#realliferett was born in a moment of deep frustration, a ‘screw you all, this is what I have to deal with’ kind of moment in the midst of yet another hurdle in the life that is Rett Syndrome.
I was lying next to Lily in her ‘sick bed’ that we set up in our bedroom for times such as this one, when Lily is unwell and we need to have her close, to hear her breathe, to comfort her, to make sure she gets through the night.
It was late afternoon and still in my pajamas, hair unwashed, black circles under my eyes, Lily crying in her bed beside me, I began scrolling through Facebook and Instagram to escape for a moment, see what the other half are doing. Every photo depicted a friend living a carefree, healthy, happy and footloose existence. I felt so alone in that room, in that moment.
It got me thinking. Why, as a community, do we feel the need to portray our lives as though they were perfect and without flaw? Why do I, more importantly? Why am I sparing my friends from the reality that is Rett Syndrome?
The answers are deep and probably universal for most of us as a general rule, but for me as a Rett parent, they are deeply ingrained in my psyche and they are things that are confronting and controversial to talk about, even to others in the same position.
That in itself is unusual because we have this amazing kinship as Rett parents. We get each other. We can talk about almost anything with the assurance that someone else, somewhere in the social media world of Rett Syndrome, will have the same question or the same thoughts.
But the things we don’t talk about to anyone, not even to each other are the feelings of shame and guilt associated with having a disabled child. Sometimes we feel brave enough to let down our guard and express our anger and resentment, but shame and guilt are taboo feelings in anyone’s world.
The guilt is probably easy to explain. It’s just very, very difficult to talk about. It’s a painful and raw place I don’t like to go to often. But the shame, for me, stems from a fear of being judged or my daughter being judged.
Sometimes, it is more than I can bear.
Call it ego if you will, unfortunately I do have a big one in spite of trying very hard to channel the principles of Eckhart Tolle, but mostly it comes from the despicable way in which our culture has come to treat people with disability as second class citizens. As less than, as somehow, having missed out on something the rest us were blessed with.
The judgement placed on not only people with disability, but the stigmatism attached to being a parent or a carer of child with disability is a hard mould to break.
It’s something that as a community, us Rett parents are trying very hard to break. We want the world to see how exceptionally beautiful our children are inside and out. We refuse to have our children be seen as one of those people you don’t want your typical child to stare at because they look different or because they dribble, or scream at the most inappropriate times, or because they make funny noises, or they invade your personal space and make you feel uncomfortable or they eat with their mouth open and spill their food all over themselves, or they fart and burp or even relieve themselves as they need to.
Society doesn’t want to ‘see’ that. Society wants everyone to be perfect and so, that is what we give them.
October is Rett Syndrome awareness month and it is a time when Rett Syndrome parents feel they can freely express their frustrations and challenges without being too much of a downer to all our friends and family, but the truth is, this is a bloody tough life this Rett life. Don’t get me wrong, I consider myself blessed and the luckiest mother in the world to have my precious girl. But it’s tough, both emotionally and physically.
The hashtag #realliferett gives us permission to be real anytime of the year, not just for October. To dispel the belief that we do this job with ease, that we were born for it. It allows us to give a real and true account of the confronting things that our daughters have to live with and what we as parents are challenged with every single day.
Being ‘real’ can be our contribution to dispelling the myths and relieving the discrimination somewhat. After all, each and every one of us is only a ‘moment gone wrong’ away from having a disabled child or being disabled ourselves.
Let’s be real and embrace all human beings. Let’s show the world not only the good, but the bad and the ugly as well!
We’ve created a set of Profilomatic frames for #realliferett, you can find them here.
It’s leading up to our second year of supporting Awareness Month as Army of Us and we’ve decided to do things a little a differently.
For many families who live with Rett syndrome, Awareness Month is an exhausting month-long reminder. A constant barrage of facts and statistics roll up your wall, as we all share images and stories about Rett syndrome, desperately trying to create awareness.
October has always been a tough month, tough to prepare for and tough to manage, with so much expectation and added responsibility for families; putting this kind of added pressure on you is just not what Army of Us is about.
This is where the idea of the Hope campaign comes from; we want to spread a positive message during October (to families and their networks of friends) that we have a lot to be hopeful for. This has been an incredibly successful year for advances in Rett syndrome and we believe things will only get better.
Rett takes so many things but we also have so much to be hopeful and grateful for.
We have known about the cause of the condition, and known about the potential reversibility for some time now, but that shouldn’t mean we stand still whilst we wait for a cure.
The past year has shown us personally and as a community why we can be hopeful and positive today.
Research discoveries about the mechanisms of long genes, and the progress with x-inactivation means potential treatments may be closer.
The orphan drug approval and results around Trofinitide mean we may be able to improve some symptoms now while we wait for a genetic fix.
These discoveries and other drugs in trial, like Copaxone and Ketamine, will help keep our loved ones affected by Rett stronger and healthier, aiding them to learn more skills and gain abilities right now.
The lower prices and greater acceptance of eye gaze and things like the Steve Gleason act in the USA are also very important breakthroughs. Eye gaze devices are changing the way people treat our girls (and boys), as we discover new ways to break down that communication barrier for Rett syndrome.
With all of this in mind, we’ve built a whole bunch of goodies that will help make your Awareness Month easier; we have redesigned our traditional “Rett fact cards”, which can be printed out and handed out at school. The Rett fact cards will be connected to our what is Rett syndrome blog.
We’ve also created a new group of cards; we call them “hope cards” and we hope you’re going to want to share them as much as we do.
Our hope cards contain the information that has brought us hope over the last year (and a bit longer), in a simple sharable format. Something we can share with our family and friends to say, hey, look what’s changing, we are beating this thing.
We have redesigned the posters and have added all the new girls, and we will keep adding more girls (and boys) through awareness month. So keep your eye out for them early next week.
The pictures we have been collecting for the last few months are filling the Army of Hope album and they have a very special purpose.
If your daughter (or son) is included in this album, please make sure you go to the album and “tag” her name in. It’s easy, click on her photo and click tag, then type her name, then leave a special message for her in the comments. Something as simple as I love you, or a few lines of your favourite poem.
If they’re not represented, please send us a picture so we can include them.
We promise you it will be worth it when October starts 😉
We’ve started a week early so you can use the stuff we make and be prepared for any of your Awareness month needs. Like printing our fact cards and posters for school or class to explain Rett syndrome. We want to make October easier for you, and we feel giving you access to our goodies a little earlier will do exactly that.
