Operation Cinema

Things have been going pretty well for Miss Z and me lately (quick! find some wood to knock on!). She’s been healthy, only visiting the hospital for planned appointments, and going to school every day. And because she is doing that, I’ve had the opportunity to focus a bit more on my work as a freelance writer and researcher. The downside of this is that I’ve been so busy with actual paid work lately, that I haven’t had as much time to blog as I would have liked.

However, I have recently written a series of blogs for the Firefly Community – a growing new social media platform for parents of children with disabilities. One of my articles was just published, so if you are keen on more news of Miss Z and the rest of us, please head on over to read it HERE.

 

Mysterious workings afoot

This is one for those of you who love to solve riddles. I don’t – probably because I’m not all that good at it – so I’m looking to you, gentle readers, to restore my sanity. Because there are mysterious workings afoot and I just can’t figure them out.

So, bear with me briefly, while I give you a bit of background information.

Miss Z is fed bags of perfectly nutrionally balanced formula, using a feed pump that delivers the formula direct to her jejunum (which is part of her intestine). Because the jejunum isn’t designed to accept large amounts of food all at once, like the stomach, she is connected to her feed pump for 16 hours a day as it slowly pumps the formula in at a rate of 60ml per hour. The continuous feed thing is a bit of a pain, but we manage pretty well and it doesn’t seem to prevent Miss Z from going places or doing things.

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Feed pump, tubing and bag of formula – all set to go

We see a dietician ever six months to assess how much formula Miss Z is receiving and if the amount needs to be increased based on her growth and activity levels. At the moment, she is on 1,000ml per 24 hours. Since the formula bags are 500ml each, we have divided it up so that she receives one 500ml bag during the day and the second 500ml bag overnight.

That all makes sense right? In fact, it sounds pretty easy, doesn’t it?

But here’s the thing, when I give her the two bags in a 24 hour period, it never, ever totals up to 1,000ml. Ever.

That too, seems completely logical. After all, there is a long tube that connects the bag to Miss Z, so even when the bag is empty, there will be some formula remaining in the tubing. The pump has a safety feature that stops it and sets off an alarm when the bag is entirely empty and air is starting to be pumped into the tubing to prevent air instead of formula being pumped into Miss Z, so she never receives the full amount in the tubing.

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A giving set – aka the tubing

However, here is the mystery. Every day I do the same routine with Miss Z: one bag of formula overnight, one bag of formula during the day, change the tubing, and repeat.  And yet, every day she receives a different amount of formula.

Some days, it comes up short. Assuming that the tubing can hold around 10-20ml of formula, you would think that it would be short by around 20ml – so she would have a daily total of 980ml. But no. Some days it only reaches 950ml or even less.

I could account for this shortfall with the formula in the tubing and perhaps a bit of spillage and wastage, except that it gets even more mysterious. Some days she receives over 1,000ml!

This morning, for example. I put on her overnight feed before I went to bed last night. She got new tubing (the tubing, or “giving set” as it is officially known, has to be replaced every 24 hours) and a new bag of formula. I filled the tubing with formula (known as “fill set”) before connecting Miss Z. This morning at around 7am, the pump stopped and the alarm went off because we had reached the point where there was air in the tube. However, when I checked the pump to see how much she had received overnight it said 522ml. So, how did she get 522ml from a 500ml bag?!

The most logical answer to all this would be that the bags of formula contain different amounts. However, I’m doubtful about this because the formula is only available on prescription and provided by a medical company, arriving in sealed 500ml bags, so it would be alarming if they weren’t careful with their volumes. To test this theory, I weighed several of the bags and they all weigh the same amount, so that is unlikely to be the problem.

So, tell me, how can I do the exact same thing when feeding Miss Z every day, but one day I only get 950ml out of two 500ml bags and the next day I get 1,050ml?

Go on then, solve my mystery and put me out of my misery!

(I should probably note that I’m not concerned if Miss Z receives slightly less formula one day and a bit more the next. She is a healthy weight and growing like a weed. Plus, none of us consume the exact same amount of calories every day, so there is no reason for Miss Z to do so. As long as we come close to the target amount, it isn’t a worry. I am just very, very curious as to why it varies so widely!)

 

Not paying for it

We moved to Australia when Miss Z was no larger than a lentil in my tummy. She was born here in Brisbane and has only left Australia once – when she did an international tour to visit her overseas relatives at 3 months old (and before we were aware of any “problems”).

