Tuesday, December 17, 2013

Believing in Girls with Rett Syndrome

When we first got a diagnosis of Rett Syndrome for Becca we did a lot of Internet searches trying to figure out what we were up against. Paula found a blog called Spirit Dances about a woman with Rett Syndrome named Karly. The blog is authored by Karly as her mother held her hand and helped her type on a keyboard. The posts are complete sentences, complete paragraphs, with a very large and diverse vocabulary.

When I saw the blog I discounted it right away. I'd read about how debilitating Rett Syndrome is, and there was no way this disabled person was writing all that. Maybe she was picking the first letter and her and her mom was filling in the rest -- maybe not even intentionally or anything, but there's lots of research examples of facilitators who in trying to help end up saying things from their own head rather than the individual's. I didn't say any of this in a comment on the blog or anything, I kept it to myself, but I definitely thought it.

I didn't know Karly (she has since passed away) so I'm really in no place to make a judgment call either way, but I've since learned why my attitude is wrong-headed -- mostly by being on the receiving end of some healthy skepticism about Becca's abilities.

Becca is reading words. She only knows 14 letters, but that's enough that she's independently read the words "bat", "bag", "fat", "leg" and "hen". Last week we were trying to come up with Christmas words for all of the letters she knows, and she thought all on her own of "elf" for "e". The next day she asked me how to spell "lion". She out of nowhere last month told us that she hates her car seat, and she told me a few weeks ago that I make her shower water too hot, then helped me find a better temperature. She can count to five and she has just started saying her own prayers at night when she goes to bed.
Hunting for a Christmas tree we all could agree on

If you've seen Becca in real life then those events will probably sound next to impossible. Becca can't talk or sit up or walk, she shakes constantly, she struggles to move her arms intentionally anywhere other than to her mouth and struggles to keep eye contact focused on much of anything. Most people would look at someone in her condition and declare those skills as far out of reach. And if we told you how we know she can do all of those things (because she told us she can) then you'd probably put us squarely in the same bucket I put Karly and her parents in before. I can't really judge you for thinking that way since I sometimes still do myself, but I can try to help you see why you (and I) should rethink assumptions.

I'm going to introduce two probably-new-to-you concepts. The first is called "the least dangerous assumption" and the second is called "presumed competence". They both have to do with the benefit of the doubt, and hopefully will help you see why it's so important for individuals with disabilities.

The least dangerous assumption is a perspective re-think. It's essentially the request to take your assumptions about a person's capabilities and think beyond to the end result of those assumptions. Let's say for example that twenty years from now someone comes up with a drug that completely cures all symptoms of Rett Syndrome. Which would be worse, hearing Becca say "I laid on the floor for twenty years watching baby cartoons. I tried to learn to read but nobody would hold the books still enough" or hearing her say "I know you tried to help me, and I did get a little out of it, but most of the time it was too hard to focus and I didn't learn much"? Henry Frost is a young man with autism who fought this battle, using typing to convince the school that he was capable of learning. The least dangerous assumption is the assumption about an individual's abilities that will cause the least amount of damage if in the end you find out you were wrong.

Presumed competence is related. It's the idea that an individual shouldn't need to earn the right to be taught, respected or spoken to. You should assume that an individual understands what you're teaching them or saying to them or about them, whether they can respond or not. Don't skip explaining things to Becca just because she looks like she's not listening. Don't skip her in activity time with the kids because she's disabled. When Becca responds with what seems like only half or a quarter of what you were told is her typical response, you should take it and believe that it was intentional. Take it the rest of the way. Call it a victory, rather than thinking "that could have been an accident". Presume competence. Don't ask her to prove to you that she has a brain in her head, that's not how you treat "normal" people. If a "normal" kid says "appow" instead of "apple" you don't say "well *that* kid can't talk." I won't lie to you, presuming competence takes a lot more work than you probably think. It involves getting over your own insecurities in addition to the child's, and re-assessing yourself again and again.

