The trial

fullsizeoutput_3674As I mentioned earlier this year, Miss Z was chosen for a trial of Epidiolex, a medical cannabis oil.  Queensland is currently running a trial of Epidiolex for children with treatment-resistant epilepsy (in other words, seizures that aren’t able to be controlled by other means).

Epidiolex is a pure plant-derived cannabidiol (CBD). It doesn’t contain THC, which is the compound in cannabis that makes you high. It is produced by a small US-based pharmaceutical company. It comes as an oil, which we give through Miss Z’s gastrostomy tube.

Although cannabis for medical use is technically legal in Australia, it can be difficult to obtain and prohibitively expensive.  The medical trial only takes a very small number of children – and as you can imagine, the demand is high.  So, we are incredibly grateful that Miss Z was accepted.

I’ve tried to approach the trial with an open mind.  Medical cannabis is not a cure. Nor is it a sure thing to stop her seizures.  It is another drug that has the potential to reduce her seizures, and in doing so, help improve her cognition and awareness.

It was a long process to be accepted on the trial.  First, we were referred by Miss Z’s regular neurologist. Then, I met with the neurologist who is running the trial and he examined Miss Z.  Then our case was put before a panel of doctors and medical ethicists who have been tasked with selecting candidates for the trial.

Once Miss Z was approved by the panel, we had another appointment with the neurologist and the nurse running the trial to go through all the paperwork. Then Miss Z had blood tests and a 24-hour video EEG (which tracks her brain waves and seizure activity).  It was typical hospital stuff – lots of waiting, followed by a flurry of activity, and then more waiting.

Miss Z’s liver function test came back slightly elevated. Not high enough to be concerned normally, but too high to start on the Epidiolex under the rules of the trial. So, we had three weeks of weaning down one of her anti-seizure medications (that also affects the liver) and then more blood tests.

After a few months of tests and waiting, the start of the trial was a bit of an anticlimax. Miss Z and I met with the neurologist who explained how he planned to increase Miss Z’s dose over the course of four weeks and answered a few last minute questions. Then we were sent to the pharmacy where the pharmacist working with the trial gave me the low-down on how to administer the drug, how to store it, things to watch out for, and what to do if something went wrong.  Interestingly, one of the requirements of the trial is that I have to return all empty bottles and unused medication to the hospital pharmacy because demand for medical cannabis is so great, they are concerned about them ending up on the black market.

We were sent home with two bottles of Epidiolex and a schedule of scaling up the dose each week. The next morning I gave Miss Z her first dose.

That was four weeks ago.  So, I can hear you asking, “is it working?!”

So far, I’m cautiously optimistic. Miss Z’s seizures have reduced considerably.  She was actually in a “good patch” when she started on the trial, but seems to have improved even further. In the past four weeks, she has had probably four or five seizures – none of which have required rescue medication.  Of course, this could be just a “very good patch” for her, but I’m hopeful that it is the effect of the Epidiolex.

Her episodes where she goes stiff and vacant and doesn’t sleep for long periods of time (we aren’t sure what they are because we haven’t captured one on an EEG – might be a seizure or something else entirely) have also decreased in frequency and episode’s length of time.

She also seems to be more alert. She turns her head to look at people when they speak to her or walk past – something she has rarely done in the past.  She has stopped sleeping late into the morning and now wakes up around 7am (I’m not entirely convinced this is a good thing!).  She is also more active and now rolls herself from one side to her back to the other side – something she didn’t do a month ago.

And best of all, she is cheerful.  The medication definitely didn’t cure her from grumpiness – she can still get cranky with the best of them – but she seems more settled and happy in herself. She has been on Christmas/summer holiday from school and has noticeably enjoyed spending time with her sister. And because Vegemite is getting more feedback from Miss Z, she is enjoying playing her her as well. That has really been the best part our of Christmas break – watching the two of them spending time together.

There is still a downside. The Epidiolex has made Miss Z sleepy and there have been days were Z has spent more time asleep than awake.

