Archive | December, 2011

Focus on the good

29 Dec

I love Christmas, I love everything about it from the cheesy decorations to the big family get together to the enormous amounts of food that is on offer. The last few Christmas’ have been pretty tough though. 2 years ago, Matilda was diagnosed with epilepsy a few days before Christmas. We were in Darwin and I felt very homesick. Last Christmas, we were a month into the Ketogenic Diet and struggling.

This year thankfully, was great. Kylie and Tim did a fantastic job of hosting the massive Christmas lunch. The food was delicious, the beers went down a treat and we even had a White Christmas thanks to a freaky hailstorm after a morning of sunshine! It was so wonderful to see all the family in particular my delicious cousins Stella and Isla who flew in from London. Tilda had a ball. We spent Christmas Eve night there so she got quality time with Thomas and Charlie and she was in fine fettle. She has recently rediscovered the word “Yuck” and finds it completely hilarious!

 After a day of eating too much and then having lots of red wine with my sister Christmas night, we spent Boxing Day at home and had a very peaceful day. The next day, we headed up to Gisborne to spend the day with Simon’s family. Simon’s sister and partner Michelle have made Tilda the most amazing present. I am blown away by their talent and thoughtfulness. For want of a better term, it is a playchair for her. Somewhere she can sit and be part of the action, have all her toys around her and be safe. Thank you so much guys. And apparently this is just the prototype!









I think they need to patent this!

General Health Update 

We had our follow up appointment at the Royal Children’s Hospital this morning which went much better. We now have a clear understanding of what the PEG operation will involve. Unfortunately, because Tilda’s eating has been so sporadic over the last few weeks, we have made the decision to go forward with a Nasal-Gastric tube while we wait for the PEG surgery. We will therefore be going into hospital next Wednesday to have it inserted and have some training on how the feedings work. Hopefully, we won’t have to wait too long before she is able to have the PEG.

I am trying to focus on the good. I know that this will mean that Tilda will easily be able to be fed and medicated. No more battles! No more anxiety about whether she has eaten enough. It will remove all of that. And, I really don’t think that it will bother her that much. She has had one before and was not fazed. She will still be able to eat by mouth (beloved Popcorn Chicken!) but won’t have us hovering around all the time trying to shovel food into her.

Focusing on the good is good. But of course, the whole reason for this is bad, bad, bad. It is yet another progression in this shitty disease. And it completely sucks.

In happier news, the topomax wean is complete and so far the new medication, Keppra, seems to be doing something. We are seeing less myoclonics which is definitely a good thing to focus on.

Happy New Year to one and all – my hope for 2012 is for a year where NOTHING happens. Boring. Quiet. No changes. Perfect.


Hospital Visit Update

21 Dec

It is important to complain. It hopefully allows for an improvement in whatever situation. And for us, that was the case. I got hold of our pediatrician this afternoon and voiced our concerns about yesterday’s misadventures. She was very apologetic and understanding but better than that, she took steps to improve the situation. We now have another appointment next week with the head doctor to fully explain what will happen in the PEG surgery and to examine Matilda. There was a misunderstanding from my end in my desire for a confirmed surgery date. That apparently was never going to happen – we are now on the waiting list and may have to resort to a Nasal – Gastric tube if Matilda’s weight becomes a serious concern.

Which unfortunately at the moment, it is. She isn’t eating much of anything except her Vitabrits for breakfast ( I am thankful for that at least) and her swallowing appears to be troubling her. I am glad that we have another appointment and can hopefully get some more concrete plans in place.

Last day of school tomorrow! Matilda came home yesterday laden down with amazing artwork, photos, presents and crafts. It was an absolute treasure trove and I am still sorting through it. We also got her report card! A snippet:

Matilda has settled very well into school routines and activities. She eagerly greets friends and staff. Matilda has a great sense of fun and a wonder laugh. We are so pleased that she has been a P10 girl this semester.

We are very proud. 🙂


More Wish Day Photos!! (and an annoying hospital visit but yay for photos!)

20 Dec

First of all, I would like to give a huge enormous thank you to the very lovely Kirsty from ruby d photography who so graciously donated her time and talent to Matilda’s Magnificent Wish Day!! I have updated the web album with all the new shots which are just wonderful.

More Matilda’s Wish Day Photos!

We are still coming down from the day. 🙂 There are some absolutely gorgeous ones, especially of all the kids. Thank you so much Kirsty!!

