Procrastination... Living in the 1%

If it were an olympic sport I would win the gold medal, I'm sure.  I don't know how many times I have sat here, starting at the curser flashing at the top left hand side of the blank screen, only to close the page and walk away.  Too caught up in the now to put it all down in words.  Even now words are not coming easy.  What to say, there's so much to tell, but quite simply, what to say and where to start?

It has now been 4 years since Aerynn was in the Royal Children's Hospital in Melbourne, and we still have no definite diagnosis.  Well...  apparently we are very close, around a 99% certainty... but we've been there before only to have it reversed...  and this diagnosis is one I don't want her to have, so until it's 100% confirmed I'm going to dwell in the 1%. 

It has also been 2 years to the day since Aerynn experienced her first identifiable migraine.  Two years that she has been plagued by the "scratchy bug" which lives in her head.  

She is still a medical enigma.  No one can really tell us what is happening, or why...  The frustration of not knowing keeps eating me up from the inside.  We know she is unwell, we know her symptoms are increasing and slowly we see some progression of whatever this is that is stealing my little girls childhood.  But even if this new suggested diagnosis is correct, it's not something we can cure.  We can hope that there may be some way to delay the inevitable though...  

To type it here makes it seem so real.  I have been toying with saying it out loud at times...  having the words on my lips cause that all too familiar choking feeling come back to my throat.  I've even broached it on Facebook once or twice to see how it fits.  It didn't fit well though.  I mean, it fits Aerynns clusters of symptoms incredibly well, but putting the name with my daughters name feels wrong.  

Even if the neurologist confirms his suspected diagnosis when we see him again in 2 weeks, it won't be the final diagnosis.  There are many tentacles to the beast that is mitochondrial disease. We will know that she has been ensnared by the beast, however not sure which arm we are dealing with for some time yet...  

There! I said it.  I typed it.  It is there in black and white.  

Dr Google is not gentle with his information in Mito or the prognosis of children diagnosed with it.  The scary thing is, almost every week we see some small sign that she has some deterioration of her condition.  I don't want to see it, and them *bam* there it is, staring me in the face...  First it was tingles in her fingers, then they spread to her toes...  then she gets numbness and can't carry her weight...  And we used to blame her being "silly" for reacting to the tingles and numbness.  Heap on that great big serving of momma guilt there.. right on top of me, because I deserve it.  

So I guess this is the update that is long over due on Miss A, but not the update I wanted to write.  I don't really know how much longer I can live in the 1%age of ignorance...  ignorance isn't bliss in this case, because deep down I know that this finally fits.  After all, I was the one pushing for a diagnosis and answers.  We finally have one of the best neurologists in the country who thinks that he has the answers on my little girl, and our paediatrician says if he thinks that this is what we are dealing with, then we can be very certain it will be correct.  I have so many questions, however, I dare not think too hard about the possible answers...

Did I bring this upon us by wanting a diagnosis and answers so badly?

How does one come to terms with the fact their daughter is never going to be getting better?  

How does one deal with their child having what is a terminal illness?  I mean, yeah, life itself is a terminal condition... but... 

I can't do this right now.  Which is why I am living in the 1%... pretending that my world isn't about to collapse all around me.  Living in the bliss of ignorance, delaying the inevitable...  I don't think I can live here much longer, but I'm going to try to for as long as I can... I simply can't deal with the alternative at the moment. 

Happy 5th Birthday Little Princess

Our little Miss A, 

Your birthday has been and gone.  Today is August 10th, two days ago you turned 5 years of age.  There was a stage we weren't sure you would see this magical number.  You have proven so much strength and resilience.  Sure, there are challenges, but we get through them, and every morning is a clean slate and a new day to discover.  

Aerynn, you are a headstrong Miss who knows what she wants.  Your teachers believe you are gifted beyond your years and work on another level compared to other children your age.  Your brain has compensated everything your body has limited you with, but in saying that you are doing soon well physically - almost reaching the accepted growth charts!!!!!  Our little princess is growing!!!!  

