Loss of purposeful hand use

In the spirit of Rett Syndrome awareness month, I'll be posting current videos of Caroline dealing with the various aspects of the disorder. Some skills have improved while others have further deteriorated. But she's still the same determined little girl!

A hallmark of Rett Syndrome is the repetitive hand movements and loss of purposeful hand use. I've posted many times about Caroline's loss of hand skills (to see previous posts, please click on the "Hands" link below this post). Caroline uses an arm brace on her right arm to keep her hands away from her mouth and promote intentional hand use by disrupting involuntary movements.

Caroline's purposeful hand use has improved a bit over the last year. These days, Caroline can hold a small light object in her left hand for up to 2 seconds. She can also reach out and touch our faces. She attempts to give us hugs by "patting" our backs when we hold her. She can "swat" at some of her musical cause/effect toys. I'm grateful for these improvements, but I can't deny the devastating impact of the loss of this skill. Caroline can't explore the world on her own. She can't point to objects or images to communicate with us. She has to rely on us for help with basic skills like eating, dressing, toileting, and even playing with her dolls. But we do our best to "be Caroline's hands" for her.

We are her hands and she is our heart.

Caroline's involuntary hand movements are constant while she's awake

Caroline playing with one of her favorite toys

Finding a communication device for Caroline

Unlocking Caroline's ability to express herself is a priority. The loss of Caroline's voice has been one of the biggest sources of sadness and frustration for us. After a long and stressful search, we are finally working with a speech and language pathologist (SLP) who's conducting an augmentative and alternative communication (AAC) evaluation to explore an eye gaze communication system for Caroline.

Caroline is smart. Recent research shows that the cognitive ability of girls and women with Rett Syndrome has been underestimated for a long time. Nobody has ever doubted Caroline's ability to understand what she sees and hears. It depresses me that she has no way to communicate with us. She can't tell us if something hurts or if she's thirsty. She can't tell us if she wants to listen to music or sit in another part of the house. She is unable to control her arms (let alone her hands and fingers) so she can't point to objects or pictures to express basic needs. All we have to guide us are her vocalizations, facial expressions, and eye gaze.

We are still modeling the PODD system for communication, but it may take some time before Caroline is able to use it expressively. The good news is that she seems very interested and focused when we use it. She just can't say anything back to us yet. Our hope is to incorporate the PODD system into whatever eye gaze technology Caroline ends up using.

Based on initial AAC assessments, the speech and language pathologist determined that Caroline is the perfect candidate for an eye gaze computer system. Before we can start "trialing" the different devices, we have to work on getting Caroline to more effectively use her eye gaze to communicate her needs and to make choices. During today's appointment, the SLP successfully engaged Caroline in an exercise to get her to look at an exact image of a toy she enjoyed to request that specific toy (pictured below). This seemingly simple exercise was incredibly challenging for Caroline. Her whole body squirmed as she struggled to concentrate on focusing her gaze on the picture of the toy.

After the appointment, I drove straight to the craft store to get materials to make our own eye gaze board. When I got home, I took pictures of five objects representing Caroline's favorite activities. Our goal over the next couple of weeks is to reinforce the cause/effect of looking at a picture to express a need.

Everybody has something they daydream about every single day. I dream that I will someday hear Caroline's "voice."

Caroline looking at a picture of the toy to "request" it

Caroline enjoying the toy

Materials for Caroline's eye gaze board (Mike's project this week!) 

Pictures I took of objects representing Caroline's favorite activities (the Sesame Street one is an actual snapshot of the TV screen while Caroline was watching the Silly Songs DVD!)

A visit to Clemyjontri Park

Caroline met Santi for a little playdate at Clemyjontri Park this morning. We love this park because it is tailored for children of ALL abilities! The park even has a swing that allows children with special needs to use it without having to transfer from their wheelchairs. How awesome is that?

Caroline was able to enjoy the park like the rest of the kids there! She tried the swings, the carousel, the slides, and other fun equipment. I even got her to bear weight on her legs without any complaints. She was clearly distracted by all the fun she was having.  

Caroline and Santi were so cute together. Santi is just such a smart and sensitive little boy. I love their sweet friendship.

Breath holding

This morning, Caroline had several scary episodes of breath holding.

Caroline has experienced some abnormal breathing episodes for a while, but those patterns were so subtle that only a Rett specialist noticed them. The incident this morning was a first. Caroline held her breath for longer periods and with more intensity. She had a panicked and scared look on her face each time it happened. I tried to get her breathe again by blowing air on her face and kissing her cheeks. It worried me that she was unable to quickly "snap out of it" on her own. Mike confirmed he's observed Caroline holding her breath with more frequency lately.

I turned to my Rett mom friends for advice. I was surprised to hear how common it is for Rett girls (especially the younger ones) to hold their breath. Some girls turn blue and even faint from doing it, though they often eventually learn to overcome it.

Caroline's condition has been relatively stable these last few months. I think we got too comfortable. I am well-aware of all the sad possibilities and progression of Rett Syndrome, but I can't live thinking about what may or may not come next. I can only use that information to make the best of what I have today.

On a positive note, it's obvious to us that Caroline loves her new school! I still can't get over how happy and willing she was to get on the school bus by herself and start this new experience. She's so open, trusting, and brave.

We stopped by the playground during our long walk today. 

First official day of school!

Caroline had a wonderful first day of school!! She came home with such a big smile. When I asked her how her day was, she giggled and vocalized in delight. I so wish I could understand what she was trying to tell me. Her teacher confirmed that Caroline had a great time!

Caroline has always thrived in a class-based setting. She LOVES learning and being around children her age. She also easily adapts to new environments. Our main concern was the thought of putting her on a school bus at the tender age of two, but the bus driver and attendants were so incredibly gentle and kind (and Caroline seemed to love the experience of riding the bus!). Mike and I are excited for Caroline as she starts this first school year. We feel that we really lucked out with the school team. 

The day was almost overshadowed by an incident that happened when Caroline was picked up by the school bus. What was already an emotional event was made worse by an angry lady who repeatedly honked her horn behind the bus while Caroline was being safely secured inside. She even attempted to drive around it a few times, but realized she didn't have enough room. I walked up to her car and said "my child is in a wheelchair" but she refused to acknowledge me. I could not comprehend how someone could be so cruel, especially towards a disabled child. I'm trying to put this incident behind and hope that it does not happen again.

Regardless, I took pictures and videos to share!

Caroline waiting for the school bus

 Video of Caroline being lifted onto the bus for the first time. A bittersweet moment.

Caroline coming back from school! (look at that smile!)

 

 Caroline had an awesome first day of preschool. She was so happy! 

UPDATE: Caroline's thoughtful teacher (pictured below) sent us pictures that made our day! 

 

 

 

 

Unimaginable loss

Many tears have been shed for the loss of two sweet souls.

Today, Anna lost her battle with Rett Syndrome. A couple of weeks ago, I was stunned to learn that Karly passed away.

My heart hurts so much for their families.