Honestly presuming competence has made all the difference in the world with Becca. Once we started introducing concepts and ideas to Becca that were beyond where everyone (including us) assumed she was, she took off. Her yes/no eye gaze didn't get really solid until she started picking from six choices of places to go (backyard, front yard, downstairs, etc.) -- and I'm betting that at least the first few times she didn't completely understand what she was picking. But we took it and ran with it and then went a few steps farther. We didn't know for sure if she'd keep up but she has, again and again. I love the phrase I found a few weeks ago, "we teach intentionality by assuming intentionality." Just as often we we find we're not teaching intentionality so much as finally trusting her enough that she feels she can rely on us for communication. Whenever we feel like she's getting stuck it's usually not because of her understanding, it's because we got sucked back into "you need to prove you know this before we move on" again, and once we fix our side she picks right back up. Will that last forever? We have no idea. But we've heard too many horror stories of girls with Rett in high school who are still working on identifying colors, and we just have to think there's something more stimulating for them than that.

Check me out, I can rock the wink like no other.
Becca's mutation (K135E) is not a common Rett mutation, so I don't know that we can really speak for a majority of girls with Rett Syndrome, but from those we've seen Becca is not on the "light" end of the Rett spectrum, and she is still so very much "in there". We have to believe that most girls with Rett are as much "in there" as Becca. I still wonder about Karly's typing skills (my bad, I know. Why do I worry so much about being "taken in" by an online exaggeration?), but I now believe she was capable of writing a blog, that she could read and understand and benefited from her method of communication. I think it's crucial that we surround our girls with people who believe in them, because I've seen how much of a difference it can make.

If you don't believe Becca can do the things we claim she can do, we understand exactly where you're coming from but please give our daughter the benefit of the doubt. If you want a girl with Rett Syndrome to see you as part of her life then get excited for her, believe that she did it even when there's not the evidence most people would expect. Someday in the future you will see how she has changed you as much as you have changed her, and that making the least dangerous assumption has brought you into a circle of trust that will change your life in significant ways -- regardless of whether anyone else in the world believes you or not.

Monday, November 25, 2013

Rett Syndrome, Long-Term Memory and Neuren Pharmaceuticals' NNZ-2566

Over the last couple months I keep getting sucked back into the question of how Rett affects long-term memory. First I saw this write-up talking about how adult women with Rett seem to have better memory for events and objects from their early childhood. Then a few weeks later I was looking up long-term memory for an unrelated project and came across mentions of our good friend BDNF. I dug in a little then, but it's coming up again now as I'm working with Becca on learning her letters, and I wanted to better understand her learning process.

BDNF
So! Here's the somewhat limited (but still long) results of my foray into the relationship between Rett Syndrome and long-term memory.

Quick refresher from my previous post on BDNF, brain-derived neurotrophic factor is a protein and gene that have various effects, mostly on the brain. I believe it is also the first identified protein that is "downstream" of MECP2 (Rett). When you compensate for a BDNF deficiency in a mouse with Rett, their heart begins to function properly, their energy and strength improve -- many of the main symptoms of Rett are lessened significantly.

Then what about BDNF and long-term memory? Long-term memory is (I believe) defined as anything that's remembered for more than around eight hours. That includes childhood memories, lessons from school last year, and what you learned yesterday in class. The process of long-term-memorization seems to be getting something into short-term memory, then transitioning it to long-term memory and strengthening its placement in long-term memory over time. BDNF does a lot for the brain and is deficient in Rett Syndrome, so that's a great starting point for research.

 The first article I found was a winner, "BDNF is essential to promote persistence of long-term memory storage". The study shows two things, 1: BDNF is necessary for long-term memory persistence as part of a process that happens at some point after the original learning experience, and 2: even when other components of the long-term persistence process are missing ("hippocampal protein synthesis" is apparently the other part of the process), BDNF alone is enough to persist the long-term memory -- "in an ERK-dependent manner".

