A new girl

Its been a few hard weeks. And by that, I mean really, really hard. We had a meeting with Miss Z’s paediatrician to discuss Miss Z’s life limiting condition and where will want to draw the line on medical intervention. Miss Z’s new carer didn’t meet expectations and we let her go after only a week. And we’ve all been sick – even Vegemite, who is never unwell. And then, Miss Z – who initially appeared to have a gastro bug – got worse and ended up in hospital. It was a pretty scary week during which she became non-responsive even to pain, no one really knew what was making her so ill and at one point it was thought that she might have an infection of her brain.

Fortunately, after three days of IV antibiotics, she started to wake up and after seven days she was allowed to go home.

And then something wonderful happened. She REALLY woke up.

We’ve never seen Miss Z so awake and aware and interactive. She looks at you when you speak to her – really looks at you. She smiles – a lot. She intently watches what is going on around her. And maybe, just possibly, we have finally heard her laugh (at her sister, no surprise there).

And perhaps my favourite part of all – she now pouts when she is unhappy. It is  the cutest little pout I’ve ever seen. Not that she’s happy when I laugh at her instead of taking her unhappiness seriously.

How can you not laugh when you see this face?!

How can you not laugh when you see this face?!

We have no idea what has made her so much more responsive to the world around her. And we have no idea if it is going to last, or if she will eventually slide back into having good and bad days, but mostly just middling along. Already, I worry that she’s sleeping more and interacting less. But I’m not going to let myself worry about what is to come – Miss Z always surprises us on that count. Instead, we are all trying to sit back, take a deep breath, and enjoy the little girl that we have right now. Because she is amazing.

Determined not to wake up in the morning, no matter what.

Determined not to wake up in the morning, no matter what.

...and then she discovers that Doc McStuffins is on tv...

…and then she discovers that Doc McStuffins is on tv…

Feeling much better after watching 'the Doc'

Feeling much better after watching ‘the Doc’

Showing support

For the past month now, I’ve wanted to write a post about why building a children’s hospice in Queensland is so important to me, to Miss Z, to our family. But after a month of procrastination and not knowing how to even start, I have come to a realisation. I’m not ready to share those thoughts and feelings just yet. And maybe not ever. Miss Z’s diagnosis as being “life limited” is something that QB and I are still coming to terms with and aren’t ready to fully share with the outside world. It is all just too new and raw and scary.

But it is important to me to talk about children’s hospice. Because Vegemite and I have been raising money for Hummingbird House, which when it is completed, will be the first children’s hospice in Queensland and the third in the whole of Australia. Which is crazy, considering there are over 300,000 life-limited kids in Queensland alone.

Of course, I wouldn’t have even known any of this if it hadn’t been for our journey with Miss Z. I think a children’s hospice is the kind of place that you don’t realise the importance of, until you need it.

Several months ago now, Vegemite and I decided that we were going to run some races. Vegemite had done exceptionally well at cross country in school and was enthusiastic to keep running. And I have been trying to get back on the wagon – the running wagon, that is. I fell off around a year ago and have struggled to start running again – there has just been too much else going on.

I suggested to Vegemite that we could motivate ourselves by running to raise money for charity. We discussed the different charities we could support and I decided to let Vegemite choose. It didn’t take her long to choose Hummingbird House.

I think Hummingbird House appealed to her because in addition to the end-of-life services, the hospice will support families with medically complex children. It will provide breaks away for the children – or for the whole family. And Vegemite understands that. Plus, she was instantly fascinated by their plans to build an accessible treehouse (yes, a treehouse that can be used by children in wheelchairs) and began to plan other features that she thought they should consider.

I needed it, too. Because I needed to do something positive, if only to take my mind off all the intensely difficult and sad developments happening around here lately. Fundraising for Hummingbird House helped me to get out and exercise rather than hide in bed feeling sorry for myself and Miss Z.

To start with, we did the usual thing of setting up a fundraising page online and requesting donations via Facebook and email. Money started to trickle in, but since it was all online and the races were still several weeks away, Vegemite didn’t feel very involved.

Then I spoke to her teacher about fundraising at school and she was happy to get involved. Vegemite made a collection box and decorated it with pictures of hummingbirds. And she wrote and gave a short presentation to her class about fundraising for Hummingbird House. Her teacher included a request for donations in the class newsletter and the weekly school newsletter.