It wasn’t until later that the issues surrounding Miss Z’s visa status became more of an issue. We weren’t Australian citizens and we weren’t permanent residents, so therefore we couldn’t access any government disability funding.

At first, this wasn’t such a big deal. We’d managed to get Miss Z some state-funded therapy and we declared that we were happy to self-fund anything else she needed. This was easy when she was a baby and didn’t require very much. It became a greater challenge when she outgrew her baby stroller and we had to buy a supportive special needs stroller – which cost $7,000. A challenge, but not impossible – especially with the help of a friend who organised a Christmas fundraiser for us.

Miss Z’s therapists were less convinced that self-funding was going to be a good option over the longer term. Miss Z will need A LOT of equipment as she gets bigger, and none of it comes cheap.

Deciding that our dreams of international travel with a young children was no longer possible due to Miss Z’s medical needs, QB and I decided that we wanted to stay in Australia to raise the girls and started the process of applying for permanent residency. Residency for three of us wasn’t an issue, but for Miss Z it was another story.

I don’t want to go into the details of the whole application process, which was generally long, tedious and stressful. I won’t share my opinions of Australia’s immigration policies in general or towards people with disabilities in particular, because I didn’t create this blog to debate politics. I will just say that we went through 18 months of very high levels of stress and uncertainty – not to mention having to demonstrate the value of our daughter’s life – but in the end, we were successful. Our family are now permanent residents of Australia. Hurrah!

And with permanent residency, comes access to funding – for equipment and therapy – for Miss Z.

It has been a serious learning curve as I’ve never applied for benefits of any kind before. Six months in, I think I’m finally getting a grip on it…

However, yesterday I took possession of our first piece of equipment purchased through one of the government funding bodies: a gravity chair. Despite conjuring up visions of something from the space station, a gravity chair is a specially designed foam armchair. We haven’t had proper seating for Miss Z, which means she spends most of her time at home on the floor or a beanbag – or occasionally her stroller (although she really doesn’t like sitting in it for long). Her gravity chair will let her sit up and interact with the rest of us as well as watch cartoons and play on her iPad while upright.

We trialled a few different chairs and the gravity chair was by far her favourite. It is also lightweight, which means we can move it around the house so she can sit in different rooms.

Still, it felt strange yesterday when the delivery man turned over the giant box and departed without also handing me an invoice. I’m just not used to having our equipment funded. In fact, it makes me feel slightly guilty. I almost feel like I need to defend our use of funding for Miss Z’s equipment. I suppose it is one of those contradictions that come with having a disabled child – as much as you want to be able to provide your child with all the necessities in life, the cost of wheelchairs, hoists, specialised seating, home modifications, bath chairs, adjustable beds, ramps, accessible vehicles, communication devices and everything else Miss Z needs makes it pretty much impossible to afford everything yourself – no matter what your family’s income.

And I’m learning how this equipment really makes a big difference to Miss Z’s life. Our raised bathtub (currently on loan to us), has enabled us to continue to safely bathe Miss Z – hands down her favourite activity of the day. Her electric bed is a huge help with everything from managing her nighttime reflux to saving my back when I lift her in and out of bed. It also means that I can lower the bed and sit beside her to read bedtime stories.  None of the equipment is superfilous – it all contributes to Miss Z’s quality of life.

Using Miss Z’s funding to make sure she has this equipment is one of my new jobs, so I guess it is something I need to get comfortable with… even if I may never be as comfortable as Miss Z in her gravity chair in front of Doc McStuffins!

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Four Eyes

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Too cute! 

A crazy thing happened when Miss Z last visited the ophthalmologist. He declared that she needed glasses.

The first thing that everyone asks me when I tell them this is: how did he figure that out?

Good question! Miss Z is non-verbal, so she isn’t reading letters off a chart or telling the eye doctor which lens makes the image look clearer. In fact, because the ophthalmologist always has the nurse dilate Miss Z’s pupils before seeing her, she is invariably in a grumpy mood and takes non-cooperation to a whole new level with the poor doctor, who is actually a very nice man.

I can’t really explain how he did it. He had a device that he held up to her eyes, he looked in the other side and then fiddled with some buttons. After a bit of fiddling and adjusting, he put the device in a docking station and it printed out a report, which told him she was far-sighted and had astigmatism. He was surprised – Miss Z has annual eye checks and they’ve never indicated that she needed glasses before – so did the tests with the device again and got the same results.