Cue the vocab lesson :-). Gathering these terms was no small feat, the neuroscientists of the world do not like to share their language with us peons.

Protein synthesis - the creation of proteins by cells. Hippocampal protein synthesis is the creation of proteins by brain cells, and is necessary for the brain to create connections. Apparently even if you suppress protein synthesis in the brain, a sufficient supply of BDNF is enough to allow for long-term memory. BDNF pretty much rocks.

Kinase - a kinase is an enzyme that allows for energy transfer. If I understand this correctly, a kinase is essentially a pathway through which actions can occur.

ERK - extra signal-related kinase. This is a specific kinase that is "activated" or used by, among other things, BDNF and growth factors (IGF-1, anyone?). This study was focused on spine growth, but showed that BDNF most definitely leverages the ERK "path" for its purposes.

CREB - CREB is a transcription factor (a protein) that helps regulate the transcription of certain DNA pieces. In other words, when a cell uses its DNA to send a message via RNA to create certain proteins,  needs transcription factors like CREB in order to send the correct message and have the protein created correctly. CREB regulates the transcription of a number of genes including BDNF.

Thanks for sticking with me, here's a picture.
CREB != crab.

PKMζ - PKMζ is another kinase. It appears that this kinase is necessary for the maintenance of long-term memory. Without it existing long-term memories seem to break down.

Akt/mTOR - Akt and mTOR are two kinases that I see lumped together a lot in the papers I'm looking at. The Neuren study implies they are a joint pathway that is inhibited by Rett Syndrome (more on that in a minute). 

IL-6 - this is a protein that is somewhat similar to BDNF and IGF-1. It does a bunch of cool stuff. I honestly haven't dug into it much, but the Neuren study suggests that their new drug may help via the IL-6 route in addition to the IGF-1 route. The only other paper I could find mentioning Rett and IL-6 wasn't directly related (but is wicked interesting and I'm going to dig into it later for sure).

Sorry, that was rough, but hopefully it'll help if you decide to dig in to any of the linked papers. I can proudly say I understand at least 30% of the Neuren presentation now :-). Anyway, back to our research. 

BDNF can facilitate long-term memory via ERK. In fact, another study said BDNF is a requirement for ERK activation (and also that CREB is necessary for correct ERK activation). There also appears to be a time element involved. In yet another study Alonso and company found that injecting rats with BDNF blockers prevented learned fear reactions -- but only some of the time. When BDNF was blocked 15 minutes before, or 1 or 4 hours after the training then the rats didn't developer the learned reaction, but when blocked at training time or 6 hours after, it had no effect. There were a few other studies that talked about time-critical moments in the long-term memory process. If there were a way to temporarily increase BDNF it seems like it could be done at a strategic time in order to improve retention when teaching new topics.

So is there anything to be done? Not sure. The whole "BDNF is necessary and also sufficient" thing is kind of a downer. I found some articles on how dopamine (2) and PKMζ increases can enhance long-term memory, but if BDNF is a must-have then I don't see how useful that is for Becca. Maybe it would help some even if it doesn't remove the main barrier, I don't know. I did also find some articles on memory strategies, the most promising of which is to try to encourage connections between existing long-term memories when introducing a new idea ("B" is for "bus", you ride the school bus every day to school), which can essentially make it more likely the idea will be pulled along into long-term memory (though somewhat paradoxically, novelty encourages attentiveness which improves retention as well). I'm trying to offer more variety while I teach Becca, pulling in both familiar and novel concepts, to hopefully do what I can to help her with retention.

Obviously long-term memory isn't completely nonexistent since Becca recognizes people from the past, she remembers things she doesn't like (her car seat) and things she does like (Wall-E, chocolate). She seems to recognize places she's been before. But when I work with her on school topics, it feels like she has a harder time making things stick between sessions. That's what this study says, too, that LTP exists in a weakened way in individuals with Rett Syndrome.