So for your 5th birthday we had a big celebration - 3 weeks early. Nanny and poppy came to visit from Tasmania so we had a birthday celebration then so they could join in the fun.  Most other little girls would love princess parties or have a disney or frozen theme, but no, Miss Aerynn insisted on a high tea with her friends.  Complete with cucumber sandwiches, cupcakes and mini cups of tea.  We went racing around town trying to find a tea set for you to use, and discovered a set which belonged to a 90 year old woman.  She had it for quite a while.  It was white, and so fragile...  you could see through the china it was so delicate.  A light blue decoration was on the outside.  I loved it, it was a reflection of you... so pale, fragile and delicate with soft blue eyes burning with intelligence and a fire from within.  It was simply perfect for your high tea.

Nanny made your birthday cake.  You were very indecisive with what you wanted...  apparently a 10 layer wedding cake was not in our budget - much to your disgust.  Instead you allowed us to talk you into a pretty castle cake, complete with turrets. Nanny stayed up ALL NIGHT decorating the cake, making it perfect for our perfect little princess.  It received so many compliments!  Mummy mad the cakes to go inside, one double chocolate mud cake and a butterscotch mud cake.  There wasn't much left by the end of the afternoon, so it must have been very tasty.

For your birthday you asked for a baby born doll.  We found a fairy one in purple - your favourite colour.  Baby born has had several different names since she has been received.  Stacey, Leesa and Toby likes to call her "poop poop".  She is a very loved dolly.  You push her around in the pram that nanny and poppy gave you and feed her from the magic bottles.  I hope that you continue to love Stacey/Leesa/Poop Poop for ever and a day and remember these times you spend playing "mummy" with your baby dolls.  magical times in any little girls life.  

So Miss Aerynn, on your 5th birthday we wish you the best.  We with you everything, peace, love, a happy time on earth, we wish you enough. We have no idea how long you will be with us.  We don't know what the future holds for any of us.  Go ahead and write your own story.  Continue to fight, continue to defy the odds and how them the person you are inside.  Our strong headed little princess who confuses and astounds everyone is growing up... Continue to do so... be our strong and beautiful little Aerynn <3 

Love Mummy xxx

New Year, Long Overdue Update

2015 has come and gone and we are now several days into 2016 so way overdue for an update.  

As I type Aerynn is throwing a massive tantrum so well on track of typical 3/4nager behaviour.  

Medically, Aerynn is still pretty much as she has always been.  We have changed her medical team and has new paediatrician, endocrinologist, immunologist, neurologist and therapists up in Adelaide through the Adelaide Paediatrics Clinic.  I can't recommend them highly enough!!!

She still has wonky blood sugars, however we are not checking them as stringently as we once were.  Her highs and lows really do effect her behaviour and mood, so I can usually tell where she is sitting with BSL, so if it continues for any length of time or she deteriorates I will check it so we can advise her doctors at her next appointment.  

Food wise we are treading carefully.  Not too much of the same foods as she still gets her tummy boating, diarrhoea, vomiting, mucous, ickiness in general and headaches.  she has started transitioning off neonate as her main source of nutrition which I hope will mean more sleep for me if we can maintain her BSL's through the night without her 2am feeds.  

We haven't had any obvious seizure activity increase - which is good!  She has a neurologist appointment in Adelaide next week so I am praying it will be a clear EEG and we can remove epilepsy from her query sheet.  She has vacant episodes still, but nothing really super scary or alarming for me.  Migraines are still an issue however, headaches are usually 2-3 per month with 1 of those developing into a migraine.  She has had an increase in medications to help keep them to this reduced amount.  I know it sounds higher than should be acceptable, but we need to balance medications carefully considering her age, size and development.  We don't want to create a zombie or get her reliant on strong meds at this stage.  Hopefully over time these headaches will decrease and we can wean her off the meds. Well, that's my long term hope!

Growth is slow and steady.  She has cracked the 1 meter mark!!  Woohoo!! Aerynn doesn't stand out as the little person in a group of kids the same age anymore.  She is still tiny (wears size 2-3) and the smallest in the group, but she holds her own.  Probably being loud and bossy helps ;)  Her hair has also started growing nicely throughout 2015 and it has passed shoulder length!!!  It's still thin, but we can get pony tails and clips in now to help it look less sparse :)

We had an intensive vision test towards the end of last year with

a behaviourist optometrist.  No issues were detected - yay!!!  Dentist is tomorrow and then another general health check at the paed next week.  All ready to start Kindergarten in February!!!!!

