Tuesday, 24 November 2015

Amazing... but bittersweet

The last couple of months have heralded a new start for Jess. One that should, hopefully, make such a massive difference to the quality of her life.

In October, I went on two courses. One was run by Rett Education UK, and the other (a two day course) was run by Rett University. Both courses were presented by the amazing Susan Norwell, who runs Rett University, and were focused on teaching our Rett girls to communicate, read and write.

There was so much information presented on these courses, and at times I felt very overwhelmed  - both at the steep learning curve ahead for me and Jess, and also the sense of responsibility of wanting to make sure we do the best for Jess, and give her every opportunity possible.

I so much want Jess to be able to communicate fully with us - to be able to say whatever it is she wants to say, whenever she wants to say it; and not be forced just to choose from two options. I want to get to know her properly. Being able to communicate fully like this would improve her quality of life - for her entire life - so much. Being able to tell us when she is in pain, who looks after her well, being able to communicate with ANYONE, not just us or those who know her well. The difference this would make to her life is immeasurable.

The courses really brought home to me was that Rett Syndrome is NOT a cognitive disorder, but a neurological, physical disorder. Rett girls are traditionally thought to have a severe learning disability, but with the advances in technology and the use of Eye Gaze computers, this assumption is being challenged. Many Rett girls, given the appropriate tools to communicate, are showing that actually they fully understand everything going on around them - they are just not able to express themselves easily. Susan had so many amazing videos of Rett girls and women communicating in a wide variety of ways - all in context and meaningfully, demonstrating that they had full understanding of the topics being discussed.

One of the factors that significantly affects Rett girl's ability to communicate is their apraxia. Apraxia is the difficulty or delay that they experience in completing a physical act on command. In simple terms it is the delay after the message leaves the brain, and then results in a physical action, even though the cognitive understanding and intention were there. This delay in a Rett girl responding to a question or command may mean their response is missed or misunderstood. It also means that people may assume that the Rett girl didn't understand the question or instruction, but this is not necessarily the case.

The other major take-away message from both courses was, no matter what form of communication your Rett girl is using - PODD books, Tobii, high or low tech - the key is in modelling it. Talk to them using their form of communication - in essence, learn to speak "symbol"! By constantly using their form of communication, they will see how to use it, as you are demonstrating it to them. They will be able to learn what lots of different symbols mean, and you will be able to really immerse yourself into the techniques required, and will see what works, what doesn't work, what symbols are missing and be able to amend as needed. However, this is much easier said than done, and this did made me feel very overwhelmed - how on earth are we going to achieve this?

There was so much information over the three days - far too much to include on the blog here - even if I had understood it all, which I didn't! So I will just put the links to Susan's two websites:

Focused Learning Solutions


Rett University

Rett University provides on-line courses for teaching Rett Girls literacy, reading and writing. If you are thinking of doing this, then definitely do look at these websites, if you haven't already. The guided Reading 1 course provides suggestions on how to adapt reading books and activities to teach the girls to read, which is something we are planning to do with Jess.

So, using the information from these days, we have started to use the flip chart books, sold through Rett University:

 Here is a link to Susan Norwell demonstrating how to use these books:

flip book demo

The first time I showed these to Jess, I showed her how to use them by writing "I love Jess". I wrote the "I" and the "love" using the core word book, and then spelt out "Jess" with the alphabet book. This is what she wrote back:

I was so touched - and amazed! She wrote "love" using the core word book, and spelled "mummy" with the alphabet book. It was such a lovely thing for her to say, it took my breath away and I was just amazed that she was able to communicate it.

Jess has used this system now several times:

This is what Jess wrote on the day her new hospital bed was delivered - she was very excited!!!

Anna is Jess's little sister.

The other amazing news is that Jess has received her first Eye Gaze device. An Eye Gaze Device is a computer that Jess can use by using her eyes to move the cursor, and click in the same way we would use a mouse. The device took over a year from being ordered to arriving (which is very frustrating). We opted for a device that could be mounted on to her wheelchair, so it will be able to always be available to her for communication:

This is the Eye Gaze Device mounted to her wheelchair

I have a few videos of her using the Eye Gaze Device at home. In the first video, she is playing a game where she has to throw snowballs at characters as they appear. In the second video, she has played a game where she has to make a spooky cake, and put creepy toppings on it. The video shows her re-telling what toppings she had put on the cake. This is a major part of being able to communicate - re-telling a story of something that has happened, so I was really impressed that she was able to do this.

This next video was actually taken at school by Jess's Speech Therapist. She is asking Jess what she thinks about the book they have just read, that is sat on her tray. However, I don't think the school knew that this is actually one of Jess's favourite books at home. In the video, Jess repeatedly says "cool" and "great", and then makes a sentence "I think it is cool". I am so proud of her.
 However, this sounds like we are steaming ahead, and everything is good. However, for every example given above, there are numerous times where Jess hasn't managed to communicate  - she's been ill, she's been too tired, she just hasn't been interested. Or I've been too tired, or Anna too grumpy to give Jess the time she needs with me to do it. Integrating all this into our already very full, overtired lives is definitely a challenge and it is going to take us a lot of time to get settled into a new routine with it all. BUT, this is Jess's future - teaching her these skills is the ONE way (bar The Cure for Rett Syndrome) that we can significantly enhance and improve Jess's life, so we have to do it. And we will. I just wish it wasn't so very difficult for us all.
 Through all this, one thing that has shone through, is despite all the medical issues Jess faces, the pain, the discomfort - my little girl is in there. She understands everything, which is amazing to know as it shows she has the skills to further develop her communications skills. But it is also very bittersweet - she is locked in, and everything is so very difficult and frustrating for her.
I wish her life could be easier.