Oh right, LTP is another vocab word. Long-term potentiation is the biological process that most likely equates to long-term memory. They strongly correlate, anyway. As I look at my old notes, I remembered there were Rett studies mentioning plasticity, so I did a quick check on the relationship between plasticity and LTP. Apparently LTP is one type of plasticity, so if your plasticity is messed up then your long-term memory is probably messed up too. Like with Rett Syndrome.

Ok, so we're basically hosed when it comes to long-term memory, we do what we can but it's a very uphill battle. Lots of studies showing poor plasticity caused by Rett Syndrome. But remember, the IGF-1-as-a-replacement-for-low-BDNF studies on mice with Rett showed significantly improved plasticity (which should also mean improved long-term memory).

In fact, that's one of the main areas of study for Neuren Pharmaceutical's research drug, NNZ-2566. NNZ-2566, they claim, is an "analogue" of IGF-1 that can be taken orally but can still cross the blood-brain barrier, which is what needs to happen if it's going to have a positive impact on brain function.

Neuren has performed studies on mice where they "knocked out" or disabled FMR1, which is the equivalent of giving someone Fragile X. Fragile X is obviously not the same thing as Rett Syndrome (they may even be on opposite ends of the spectrum), but there are both genetic disorders with neurologic impact and do have some similar characteristics. Not sure I'm reading things right, but it seems like Fragile X *might* have the same problem of too little BDNF in the brain... The relationship between Fragile X and BDNF (search results) doesn't seem as cut-and-dry to me as with Rett, but I'm having a harder time understanding those results so don't read too much into that.

As far as the Neuren study goes, the Fragile X mice showed poor long- and short-term memory in different mazes when compared to wild-type mice. When placed in a simple maze 10 minutes and 24 hours after an initial exposure, the Fragile X mice were exploring the maze anew while the wild-type mice more quickly re-settled into their environment. In a different maze with essentially tall cliffs, the wild-type mice spent significantly less time on the anxiety-inducing cliffs than did the Fragile X mice. When given NNZ-2566 the Fragile X mice performed basically the same as the wild-type mice in both mazes.

Only one of these things has a head
full of fluff.  Also, stripes are cuter
than polka-dots.
So that's potentially very promising. It says to me that NNZ-2566 as an IGF-1 alternative may be a sufficient supplement for a BDNF deficiency as far as memory is concerned. We know from the other study I linked to before that IGF-1 positively affects brain weight and plasticity of Rett mice, so maybe that's enough of a correlation to be hopeful for Rett in addition to Fragile X. 

At any rate Neuren is working on Phase II of a clinical trial for NNZ-2566 as a treatment for Rett Syndrome. They are looking for adolescent and adult subjects (if I remember correctly, they're targeting older subjects because IGF-1 is a *growth* hormone and they don't want any confounding factors from younger subjects who are still growing), though if the drug is effective they will obviously work for approval for younger cases as well. They claim that in pre-clinical models of TBI, Fragile X and Rett there was a "normalization of Akt and ERK activation profiles" (you know what that means now, sort of!). Hopefully the clinical trials will back up their previous research.

What do the rest of us do in the mean time? Be patient is all I can suggest :-). It's been a tricky balance as I've been working with Becca on "school time" because she seems to remember enough to get annoyed if I'm too repetitive or spend too many days on the same subject, but it's also clear there are holes in her understanding that I need to fill before I can go too much farther. It does help to know there's the potential for learning, and that I'm not crazy in thinking she's getting it albeit sometimes at a slower pace. Even with all of her constraints she still surprises us quite often with what she knows or remembers, we just have to believe in her and keep remembering to ask.

Wednesday, November 20, 2013

School Time Update (Becca read a word!) - 11/20

Whew, it's been another month already. Lots of after-school adventures to recap. I was out a couple weeks for some work trips and things so it wasn't quite every day.

We've mostly been working on letters for the last month. Some of the non-letters work we've done was on matching baby animals to grown-up animals. Becca thought that was fun. ...actually that's the only non-letters thing I can remember doing. I need to be better about getting more non-letters things mixed in, it's just that letters is taking a lot of time and review.