Profilomatic has been totally rebuilt and now works on mobile devices 🙂 You can upload your favourite picture from your phone, apply an Awareness Month frame, then share.
More hope or fact cards
If there are other hope cards (or fact cards) that you would like to see made (or translated) please give us a shout (firstname.lastname@example.org) and we will do our best to make it happen.
As well as our HOPE campaign we will be supporting other campaigns from our friends too; there will be a host of profilomatic campaigns like the #realiferett for #rettober campaign in Australia and #nomoreemptyarms here in the UK.
In August, my daughter turned three and it marked one year since she has been using a communication device, namely the Tobii. Today I was watching her and thinking about how far she’s come and also how far she has to go. It also got me thinking about how many gains she’s made in other types of communication.
When her diagnosis was confirmed, I remember feeling completely overwhelmed by the work I knew was ahead of me. I had wished that there was a manual telling me step by step what to do. Sadly there is no ‘How to Teach Your Daughter with Rett Syndrome to Communicate” book. However, after one year of piecing together opinions and advice from other parents, teachers, slps, cdas, communication experts, and from my own experience and intuition as a parent and teacher, I thought I’d pay it forward for the newly diagnosed parents out there wondering where to start.
Note: this blog post is simply a summary of what we’ve done- take it or leave it! I make NO claims that what we are doing is the ‘right’ way to do things- in fact, I often second guess myself and lose sleep wondering if I should be doing things differently, or if there is some other magical method out there to ensure she communicates effectively and will guarantee that she is fully literate later in life. Because, above all else, that is my number one goal for Ava- if she can read and write (and learn to use the Tobii proficiently), she will be able to communicate with no barriers. My friend Jo is proof positive that IT IS POSSIBLE. It’s up to us to figure out how to make that happen.
So. Here it goes…Learning to communicate with Rett Syndrome: what we did during the first year…
Engagement and interaction:
Engagement and interaction is key to any communication and a good starting point. And while this was officially a goal for Ava, the real work here happened on my end. When Ava was going through her regression, I went through a sort of regression of my own. Call it depression, sadness, despair… it affected MY ability to engage and interact with Ava. She checked out and in some ways, so did I. I learned quickly that I needed to get over myself. Engagement and interaction go both ways and they are largely reciprocal. We had to find what interested her (which in itself was a difficult task) and use that as a starting point.
This was particularly challenging as we began this work (unbeknownst to us) during the regression phase. I do think that developing her engagement in the world around her was key and allowed her to be open to learning the other methods of communication. We kept at it but also read her cues. It was important to push her to interact with us, but not push too hard that it would turn her off.
Goals: Making and maintaining eye contact, noticing and acknowledging new people entering the room, participating in an activity or conversation by actively looking.
Tools/Method: Highly animated voice and facial expressions, facial proximity and vocal volume during conversations and salutations, and highly motivating activities like bubble blowing.
Example: Ava responded quite well to bubbles. We would use the bubbles as a way to solicit eye contact. Using an animated voice and facial expressions we would say: “Ok- I’m going to blow the bubbles. 1, 2, 3, Blow!” We would do that several times. Then we would do the same script but take an audible breath and pause before the word “blow” and wait a few beats to see if she would look up.
As soon as she did (even if only for a split second), we would blow the bubble. She learned that the quicker she looked, the quicker she would get the bubbles. Again- this activity was done during her regression phase when she was 1.5 years old and we had no diagnosis. I’m sure there are people who would disagree with this method, but I really think it helped Ava relearn her social skills (and got her to smile and giggle again!)
Eye Pointing and Choice Making
Before I get jumped on, let me be clear about choice making! I do not think choice making should be the only mode of communication available to anyone. Period. However, it was particularly helpful in helping Ava understand that there was power in communicating (especially when she was under 2 years old), and frankly, it was helpful to know what she wanted!
I look at choice making as part of communicating. With my older, typically developing daughter, I give her choices all the time. Do you want strawberry jam or apricot jam? Go to the park or go for a bike ride. Choices happen all the time, and they are an important part of communicating.
Goals: make decisions for foods and activities.
Tools/Method: laminated pictures (we used actual photographs at first) of a variety of foods (fruits were a big motivator), activities including swing, stroller, toys and TV shows. At first, we took looking at the card as her indication, then moved on to looking at the card then at us for confirmation. This was a bit of a hit or miss so we transitioned to aural choices using non-verbal yes (see below).
Non-Verbal Yes/No (and low tech communication)
Developing Ava’s Yes/No response has been the single most useful communication tool this year. There are many different schools of thought on the method of achieving the ‘yes’ and I’d encourage everyone to read all of the information out there. Developing a clear yes makes things easier and opens up new possibilities for communication.
Many types of low tech communication programs depend on having a yes/no in order to implement partner assisted scanning (basically someone reads off categories/vocabulary, and the person indicates which word they would like to say by giving a yes response when they hear the one they want to say). Some types include Flipbooks (for communication, literacy/writing), customized vocabulary books/binders, and the PODD system.
Goals: consistently indicate a clear yes response. Lack of yes response= No
Tools/Method: Identify the natural yes (for us it was looking at the speaker), verbal prompt (“remember to look at me to say yes”), verbal affirmations (“good looking at me to say yes”), reinforcement and practice by playing games, and throughout everyday activities. An assortment of partner assisted scan low tech books for a variety of purposes (communication, storytelling, ABC flipbook…)
Example: Here is a video that shows the non-verbal yes (and no), basic partner assisted scanning, and the ABC flipbook (a.k.a. an alternative pencil, although in this case she is not using it for spelling but to select which letter video she wants to do next)
So off the bat, I will once again say that there are a variety of devices and programs available and the following is simply what we did.
My husband and I attended the RSO conference in Washington for the main purpose of learning about eyegaze communication. I signed up even before we had a diagnosis because I figured even if it wasn’t Rett, eyegaze was going to be a key player in her communication.
Listening to Judy LaRiviere and Susan Norwell was enlightening, especially in light of all of the literature I had read claiming girls with Rett had severe cognitive impairment. I saw videos of girls not just communicating but reading and spelling!
This got my teacher wheels spinning, and in some ways helped me cope with the grief of the diagnosis we had received two weeks prior. We purchased a Tobii Eyego for her second birthday without having trialed anything because it seemed that the majority of support available online (from other parents and from some of the communication experts) for Rett Syndrome were for Tobii devices.