Coming home to the photos was a welcome relief from the annoyance of our earlier hospital visit. I understand that, with the new move, the Royal Children’s Hospital is currently in disarray but the new system for seeing a specialist at a clinic is nothing short of awful. Doctors from all genres are lumped together in one waiting area with receptionists who have to know what is going on with all of them. The waiting areas are packed to the brim with parents, children, prams, wheelchairs and when doctors come out to call for a patient to come in, you have to strain to hear over the noise to find out if they are calling for you. After an hour of waiting, we finally got called. But only for Matilda to be weighed and measured. We went back out to the waiting to strain to hear for the next time her name was called.

I have been dreading this appointment. I have been putting off the idea of a PEG even though I know that ultimately it is the best thing for her. So I wasn’t really going into the appointment in the best frame of mind. What I did want out of it though, was answers and a date. My mood did not improve when the ‘specialist’ who actually was a visiting trainee, first of all kept referring to Matilda as ‘he’. Then didn’t really seem to know why we were there. Or anything about Matilda and her history. Then did not even examine Matilda at all (why the hell did we have to bring her?). Then went off to talk to a consultant, who was busy. Then came back and said that they will liaise with our pediatrician but it will probably be 3 to 6 months. We left feeling incredibly frustrated with what was a complete waste of time. We have left messages for our neurologist and our pediatrician who will hopefully get back to us in the next couple of days to let us know what is happening.


But as I said, yay for photos! And this one is my favourite.

Thanks for letting me vent. I am feeling better than I did earlier. Photos, and wine, have helped! If I don’t get a chance to write another post before –

MERRY CHRISTMAS EVERYONE!!!! Wishing you all a very happy and safe Christmas and New Year.


Thank you

16 Dec

Thank you for all your wonderful comments, emails and messages after the last, fairly sad, post. They are an absolute balm and I appreciate them so much. There will be times of sadness, times of okayness and times of happiness. And that is just the way it is. We can feel you all out there though and that drives us everyday.

We are trucking along okay – very much looking forward to the end of the term and a well-earned break. I will be working in between Christmas and New Year but then have the whole month of January off! Tilda is getting a bit tired so a break from the hurly burly world of school (and waking up for school) will be most welcome.

We had our appointment with the neurologist yesterday and have a plan to try a new medication, Keppra, for Tilda’s seizures. Our neurologist showed me a copy of Tilda’s last EEG – out of the 10 seconds that were shown, there was only 1 second where she wasn’t seizing. I honestly don’t know how she copes with all that is going on her head. And with a smile! I am eternally grateful for that.

Our next appointment is Tuesday to talk about the dreaded PEG feed which has now become inevitable. Tilda’s eating had improved but unfortunately, she has gone back to her old ways, only eating dinner 1 night out of the last 3 and that was because it was Popcorn Chicken! Child cannot live on Popcorn Chicken alone. And I am sick to death of the very sight of the stuff! We did get a great recommendation for a supplement that has no flavour so can be added to sweets and savouries. I shall certainly be giving that a try.

To close, I would like to show off some more photos taken by the lovely Nancy from who certainly captured Tilda’s incredible spirit. These photos will be treasured forever. Thanks Nancy! And thank you to the amazing Heartfelt organisation for making it possible. Heartfelt bring together amazing photographers (like Nancy) to give the gift of photographic memories to families like ours.

Not always roses and sunshine

11 Dec

I woke up sad today. Tilda was smiling as usual but it failed to arouse the usual good cheer. I watched her as, just like every morning, she flopped around the bed. A gargantuan effort to get to sitting position followed by uncontrolled falling. Sometimes on my head. I keep one hand on her chest to keep her upright. Falling does not deter. More of a struggle to standing, holding on to the the bed head. Success celebrated with jumping. Followed swiftly by falling. I grasp at her pyjamas to stop her falling off the bed. Cuddles and smiles. Inside, I am so sad. I get frustrated. Stop jumping. Knowing you will fall. Stop struggling. Don’t stop struggling. Don’t stop fighting.

I logged on to Facebook and found that another child in the Batten community passed away this morning. Passed away. Earned her angel wings. Left us. Died. Stopped.

A confession. Whenever I hear of a Batten child dying, I become quite obsessed with finding out as much as I can. I search for clues. Watch videos on Facebook. How old were they? What type of Batten did they have? At what stage? What were they like just before they died? Are they similar at all to Tilda? Could she be next?