And the donations came flooding in, much to Vegemite’s delight. We ran our races – the first where it was a team effort over an obstacle course and the second where we ran different races, making it Vegemite’s first road race on her own.

Today was Vegemite’s school athletics carnival, so I ended up chatting with a lot of other parents. They all knew about Hummingbird House because everyone in Vegemite’s class was talking about it. And better still, parents were telling me how their children understood the importance of the hospice – and the importance of supporting Vegemite. Several actually donated their pocket money – their own personal money rather than just asking for their parents to give them money to donate. One girl scoured her house and car for spare change every morning. Everyone got behind Vegemite for the cause.

Her teacher also told me how happy she was with the class. They donated money knowing that they weren’t going to get anything back – just the knowledge that they had done a good deed.

I’m hugely proud of Vegemite. Not only did she rock her races (she had a great time at both) but she raised a lot of money for an important cause.  I’m also so happy that her class – and the whole school – supported her fundraising. I hope that if/when we have difficult days in the future, Vegemite knows that she did something great for her sister and other children like her. I also hope she remembers that her friends, classmates and teachers are there for her – something that I think is a big deal for a sibling of a child with special needs.

There is still time if you would like to support Vegemite’s efforts to raise money for Hummingbird House. You can donate online here: https://give.everydayhero.com/au/anya-and-sarah-run-for-hummingbird-house .

Team Z, aka the Wonder Girls, in our pink

Team Z, aka the Wonder Girls, in our pink “running tutus”

I wasn’t expecting that…

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Unimpressed with another visit to the hospital

Months ago now, Miss Z had an appointment with her neurologist. I love her neurologist – not only does she obviously care about Miss Z and actually take the time to have a discussion with me, but she is also a sincerely nice person. Plus, she recognised us in a shop one day – completely out of the neurology context and remembered both Miss Z’s name and mine, which reinforced my belief that Miss Z is more than just a patient number to her.

At the appointment, the issue of Z’s genetics came up again. We’ve taken a long break from trying to figure out the cause behind her issues and have instead embraced the clinical diagnosis of ‘atypical Rett Syndrome’. At first, we decided to take a break because Miss Z was in a good patch, and we just wanted to enjoy the good times and not spend more time with doctors. And then she hit a bad patch and we were too busy dealing with her health issues to think about doing anything.

The neurologist has always been a proponent of figuring out the genetic origins of Miss Z’s condition. So, when the conversation came up, I asked what the next steps would be, since after testing for Rett and CDKL5, it felt a bit like we’d come to a dead-end.  She mentioned exome and whole genome sequencing, but QB and I aren’t particularly keen as it is very expensive and we feel that the money could be better spent on therapy, equipment and activities that would directly improve Miss Z’s life.

We chatted some more and she suggested that she refer me to a paediatirican who specialised in – amongst other things – genetics. I’d heard of him on some of my Facebook forums, so agreed. Then I kind of forgot about it.

So, when our appointment came round yesterday, I wasn’t quite sure what it would cover. Initially, I’d suggested to QB that he take time off work to attend, and then later worried that perhaps I was making out to be more than it really was, so suggested that he not come.

I wasn’t actually at the top of my game for the appointment either. I was feeling a bit hot and sweaty since I’d over-dressed for what was a lovely, warm winter day in Brisbane – and the hospital was equally unprepared as they seemed to still have the heating on. I was distracted because I ran into the neurology fellow in the waiting room and was engrossed in discussing the pros and cons of putting Z on the ketogenic diet when we were called back to see the doctor. And Miss Z was in a less than cooperative mood – having been plucked out of school early for the appointment.

The appointment exceeded my expectations. He did a case history, and asked a lot of seemingly random questions about Miss Z. He asked to see photos of Vegemite and QB on my phone. He was fascinated by the fact Miss Z’s hair is striped – each individual hair has darker and lighter bands of colour. He examined her legs, which are quite furry and asked if I thought they had been like that from birth or occurred later. He commented on her eyebrows. He asked several questions about her osteopenia. His questions all seemed a bit random, but he was obviously considering and ruling things out as he went along.