We went this route because it was clear that getting a device at Ava’s age in Ontario was not a common occurrence (although the system is hopefully starting to improve). This was a bit of a reckless gamble on our part but we were lucky that Ava was able to use the Eyego.
I reached out to Susan Norwell and set up a trip to visit her in Chicago. She helped us set up the Eyego with my dad’s old laptop (using the free trial software of Communicator, Sono Primo, and Sono Flex) available on the Tobii website, and had some conversations with Ava (!!!) and away we went. One reason we follow Susan’s methods (aside from her extensive experience) is because of her unwavering belief in the abilities of girls with Rett. No matter who you choose to work with or what method you follow, find someone and/or be someone who believes in your daughter.
Some general comments here- it takes time and patience. It is a lot of work and sometimes I want to bang my head against a wall in frustration. I have to constantly remind myself of her age, and that just because she has access to hundreds of words, does not mean she is going to immediately start using all of them accurately.
Kate Ahern, another communication guru, often likens it to the way a baby learns to talk. We don’t stop talking to a baby because they don’t answer back. It takes time and lots of modeling.
Goals: to access and make selections on the screen, to understand that her eyegaze is controlling the screen, find an appropriate dwell time range, play independently, enjoy Tobii time, communicate with some consistency, use core words, and begin to make word combinations.
Tools: Tobii PCEyeGo (at first), Tobii i12, Sono Primo, SonoFlex, literacy videos, engaging activities, support from professionals and parents on various FB groups.
Note: If you have an i12, SonoPrimo and SonoFlex are already installed IN your device. Go to ‘readymades’ and you will find them under the Symbols tab.
Methods: We started Ava with a grid of 16 words using the Vocabulary pages in SonoPrimo. We gradually moved onto the 32 page vocab once it became clear she understood the concept. We used motivating activities and honoured every word she selected. For instance if we were playing with bubbles and she selected ‘big’, we blew big bubbles while constantly modeling.
If we were eating dinner and she said “drink”, we shoved the drink in her mouth so that she understood that when she selected a button, she was communicating. She caught on very quickly using this method.
Here is some early video of Ava using SonoPrimo after 1 month of using her Tobii:
SonoPrimo is fun because it has games and correlating vocabulary pages. We found (and still find!) that Ava does better doing the actual game in real life and interacting by having the vocab/context page open so she can communicate while the activity is happening- great for reinforcing the meaning of words and introducing new vocabulary. A great activity (you’ll notice a theme here!) is bubble blowing using the vocabulary 16 or 32 page.
When we transitioned over to SonoFlex, the program she now uses most of the time, it was overwhelming for Ava and for me. The difference between the two programs is that one is a ‘robust language system’ giving her access to all of her vocabulary (SonoFlex) vs theme-based which gives her access to some core words and theme vocab all on one page.
Though we do use SonoPrimo sometimes, the disadvantage is clear when she wants to change the subject or if what she really wants to say is not on the page. With the bubbles for instance, in SonoPrimo she can say lots of bubbles related vocabulary, but if she wants to ask for a drink or if I’m talking too loudly and she wants me to be quiet, she has no way of communicating that. Let me be clear though- SonoPrimo has its place. I loved SonoPrimo and there are days I wish we could just use that. SonoFlex is HARD and Ava is only 3. But she’s learning…
Model, model, model, and then….model some more.
There are some days I’m pretty sure I use the Tobii more than Ava, but the way I look at it, she’s not going to figure out how to use it if she doesn’t see others (including her family members) use it. At first it was daunting. I didn’t use the program for the first few months because I couldn’t figure out where anything was! The thing that helped me the most in that department was to take books and try to read them ONLY using SonoFlex.
I also had an imaginary conversation with myself one evening after everyone had gone to bed (and yes, there was wine involved!). Not only did this give me an idea of where everything was, but it also showed me how limited the program is. So if Ava doesn’t make perfect sentences, who can blame her?
A lot of people use the Pati (King-DeBaun)/Judy (LaRiviere) pages which are phrase based, meaning she doesn’t need to put words together- just select the phrase she wants to say. A lot of girls have success using them. I’m going to admit I’m not the greatest with exposing her to and modeling these pages- but it is on my list of goals this year. Because as Susan once told me, sometimes a kid wants to shout something quickly and navigating two buttons per word to create a sentence just won’t cut it. There- I wrote it down, so hopefully this will make me use it more.
The few times she has used it, she has used it well (I remember when one of her slps came over and was wondering if she would be able to navigate from program to program, Ava got out of SonoFlex, opened the P/J pages, requested a drink then promptly looked at the fridge!) The nice thing about these pages is that the pictures are pretty self-explanatory and the meaning, for the most part, is easily understood just by looking. I wish the Sono vocabularies would use those images instead of the Symbolstix.
When Ava started putting words together, we celebrated (and re-modelled some more!) everything she said, even if it didn’t make a whole lot of sense. ‘Presume Competence’ is a phrase that is tossed around quite a bit, especially by me! What does it mean practically speaking? If she selects a word or puts several words together, I assume she did it on purpose and that there was meaning attached to it.
I talk about her selection, remodel what she said, then muse out loud what it could mean. Sometimes she gets her message across and sometimes she doesn’t. Sometimes she wants to say things and other times she wants to explore, and sometimes it’s hard to differentiate which ‘mode’ she’s in. However, I don’t ever doubt that she’s learning and paying attention.
Here is an example of presuming competence. At the beginning of this video, I wasn’t paying too much attention, I admit, and was just kind of throwing ideas out there. Then this happened… Presume Competence.
Side note: We take a lot of videos of Ava using her Tobii not only to show our friends and family how well she’s doing but to document her progress once she starts school. Apraxia is a tricky thing and she doesn’t always want to perform, especially if she’s feeling pressured. I’m a skeptical person by nature, so seeing is believing and I want to ensure that everyone she is exposed to in the education system believes that she is smart and has lots to say.
This one is probably the most important thing. We read with Ava all the time and while she loves books, this wasn’t always the case. There was a long time where I doubted Ava was even listening to me (let alone looking) while reading books. We kept on reading, made sure we gave her lots of funny stories, and used our best acting skills to make the books exciting. She loves story time now. We do a lot of work with letters by showing her various videos and playing games with letters and letter sounds. This is a developing skill and we are trying to go about it the same way we did with our older daughter.