Basically we introduce a new letter every couple days. I hold up a uppercase and lowercase version of the letter and we talk about it, draw the shapes, make the letter sound, and think of words that start with that sound. I always have at least one picture that starts with the letter, where it's the picture and also the word typed out with the first letter highlighted. We're trying to work on reading with her inner voice, so I prompt Becca a lot to say the letters in her head or make the sounds in her head.

When we review old letters she seems to do a pretty good job of saying whether she recognizes the letters. That is, she says she knows B and A and their sounds really well, but she struggles remembering D, and the fact that C makes a "kuh" sound is hard for her to remember too. The newer letters are still pretty hard to recognize. We've played a sound matching game a couple times, where we stick pictures on her board and then I ask her to find the picture that starts with the letter I hold up. A couple times on B and S she's done it without any additional prompting, but usually she needs to hear the sound the letter makes, and then she can find a picture that starts with the sound.

She pretty consistently (eighty percent? I haven't been counting) can find a correct picture (sometimes there would be more than one) by looking at the picture and then back at me. She does tend to look around at all the pictures, and sometimes she gets caught on one even if it's not the right sound, but with some additional prompting she'll usually keep looking. When she wouldn't engage well I would then scan through the pictures with her, saying the picture out loud and then asking if it had the right sound. There were only a few times we had to get to that point, but then we ended up at the right answer. Probably twice she selected the wrong picture before scanning, and she either changed her mind when I tried to confirm, or when she confirmed the wrong pictured and I said that didn't match she laughed. I'm pretty sure at this point she likes to tease.

We've also been starting to spell out words. I cut out each of our letters we've been working on with a thick edge at the bottom so I can hold the letters together more easily. Then I hold them up for Becca and we try to figure out the word. First we go through each letter and say what the letter is and what sound it makes. Then we make each sound one by one, faster and faster until we get the word. It's really cute to see her "get" the words, it's a very clear reaction where her eyes get a little wider and she looks up at me (when she's well-engaged). Sometimes it's not until I've fully combined the letters, but sometimes it's sooner than that. Once we figure out the word we celebrate.

A couple times when she reacted before the word was fully combined I'd ask if she thought she knew the word already. If she said yes then I'd say let's try and find the picture. I'd hold up different pictures and ask if they matched the word. The three times we did this she correctly picked the right picture.

One time was particularly awesome, I held up b-a-t and she reacted before I'd pointed out any of the letters or anything. I asked if she thought she knew the word and she said yes. I held up a cat and asked if that was the word, and she didn't say yes. Then I held up a sun and again she didn't say yes. I held up a picture of a bat and asked and she said yes. We celebrated a lot on that one :-). Becca read a word!

So far we've worked on the following letters: B, A, S, T, C, D, E, N, U and G.

Thursday, October 24, 2013

School Time Update - 10/24

I think you all get the idea now on what we're doing with Becca, and I have the evidence I wanted in case I need it for school, so I'll chill out again now. Over the past two weeks we've done a bunch of different things with Becca. We worked on matching animal babies with their parents, picking items from a list, some more letter work, and pushing vs. pulling. Each time she answered our questions correctly at least 75% of the time.

A highlight for me was when we put 12 pictures on a board and had Becca select pictures from the grid using partner-assisted scanning. Paula worked with her on it first, just asking simple questions and finding Becca's answer. It was more freeform than we've been able to be with her before because there were so many different choices (some food, some equipment, some TV shows, some locations). The second day was right after a trip to grandma's with lots of driving.

I asked Becca to tell me what she wanted to talk about. She picked her carseat. I asked if she liked her carseat. Immediate no. I asked if she wanted a different carseat. Immediate no. I asked if she wanted something else instead of a carseat, I joked about putting a bed in the car. Immediate yes. She looked at me like, "that's an option?!" I think she has so much she's going to start saying as soon as she has a better opportunity to.