We concluded the appointment with a plan. At Z’s next pamidronate infusion (when the line nurse is inserting the cannula), they will take some blood samples. The doctor thought there were a few tests that hadn’t been run yet that were fairly quick and easy. He also wants a hair sample to send to a specialist in hair pathology. And if none of that turns up an answer, he will aim to get Miss Z onto an upcoming medical trial that involves exome sequencing – and since it is a trial, that means the test will be free.

To be honest, I had given up ever having a diagnosis for Miss Z. Of course, even with our new plan, we may not get an answer. It feels good, however, to be back looking. After trying to make my peace with being undiagnosed, I wasn’t expecting that…

Everything is a-okay

I am one of those perpetual people-pleasers who never wants to disappoint anyone. Keep them all happy and they’ll like you.

I am also one of those people who doesn’t like to share my feelings. I don’t like to do it and I don’t even really know how to do it, even if I wanted to. Which I don’t.

And this all makes it very uncomfortable when I have bad news. I don’t like to tell people bad news. I learned this a long time ago, back when QB was diagnosed with leukaemia and had a bone marrow transplant. Keeping friends and family updated usually meant that I stuck to the facts – the treatment plan, what the doctors had said that day, how QB was feeling, that kind of thing. Always with a positive twist, a bright point to the day. Because after all, no one wants to hear anything bad. And I didn’t want to tell them anything bad. After all, what could they do if I did tell them just how much it sucked?

I was, rightfully, superfluous to the update. If anyone asked, I mentioned how busy it was balancing work and hospital or the different meals I was cooking to take into him every afternoon. If pressed about how I was feeling, I would say I was tired – and stress eating Malteaser ice cream bars.

But that is nothing compared to trying to share news about Miss Z.

Most of our family and friends are overseas. Do I send them an email? Message them on Facebook? Call and casually bring it up in conversation? Or let them find out by reading my blog?

Or not tell them at all? After all, there really isn’t any news to share – its not like anything has changed from last week or even last month.

How do you share bad news about your four-year-old daughter? My few attempts so far have more or less been total failures.

Although it probably isn’t nearly as bad as trying to tell people in person. I have the distinct feeling that several people wonder if I’ve lost the plot. After all, who remains positive and upbeat after telling you their child is unlikely to ever recover from her chronic respiratory problems and as a result is expected to be ‘life limited’. But that desire to please kicks in and I smile and mention all the good things – the communication revolution, success at school, an upcoming holiday – rather than lingering on the bad news.

One of Z’s therapists asked me today how I could still be so positive and I replied “denial”. After all, there is a healthy dose of that, too.

I keep thinking that maybe I am making something into more than it really is. Perhaps I misunderstood what her doctors have been telling me. And after all, no one really knows what is going to happen, so why dwell on the negative?

Except the negative is a big weight hanging around my neck, distracting me from everything else.

Which is ridiculous. Its not like QB and I haven’t suspected this could happen for a long time now. Children with complex medical issues like Miss Z don’t always go on to live long and healthy lives. We know this. Last year, after she had a status seizure and was nearly intubated because her sats dropped so low, QB and I acknowledged to each other that it could happen again that she might not come home on that occasion. So it isn’t like we were totally unaware.

But it is also like when we were first told by a doctor that Zoey had permanent “problems” that she wasn’t going to ever outgrow. We knew (or at least strongly suspected) that before we had taken her to the doctor – after all, that is why we had gone to see him, because we knew there was something wrong. But we were still devastated to have it confirmed by a doctor. So, we knew it was bad that Miss Z had been suffering from recurrent chest infections since December, but it doesn’t make it any easier to hear.

What feels hardest for me at the moment is feeling like the rug has been pulled from under my feet – again. I had come to accept that Z would always have special needs, that she would always need assistance, and that she would always live with us. I have tried my hardest to embrace that new scenario over the past four years and it no longer seemed scary or unpleasant – it was full of new opportunities. I was inspired by parents of other special needs children who emphasised if we could only do the right therapies the right number of times, Miss Z could sit, walk, communicate. I met parents of young adults with complex disabilities who were living happy, fulfilling lives – and equally importantly, so were their children.