School in itself is a big and scary thing for us, knowing that we are posing her immune system to all the bugs and the likes that comes with kids being in a close environment, but I'm trying to be positive and hope that she won't be as susceptible as expected.  The school has been fully updated with her story and we hope that all the appropriate steps have been covered in the run up to starting school.  Time will tell if mainstream school (albeit super small) will be workable for Miss A or if I need to look at homeschooling her.  It's a waiting game and a big lesson in trust, faith and patience! 

So that's pretty much where we are at the moment.  A year of NO HOSPITALISATIONS!!!!  Lots of tests, investigations and more questions, no answers but we're pretty used to that now.  

Needing Answers

And soon... please! 

It's heartbreaking to see our little girl in pain and not understanding why she is hurting.  We can't tell her why her head hurts, and why she feels sick all the time.  We can't tell her it's going to get better, we simply don't know what tomorrow is going to bring.

We can, however, enjoy the times she is happy and bubbly and downright cheeky.  We can enjoy her laughter, her smile and her gorgeous pixie voice.  

We've had another rough 12 hours with Aerynn waking at 12.45am with disorientation, lots of scared tears, glazed over look on her face, non-responsive to questions, full body tremors which slowly eased to just hand and feet tremors by 1am, when she fell into a deep deep sleep and was not stirring even when being picked up and moved.  She slept 9 hours straight...  woke, bright as a button... only to be sleepy within the hour and needing 3 naps during the day.  No headaches and no remembering what happened during the night. 

We don't know what is happening.  

We are worried, but trying not to worry at the same time.  

We can increase her medication for migraines to the full strength if we feel it necessary... But I don't know if these episodes are migraines or not.  We suspect they are seizures.  

We have an urgent appointment with her paediatrician in 2 weeks time... it can't come soon enough for me!

Upping the Ante

Our little girl isn't doing so well at the moment.  She has had an increase in the intensity and frequency of her headaches in the past fortnight in particular and we suspect she may also be having some seizure activity :(  We have always been on the alert for signs of seizures as Hubby had epilepsy as a child and there it is on my side of the family as well which increases our risks of the children inheriting it quite substantially.  

Today we happened to have a GP appointment just as Miss A was in the middle of a migraine.  It wasn't pleasant travelling the 38kms to town to have her seen but I'm glad we pushed through.  

On arrival Aerynn promptly vomited several times at the reception desk, so I guess they knew we were being serious when I was saying she was unwell with a migraine.  Poor little mite ended up moaning and unable to contain her pain :(  It's so hard to see her like this and not able to help her...  

Our lovely GP saw her and agreed that it was more than a headache, our little girl is having migraines.  She also agrees that we are very possibly seeing seizure activity.  We have an urgent referral for her to see the paediatric neurologist who visits here every 3 months.  I believe they are here in December so hopefully if they haven't requested she be seen in Adelaide by then she will see him then.  Aerynns medications have been increased and we will get her up to the maximum dosage of it in order to try and reduce her migraines and their severity.  She has been prescribed painstop to help deal with the pain, unfortunately there are no anti-nausea meds she can take in conjunction to her meds so we are hoping she levels out soon.  

Our Gp was also not impressed that her next appt with the paed is next May and has called the peads rooms requesting an urgent appointment within the next month and to be for Aerynn to be under the care of another paed

whilst our regular paed is on maternity leave.  

We are trying not to worry too much.  We don't know what is happening inside her little head at the moment but at least the neurologist can rule out anything sinister and run the necessary tests to help our little girl.  

Headaches..

Aerynn, October 2014

Our Aerynn still isn't in peak condition.  She is slowly growing and appears on the surface to be a wonderfully happy and well adjusted 3 year old but she is still small for her age, still has dietary limitations and on neonate formula and supplements.  Her blood sugars are stable most of the time but have a tendency to drop super low or swing high and she is still getting severe stomach pains and migraines.  

She has beens started on some medication to try and combat the migraines.  Her paediatrician believes some of her sever stomach pains are related to her migraines as well.  She has had a week of the new meds and already had another migraine last night with swinging sugars, so we are back starting BSL, food and activity monitoring to try and work out any triggers to these attacks.  

Picking Bunny Tails (what Aerynn calls "clams") October 2014

In the meantime she is still our gorgeous Aerynn.  She is blossoming into a wonderful character full of love, a wonderful big and little sister and knows how to put us in stitches at the oddest times :)  

October: Dwarfism Awareness Month