Miss Z has CVI – cortical visual impairment – which means that even when her eyes work properly, her brain doesn’t always interpret what she is seeing correctly. So, even if she didn’t need glasses, things might appear blurred or out of focus to her. It also means that her sight can be variable – one day her vision may be good and the next very poor. I find particularly after she’s had a few bad seizure days that she rarely makes eye contact or looks directly at a book or the television. I interpret this as Miss Z having a ‘bad vision’ day. Other days she makes great eye contact and stares fixedly at things, suggesting it is a ‘good vision day’.

Fortunately, our ophthalmologist recently attended a conference where one of the speakers was a strong proponent of kids with CVI wearing glasses if they needed them – and her argument convinced him. Apparently, some eye doctors don’t think it is worth the effort of prescribing glasses for someone who already has a vision impairment.

Of course, in true Miss Z fashion, getting a prescription for glasses was the easy part. We then had to find an optomitrist who could fit Miss Z with glasses that would actually be comfortable and virtually unbreakable. It took a bit of effort, but we found a place in the end that carried a wide range of children’s glasses.

Trying on glasses took Miss Z’s refusal to cooperate to an entirely new level. Fortunately, Miss Z’s stylist (aka Vegemite) was there to make the big decisions on style and colour.

So, Miss Z now has glasses. And they make her even cuter than she was before, if that is even possible.

She’s not crazy about wearing them. Some days she doesn’t seem to mind, but other days she will spend all her time and energy on trying to knock them off. They have a band that goes round her head, so it is difficult to get them off completely, but she can still knock them skew-whiff.

It is hard to tell how much of a difference they make to her. I suppose that with her CVI, the glasses make a different difference to her every day – on bad vision days, they won’t help her to see any better, but on good vision days, they may help a lot. And her vision is becoming increasingly important as we focus more and more on communication through eye-gaze (more on that later!).

 

So, we’ll keep putting them on her, in hopes that she’ll get used to them and be happy to wear them more often. And in the meantime, at least they provide a bit of comedy.

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Keto Fail

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Home after yet another visit to the hospital

Back in February, Miss Z started on the ketogenic diet. For those of you who have forgotten, the keto diet is a special high fat, no carb diet that can help to control seizures. It works for approximately 50% of people – 25% see seizures stop completely whilst the other 25% have fewer and less intense seizures. There is no way to tell if the diet will work for someone without trying it.

Miss Z is part of the other 50%. The 50% where the diet has no effect.

She was on the diet for four months. During that time, her seizures actually increased in frequency and intensity. She went from having tonic seizures (where her arms and legs go stiff) to having tonic clonic seizures (where her arms and legs stiffen and then start jerking – it used to be called a grand mal seizure). She also dropped in weight from over 20kg to 18kg – and started to look quite thin.

I don’t think the increase and change in seizures was caused by the ketogenic diet. I think that it was just part of Miss Z’s seizure cycle (we have good control for a period of time and then she goes steadily downhill). She also had a number of health problems during the four months, including a central line infection (which eventually resulted in her portacath being removed), a nasty chest infection and gastritis (which caused her to vomit blood). My (completely unprofessional) opinion is that the diet just didn’t make a difference, which meant all the other factors contributed to an increase in seizures.

By the end of four months, it was clear it hadn’t made any difference. In the end, it wasn’t a huge disappointment. I think the realisation that our diet experiment had failed crept in slowly over the course of several months, so that by the time QB and I decided to take her off it, we had already overcome any  feelings of disappointment and sadness. In the end, I was just glad to not have to wash bottles and mix formula.

Miss Z is now completely off the ketogenic diet and back on her usual (and much more nutritionally balanced) formula. Coming off the keto diet hasn’t made any difference to her seizures, which she still has nearly every morning.

One of my top goals for the diet was to try to wean Miss Z off some of her anti-seizure medications. She is on four different drugs (Epilim, Keppra, Topamax and Frisium) at the moment, which is a lot of drugs affecting one small girl’s brain.

Yesterday we had an appointment with Miss Z’s neurologist. We discussed reducing her medications and the doctor pointed out that there is no sense keeping her on four drugs if she still doesn’t have seizure control. So, we’ve come up with a plan to try to stop some of these morning seizures (by increasing one of her medications) and then begin to wean off another medication.

It feels good to have another plan. Of course there is no guarantee that this plan will work either. But there are never any guarantees with Miss Z. And we can only carrying on trying until we find something that works.