It also has had no effect on Miss Z’s movements – where she will suddenly freeze and go very stiff for up to 30 seconds before her body relaxes again. They aren’t seizures, but they have a neurological cause, so the neurologist and I were hoping the Epidiolex might reduce them. So far, it hasn’t.

And there is another reason for cautious optimism. Some children on the trial have initially shown positive results, only for them to slowly dissapate over time. So, Miss Z’s progress may not last over the longer term.

I am trying not to think too much about the downsides of the trial.  Nothing about Miss Z and her life is certain. So, for now, we are enjoying the brighter, happier Miss Z.  May we have much more in 2018!

IMG_0301

Enjoying the school holidays by hanging out with the cat…

 

Advertisements

Not so Wonder-ful

fullsizeoutput_3658

Giving you attitude, not inspiration

I’m meant to be working on a report with a pretty tight deadline at the moment, but I’m feeling so annoyed that I just have to get this out of my head before I can concentrate on anything else.

(By the way, there are some spoilers for the book/movie “Wonder” below – you’ve been warned).

Social media has started swamping me with advertising for the upcoming movie, “Wonder”. For those of you who haven’t read the popular book by R.J. Palacio, it is about a boy named Auggie who has a rare condition, Treacher Collins Syndrome, that has caused him to have facial deformities. He’s been home-schooled his whole life but is about to start attending school when the book begins. The story is told from not just Auggie’s perspective, but also the perspectives of his family and friends. As you can imagine, they all face various challenges, but Auggie – and his friends and family – ultimately emerge triumphant.

I read the book a few years ago, back when I was searching for good books that had a character with a disability to read to Vegemite. Overall, I thought the book was good.  The concept of a book about the life and feelings of a child who looks different and wants to fit in, aimed at a pre-teen readership, isn’t a bad thing and something that probably a lot of kids can relate to in various ways.

However, the end made me want to scream and pull out my hair. The final scene is at an end of year awards ceremony, where Auggie receives the school’s most prestigious prize for… attending a year of school. Everyone cries and reflects on how inspirational he is and give him a standing ovation. For attending school.

If memory serves, Auggie even acknowledges in an internal dialogue that he hasn’t, in fact, done anything to deserve the award or the applause but “everyone deserves a standing ovation at least once in their life” (or something to that effect). And it made me want to rage at him “but wouldn’t your standing ovation be more meaningful if you had actually done SOMETHING to deserve it?!”

I’m not trying to undermine the challenges faced by children with facial – most certainly not.  But what the other kids in the school were meant to learn was to not judge him by his appearance and that once they got to know him, he was just another ordinary kid. Except he wasn’t an ordinary kid because they’ve just stuck him on a pedestal as an “inspiration” to the whole school. And when you think about that, its pretty demeaning.

To paraphrase social activist, the late Stella Young, using Auggie as a feel-good tool and “inspiration” is based on an assumption that he (or disabled people in general) have terrible lives and that it takes some kind of extra courage or heroism to live.

According to the book, he doesn’t have a terrible life at all. He has a loving, stable family, he makes friends and he has a passion for Star Wars. And if my memory is correct, he gets a puppy towards the end of the book. In fact, most of the problems in his first year at school come from ignorant, unkind people and unjust social expectations.

However, by giving him an award and ovation for being inspirational, all the school has done is objectify him. And in a way, aren’t they applauding him because he managed to withstand a year of them being cruel to him?

This makes me think the term “inspiration porn”, which was coined by Stella Young. Talking about “inspirational” memes and videos about disability on social media, she said:

And these images, there are lots of them out there, they are what we call inspiration porn.  And I use the term porn deliberately, because they objectify one group of people for the benefit of another group of people. So in this case, we’re objectifying disabled people for the benefit of nondisabled people. The purpose of these images is to inspire you, to motivate you, so that we can look at them and think, “Well, however bad my life is, it could be worse. I could be that person.”

Now the book has been given the Hollywood treatment, complete with Julia Roberts  and Owen Wilson as Auggie’s parents. I haven’t seen the movie, but I watched the trailer and unsurprisingly, the movie looks like it plays up the “inspiration” side of things even more than the book.  I’m not surprised, since Hollywood really only seems to have two types of disabled people: the inspirational person or the bitter and twisted person.