Now, I’m not sure if that is where I fit any longer. Of course I’m not going to give up on the therapies or the hope of a bright future, but suddenly the medical side of things outweighs everything else. Whereas before it was about using therapy and determination to expand and improve Miss Z’s world, it is now about medically managing her from home in order to avoid hospital admissions.

But for now I’m trying to live in the moment and not worry too much about what might – or might not – happen in the next week, month, year, decade. So, if you ask me how I am and I say “fine” that is because it is. Right now, everything is okay. And here’s hoping it stays that way.

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The (communication) revolution will not be televised…

As I’ve mentioned several times, we are launching a communication revolution here. And I couldn’t be more excited.

And in light of all the bad news we’ve had lately, its good to be able to focus on something positive.

First, let me give you a little background into Miss Z’s communication and what we want to achieve with augmented and alternative communication (AAC).

Miss Z is non-verbal. She makes lots of vowel sound noises (mainly ‘aaaah’ and ‘ooowah’), but doesn’t articulate other sounds. This doesn’t mean she isn’t able to communicate – in fact, she can be surprisingly effective at getting her point across. As I regularly assure therapists, nurses and doctors, if she doesn’t like something, she will lose no time in telling you very clearly. A small few of us (who spend a lot of time with her) can also understand when she’s happy, very happy, bored, in pain or frustrated, mainly through a combination of the tone of her voice and her actions (arm waving and head shaking are happy signs, ear scratching and hair-pulling mean unhappy).

Miss Z also has very limited use of her hands. She can’t independently isolate one finger from the rest (for example pointing or giving a ‘thumbs up’) and can really only do big, gross motor movements. For this reason, sign language isn’t very successful for her. She is able to do a few basic signs and understands several more if she is doing body signing with a therapist (where they do the sign together), but it is never going to be her easiest or preferred mode of communication.

So, this is where our Communication Revolution begins. We need to find a system of communication that is right for Miss Z.

Fortunately, Miss Z was born at the right time. Even less than 10 years ago, there was very little out there to assist with communication, apart from some specially-made communication devices that were prohibitively expensive or home-made communication books (essentially a book with a bunch of words, pictures or the alphabet that a person would point to). Then the iPad came along and suddenly there was an affordable piece of technology that could assist communication – and a whole bunch of programmers writing apps to do just that. Of course, the iPad isn’t the only new means of communication, but it is a good place to start.

So, for her fourth birthday, Miss Z got an iPad and we are hoping that she will use it to communicate.

This is the start of a revolution that could change Miss Z’s life. And that is not in any way an exaggeration. Until now, she has been unable to communicate almost anything with the outside world. For the past four years, her main mode of communication has been crying when she is unhappy / uncomfortable / in pain / bored / frustrated. Essentially the level of communication of a baby. But she’s not a baby any more – she’s four years old – and it must be unspeakably frustrating to not be able to communicate even basic requests such as “I’m cold” or “I’m hungry”. And I know that she has a lot more to say than just that. I have no doubt she wants to tell her sister to move from in front of her when she’s watching cartoons. She wants to tell us she’s bored sitting in her beanbag and would rather roll on the floor or go for a walk.  She wants to say that her back brace is pinching or her nose itches. And she definitely wants to tell the doctors and nurses a few words that probably don’t appear on children’s communication books or devices…

By finding the best means for her to communicate, it will open up the whole world to her.

Here is what I’d like to be able to write:

In the two months since Miss Z got her iPad, an overnight revolution has occurred. We purchased an expensive and extensive AAC app that she swiftly mastered and has been communicating with us in increasingly complex ways ever since. It took her very little time to learn how to use the app and she now is better at it than we are. We take it with us everywhere and she is constantly using it to express herself. She has learned to isolate her pointer finger and make selections on the screen swiftly and easily. Using her iPad as a communication device is the best decision we’ve ever made. 

But here is what actually happened:

It is a slow process.

We bought the iPad. I set it up and downloaded Miss Z’s favourite songs and cause-and-effect apps on it. There were then several weeks where we used the iPad mainly to play music for Miss Z.

Then we had an appointment with her Speech Pathologist (speechie). He and I basically spent the hour playing around on the app store and debating the pros and cons of several different apps. There was one that would be ideal, but neither of us could get it to record words, and without sound, it was less motivating to Miss Z. We finally decided on an app that looked appropriate for Miss Z, called Go Talk,  and downloaded the (limited) free version of it.

