles. Then her sister picked between the guppies and Cha-Cha, she picked the guppies, then we talked about why she liked the guppies better. 

After that we tried to identify which fish appeared in the story in which order. Becca and her sister took turns picking between two options, and they both picked correctly every time. Then I asked Becca which fish the goldfish liked better, Gracie or Cha-Cha. Becca picked Gracie, which makes sense since Gracie is also a goldfish and the goldfish likes swimming around the pool with her. Then Becca and her sister took turns putting the fish in order again, and there were no mistakes that time either.

I wasn't sure what to expect from this exercise, but by keeping the tone very conversational rather than making it sound like a test we did a lot better than times I've tried before. I think we're in for some more surprises as we keep trying to help her get out of her shell :-).

Becca's Schedule - May 2013

This schedule is a couple months old, but I wanted to get it out there since it's all going to change a lot now that Becca is heading into first grade. This summer has been all over the place with sickness and summer school and family trips, so I wouldn't say it's been consistent enough to even call a schedule. I know that's been hard on Becca and I feel bad about it, so hopefully we'll get back into a routine for her soon.

4-6am: wake up.

Depending on the week Becca has been waking up earlier than she used to. We get up, slide her down off her pillow and give her a toy. She plays quietly until we get her up.

8am: get Becca out of bed and have breakfast

Becca has been having a tub of yogurt for breakfast. Depending on how well she at the night before she'll open her mouth pretty wide, and she usually eats the whole tub. The we give her her trileptal, two tubes of formula and some Miralax in water to make up the difference of what she should be having for breakfast.

9:30am: nap.

School has been in the afternoon this year, so Becca's been getting into the habit of falling asleep not long after breakfast.

11:30am-12:00pm: get up from nap. lunch through a tube. get ready for school.

Becca gets four tubes of formula for lunch, and the rest of the Miralax from the morning. Then we get her dressed if she's not already, do her hair and put her shoes on.

12:30pm: head outside and wait for the bus.

Becca's new wheelchair is great (and it still fits in the van! hooray!), it's almost the same as the loaner from the school, but actually the right size. We keep it either in the living room or in the garage, then wheel it out to the front porch and carry Becca down the stairs to put her in. We keep talking about getting a ramp, but haven't done that yet.

4:30pm: arrive back home on the bus. standing time if she's up for it.

Depending on how Becca's feeling when she gets home (and how much else is going on at the house, unfortunately) we try to get Becca to have some standing time before dinner. She usually tolerates 30-40 minutes in her stander with a show on. Sometimes she'll get frustrated before then, but usually she's a good sport for standing time.

We also try to get another two tubes of formula into her long enough before dinner that it doesn't ruin her appetite.

6:00pm: dinner.

I feel like we've made a lot of progress on the dinner front with Becca. Almost without exception now she eats whatever we're eating. We got a new chopper (and have learned a few tricks for better chopping) that lets us puree anything from pizza to quesadillas to chicken and rice to hungarian goulash. We use milk (or bread for soups) to get it to the consistency of thick pudding, and Becca will eat a healthy serving of most things. There are definitely some foods she likes better than others, but she's definitely the least picky eater of all our children :-).

7:30pm: get ready for bed.

Becca gets two melatonin pills by mouth every night (as long as we remind her to try not to choke she usually doesn't), a dose of trileptal and another four tubes of formula. We change Becca into a larger "sleep" diaper for the night because she's been leaking through more often lately in the night, then we say a family prayer and it's off to bed.

8:00pm: bed.

We put Becca in bed, brush her teeth, turn on her night light, sing her a song and turn on her blow-by oxygen to run in her tent. She usually falls asleep within five minutes of going to bed.

We've flipped Becca's bed around recently to try to help at least a little with her hips. She always ends up sleeping on the same side. Obviously this new side hasn't been her preference, so sometimes she gets herself in weird positions trying to get comfortable. She doesn't like laying on the side so she'll try to roll the other way and get all twisted, or try to roll onto her stomach and get two-thirds of the way there with her neck all arched back. Not sure if it's actually better or just bad in a different way :-/.

And that's it! A day in the life of Becca from two months ago. There was recently a bunch of people in a private Facebook community for Rett parents talking about their schedule that I think made both of us feel guilty that we don't fit more therapy in, but honestly I don't know where it would fit in our days anyway. One more thing to work on, I guess.