Auggie is obviously a Hollywood inspirational character. This isn’t helped by Julia Roberts saying in an interview that it should be considered a superhero movie “because Auggie is a superhero”.

Why is he a superhero?  Because kids are cruel to him and he goes to school anyway?  I suspect that applies to a lot of kids. Because people stare at him?  Because it’s just so amazing to see someone with a disability attend school?

This is something that obviously touches a nerve with me. There are obviously some positives to the movie, including raising the profile of Treacher Collins Syndrome and perhaps making a few people think twice before they point, laugh or stare at someone who looks different.

Miss Z looks different, because she uses a wheelchair. And she acts very differently – she has muscle spasms, seeks sensory input by rubbing her hair and mouthing her hand, and often gets stuck in repetitive movements, such as kicking her feet and circling her hand in the air. She sounds different because the only sounds she can make are “woouaaa” and “aaaaahhh” sounds and some lovely shrill shrieks if she’s frustrated or sad.

And yet, she manages to go to school most days.  And it isn’t particularly inspirational. Some days she does amazing things at school and comes home bubbly and smiling. Other days she’s a ratbag in the morning because she doesn’t want to get up, gets on the bus grumbling, is obstinate and grumpy at school, and falls asleep on the bus home. And some days she simply isn’t well enough to go to school.

Miss Z has received some awards from school this year, and I’m hugely proud of her.  None of them were for simply turning up. One was for “Magnificent Maths” and working very hard when learning about patterns. The other was for “Wonderful Writing” where she “looked, listened and effectively used her PODD (communication book) to communicate with others”.

Today at school, she very clearly communicated on her PODD that she thought one of her classmates was “noisy” and “boring”.  I’m not sure that is going to win her any awards (particularly with the classmate), but it makes me proud of her – both because she’s working so hard on her communication and because she has the most sarcastic attitude of any 6-year-old I’ve ever met.  But I don’t look at her and think I shouldn’t complain about work because Miss Z has to work so hard to communicate. I’d never tell Vegemite to suck it up because her sister has it harder than she does. Miss Z is just a 6-year-old going to school and doing the best she can with her abilities.

However, the thing that has really irritated me about the whole “Wonder” movie phenomenon has been the “Choose Kindness” campaign that has been tacked on to the movie. At first, I thought it might not be so bad. After all, if there is one thing this world needs at the moment, it is a reminder to be kind to others. Especially others who may not look like you.

However, when I clicked on the “Choose Kindness” campaign website, it was nothing more than a big marketing campaign encouraging people (kids, I guess) to “be nice”. It is the 2017 version of “just say no” – a meaningless slogan. It could have raised awareness about Treacher Collins Syndrome, suggested ways to be more inclusive, or a million other worthy things. Instead, it encourages you to sign an online petition promising to be kinder (and receive more information about the movie). And it provides a bunch of catchy little “precepts” such as “don’t judge a book by its cover” and “throw kindness around like confetti”. Because that’s gonna make a bully re-think his or her actions…

So, there you have it –  my frustration and outrage at the fact that most of the world doesn’t seem to be able to talk about disability or difference without launching into inspiration porn and throwing puerile slogans around like confetti.  I’m feeling better now that I’ve had a rant – even if I’m even further behind in my work…

I will leave you with this:

If you are planning to see the movie “Wonder” (or read the book), that’s great, but might I suggest you watch this outstanding TED talk by Stella Young first.

If you are looking for good books for kids with characters who have a disability, I recommend Rules by Cynthia Lord and Out of My Mind by Sharon M. Draper.  And I’d love recommendations of more books if you have them!

If you want to watch something that more accurately portrays a family where a child has a disability, I suggest you check out Speechless. Because honestly, parents of a child with a disability are more likely to be a bit crazy, disorganised, hilarious and neurotic than we are “inspiring”. Just think of Z and her sarcasm…

And just in case the concept of “inspiration porn” still hasn’t sunk in, here is a brilliant Speechless explanation of it:

Having a laugh

IMG_0106

A rare smile from Miss Z

Miss Z doesn’t laugh. Maybe it’s because she doesn’t find my jokes funny, but more likely it is a developmental thing. Even as a baby she never laughed, and rarely smiled.