I can confidently say that she knows her ABCs and is starting to learn to identify beginning letter sounds. I am desperate for her to be fully literate as not only will it have a direct impact on her communication skills, it will also improve her quality of life and allow her to learn and study along with her peers. I can’t possibly write down everything we’ve done here, but one thing I found incredibly helpful was the Emergent Literacy course through Rett University.
Here is a video of Ava using her Tobii to comment while we read one of her favourite books:
A Final Note
This year of learning to communicate has been tough but also very rewarding. I want to also make clear that I haven’t been able to do all this alone- I have a lot of support from my family and some amazing therapists we have met along the way. There have been days (and there will continue to be days) where I doubt I can rise to this enormous challenge of teaching Ava to communicate and wonder how she will possibly be able to be fully communicative given all of the challenges that go along with Rett Syndrome.
I read and hear people talking about all girls with Rett being severely cognitively impaired, how they think the girls are not capable of communicating and it’s tough to keep on going, especially if we’re having a bad day. However, when I look at Ava and watch back some of her videos (another perk of documenting) I can see the progress she’s made. I can see how smart she is. And I know I owe it to her to try my best to ensure she reaches her full potential.
I would love to hear more about what other parents are doing to get their daughters communicating! Please leave a comment below or consider writing a guest blog post for Army of Us!
Useful vocabulary to learn:
AAC: Augmentative and Alternative Communication PAS: Partner Assisted Scan Core Words: High frequency words that are applicable to all topics. Eye Pointing: Looking with intent Eye Gaze Device: Computer that is controlled via the user’s eyes (the eye acts as a computer mouse) Dwell: The amount of time the eye needs to pause in order to make a selection Robust Language System: provides access to core words and a large variety of ‘fringe’ or theme words, and allows the user to create grammatically correct sentences Phrase-based: selections are phrases rather than individual words PODD: Pragmatic Organization Dynamic Display Modeling: Going through the motion, showing SLP: Speech Language Pathologist CDA: Communication Disorders Assistant Alternative Pencil: Providing a way to write not using a pencil (making selections in an abc flipbook, using an eyegaze keyboard…)
Technically, because Rett syndrome affects fewer than 1 in 2000 it is listed as a rare disease.
I’ve been making collages of people affected by Rett syndrome and although I have a load of girls (and boys) in these collages, it’s not even a micro-speck of the real numbers of people affected by Rett syndrome.
It’s important to me to [em]separate the girl from Rett syndrome[/em] but also to show that Rett syndrome affects so many people.
She is a unique and amazing individual, she doesn’t need a disease to define her as rare.
We often talk about Rett syndrome being a rare disease, but what does rare mean?
We have two stories we tell, we tell our friends and family what Rett syndrome is and how it affects our child, the child they know and love and have exposure to. Through social networks me meet and see other children, but for many, bar parents, contact remains limited.
The other story is getting people to understand that thousands, worldwide, are affected by this disorder.
Rett syndrome is bigger than people think and I think we do damage to our need by calling it rare.
By saying this thing is rare, we create the impressions that there are other more “common” things that need to be addressed before Rett syndrome. It’s how, as humans, we try to prioritise. If something affects more people, we “prioritise” and say lets go sort this more common occurrence out first.
Applying the rare label to Rett syndrome creates a priority in the minds of people who have no connection to Rett syndrome. I’m not calling them cold, I’m not calling them callous. Their priorities are built on the perception of need.
We need to find another way to prioritise their thought process, of how we can move a “rare” disease up the chain of priority.
We are making some headway in this regard with things like the Defense Appropriations Bill which will potentially have a huge impact on our Rett syndrome studies in future. The strength of funding will bring more doctors and researchers to the field of traumatic brain injury. I would love to see more effort made in aligning ourselves with studies like this.
Let’s talk about rare.
Let’s break Rett syndrome down, Rett syndrome is considered rare, when 17 children every day are born with it. Figures now estimate that 1 in 10 000 people are affected by Rett syndrome. I’ve extrapolated this before in my Rare Disease Day posts, but based on the statistics there could be around 700 000 people in the world that are affected by Rett syndrome.
You might think that’s small according to population statistics (I don’t think 700 000 people should be classified as rare), but let’s consider what makes up Rett syndrome. Among other symptoms, Rett syndrome includes :
Loss of speech (not rare)
Loss of motor control (not rare)
Scoliosis and fragile bones (not rare)
Seizures – including Rett episodes (not rare)
Compulsive hand movement (not rare)
Irregular breathing (not rare)
Apraxia or Dyspraxia (not rare)
Extreme anxiety (not rare)
Gastrointestinal issues (not rare)
Impaired circulatory and cardiac function (not rare)
Parkinsonian tremors (not rare)
Do you see what I’m getting at?
While Rett is considered a rare disorder, the symptoms of Rett are most definitely not.
1 in 26 people have epilepsy
3 in 100 have scoliosis
6.3 million people have Parkinson’s
I can’t even begin to get the numbers of gastro issues and irregular breathing.
That means that hope for Rett syndrome does not only come from our field of research, it comes from others too. While our scientists tackle a “cure” and also try to treat other symptoms, other areas of research will and are having an impact on Rett syndrome. Just as Rett syndrome will have an impact on other disorders and treatments.
8 years ago (in 2007) Sir Adrian Bird reversed Rett syndrome in the Lab. 16 years ago (in 1999) Huda Zoghbi discovered the gene responsible for Rett syndrome.
As a community we need to be smart, we need to be collective, working together towards the only meaningful goal, and that is progress.
With all this in mind, I’ve been working posters that people can use to explain Rett syndrome. They will be available for sharing on Facebook, but I’ve also been making sure they are available for you to download and take to your favourite printer.
I will make sure there is a version that has space for you to add your own information, to raise for your daughters needs, or your favourite charity. The choice is yours.
I will link the big download poster once I am totally sure I have no duplicated pictures and also once I’m happy that I have all the girls (and boys) included.
Please message me if I you sent me a picture and I haven’t included it.
Also, I can make more posters, so if you never saw the initial request and you want your daughter to be included. Send me her picture for followup posters.
Neuro-habilitation is a young field; its roots are found in the Neuroplasticity. We are constantly discovering more neuroplastic programmes that are having an effect on Traumatic Brain Injuries and also possibly on Neurodevelopment Disorders like Rett syndrome.