On the letters I noticed Becca has a hard time engaging for a long time, probably because it feels so repetitive. I ask her a lot to say words or sounds in her head (a la the nonverbal reading approach) and it's not exactly fast-paced. I try to explain to her why we're working on it, and that does help some. Also I've noticed when I get Becca's sister involved and have her practice, Becca does better as well. For example, we worked on matching pictures to the words describing those pictures (candy vs. sit vs. books, working on starting sounds). Becca made it through and matched each picture to the word based on the starting sounds with only one mistake that she corrected on her own, but the last one was hard, we had to play a hand game first to help her re-engage. Then when I asked Becca's sister to go through the same matching exercise I noticed Becca would look very attentively at the right answers, even though it wasn't her turn. I need to remember this trick. I know I wouldn't like being put on the spot all the time, in school I definitely preferred answering on my own with some other kid in the hot seat.

Anyway, lots more to learn, but that's the latest update.

Friday, October 11, 2013

School Time 10/11 - Describing Family

Yes to school time. I told Becca I wanted to use the same family pictures but use them to describe our family members, and then we would talk a little about some animals and describe how they look too. She said yes, that sounded good. Becca had a hard time with the session today, she was very tired and had lots of coughing, both of which made it hard for her to engage and for me to be sure I was seeing her eye choices correctly.

I got out the board with our pictures still on the left side (her left). Then I pulled out four pictures: a boy, a girl, a big person and a little person. Each time I would hold them up and describe them to Becca. Then I attached them to the right side of the board and said I wanted us to try describing the people in our family.

I went first and pointed to myself. I said daddy (I pointed to my picture) is a boy (I pointed to the boy) and is big (I pointed to the big picture). I went over what the pictures on the right stood for one more time, then I let Becca have a turn.

She looked at Becca first, and I said great, let's do Becca. What can you tell me about Becca? She kept looking at the people side, but not long enough to say any of them were choices.  Then she had a coughing fit so we had to wait. After that I asked if Becca was a boy or a girl. She looked around, but on the second prompt she picked girl. Then without prompting she picked big, not little. I said that was right, Becca was a very big girl.

Next we let Becca's sister go. She picked herself, and said she was a big girl, touching each picture as she went. I repeated that a couple times.

Next I held it up for Becca to go again. She picked her brother this time. I asked if he was big or little, and she said big after a short delay. I asked if he was a boy or a girl and she was slow to answer. Actually I think she was slow to answer that question every time. She wasn't super engaged and she might have been a little annoyed and thought it was obvious question, not sure. She did eventually pick boy.

I tried to model again how we can use picking different pictures to make a sentence. I did Becca's brother again, showing that we could say he was a big boy by selecting those pictures one by one.

I held the board up again for Becca and asked if she wanted to talk about anyone else. She picked mommy this time. I asked if she wanted to say anything about mommy and she quickly chose big. I asked if she wanted to say anything else about mommy. She had another coughing fit, and after getting re-settled again she picked boy. I laughed and said that was silly, mommy is a girl, not a boy. I asked if she was trying to trick me. She looked away, not sure what that meant. I modeled saying mommy is a big girl again.

I asked if Becca wanted to talk about anyone else, but she wouldn't look at the board anymore. After that we looked at a couple pictures of animals but only because I'd promised to. I told Becca we'd work more on the animals next time, but that she seemed very tired and we should probably be done for now.

School Time 10/10 - Becca Says

School time was a go. I told Becca we were going to play a game called Becca says, where she gets to pick a person in our family and have them perform an activity. I showed Becca a picture of each member of our family, then attached all five to the left (her left) side of the plastic communication board. I let her look over them and she looked around at all the pictures in a circle a couple times, then back at me. Then I showed her three pictures, one of a person dancing, one of a person singing, and one of a person jumping. She would look at the picture and then look at me, and I'd say what each of those pictures meant. Then I attached all three actions to the right side of the board.