Rett Syndrome and Apraxia

We went to the Rett conference two weeks ago in Midway, UT. I'll talk about the conference in general in another post, but one of the recurring trends that I picked up on was actually an eye-opener for me. Multiple presenters and some of the parents I spoke with talked about "apraxia", which was a term I probably should have known already but didn't (I blame it on the fact that it's currently missing from the Wikipedia article on Rett).

Apraxia is a stubborn body that refuses to listen to a willing brain.

Apraxia means a failure to carry out purposeful movements that a person is actually capable of performing. It's not the same thing as ataxia, which is an inability to perform purposeful movements at all, typically because of a neurological disfunction. With ataxia there's not even the ability to move on purpose, but with apraxia there's the ability, but something is jamming the signals along the way. Our neurologist used the word "ataxia" a lot when first diagnosing Becca, but "apraxia" was a new one on me.

If you do a search for "Rett apraxia" some of the first results that pop up are by Linda Burkhart talking about just how much of an impact apraxia has on girls with Rett. You'll also see comments from Boston Children's Hospital, National Institutes of Health, etc. calling apraxia "one of the most disabling characteristics of Rett".  Linda's tone very closely resembles Judy Lariviere and Pati King-DeBaun who presented at the Rett conference (so much so that I thought Linda was Judy when I first found her web site). Linda (and the others) talk a lot about how apraxia in Rett girls is made worse by demanding or asking for a specific action, which leads to problems with assessment in school (since testing is almost always done in a "now show me you can do this" manner). In other words, if you ask a girl with Rett to stick out her tongue she probably won't do it even though she may be perfectly capable of sticking out her tongue when she wants to.

Probably an easy way to understand apraxia in somebody like Becca is to think of Becca's body as a defiant teenager and Becca's brain as the teenager's mom. The teenager is perfectly capable of taking out the trash, but as soon as mom says "Johnny, take out the trash already! You're killing me!" then it's a contest of wills and Johnny does everything in his power not to take out the trash -- at the very least not right at that moment. That's what's going on with apraxia, sometimes the action just takes a lot longer than it should or than it normally does, and sometimes it just doesn't happen at all. And demanding action only makes the problem worse. In the case of Rett it appears to have to do with the neurological characteristics of the disorder gumming up the brain-to-body communication somehow.

"Don't talk back"

If she doesn't think too hard about it, Becca has enough gross motor control right now to reach out and hit her toy piano or knock over my glass of water (and subsequently get a huge smile on her face). But if I ask her to look directly at something, or to hit a specific button on purpose, that's a *lot* harder for Becca. Here's a video from Becca's second birthday where you can see just how much Becca wants to eat a cupcake, but her body just isn't doing the things it's supposed to to get it to her mouth -- though when she would play on the ground Becca was totally able to grab toys and bring them straight to her mouth.

Even in the couple weeks since the Rett conference I've been changing the way I ask Becca questions or try to get her to express herself and I feel like it's helped. I don't ask her to look directly at things, I let her know that things are there and that she has the option to look at them, but I don't demand action. I don't have hard data but it feels like she's making eye contact more this way than she did before.

It was also a really good reminder to me that when Becca is being "stubborn", it may be just her body that's being that way. Becca has been pretty sick the last few weeks and whenever she gets bad she gets a ton of gunk in the back of her throat. She'll cough it forward, but then she doesn't swallow or anything and so it settles right back where it was before. This drives me nuts, and I find myself saying "Becca, you need to swallow" over and over again until I start to get really frustrated. Meanwhile Becca just looks at me like "what do you want me to do?" Pretty sure that, at least to a degree, that's her apraxia kicking in in full force. I need to do a better job keeping apraxia in mind as a factor when Becca isn't doing what I want her to be doing. I still think she can just be stubborn at times -- just like any other kid -- but it's not always because of a defiant personality.

The comments I heard at the conference made it sounds like apraxia most often rears its head as a major problem in school settings -- which I guess makes sense. Girls with Rett get labelled with behavior problems because the teacher or therapist has seen the girl perform certain actions, but they refuse to perform then on demand. Then they fail tests and get labelled as not progressing.