There are habilitation and rehabilitation programmes that will have an effect right now and in future.
Two techniques spring to mind, Feldenkrais and CME, both of which are used to reset neurology in patients, both of which have shown some incredible results in the field of Neuro-habilitation.
I’m going to blog about both of these in the next few weeks, as well as the importance of PT.
The eye gaze device has given girls with Rett syndrome a voice that they can control.
They get to intentionally choose what they want to say; it’s not interpreted by a parent or a carer.
Some girls with Rett syndrome have always been able to communicate, but it’s rare. Rett syndrome blocks so many communication avenues that it makes it practically impossible.
For the first time in Rett syndrome the playing field is being levelled. The eye gaze device is allowing more girls to access a voice, something that has long been denied to the vast majority of girls.
Getting your mind around the eye gaze device is hard.
The hard part is understanding what needs to be done to help your child communicate, and building the screens to do it. There are also time commitments and the stubborn need to stick to routine.
Yeah, you know this…so what’s the point?
I think the most important thing when I talk to families about Rett syndrome is honesty.
Honesty is very often lacking in our discourse in this community, not intentionally, often because the truth hurts and nobody likes to pile hurt on.
Although sometimes we might be too honest, but that’s another story.
So let’s talk about this.
My advice (which will be controversial)
Raise for an eye gaze device before anything else
Raise more than the cost of the device.
You are the gate keeper.
Don’t stop at low tech
Eye gaze device first
The first thing you should raise money for when you get a diagnosis of Rett syndrome is an eye gaze device.
It is one of the most expensive things you will be raising for.
For many people the first time you raise money will be the most you ever raise, by a considerable amount, if you give that money away to another cause (however worthy) you’re going to struggle for a long time to raise that amount again.
Don’t get suckered into distant promises, attend her needs now.
It’s more than just hardware.
When you get it, you will need to learn how to use it.
Make sure you add money to your raise amount which will cover local conferences and/or access SLT’s or organisations that can support you. There are also online courses that are designed to help you. Don’t be shy to do this. Otherwise your brand new eye gaze device is just an expensive paper weight that will generate a ton of guilt.
When someone tells you she is too young, she has to prove competence, blah blah blah, tell them get stuffed.
That’s like saying don’t buy your child books until they can prove they can read.
Quite frankly it’s bullshit. Don’t let people stand in your way to giving your child a better quality of life.
There is no such thing as too young.
Yes girls regress in Rett syndrome, but childhood is also the most vital stage of learning for children. Whilst she might be going through regression, or just have gone through regression, her brain is in a chemical state of hyper learning.
Don’t lose this vital opportunity to take advantage of those chemicals. You need to burn pathways in her brain and this is the best time to do it.
Treat the eye gaze device like you would learning a new language, the earlier you start the better.
That said, it’s proven that girls with Rett syndrome never stop learning. She is never too old to access these devices. The older girls already understand, so they’re going to pick it up faster than learning a new language.
You are the gate keeper.
The eye gaze device is not easy, it’s as big a learning curve for you as it is for her. You are going to have to invest time and make a commitment to training yourself.
Not just in how an eye gaze device works, but what are the best methods to use it. Then once you are trained, you are going to have to FORCE it into your routine.
You are the gate keeper to her potential.
I realise that a lot of parents have to live with the guilt of feeling like they have to be a super person, and I’m truly sorry that you are in this position. I get it, it’s hard. It’s turned your life upside down.
Some people are going to have a go at me about how difficult this is and how I don’t know what it’s like to live with the torments of Rett syndrome on a daily basis.
You’re right, I don’t, I have no idea what it’s like.
It’s easy for me to say that the changes you make now will make a fundamental difference to your life in future and more importantly hers.
Ultimately the responsibility for this decision lies with you, because you are the gate keeper. You choose whether to open the gates or not.
Don’t stop at low tech.
Low tech is incredibly useful to establish things like a clear “yes” response; it doesn’t run out batteries; it doesn’t explode when it gets wet. But low tech is also an interpreted voice.
It relies on someone interpreting intention.
Which is very different to her controlling what it is she wants to say (even if the boards on eye gaze device are augmented). *I’ll let Jo explain later 😉
If you truly want to give her a voice, then invest yourself in the needs of the eye gaze.
Alternatively you will find yourself settling for something that will ultimately be a source of frustration. Until the eye gaze, low tech was the only recourse, but now you have the ability to take this a step further.
Once again, don’t let people stand in your way to giving your child a better quality of life.
About eye gaze devices
There are a bunch of eye gaze devices available and people often ask which is the best device to get.
In my opinion the best device is the iSeries+ from Tobii Dynavox, there are various other devices. but you will always find yourself yearning for the iSeries.
The other models like the PCEye Explore will only ever prove competence (a pet hate of mine) and PCEye Go is a great supportive or mobile device. If I was a parent though, my end goal would be an iSeries.
It’s an expensive beast and it’s up to you whether you want to compromise on cost; the EyeGo is substantially cheaper.
Why do I hate the “prove competence” statement?
Because it’s essentially bullshit and an argument people use to block girls from accessing something that should be provided without question. Competency testing is a crutch for asshats to deny voices to people, under the guise of it looking like it’s a “considered process”.
When you watch girls expressing themselves on eye gaze devices on the internet, they are not lucky, they are not lead or pushed in that direction. These girls are responsible for their decisions on the eye gaze, they are in control of what they say.
The faster you learn to accept that statement the easier this process will be.
Simone (Jo’s Mom) says [highlight]“If I don’t believe in her, who will?”[/highlight]
Believe in her. Believe in her ability.
The importance of literacy.
David Koppenhaver spoke about the importance of literacy in a Webinar about AAC (Augmentative and Alternative communication) last year. How (AAC) users can never truly express themselves unless they have not only the ability to read but to write as well.
You can have 100 pages of symbols that you understand but if the words you want to express aren’t there you’re in trouble.
This is only the first step in enabling our ambitions for people who suffer with Rett syndrome, we have an end goal…literacy.
“Kind of a big deal”
I’m incredibly lucky to be friends with a very special young lady on Facebook, who uses this technology to voice her fabulous sense of humour and her incredibly astute observations from her perspective.
In a very short time Jo has forced me to question how I perceive Rett syndrome and how I treat people with Rett syndrome.
This is what Jo (who incidentally has Rett syndrome) has to say about AAC.
