Archive | August, 2011

The kindness of others and other random thoughts

31 Aug

What has been truly overwhelming in the most positive way since this terribleness hit is the generosity and kindness of so many people. From lovely cards people have written, calls made, food cooked, presents given and tears shared, it has been such a panacea to the sadness.

Thank you.

Today’s update is that things are holding a steady course. Which is damn good news! We are weaning the Topomax very, very slowly and it seems to already be making a difference. Topomax causes weight loss and difficulty in finding words – two things we absolutely cannot afford for Tilda to experience. Her appetite has been pretty impressive over the last week or so – KFC popcorn chicken is a huge hit and we have gone from the kiddie size to the regular size to the gigantic oh my god, I can’t believe she is eating all of this size! Fantastic! Not to be flippant but there is something quite liberating about not worrying if your child is eating too much junk food! Chips Tilda? Sure! Deep fried spring rolls for two dinners in a row? No probs!

You take the good where you can get it.

There is not much else to add today so I shall instead show another of my favorite videos of Tilda. This was taken in Darwin again, before all this poohness began.

She’s so awesome.



Your daughter is a delight

27 Aug

On Wednesday, we had arrived at Tilda’s school and had our meeting with a social worker about possible services in the Eastern Suburbs that we could link in to. I am all about getting whatever services are on offer – the more people in Team Tilda, the better! So we had a good meeting and our lovely social worker is going to do some investigating for us. In particular regards to possible school holiday programs for Matilda.

So anyway, I was due to go swimming with Tilda but we were a touch early so we wandered down to her classroom to find her. As we were giving her a cuddle and getting her bag sorted for swimming, one of the lovely teachers came by and said ” I just have to tell you, that your daughter is an absolute delight, we all love her and are so happy that she has come to school here”.

Well, I just about burst with pride. I mean, I know that she is a delight but it certainly nice when other people can see it too.

Let’s not get carried away of course – she can be a pain in the arse – stubborn, whingy, frustating – all of those things and she is far from perfect. But she is still a delight.

I attach for your listening pleasure, a recording I took of  Tilda in the Morning just after she woke up. She has an infectious giggle that usually leads to hiccups and coughing but it is a pretty great way to start the day. (note: if you are using Google Chrome this link will not work correctly)


The Tildaness of Tilda

24 Aug

I have been putting off writing this post because I know it is going to make me cry. But there are thoughts swirling around in my head that I think should be set down.

The complete bastardness of this disease is not just the fatality of it. It is having to watch her no longer be able to do the things she used to.  It is watching her lose her words, her ability to walk and play, even sit. I can feel strong at the moment because the Tildaness of Tilda is still there. We get the cheeky smiles, the giggles, the jumping and of course the roly poly’s. Her personality is still shining through and I am hanging on to it with all my might. My greatest fear about all of this is losing the Tildaness of Tilda. Most of the time, my coping mechanism is pure denial. I think that is the only way to live through this. Day by day, not thinking too much about the tomorrow.

Some days though, my thoughts drift to the past and is it at these times, that my grief overwhelms me.

I wanted to show my very favourite video of Tilda. It was taken on her third birthday, exactly 1 week after her first seizure at the pool. At that time, we were in ignorant bliss and believed that it was a febrile convulsion, never to be repeated. A childhood anecdote that we would talk about in years to come. She is bright-eyed, she is tanned and blonde . She is wearing her favorite dress  and those bright red shoes. She is totally delicious.

This morning, I carried Tilda from her room to her play area. I carried her because she was seizing so much, she couldn’t stand up straight. She sat in her chair, whilst Simon spooned her cereal into her mouth, a bandanna around her neck to stop the drool from getting her soaking wet. She is still Tilda but I don’t know how long for and it is that thought that wakes me in the night, that thought that sits on my chest and squeezes my heart until I can’t breathe.

Fuck you Battens.

1 in 4 / Genetic Stuff

17 Aug

First of all, thank you all very much for the positive feedback on the blog. Feel free to comment lots! I like the validation!