I can see that becoming a problem for us. When I look at Becca's existing IEPs it's about performing a specific action on demand on multiple attempts throughout the year. Things like locking eye gaze on a specific target when requested. That's obviously not going to work, and honestly it hasn't in the past (Becca's "grades" have typically been around 60%). We're going to have to be careful the way we word IEPs going forward to make sure the assessments are happening in ways that will actually allow Becca to be successful.

Discouraged

I’m having an overwhelmed day.  How can a little girl that is this cute cause any kind of stress?….

 
Becca was a farmer for Halloween and her siblings were her farm animals.

Well, it started with a cold.  A cold that got passed through each of our children, but when it got to Becca it was quite a different experience for her.  Then, after a week of being sick she had a seizure, threw up, and aspirated (after she went to bed).  We were really close to taking her in to the hospital that night because her breathing was really labored.  I ran to the store to by a pulse ox while Brian woke her up to see if we could get her breathing better.  After a blessing from her Daddy we took turns sleeping next to her to make sure she was breathing ok.

The next day I took her in for a chest x-ray which showed a small amount of aspiration which they ended up diagnosing as aspirated pneumonia.  The next morning she had a fever and another episode of very labored breathing that almost landed her in the hospital.  Suctioning wasn’t helping.  It was scary.  I even packed a bag and called my mother-in-law to head over. Then she got a little better after we turned on Mickey Mouse.  We did this for the next couple days…

Notice suctioning behind Daddy, pulse ox in front along with oxygen which she was on 24/7 for the next week.  I’m so grateful that Brian was able to stay home most of the week to help.  I wouldn’t have been able to do it on my own (she would have ended up in the hospital).

It’s a scary thing to see your child struggle to clear their congestion…very scary.  It’s frustrating to sit there wishing I could cough for her and just hope that she’s getting enough oxygen.  The cheap ($50) pulse ox that we got wasn’t very accurate but we tried our best to monitor her O2 levels. 

 
Here she is after being on her strong antibiotic for a day.  It really made all the difference.

Now here’s the kicker.  After taking her in for a second chest x-ray we found out that it was very mild aspiration.  Her second x-ray was quite clear and the radiologist said it wasn’t clear pneumonia on the first x-ray.  Wow.  If this is what happens when she gets a mild virus with mild aspiration what will happen when she gets a bad virus with lots of fluid in her lungs.  Well, I know what would happen.  She would be in the hospital fighting for her life.  I hate Rett syndrome.  I mean hate.  I’ve never been one to hate, but I HATE Rett syndrome. Our sweet angel Becca is so fragile and I hate it. 

I’m a little teary today.  Becca has had a hard time being back at school.  A dumb cold has completely changed her school experience.  I get notes from school every day saying that she coughed a lot. No longer am I getting happy reports.  Her bus drivers are scared to drive her because they say she has so much congestion they’re worried she’s going aspirate at any moment.  I am super sensitive about Becca.  Today the bus driver said as we packed her on the bus that “he didn’t know if I would send her today because it’s swimming day and with all her congestion/mucous she has…”  I said as politely as I could muster, “Well, unfortunately this is her lot in life.  She’ll likely be this way for the rest of the winter.  I have no idea what to do.”  And then the afternoon bus driver had to bring her home first even though it was way out of order because she was “about to aspirate”.  It makes me feel like a horrible parent.  I know they’re just being nice and trying to help out but it feels like they’re wondering why I send her when she’s congested/coughing and at such a high risk to aspirate. 

Becca’s chest x-ray shows chronic aspiration.  I don’t know what to do about that.  Becca lost 2 pounds since March and 3 since last summer (she weighs 34lbs).  I don’t know what to do about that.  She hasn’t been to physical therapy in months and her joints are stiffening.  I sure feel like I’m failing.  I know that’s dramatic but it’s the way I feel today.  I’m going to go cuddle with my Becca now.  She makes me feel better, it’s that dang Rett syndrome that nags at me.

Rett Syndrome Research and the Boston Studies

I've been doing a lot of research over the last month on papers published relating to Rett Syndrome. There's been plenty of talk about reversing or lessening the symptoms of Rett, mainly because of a drug trial going on at the Boston Children's Hospital. I heard there was a study published a couple years ago talking about some promising results in mice that were given Rett Syndrome that lead up to the clinical trial, but it wasn't clear how promising the research was. I thought I'd heard that the hormone they were using was happened upon by accident, and they weren't even sure what its effects would be, so I decided to dig in and do a bunch of research of my own. This post is a place for me to collect my thoughts and summarize the findings I came across. Maybe it will be useful for someone else as well.