A lot of people have been asking me what my opinion is on having a device be phrased-based instead of selecting or typing out individual pictures or words. I believe there is a time and place for both.
My software allows me to save and memorize sentences and words as I type.
I decide what “phrases” are readily available for me to re-select.
This is no different than the auto predict on your phone, used to quickly type something.
A communication device is not intended for you to hear me say what you want to hear. It is meant for me to be able to able to express myself freely. Unless you are a mind reader, how do you know what I want to say? How can anyone predict every single thing someone wants to say?
If I only have the option of selecting pre-made phrases someone else has written then I am only independently choosing what you think should be said and not what I want to say. BIG difference!!
Having certain sentences close by to quickly say in a hurry – great. Being subjected to your opinions and not being able to express myself authentically – not cool.
Just my opinion….
Thank you Jo, your opinion is gold 😉
I’m sorry it’s taken so long for me to blog, as some of you know I’ve recently started a new job. I was meaning to write this blog a long time ago, but sometimes life happens 😉
Humans are designed to stand, walk and run; it is a primary function of our physiology and why we are built the way we are. Our ability to stand upright differentiates us from so many other animals; it also means it’s vital for our natural state of growth.
We need to stand because it:
Strengthens our bone density
Aids digestion, bowel and bladder function
Facilitates hip formation
Improves skin integrity
Strengthens our core
These 9 points all lead to improved wellbeing, alertness and sleeping patterns.
Whilst the simple act of standing does all these wonderful things, the upgrade is walking, but learn to stand before you walk.
Why is it necessary for us to keep people with Rett syndrome walking or trying to walk?
When I say walking, I include the therapeutic aids like treadmills, harnesses and the Upsee.
Walking is Therapy
There is a lot of convincing study happening around the world that is proving that walking is therapy for both the human body and mind. Conscious walking is being taught as a way to slow down the onset of Parkinson’s Disease.
These reasons alone are enough but there is more.
Walking is a superb conditioner for our brain, there is scientific evidence that walking improves concentration, learning-ability and abstract reasoning.
Walking is not a linear progression, the elements we use to learn to walk all overlap so it’s okay if it’s mixed. Walking requires balance, muscle strength, body & spatial awareness and most vitally the brain.
The first thing a child has to learn in order to walk is balance. Help your child master balance and then move onto gait. If balance is mastered the gait is easier because the balance networks are active and working.
Walking memory (The brain)
As we develop skills our brain creates synaptic pathways which facilitate the “memory” of a learned skill. Learning a skill is a linear process, where the synapses fire along a forming pathway. When the pathway is formed, the skill is learned.
It has been proven that when we don’t practice a learned skill after a period of time we will lose that skill. This is called learned nonuse, which is why it is so important to maintain the skill.
You can also lose a skill due to brain damage but it has been proven that the skill can be re-learned by using another undamaged part of the brain. It is the power of neuroplasticity that allows one part of the brain to do what another part was “designed to do”.
This process is called Neurorehabilitation. In Rett syndrome, quite often walking has not been learned, which is why the process of teaching this skill is called Neuro-habilitation – because it’s about teaching new abilities, not relearning abilities.
The way I understand this is to think about walking from my house to my best friends.
The first time I walk, I figure out how to get there, traversing possible obstacles and sometimes even backtracking to choose an unobstructed pathway. Once I have made it to the house, it’s a case of remembering how to do it again.
Over a period of time I begin to wear away a path. That path get’s wider and wider as I use it, until it’s completely defined. Walking to the house is now second nature and carved firmly into my memory.
What if we were to move to another house?
I would have to learn a new path. The knowledge of walking remains, but I have to find a new pathway to quickly and efficiently get to my goal.
The principles of how our brain learns to walk are explained especially well by Dr Steve Kaminsky here:
Four phases to building networks.
Phase 1. Unconscious incompetence.
Watch a baby move his/her arms, legs and head. They are moving them all the time and although they do not know what they are doing, their motor sensory networks are being set up.
These networks are the foundation of all movements that they will use later in life.
Phase 2. Conscious incompetence.
The baby or child knows what they want to do but are incompetent in achieving their goal. Reaching for their spoon, or trying to get the spoon to their mouth, or in the case of walking they can stand and take a few steps and fall.
The networks are being built and the individual has to really concentrate to make the action happen, but the networks are not completely formed.
Phase 3. Conscious competence.
The child knows what they want to do and they can do it but are actively thinking about every movement. The child walks across the room without falling.
The networks are all firing and they are being wired for the future.
Phase 4 Unconscious competence.
The child no longer thinks about walking, it is happening without thinking about it. They can change direction without thinking about it, they can do something else while walking.
The network is fully integrated into the central nervous system.
With Rett we have to work harder than normal because the available pathways are fewer in number. So our hill is steeper to climb because we have make those networks and continually fire them to make sure they stay wired. Yes, it is hard but it is why the physical therapy, occupational therapy and cognitive therapy are so important. These activities build the networks and keep them active.
It is important to remember, as with any child, failure is part of learning and there cannot be success without failure. Do not be afraid of failure because that makes the final success more meaningful when it finally happens, for you and your child.
Maintenance of therapies
These walking and standing therapies are incredibly important to the state of your child’s health, but I do know that it’s not as easy as this. Some of the girls develop twisted feet, or taunt tendons which make standing and walking impossible, even in braced or harnessed environments. Sometimes scoliosis or kyphosis will bend her so she finds it impossible to stand straight or without pain. The decision on how you tackle these symptoms is yours to make as a parent.
Physiotherapy and massage are important for muscle development and integrity. When you take your child to the therapists ask questions to understand what they are doing and why. Ask if it’s something you should be doing at home too.
You’ll be surprised how much the body can handle, as an ex-sportsman one of the things I used to (and still) hate was a sports massage, they are incredibly painful, torture in the hands of a master, but there was no denying how beneficial they are. Sports massages are deep muscle massages designed to release the fascia (fine sheath that covers your muscles) and encourage fresh blood to the muscles, to get rid of any toxins, like lactic acid.
I’m not saying rush out and start to torture your child (anyone who’s had a sports massage will know what I mean), I’m saying that it is surprising how hardy we can be.
Taking care of a child or person with Rett syndrome is already a full time job, I know how busy you are. I know that it’s a struggle to find the time to do all these things.
Try combine existing opportunities with these practices. Like give her time in the standing frame when she is watching TV and give her muscle massages while you’re bathing her.