On to today’s thoughts:

For a lot of people, when they hear about Matilda, one of the first questions is whether or not we are going to have more children. It was one of our first questions too. The whole genetic side of things is a bit confusing but a few weeks ago we had an appointment with a genetic counsellor which clarified things quite a lot.

Basically, both Simon and I are carriers of the defective gene. This means that any child of ours has a 1 in 4 chance of inheriting the disease. A 1 in 2 chance of being a carrier like us and 1 in 4 chance of being totally unaffected.


1 in 4. It can be looked at in so many ways. If you were putting a bet on something with a 1 in 4 chance of winning, you would think that was pretty good odds. But then 3 in 4 of being either a carrier or unaffected is even better odds. The good news is that there is prenatal testing for the gene. The bad news is that it isn’t done until about week 14 or later. The harsh reality of it is that if we do decide to try for more children and we have the testing and we find out that it is positive, we would undergo a heart wrenching and devastating termination. Personally, I have been struggling with how I feel about having more children. I oscillated daily between thinking of a future with them and thinking of one without. Truthfully, I could see some advantages to living a child-free life – the freedom, the ability to go anywhere, do anything. I thought about how hard it would be with a newborn and with Tilda. I thought about how hard and unfair it would be for a brother or sister to lose Tilda too.

But thinking like that made me feel like I was giving up. Because a life with children is hard and painful but it is also full. I don’t know whether we are even able to have more children. Before the diagnosis, we had been trying for a year without success. But I don’t want to not try. And that is about all I can know at this point.

Much love


Daily Tilda Routine

15 Aug

I thought I would write today about our usual daily routine with Tilda as it might be something of interest.  Currently she is attending school Monday through Friday so the schoolday routine goes a bit like this:

7:00am: Hit the snooze button

7:15am: Matilda wakes up, morning cuddles then she points towards the door and states quite explicity: “I need to watch Dora”

7:30am – 8:30am: Matilda is ensconsed in her high chair watching Dora whilst we run around preparing for the day. Tilda has difficulty feeding herself so breakfast can take a long time (usually vitabrits plus cream plus milk – we try and sneak cream into as many of Matilda’s meals as possible to stave off the inevitable peg feed).  After breakfast, there is the usual struggle to take the morning medications. 7ml Epilim, 15mg Clobozam, 1mg Artane, 25mg Topomax, 50mg Lamictal. We are hoping to wean some of these over the next few months in particular the Topomax which we don’t feel is having too much of an effect. School lunch gets made, entry written in the communication diary, bag packed for the upcoming school day. Tilda is dressed and ready to go for the school bus which arrives at about 8:30. There is usually a mad flurry around this time as we see the bus come up the hill and haven’t quite got everything together yet! Thankfully, they always wait for us 🙂

8:30am – 3:30pm: Tilda is in her fabulous school. Different activities on different days but on a Wednesday, I get to go swimming with her and the rest of her class in the afternoon. She absolutely adores the water and Glenallen’s indoor heated pool is wonderful. Other activities include Art, Music, OT, Speech, Libary time, using the interactive whiteboard etc. The communications diary is such a wonderful tool. Each day her teacher writes in it, letting us know how the day has gone. They even include photos on some days and it is something we really look forward to reading.

3:30pm: The bus drops Tilda off. She is invariably cheery but weary after a big day at school. We chill out for a while. Depending on her energy levels, it might be roly poly’s on the floor, jumping on the trampoline or playing in her room. She loves the bath at the new house and can happily spend up to an hour in there playing.

5:00ish: We try and give her dinner pretty early and depending on work schedules will either all eat together at the table or if it is just one of us, let her eat whilst watching more of her beloved Dora/Maisy/Charlie and Lola/The Wiggles. Tilda can be a great eater or a not so great eater totally depending on the day. It can be very frustating when she doesn’t eat so we usually go for things that we know she likes – spaghetti bolognaise/mac n cheese/pizza/creamy pasta – high fat is good, good, good! After dinner, once again we have the usual struggle with medications. At night, she takes 7ml epilim, 15mg clobozam and 40mg topomax.