A few years ago, when she was seriously ill with chronic chest infections, Miss Z had a breakthrough day. She’d had a big course of IV antibiotics and suddenly she woke up one day and was much more alert and engaged than usual. I wrote about it here. It was wonderful. But it only lasted a day or two and then it was gone.

During her breakthrough day, she laughed with her sister. It was the first time I’d ever heard her laugh, and at first I wasn’t even sure it was a laugh. I got one video of her laughter from that day and it still makes me smile every time I watch it.


<p><a href=”https://vimeo.com/237032008″>IMG_6200</a&gt; from <a href=”https://vimeo.com/user22250250″>SEMeyers</a&gt; on <a href=”https://vimeo.com”>Vimeo</a&gt;.</p>

And then she stopped laughing and hasn’t done it since, until a few weeks ago. I’m not sure what changed or why, but she is laughing again. Not every day – it is still quite rare to hear her laugh – but she does it.

What breaks my heart is that I’m not sure if it is a “real” laugh or if it is a new type of seizure. There is a seizure type – “gelastic seizures” – which are characterised by uncontrollable laughter or crying. The first time she laughed, we were getting her ready to go to the hospital because she’d had two days of a “vacant episode” that I was concerned might be a prolonged absence seizure. QB and I were both a little stressed and getting her dressed for a night-time trip to the Emergency Department, when she burst out laughing. I didn’t know whether it was hilarious or horrifying.  It was completely out of character and there wasn’t anything obvious that she was laughing at (apart from her stressed out parents). In my mind, I wrote it off as a seizure.

Since then, she’s had more giggling spells, and these seem to be more in context. She laughs at her sister – a lot. She giggles when she does something to get QB or I to come check on her after she’s gone to bed in the evening. She chuckles when you give her a kiss or blow a raspberry on her. And her eyes sparkle a little, making me think that it is a sincere laugh.

We all love it when she laughs. You can’t help but smile when you hear it. One of the most rewarding and fulfilling things about being a parent is seeing your child smile and laugh, and it has been difficult to always see Miss Z so solemn.

But still, I can’t help but wonder. Her laughing is rare, and occurs mostly around the time of her vacant spells (which we still haven’t gotten to the bottom of, but that’s an entirely different story).  While I love it when she giggles, there is always a small part of me wondering if I’m smiling and laughing while Z has a seizure.

There really is no way to tell. At some point in the coming months, Miss Z will have a 24-hour EEG, so if she has a laugh during the test, the doctors might be able to tell. Otherwise, we will just have to go on guessing.

Click here to see a video of Z laughing that will definitely make you smile.


<p><a href=”https://vimeo.com/237662030″>Laughter</a&gt; from <a href=”https://vimeo.com/user22250250″>SEMeyers</a&gt; on <a href=”https://vimeo.com”>Vimeo</a&gt;.</p>

More modifications

The girls headed back to school on Tuesday after two weeks of school holidays, leaving me to enjoy a week of calm. A week where I’ve been able to work a full day and not have to stop and start and then put in extra hours in the evening. And I need to enjoy it, because I may not have another week like this for a long time to come.

On Monday, the builders will arrive to begin ripping out our main, upstairs bathroom. (Our house is a Queenslander, which means all the living areas and the girls’ bedrooms are upstairs). It is going to be a massive job, involving removing all the bathroom fittings and tiles, moving a wall, lowering a floor and reinforcing the walls before rebuilding an entirely new, larger and accessible bathroom.

IMG_0637

This won’t be around much longer!

We are in desperate need of an accessible bathroom. Over the past year, Miss Z has gone from relatively easy to lift to long, gangly and heavy. Both QB and I have problems with our backs (necks, shoulders and hips) as a result of lifting her. And nowhere is it harder to lift her than in and out of the bath.

Until last year, Miss Z was able to have a bath in the bathtub with us lifting her in and out. She is unable to sit or stand, which has made it increasingly difficult. For a long time she used a baby bath chair to support her, and once she outgrew that, I didn’t fill the bath as full and propped her head on my feet. However, as she got bigger, it became increasingly difficult to lift her and step in and out of the bath safely.