I started with what I understood was the Very Important Paper. I found a link to it on Wikipedia, so I knew it was a big deal :-). Actually there were a bunch of links to it, some of them related to the Boston study.

The Very Important Paper is titled Partial reversal of Rett Syndrome-like symptoms in MeCP2 mutant mice and was written in 2008. It starts by talking about how they've had a hard time finding exactly what things are downstream effects of the MeCP2 mutation that causes Rett, one that they have found is BDNF (brain-derived neurotrophic factor) regulation.

Before I keep going on that paper I had to switch to another paper, The Ups and Downs of BDNF in Rett Syndrome, since unlike the authors I didn't know anything about BDNF or how exactly Rett affects it (researching protip: "etiology" means the study of causation or origination). As explained in the second paper (something of a summary paper), the BDNF connection was first discovered in 2003. In something of a surprise, BDNF levels are actually lower than normal (this is the important part), in spite of the fact that the MeCP2 mutation causes BDNF repression to fail. In other words, MeCP2 normally helps keep BDNF levels in check, so it seems strange at first blush that an MeCP2 mutation would causes lower levels of BDNF instead of higher levels. It turns out that BDNF levels actually start out high for Rett mice and drop to lower levels some time after that. The paper never actually concludes why the levels drop, and I'm still digging for more studies on that, but at least I had what I needed on the relationship between BDNF and Rett.

Back to The Very Important Paper.

The paper continues by referencing another paper, The disease progression of Mecp2 mutant mice is affected by the level of BDNF expression, that talks about how giving mice extra BDNF fixes a bunch of Rett symptoms including "locomotor activity levels". Unfortunately, BDNF doesn't cross the blood-brain barrier well, so it's hard to create a treatment using BDNF.

However. IGF-1 (Insulin-like Growth Factor 1, which incidentally is used by body builders) is a hormone that has a lot of the same results as BDNF, and is much better at crossing the blood-brain barrier. The point of this study was to try using IGF-1 as a treatment for mice with Rett.

The results were very positive. IGF-1-treated mice have a longer lifespan than untreated Rett mice, their physical activity is almost back in line with "normal" (they called them "wild-type") mice, and their breathing variability (Rett causes a lot of breathing irregularities, especially when sleeping) and heart rate (there's lots of weird heart problems related to Rett, e.g. Long QT Syndrome) are also improved.

Next came the really interesting stuff. Rett mice typically have less-heavy brains than normal mice, but when they were given IGF-1, brain weight also increased. That's very promising for something that is clearly a neurological disorder.

Rett is known to result in immature synapses in the brain. For a long time it was assumed that this was permanent, and that there would be no way to improve synapse development for girls with Rett should a cure ever be found. People assumed if a treatment were ever found, it would have to be given *before* Rett presented in order to do much good. (here are two sources for this statement -- though it's one I didn't find until later in my research)

However, as part of this study, the team tested "cortical plasticity" of MeCP2 mice. Plastic-what? Here's the deal: when you're young, your brain is pretty flexible and adjusts to changes very quickly. This is called "plasticity". As you get older and your brain is more developed, it can't adjust as quickly. So to study this, the researches recorded the eye dominance for a bunch of normal and MeCP2 mice. Then they stitched one of their eyes shut. Young mice should be able to adapt quickly to a change like that and shift eye dominance to the open eye, and old mice wouldn't adjust very well. Since Rett causes immature synaptic development, an old MeCP2 mouse should be more like a normal young mouse, and should be able to adjust quickly to the change. That's exactly what happened -- except for the mice that were given IGF-1. IGF-1-injected Rett mice responded like the normal old mice, which suggests that IGF-1 helps to "mature" brain synapses to more like what they're supposed to be.

Remember, that's a restoration. The immature synapses that are seen in Rett cases can actually be addressed, which means treatment can help existing Rett cases, not just brand new cases. This sounded like big news to me.