6:30: After a long day at school, she is knackered by this time so it is off to bed with a story. She usually goes to sleep very quickly until about

2:00am ish: Tilda will call out and I will go in to her and sleep the rest of the night in her bed. I started doing this a few months before her diagnosis and it had got to a point where I thought I should probably wean her and myself of this routine. Since the diagnosis, however, I have decided that I actually don’t want to give it up. I love waking up with her in the morning, she is a cuddler and a snuggler and it is a time to be enjoyed. In the past few weeks, it has also been prudent that I have been with her as she has awoken in the night with febrile convulsions. We worked out that she was overheating some nights leading to the fever and subsequent seizure activity. When this happens, we administer Midazolam and give her Nurofen to bring the temperature down. It turns out that Tilda is one of those where Midazolam doesn’t have a sedative effect! We know now that it will stop the seizing but will result in a sleepless night for all as Tilda lies in her bed, chatting away and pointing at the imaginary ducks on the ceiling. She bounces back from each episode seemingly unscathed.

7:00am:Hit the snooze button and it all starts all over again!

General Health Stuff

Seizure activity throughout the day: Matilda is almost constantly seizing. Absences, myoclonics and general ataxia cause her to be very unstable on her feet requiring assistance to walk any length. It is both a blessing and a curse that she remains as active as she does – her fearless and gutsy attitidue means that the only time she is still is when she is watching TV. Otherwise, she completely ignores the fact that she is wobbly and likes to be on the move. Stairs (at my parents and my sisters house in particular) are a huge favourite – meaning we cannot turn our back on her for a second. It is similar to having a very tall 18 month old roaming about!

Speech: Sadly, Matilda is losing her words. It takes her a while to get a sentence out but sometimes she will blow us away with the connections she can still make. Yesterday was Simon’s birthday and we were at my mum and dad’s for a celebratory meal – there was cake and Matilda helped Simon blow out the candles. When I was putting her to bed later that night, she managed to get out “I blow candles” which was very impressive. Some days again are better than others in regards to her speech.

Vision: We know that one of the key symptoms of Batten Disease is the loss of vision but have been given the very happy news at our opthamologist appoinment on the 8th August, that there is no sign of vision damage at the moment and Matilda’s sight appears to be in good health. This is excellent and very welcome good news.

Upcoming appointments are on the 22nd September with our pediatric neurologist and 5th October with the developmental pediatrican. We have been accepted into Very Special Kids and are looking forward to meeting with them to see how their services might help us. We are still thinking about a Wish for Matilda through Make A Wish but haven’t decided on anything as yet.

That’s about it for the moment. Updates as they happen!

Much love


First post!

12 Aug

Today it has been 10 weeks and 1 day since we received the news that Matilda was not just fighting a particularly malovelent form of epilepsy as we had thought. She was fighting a far greater battle, an insidious genetic condition called Batten Disease. Thursday, 2nd of June, whilst talking about other treatment options for the epilepsy, a piece of paper with some recent test results was handed to our neurologist. Her face went white and we pretty much knew straight away that the news wasn’t good.

Since that time, I think we have experienced every emotion under the sun. The first week following the diagnosis is still a bit of a blur. I remember walking out of the neurologist’s office. I remember Simon and I breaking down in the car park and going home to look at one another and ask how the hell we were supposed to deal with this. I remember drinking quite a few beers. I remember the kindess of friends, family and work colleagues who all rallied around and provided an ear, a comforting word even presents for Tilda and food for us. Thank you all so much for that.

Since that time, we have come to a better place. We kicked into high action – moved house, enrolled Tilda in the truly special Glenallen school, shed our fears about the future and concentated on the now.

The now is pretty good! Tilda is having a blast at school – photos to come once I work out how to do that! Our new house is roomy, with carpet for her to do her ever constant roly poly’s. I have settled into my new hours at work, Simon is currently playing house husband before starting back at his new work on the 29th August and most importantly, Tilda is cheery and happy and well.

My idea is to try and update this weekly to keep everyone up to date. Once I figure out how it all works, I will add more features, photos, links etc but right now we are working with the bare essentials!

Thank you for being a part of Team Tilda.


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