At this point, we were advised that the best thing to do was get a shower chair and wheel her in and out of the shower. But Miss Z loves her baths. I mean she really, really loves her baths. And there isn’t much that Miss Z really loves and enjoys, so we didn’t want to take her baths away from her.

And so an organisation that supports Miss Z loaned us a raised bathtub. It just about fits in our bathroom and it means that we have to have the sink tap permanently connected to a hose to fill the bath, so it is far from ideal. But it was much easier and safer to lift her in and out.

IMG_0390

The raised bathtub

However, this year, lifting her from the floor (where she has a changing mat) and into the tub and back again became a greater and greater challenge. And with that challenge, came an increasing number of injuries, aches and pains for QB and me.

And so, once we competed the lift (elevator) installation earlier this year, we turned to planning the bathroom. It has been a very long, difficult process, involving a surprisingly large number of people. It started with just QB and me, talking about what we could do. Then we consulted her occupational therapist, who has been invaluable in the process of making sure we’re designing it for Miss Z’s long term needs, advising on all the specialist equipment, and organising the few bits of funding which are available to us. Miss Z’s physio also made suggestions – and in fact was responsible for the idea that transformed our thinking.

And then there were the equipment providers. The first one came, spent over an hour discussing how we could install a ceiling hoist, and then never followed up, despite numerous attempts to get a quote from him. Apparently, he just wasn’t that into us.

So, after a few months of trying to contact him, we had to find another ceiling hoist provider. Once we did, their equipment guy came round, then he came round again with an installation specialist, then he came round again with the installation specialist to meet our preferred builder. QB, who is great at designing and project managing these types of projects, came home from work early many days to meet with them and sort out the details. Over the past months, I’ve become accustomed to finding my husband and two or three other men hanging out in our bathroom, having a conversation…

And then there were the funding people, who also had to come round to interview me and then come round to inspect the bathroom and discuss the modifications we wanted to make. By this time, QB had a very clear vision of the design in his head – and on paper.

Of course there was also a lot of wandering around bath shops and looking online at various bathtubs and toilets and vanities and taps and showerheads… Again, I’m grateful that QB is good at this design stuff, because to be honest, I suspect I have rather random taste, although the $10,000 bathtub I spotted in one of the shops was truly lovely (and well above budget and probably wouldn’t have fit a bath support chair, but that’s beside the point…)

All this time, we still lifted Miss Z in and out of the bath every night. And then it became every other night, because we were both suffering from sore backs… But at last we’re nearing the end of the lifting.

If all goes to plan, we will have a larger bathroom (we’re taking out the walk-in closet in Z’s bedroom to extend the bathroom). The floor will be lowered so that she can be wheeled directly into the bathroom. There will be a ceiling hoist to lift her out of her chair. There will be a changing table to get her undressed. And then she can either be hoisted into the bathtub or there will be a hand-shower over the changing table, if we’re in a hurry and just want to give her a quick wash.

During this process, there have been two things that have frustrated me (although once the building begins, I’m sure there will be many more). The first is trying to make everyone understand that we don’t want the main room in our house to look like a utilitarian, public disabled toilet. This is our house. This is our main bathroom – it isn’t just going to be used by Miss Z. We want it to look nice as well as be functional. It took a lot of effort to get that message across to everyone.

The second was the constant insistance that Miss Z needed to be able to take a bath. Yes, the easy option is a shower, Yes, it would be cheaper. And I admit, that as she gets bigger, we may end up showering her more than bathing her. But the girl loves her baths. They soothe her. Some nights she lies in the bath with her eyes closed and is a picture of pure relaxation. Other times, she’s had multiple seizures and is screaming and a bath is the only thing that calms her down. Lying in a bath, listening to music is probably the best part of her day, and we are not going to take that away from her.

IMG_7035

Happy in the bath

So, now we’ve won our fights. We’ve chosen our taps. We’ve decided where the ceiling hoist track will run. All that is left to do is suvive the build.

Let the construction begin!