If you have an Upsee, from FireFly, write a plan for walking practice. The Upsee is therapeutic training, not just an awesome way to enable your child.
Through intense physical therapy, occupational therapy, and cognitive therapy we may be able to form new networks that aid in Rett syndrome. If any of the drug therapies correct Rett biology, i.e. form more networks, then we have an active program to be ready to fire and wire those networks.
On the subject of walking
While I was writing this blog a friend of mine, Eryn Rae, shared a story by Shane Burcaw who suffers from Spinal Muscular Atrophy and is confined to a wheelchair. In the story, a young girl asks Shane “What is the first thing you would do if you could walk?”
He replies “Skateboard”.
Then he spends some time thinking about it later and after much consideration, he says “I’d walk to my kitchen and make myself a cup of coffee.”
I think sometimes we think about Rett syndrome we think about all the amazing things it takes away, the ability to skateboard, to surf, to ski. What Shane’s post brought home to me is that Rett syndrome often steals the ability to perform the simplest tasks, not the exciting tasks.
Rett syndrome steals lifes simple moments, as well as lifes precious seconds.
Rett syndrome education and how it’s helping us all.
We all believe in the potential of our children; we all hope that our child will achieve something meaningful in their lifetime. We also have different ways to measure success, and sometimes it’s incredibly hard not to pressure your drive for your child’s success onto their shoulders.
Children with Rett syndrome are no different. For a long time parents have been saying “You’re under-estimating her, your understanding of her intelligence is wrong.” And at last we are slowly getting the proof that we need.
Products like the eye gaze from Tobii are not only bridging the gap in communication between Rett syndrome and the world, they are also allowing us to more accurately prove intelligence. These devices are not the only thing making the difference; it is the belief of Educationalists and Speech and Language Therapists like Susan Norwell and Sally-Ann Garrett that are pushing the boundaries of our perception. As our learning and teaching techniques get better, it drives change in the base. The acceptance of that change is sometimes slower than anticipated or hoped, and we are left dealing with the legacy of old experience and beliefs.
Steve Kaminsky believes that we can’t measure the intelligence of people with Rett syndrome right now, because we haven’t developed a method to do it properly. So right now, anything anyone is telling you is based on limited knowledge and even more limited techniques.
This created a problem for any of the older parents and girls, who’ve not only had to fight the symptoms of Rett syndrome, but have also had to deal with the lack of faith in her intelligence.
The fact is, the more we get to crack the prison of Rett syndrome, the more ability we discover. We’ve also learned recently that Rett syndrome doesn’t stop people progressing, and that they keep learning after regression has passed. Focus on what we are learning, not on what we used to know.
This is something the internet can change, and not just the internet, organisations too. Which is why I want to talk about Rett Education UK and why it’s so important to our future as a community.
Education drives change; it drives change in belief and understanding. It creates new understanding, changes perception and sets new expectations. As a community, we fight to change the perception of Rett syndrome on a daily basis, but great education enables group change, and massive shifts in thinking.
Part of the problem we have in Rett syndrome right now is awareness based.
This is not just the awareness in general of Rett syndrome, but more importantly the conservative and stagnant knowledge built on old information from before a time when we could access just a little bit of what people with Rett syndrome are capable of.
Not only are we educating the world about Rett syndrome, but we are re-educating the people who should know.
I don’t blame these people for not having the latest information, because really the onus should actually be on us to change this. Rett Education UK is doing that; they are focussing on change at a professional and family level.
Let’s face it, very few people can access all the latest and greatest stuff out there. Staying on top of Rett syndrome is a full time job, so when an organisation like this comes forward and shares knowledge for “everyone”, then this is a good thing. Personally, I can’t thank them enough for it.
At a time when our professional pool is shrinking, we need to be taking the best possible care of the people who can help us.We need to be getting them the most current and the best information we can, to make sure our girls are getting the best possible treatment all the time.
The feedback I’ve had from the Birmingham Rett Education Event last year was incredibly positive and I’m thankful that an organisation, made up of people who are clearly passionate about this, have taken it on.
Rett Education Conference 2015
This years Rett Education conference shares the weekend with RettUK’s family weekend, the whole event is covered here, and I thoroughly recommend it.
The Rett Education conference is at the Hilton Hotel in Northampton during Friday 9th October, 9am to 5pm, with its focus on communication and education.
Tickets for the Rett Education UK day are available from their web site at a cost of £79. www.retteducation.com
Susan Norwell – inspirational and informative presentations on education and communication topics.
Dave Hewitt – importance of intensive interaction as a communication strategy
Hector Minto and Kim Elliot – will be explaining many aspects of eye gaze technology and describing its benefits
Sally Chan – funding for eye gaze equipment.
Shonette Bason-Wood – is an early years (EYFS) motivator and international educational trainer and motivator.
A rare disease is defined when it affects fewer than 1 in 2000 people – statistically speaking that means that around 3.5 million people could be afflicted by the same disease and it would be considered rare.
That is an awful lot of people.
Based on the extrapolation of statistics we have on Rett syndrome, as many as 700 thousand people could be affected by Rett syndrome, a rare disease…
Rett syndrome is awful; although it affects the entire body, it is primarily considered a brain disorder. Among many other symptoms, it affects the ability to walk, breathe and to communicate.
Rett syndrome symptoms include:
Loss of speech
Loss of motor control
Scoliosis and fragile bones
Seizures – including Rett episodes
Compulsive hand movement
Apraxia or Dyspraxia
Impaired circulatory and cardiac function
The last day of February every year is Rare Disease Day. Rare Disease Day is used to highlight Rare Diseases and create awareness in the general public and decision makers, about the impact a rare disease has on the lives of the affected.
We are slowly breaking down the wall created by Rett syndrome, but like many other Rare Diseases, we need you help.
You can help by spreading awareness for Rett syndrome and Rare Disease Day, you can even donate to any one of the excellent charities out there that support Rett syndrome.
Thank you so much for your efforts, we truly appreciate any help we can get.
We need to change, we need to change what people believe about Rett syndrome.
When Carys was diagnosed I went onto the internet and started to read, I now think that wasn’t a good idea. Those initial weeks of reading created a host of boundaries in my head of all the things I read she would never do.
Can’t use hands
Can’t breathe properly
It was a list of boundaries, boundaries I would start to apply to my God Daughter. At a subconscious level I stopped believing in her ability.