It was at this point that I started checking more sources, and came across more research. I thought the Very Important Paper was where they concluded that Rett symptoms could be reversed without permanent damage, but there were actually two prior studies that had already shown that: Reversal of neurological defects in a mouse model of Rett syndrome (full text not available) and Partial rescue of MeCP2 deficiency by postnatal activation of MeCP2. Those two studies focused on actually restoring the MeCP2 deficiency itself. But getting exactly the right amount of MeCP2 is very difficult and potentially dangerous, which is why work is being done on things like BDNF and IGF-1, for example.

Anyway, this all led me back to the Boston study, which I now felt like I understood the reasoning for. I'll summarize according to my understanding now.

This is the new Rett dude at Boston Children's

Boston Children's Hospital's Rett Team is running a multi-phased clinical study of the effects of IGF-1 on girls with Rett Syndrome. The first phase of the study, why has already concluded, was focused on making sure there were no major negative side effects that came from giving girls the IGF-1 hormone. It was originally a 4-week study with 12 girls that, after it went well, was extended an extra 20 weeks in which time all the girls (even the control group from the 4-week study) were given IGF-1. I missed the webinar where they summarized the results, but it sounded like there were no major concerns. The only thing probably of note is that the study was limited to girls 12 and under, since there are concerns with regularly prescribing growth hormones to more matured individuals (but apparently a separate study for a different disorder is already investigating this, so they're waiting on the results of that study... I'll see if I can find a link and post it here).

Phase two of the study hasn't started quite yet (I think it got delayed after the Rett dude at Boston Children's ended up leaving for family issues), but should start relatively soon. I just got an email update on it via the private Rett mailing list, RettNet. The age limits were restricted to between 5 and 10 to try to get a more homogenous set. The goal with phase two is to actually examine and measure the results of regular IGF-1 intake on Rett symptoms (behavioral changes, motor impairments, seizures, breathing abnormalities, hand stereotypies, etc.). Once that's done, if it looks like it actually helps, they're hoping for a phase three, which would be a major stepping stone toward getting FDA approval of the drug for treatment of Rett.

Anyway, that's what I got out of the research I've done so far. I have more digging I want to do for sure.

I Never Knew Sleeping Could Be So Dangerous!

I have been meaning to do a post about Becca’s sleeping habits for a long time.  It has definitely been the subject of stress in this house. Brief recap: Back in the beginning of last year Becca had a sleep study that showed she has severe obstructive sleep apnea.  Her apnea is characterized as centralized sleep apnea which, at least in part, means that the part of her brain that is supposed to tell her to breath doesn’t function properly while she’s sleeping.  Pretty much her brain forgets to tell her to breath periodically throughout the whole night.  I know, rude! As a result (from both her apnea and obstruction), her oxygen levels dropped pretty low.  Anyway, since her sleep study results were not so good we decided to get her tonsils and adenoids removed to see if it would help.  That happened last June and turned into a month long stay at the hospital (see this post for details).  Since the surgery, Becca has had a great recovery and has been really healthy.  End of recap.
Oh and here’s a picture of how Becca has been sleeping since her first sleep study:

 
Since she won’t keep tubes in her nose we do blow by (high concentration of oxygen is blown into her tent by her face).  The side flap is usually down.

So, a few months ago we did a repeat sleep study to see if the tonsillectomy helped with her breathing/oxygen levels.  And the results came in….nope.  Not at all.  Her study was still just as bad.   The doctor told me that basically she’s having an apneatic/obstructive event about every two minutes through the night with her oxygen levels dropping low.  It was kind of sad considering all that we went through to get her tonsils/adenoids removed.   

The next step was to see what her oxygen levels are like while sleeping in her magical oxygen tent.  A few weeks ago we rented a pulse oximeter and tested Becca through the night.  And hooray! The oxygen does seem to be helping as her levels stayed good and high.  Whew.  That was a relief.  So after all that, we just keep doing what we have been doing.  But, I’m ok with that.  I'm just so relieved that the oxygen is helping and she doesn’t have to wear anything on her face. 
I’ve had times when I’m very worried about Becca and her unconventional sleeping habits.  As you may or may not know, many girls with Rett pass away in their sleep (without any warning and at any age).  Brian and I both have times where we go to check on Becca and we have a brief moment of sinking terror when we don’t see her chest rise right away.  At least it helps a little to know that the oxygen is helping!