 

 

Hanging on the telephone

One of the outcomes of the big, multi-disciplinary meeting we had with several of Z’s doctors last month was a decision to push harder for Miss Z to join the medical cannabis trial that is starting in Queensland.

For those of you who might not follow the developments in the treatment of intractable epilepsy, it has been discovered that medical cannabis can reduce seizures and increase cognitive function in some kids with uncontrolled seizures. After a long battle in Australia, the use of medical cannabis has been legalised – hurrah!

However, there are still some hurdles to obtaining it, and when you can get it, the cost can be prohibitive (unless you’re buying from unregistered suppliers).

All the doctors I’ve spoken with are confident that within a few years, medical cannabis will be available for epilepsy as readily as any other antiepileptic medication. However, until then, the easiest and safest way to access it is through a medical trial.

So, when I heard that the Lady Cilento Children’s Hospital, where Z receives her medical care, was going to run a medical trial called the Compassionate Access Scheme, I signed her up – along with pretty much every other parent of a child with uncontrolled epilepsy in Queensland. Our neurologist was a bit skeptical at first – mainly because it isn’t a real scientific trial – all the kids receive the medical cannabis oil, no one gets a placebo. However, the promising early results made her decide that Z would indeed be a good candidate.

A week after our multi-disciplinary meeting, I got a call from the trial to arrange an appointment for Miss Z. I was so excited that I immediately took to Facebook, declaring Z had been accepted into the trial. Cue lots of celebration.

Alas, I was a bit premature. The appointment, it turned out, was only step one to joining the trial. And I also found out some fairly surprising news – that only 30 children across Queensland would be chosen for the trial. Only 30!

Miss Z was her typical self during the appointment. She was seriously unimpressed about being dragged into an early morning hospital appointment on her school holidays. So, she scowled at the doctor, and grizzled, and tried to kick him when he examined her. There was none of her typical Z-charm was on show that morning. In fact, the doctor asked me if she was always “so distressed” – which I tried to answer honestly without actually saying “no, it’s just you”.

The appointment established that Miss Z definitely qualified for the Compassionate Access Scheme. However, the next step is that her case – and those of other candidates – would be considered by a panel of doctors who would choose which children would be offered a place on the trial. If we aren’t offered a place on the trial, then we can go on the waitlist, so that we may be offered a place if another child drops out.

I left the appointment with a grumpy child (who cheered up immediately once we left the doctor’s office) and a promise that they would contact me when the panel had made its decision.

And so, now we wait. I feel like someone who has just been on a first date and is waiting to see if he liked me enough to ask me out again. Seriously. Did he like us? Will he choose us? Will he call?

I’ve even resorted to getting my friends to ask his friends if he is planning to call – although instead of friends, I’ve used nurses and her neurologist to try to find out when a decision might be made. QB is like my bestie, reassuring me that the doctor will call me – after all, I did three pages of seizure charts and graphs for him!

I keep running through the appointment in my head, thinking of how I could have made Miss Z’s case more compelling, better arguments for why she needs the treatment, examples of what she can achieve when she isn’t suffering multiple seizures a day.

Logically, I know the decision isn’t going to be made because he likes or dislikes Z, it will be on a medical basis, considering things like contra-indications with the drugs she currently takes, the risk of side effects and the options still available to us. But I still can’t help but feel like we are party of a beauty parade.

And is it even something we want to win? If she gets on the trial, it will be a big, fat declaration that yes, Z’s seizures really are that bad. It can be difficult to know what is bad versus manageable when you have a child with uncontrolled epilepsy. What is bad for her might not be so bad for another child, or vice versa.

Plus, despite positive results for some kids, it is important to remember that it doesn’t work for everyone. Medical cannabis is like the Ketogenic diet or the vast range of anti-epileptic medication Z has taken since she was 6 months old – it works great for some, OK for others, and not at all for others. And there is no way to know if it will work for Z until we try it.

So, I keep my phone close and wait to see if we get a second date.

IMG_0439

Sleepiness is one of the side effects of seizures for Z. If she has a big seizure or a cluster of them, she will often spend the rest of the day sleeping.