Then I explained again that we were going to take turns picking a person, and then picking an action for them to do. I asked her to first pick a person and she looked quickly and for a long time at mommy. Then I suggested she pick an activity for mom to do. Her eyes would dart quickly back and forth between the people and the activities, and I wouldn't count it unless she sustained gaze, but she darted back and forth quickly a lot for this activity ("Mommy jump daddy sing brother sing sister dance" in like three seconds). After some prompting she looked long at the "jump" picture so I called out to mommy and asked if she would come jump for us. She came over and jumped up and down. Becca got a big smile on her face.

We took turns letting Becca and her sister pick (and her brother occasionally when he was interested). Becca chose difference people and different actions clearly. She would spend extra time looking at the people side, and sometimes it seemed like she wanted to change her person choice, but I wouldn't let her, I'd tell her that we'd already chosen a person and now we needed to pick an activity. After some more prompting she would pick an activity.

After the first time Becca chose to have her brother sing a song (which he did, with a lot of help), daddy dance, Becca sing a song (we sang one together), mommy jump again, brother dance, mommy sing a song, and maybe a couple other choices. Becca's sister would also choose some, though the only one I need to bring up is when she had daddy jump. Becca got a really big smile on her face and the next time it was her turn she had daddy jump again. One time in there I also picked and had Becca dance, and we danced together. She liked that, but she didn't like "jumping" in her chair when her sister picked that, it stressed her out a little bit.

Wednesday, October 9, 2013

School Time 10/9 - Plus One Again

I told Becca I wanted to work on plus one again, she said yes to school time working on plus one. Huzzah!

We got out the plastic board and did the same approach as last time, "__+1=___" on the bottom, with three options on the top. I first went through 1-4 in order. I'd fill in the first number ("1+1=___") and then ask Becca what came next ("one plus one equals... what?"). I'd read off her three options at the top, mixed up each time. If she didn't answer right away then I would talk about how plus-one is like counting, we try to figure out what number comes next. Then I'd count up to the number and stop right before saying the next number (i.e. "four plus one, that's one, two, three, four...").

For 1+1 she immediately looked at 2 (even before me pointing out the choices) and back at me. I moved 2 to the right spot on the board and we celebrated (I would wobble the plastic board so it made a funny noise and sort of fanned her face, and I'd sing the number to the tune of the Braves' war chant, "twoo-ooooo... twooo-ooooo"). This made Becca smile every time.

For 2+1 after I paused, she didn't pick. I then showed her the choices, and asked again. She started right at 3 for a long time. I said that was right, moved 3 to the answer spot, and we celebrated.

Becca's sister was a little jealous
For 3+1 after I paused she didn't pick. I showed her the choices and asked again. Still no choice. Then I did the counting thing. After that she looked at 4 and back at me.

For 4+1 she immediately picked 5 after I showed her the choices.

Next I said I wanted to review some of them and asked if that was ok. She said yes. We started with 2+1 again. She wouldn't pick, even after me showing her options and counting up to 2. She kept looking away instead of at the numbers. It could have been because her little brother started saying "tree" after my "one, two..." every time. Or it could have been she was annoyed that I'd already asked. Either way, I eventually said it was three and we drew a 3 in the air with her hand. I asked if she could find the 3 and she looked at it and back at me. Celebration song again.

After that I asked Becca if she understood why I was asking some of the same questions again. She wouldn't say yes, so I explained that for math we have to answer the same questions more than once so we can learn to answer it very quickly. After we practice enough then it gets easier to answer questions fast, and then we can use that to answer even harder questions. (Side note: I have no idea how to teach someone with no motor control how to add two-digit numbers. How do you teach something like carrying? Just tell them to keep it all in their head? Something like 4+5 is going to be hard enough..)

She's going to be so bored in school..
Next I did 1+1 again, and she rocked it with a smile on her face, no prompting other than to ask "what is 1+1". Next I said I had a hard one, 4+1. Again she picked the correct answer without needing them to be listed. Celebration again. I told Becca she was really smart and I was impressed she was getting better at her plus ones.