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 :-).

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.