Carys, and other girls, have since corrected me, I learned very quickly that communication is a two way thing, and if I wasn’t going to listen, she wasn’t going to engage.
Now I do listen and she does engage, I’m not the best at understanding what Carys’ needs are, her Mum is, but that’s not Carys’ fault, it’s mine because I need to learn her language. There are tools that are changing this barrier in communication, I can’t wait for Carys to tell me to shut up because I talk too much.
I got corrected again this week, by a young lady called Jo. Jo used her eye gaze to make me see that even though many boundaries had been broken, I was still applying them by using words like “won’t write” and “can’t talk”.
I got the message Jo, from a young lady not confined by the boundaries the world of Rett syndrome has applied to her.
I’ll be doing my utmost to make sure I am not someone who applies boundaries to potential. I want to be a part of the change of how we, as a community, perceive Rett syndrome. That doesn’t mean I will stop being a realist, because Rett syndrome is a very real thing. It does mean that I won’t let Rett syndrome define boundaries, I will let her be what she can be.
I saw a picture of Ava this week, one of the girls responsible for changing my initial understanding of Rett syndrome. It was just beautiful, engaging, bright and so full of connection.
I looked at that picture and it pretty much described why this is so important to me, it is the picture I have shared with this blog.
One of the vehicles for our changing attitudes towards Rett syndrome has always been eyes, they’ve been described as having their own vocabulary. So what better way to show why we have to change than using the “windows to the soul”.
Deep in the soul of me you can find the whole of me unfettered from complexity free of my biology deep within the heart of me you can hear my voice straining to be set free
I believe in her, and hopefully, she will believe in me.
Army of Us is on a mission, we want to change what people read about Rett syndrome. We want to accurately reflect the stories of people affected by it, to make sure that people understand that what the read about Rett syndrome right now, is not their destiny. So that when others go onto the Internet, their journey is not decided before they’ve even taken the first step.
So we’ve started to surround ourselves with people on the same mission, we are building up a blogging network to talk about Rett syndrome and talk about the strides medicine, technology and study have been teaching us.
We are looking for people who want to share this mission with us.
Our first new blogger is my friend Jen, who along with her daughter Ava has already accomplished so much on this journey, her blog is inspiring: Angel Ava – Believe
When Ava was first diagnosed with Rett syndrome at 22 months, I had a lot of preconceived ideas about what she was going to be like. In the months leading up to the diagnosis, I had spent a lot of time conferring with Dr. Google who informed me over and over again about all of the things girls with Rett can’t do. They can’t talk. They can’t walk. They can’t play. They can’t use their hands. They can’t learn.
It really did a number on me and when she was finally diagnosed, it was a huge blow. To be clear though, the blow was not to her. As my husband and my parents kept telling me “Ava is still Ava.” Rather, the blow was to me and to my expectations of what her life (and mine) would be like.
Interestingly enough, immediately after that appointment with the neurologist we had an appointment with her amazing physiotherapist, Simona DeMarchi. As soon as we walked in, I blurted out that Ava had Rett and broke down in tears. She gave me a long, emotional hug and said: “Now we know and we’re going to keep working.” And that was that. She didn’t suddenly change her approach or make the exercises easier. She kept on expecting Ava to rise to the challenge. And rise to the challenge she has.
In and around the same time, I came across this video of another little girl with Rett, Cammy Can, using a Tobii to communicate and I was floored.
This was in stark contrast to the image I had conjured up when I read descriptions of Rett. My husband and I agreed that if there was the slightest chance Ava would be capable of communicating, we were going to make sure we gave her every opportunity to succeed. As a teacher and someone who speaks three languages, I was hell bent on getting her started as early as possible. We researched and figured out that we could bypass the stress of waiting to be approved for a device by purchasing the Tobii PCEyeGo.
We took a road trip to Chicago to see Susan Norwell, and it was life altering- not only for Ava but for me as well. At first, Ava was not too happy about the Tobii, but eventually she calmed down and started making selections. Then an amazing thing happened. Susan had a conversation with Ava. I remember saying to Susan, “surely she’s not intentionally selecting these buttons- she’s never used symbols, let alone these ones.” And Susan very firmly (and almost irately!) said: “Well how do you know she doesn’t mean what she’s saying? She can see the pictures and she’s smart!” From the moment Susan met Ava, she believed in her abilities. She expected her to communicate using the Tobii. And communicate she has.
It took one final group to kick me into high gear. I was lucky enough to virtually bump into Eryn online. We had both posted questions about the PCEyeGo in an online group, so I sent her a private message. Turns out she was also Canadian and worked closely with a 16 year old girl with Rett. We became facebook friends and shortly after, she posted something that changed my entire way of thinking:
“For all of you who have been asking how Jo has been doing with her new eye gaze communication device. Here’s the conversation Jo had with her mom this afternoon;
Jo: “I love you mom” “How’s it going?”
Simone <Jo’s mom> “I love you too Jo. I’m pretty good thanks, how about you?”
Jo: “My servant woke me up early again. I really think we should consider firing her!”
Well that pretty much sums it up. ROFL”
My jaw dropped reading this. Here was a girl with Rett clearly communicating more than just needs. I immediately stalked, I mean sent a friend request to her and to her mother, and a wonderful friendship has since ensued. Jo is incredibly smart, is a talented writer and is very well spoken. She advocates on a city youth council for underprivileged, underserved, and undervalued youth in society. She has great taste in music, enjoys skiing, and loves bobsledding (both for the sport and for the eye candy.) She has a wicked sense of humour. She is quite simply the most amazing 16 year old girl I have ever met, Rett Syndrome or not. I would like for both of my girls to aspire to be like her. (Well, maybe minus the potty mouth!)
I asked her mom how she did it. And her reply was simple- If you don’t believe in your kid, no one else will.
Those words have since become my mantra. And how true they are. I believe in Ava, and I expect her to work as hard as she can.
I am amazed by all she has accomplished in the 8 months since receiving her diagnosis. I’m not going to lie- it’s not all rainbows and unicorns. Rett Syndrome sucks. Sucks. I still have days where I am incredibly sad and angry, and would rather not get out of bed in the mornings. But those days are becoming fewer and farther in between. I’m trying my best to work as hard as I can to help Ava so that she CAN do all of the things her older sister does like talk (on her Tobii or with her eyes), walk (with some assistance), play (with a little help), and learn (with accommodations). Because she expects it.