There are no answers because every Batten child is different. Whatever I do, I cannot stop this. There is no cure. There is no miracle. There is only looming and sometimes, like today, that looming becomes too much to bear.

So I have done some cooking. Thank you to Mum for the stew recipe – Tilda tried, tested and approved of. I am not a cook at all but there is something very comforting about chopping onions and carrots.

We are having a quiet day. Matilda is watching enormous amounts of television. She is still in her pyjamas. That’s ok. She is not sad. She is not in pain. She is extremely content. Perhaps a bath later.

Perhaps I will bake a pie.

Neurology appointment later in the week. Before that, a get together with the mums of Tilda’s class. Including the mums of Charlotte and Dylan who passed away late last term. It will be nice, and hard, to see them. I am sure there will be lots of tears and hugs.

Tears and hugs.

Adapting and living a new normal

7 Dec

Sometimes I look back over the last two years and can’t believe what we have gone through. I wonder sometimes whether its gradual unfolding gave us time to get used to things. It wasn’t a one minute she was fine and the next she wasn’t. It has been a long road and we have seen her change so much over that time.

Up to age 2 –  Normal child. Normal child behaviour.

Between 2 and 3 – ok, some speech issues , nothing too drastic, a bit behind, start speech therapy

age 3 – ok, seizures. Well, they suck but epilepsy is treatable, we can deal with that.

age 3 1/2 – ok, not just any old epilepsy but Doose Syndrome – one that is catastrophic in medical terms and difficult to treat. Alright, we can deal with that too – we will try medications, diet, whatever might work. If all else fails, there is the chance that she could grow out of it.

age 4 – ok, medications and diet is not working, she has a particularly severe case of Doose. Looking at long term developmental problems

age 4 1/2 – shit. shit. shit. shit. shit. shit. Shitty bloody Batten bloody shitty Disease.

And even since the diagnosis, things have changed. Matilda has stopped walking on her own and she has lost most of her words.

What strikes me most about all of this though is the ability we seem to have to adapt to each new change.  Each new phase becomes our new normal. We work out what is possible and what works and we adapt.

It seems strange to say, but life at the moment is actually really lovely. This peeling back to the simple aim of making Matilda happy has led to a fairly blissful existence. We know what is possible and we work our days around that. Matilda is cheery and funny and happy.

Exhibit A:















You just can’t be unhappy with that. This photo is courtesy of the very lovely Nancy Morrison from who came and took photos of us last Saturday. More to come!

Even eating , which has always been a battle, has just recently come to a pretty good place. The wonder of cereal plus Pediasure means that we finally have something we know she will eat that also fills all of her nutrition requirements. I have just come off the phone with the dietician from the Royal Children’s Hospital who is going to do some number crunching for us to get the exact ratio for her needs. To date, I have unscientifically been dumping a few scoops of Pediasure into her Vita-brits of a morn so would like a bit more direction!

Speaking of Royal Children’s Hospital – we had our first visit to the swanky new building yesterday. It is very nice but very chaotic at the moment. We were actually the very 1st appointment with our pediatrican there so it was all a bit all over the shop. Suffice to say, the appointment was fine. There is nothing that she can really tell us so I think I will cancel the next one unless there is something vital that needs discussion. It seems a lot of effort getting there etc for not a whole lot of gain.

Next dr’s appointment is with the neurologist next Thursday where we shall be discussing Tida’s seizure vs ataxia activity but before that we have much more fun things to do. The first of which is a visit to the zoo tomorrow night. Should be great!


Still no comedown!

6 Dec

Nope, the fun has just continued! Tilda is in absolutely terrific form at the moment – in excellent cheer, eating pretty well (for her!) and generally a joy to be around. Except for medicine time but even then, she is quick to forgive.

The week started quiet enough – after the HUGE weekend, we thought some quiet time was a good idea. But it hasn’t lasted.  Last Thursday night, the fabulous Glenallen School had their primary school Christmas Concert which was just gorgeous. The school choir sang songs, kids played with balloons, families joined in the singing and Tilda spent a lot of the time on the ground admiring her shoes!

Please excuse my terrible singing!

Then the big excitement of the weekend was THE WIGGLES on Saturday!!! Thank you very much to my lovely uncle Jeremy for the tickets. We had amazing seats and we had an amazing time. Those guys really do know their stuff.

We are off to see the Developmental Pediatrician this afternoon so shall report back.


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