I played around with it, adding some photos and words. And I played around with encouraging Miss Z to make selections from it. She wasn’t particularly interested.

Then we had another appointment with the speechie and he changed the set-up. On the free version, we only have five pages to use. The speechie prioritized things that will motivate Miss Z, so we selected four activities: bouncing, swinging, listening to music and watching tv.

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On the second page, we gave her the opportunity to refine some of her choices. So, if she was bouncing or swinging, she could ask to: go fast, go slow, have more of the activity, or stop.

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If she was listening to music, she could select which song she wanted to listen to – the app is great in that we were able to set up the music to play as soon as she touches the button.

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And if she wanted to watch television, she could select which programme she wanted to watch. At first we had to let her make a selection and then find it on the tv, but I worked out how to immediately play brief video clips when she makes a selection. Instant results are always best.

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The final page has her family (including her au pair!). Although we don’t use this page much at home at the moment, they discuss families at school, so I’m hopeful she will use it more there. In fact, I’ve provided school with a huge number of photos so they can set up some picture boards for her.

Our first time trying the app, we put Miss Z on the trampoline and encouraged her to choose how fast or slow she bounced. It was something of a challenge to position her so that she had an arm free to touch the iPad, but was still comfortable on the trampoline. There was a lot of encouragement, a bit of help, and a few moderate successes (I treat everything as intentional, even though sometimes she might be touching the screen accidentally).

Since she fractured her leg, the bouncing and swinging are out, so we’ve focused on getting her to choose music and tv shows. Sometimes she seems to choose something very intentionally. But most of the time, she either swipes randomly at the screen or ignores it completely.

We have also started trying to get her to make yes/no decisions through the use of cards and a different app on her iPad. “Do you want to listen to music, yes or no?” Again, we are usually lucky if she actually gives us an answer, but we just need to keep persevering. Even being able to communicate yes and no will give her a greater say in her life.

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Miss Z has dyspraxia, which means the messages from her brain aren’t transmitted properly to her body, affecting her ability to move. This also means it takes a considerable effort to strike the right square on her iPad. And it can take time. And sometimes her arm or hand needs support to help her to do it.

Today, we saw a faciliated communication specialist who spent a very, very long time waiting for Miss Z to get her muscles organised to be able to reach out and answer when he asked her a question. He helped her by holding her hand with her index finger extended, helping her to control her arm, but importantly leaving her to initiate if and where she points. Our communication revolution is not going to be rushed – it will arrive at the pace of a snail. Patience is a necessity.

The process of holding or touching a person so they can focus better to communicate is called facilitated communication. It is a somewhat controversial means of AAC, because the person doing the holding or touching, if not trained properly, can influence what is being said by directing the speaker to particular words. However, it can also help people to communicate who might not be able to do so on their own. If Miss Z ends up using an iPad to communicate, I think she will certainly need facilitated communication because her dyspraxia and her tendency to get distracted by repetitive actions mean communicating completely on her own would be very challenging – particularly at first.

But, it might also emerge that the iPad and facilitated communication isn’t the best communication tool for her. Another form of AAC is eye gaze technology, where the user looks at a picture or word, instead of pointing to it, and the computer tracks their eye movements. This technology has been made famous by Stephen Hawking, but in recent years (who has his eye gaze devices made specially for him by Intel) has become increasingly more accessible to everyone. So, the facilitated communication specialist we saw today is going to put us in touch with an eye gaze specialist, so that we can also give that a try – which quite frankly, would be awesome.

I have no idea at this point which approach will work better for Miss Z. So, the plan is to test out both systems, consult with specialists on both, make a decision, and then give it a go. In the end, we may also find that there is no ONE system that works best for Miss Z, but we will instead use a combination of different approaches.

So, the great communication revolution isn’t starting with a bang, but rather a long, slow consultation process, a lot of trial and error, and even more patience. But I am determined we will get there in the end – and excited about the process. The revolution may not be televised, but as it progresses, you can be assured there will be plenty of video clips on here to watch!

And in the meantime, have a look at how eye gaze technology works: