Sorry Sammy :0(

We live 5 minutes away from Sam's garden (the cemetary). We visit often. We
NEVER drive past without popping in. But today, I said to Chris I couldn't go there.

I am afraid I will just break down at Sam's . You see, it's a family plot. So there's room. But it was meant to be for me and Chris a long time in the future. Not Hope!

I don't want to have to have pink writing added to the pale blue. To have to make room for more toys :0( I really don't have the strength.

I know noone imagines they would. But Before, well I guess I didn't give it much thought. I never really believed Sam would die (though equally, I also never imagined Sam coming home). Last time, we kinda just got through.
But now, I know
And I know I can't do it again

I love you Hope
I need you
I promise to always look after you
Forever
I'll spend the rest of my life caring for you
I'll never resent doing that
Or wish my life was different
Just don't ever leave Mummy
Please Princess Plonkey Pie

Pyruvate dehydrogenase deficiency

That's what I think it is. Now I'm wishing and hoping it's MELAS. But so
Much seems to ring true of pyruvate ...
Microcephaly- check
Ventricular diAlation - check
Lactic acidosis - yep
Hypoplasia of corpus callosum-
check
Loss of head control - kinda
Failure to thrive - yeah previously
Seizures - yep

If it is this, and I am on my knees begging and praying it's not ... We won't have her long :0(

Shit

The only thing keeping me going is the hope that I am wrong.
That this would have been picked up on the amniocentesis (but I'm not sure that it would have because although genetic, it is evident on the Mitochondria and not the chromosomes- I think)

Apart from loosing my precious lil girl, whom I cannot imagine life without...
I can't stop thinking about Jordan and max. How will they cope? How do I tell them? How can they recover, go to school, be children, be happy?

Oh my, how my hopes have changed over the past few months:
In the beginning I hoped the medical profession were wrong.
I hoped she'd walk and talk
Live a normal life
I said goodbye to those hopes a whole back.

I them hoped for independent living
Mainstream schol
For her to be happy
But knowing I'd always care for her
I said bye to independant living and mainstream school a few weeks back

Earlier this week,
I hoped for adulthood
Teenage years even
I still do
But think I may have to lower my expectations and hopes again

What is left?

Just to hope I'm wrong!

Her name now seems rather ironic :0(

Keeping busy

I haven't had much chance for feeling gloomy today; Max had a 10:30 kick off so it took great effort to get out the house that early (God, I'm i'm in for a shock when they go back to school on Tuesday lol).
I managed to give her honkness her cereal, but her 1oz of infatrini with her meds in ...well, it's beyond a joke. I took it to footy, I tried, Jordan tried, chris tried, and even Sam (a fellow soccer mum) tried. The match was over and there was still more than 3/4 of an oz left, so we came home and she finished it.

I was concerned that her getting the full dose of meds nearly 2 hours late would result in lots of seizures, but thankfully not. Infact, and I suppose I overlooked this is my doom of yesterday... Yesterday was ....
SEIZURE FREE!
And touch wood...
So is today (so far)
That's on 800mg of vigabatrin.

Hope was given some cash my her god mother the other day. I was going to bank it for her, but yesterday decided to go and buy some nice things.

After much deliberation and trying out, we bought a 'babasling'. It's fab! Today Honk has been helping me round the house whilst breast feeding! And then in 2 other positions too so she can look about. I'm really chuft with it.
We also bought a BEAUTIFUL grey knitted Humphrey's corner dress. It was more than I'd usually spend on an outfit for Hope, but soo worth it!
And we bought some lil noisy toys for her too.

We've been to town too. A bit of last minute getting new school stuff.
Then we've come Home and hope has just been in a delightful mood so we've been doing lots of playing.

Hope has been rolling from her front to her back (mainly because he Hates being on her front). I've been getting her to look towards sounds (not much I know, but this is something she really needs to work on). Also, I've had her sat right up in her rocking chair, which positions her feet just at the right position for her playstation ( the fisher price superstar thingy). She seems really pleased with herself when she makes the music go off.
Infact she enjoys it so much, that we are all curently sat in darkness listening to 'if u r happy and u know it...' so she gets the full effect of all the flashy lights.
She's really going for it now!

Now, u may think I'm insane, but we r all pretty convinced Sam was playing with his lil sis earlier. She was in her rocking chair in Sam's corner ( where his photos and special things are. Hope had fallen asleep in her chair so I turned off the swinging motion. A few minutes later it was back on. Chris turned it off. It came back on. Then Max turned it off. Guess what! So we decided it must be Sammy being a monkey. How cool :0)

Over and out
Xxx

Dead inside

Can't believe there can be something sooo wrong :0(
I Just can't imagine life without Hope
Or seeing her get really poorly

My heart feels dead! I'm just going through the motions of life. But it's not fun

I keep trying to think positive. ...That perhaps it won't be that bad...
But they r bad!
I can't see past the dark clouds
My heart is heavy :0(
I wanna cry, but can't for sake of kids

The more I read, I just can't pin down which one of the 4 she has. But I suppose it explains a lot; her failure to thrive, her need to feed almost constantly, the fact she still doesn't sleep through the night on a regular basis.

I just hope we get the results soon
Then I can learn more
And find out how long
:0(

I can't do it

I can't do it.
Again.
I can't....
.. kiss 2 photos goodnight.
... Have 2 special stars in the sky.
...Kiss cold lips goodbye.
...Watch my dreams lowered into the ground
...Break Jordan and max's hearts again
...Watch them grieve
...Helpless
...Unable to fix them
I just can't


I know I said I'd not research/obsess anymore.
I have.
It's not good
There are 4 possibilities
None are great
2 are nasty.
Painful. Premature ....
1 I can rule out because of the ultrasound revealing normal kidneys and liver
There's one I guess I'd rather it be, but knowing our luck, it won't be.
And still, that one is not good
Far from it

To top it all, 2 of the 4 involve an auto recessive gene pattern
Which would mean I'm a carrier
All my kids would have 25% chance of having it
Or being carriers

What on earth did we do to deserve this?
This life stinks

Even Chris is walking slower
He knows we can't do this
Again


I think this time, would be worse

If that's possible

:0(

I was so prepared to care and look after Hope. To dedicate the rest of my life to looking after her. Now I may not even get that honour

:0(

Life goes on

Well, I've pulled myself out of my
Big black hole once again. No doubt I'll find myself back in it again some time soon, but until we get results, I have to get on. I know we are not going to get good news, but until we know which condition it is, I need to stop researching/obsessing etc. But at the end of the day, there is something seriously wrong. The lactic acidosis which has led dr ch down this path is a process involved in rigor mortis :0(

We had an early start today. Lil Max (8) was picked up at the crack of dawn and is now in London to play in goal for Swindon Town F.C against Fulham. Incase u don't already know, Max was scouted last year and has quite a reputation for astounding goalkeeping.(Proud mummy gloat, but think I'm allowed).

Jordan's Auntie Tracey offered to have her today. Thanks Trace. So they were going to town and swimming with lovely lil Faith (my niece).

Which left Honk and I free to go to her first physio and occupational therapy assessment. I'm not quite sure what I was expecting, probably excercises and manipulation, but it wasn't. The 2 ladies were lovely and very tuned in with H. They basically just played with her, seeing what she could do. They said I am doing all the kind of things I should be, that Hope is quite strong. She proved her dislike of being on her front. She impressed them by rolling over. We need to work on head control. Which I knew. But they said her head doesn't lag, her main problem is midline control (keeping it straight). But then Hope is gonna struggle here because the cerebellum
(which in hope's brain is hypoplastic- under developed/small) is responsible for balance. Plus because she is visually impaired, she isn't getting visual input to help her. Overall, though it was a nice experience. They are going to see her again in October.
Oh, and they are getting her a special chair (like Jude and Kendall's). They think she's held too much- lol. Can't say that's going to change much :0)

Had a bit of an embarrasing moment just now. Anyone who has breastfed will sympathise. Just waiting for bus home and felt the 'let down'. Because we've been out it's been a long time since Hope fed, so I get in the door and see I have 2 big wet patches on my top. Oh well

Well, I have to say, I have been overwhelmed by all the messages of support and kind words. I actualy had a really nice message last night from an uncle I haven't seen for many years (hello uncle rick and kim), and so many messages from friends just saying that they are thinking of us. My phone is going off even more than usual. And that's a lot. I know you all feel powerless to help us. And really there is nothing anyone can do to change how things are, but it does mean a lot. At some point, I will have to go through replying to u all, and asking for your permission for me to copy and paste your messages onto here. I'd like to do that, so they are all in one place iykwim.so if u r reading and u don't mind me doing that it would be great if u could just drop me a message saying u don't mind, it would save me a lot of work. On a less positive note, I suppose, there are some people I'd have hoped to have heard from, and haven't. But hey ho.. U win some ....

My house is an absolute shambles! I need a maid. Or something. And a chef to mention it. But I guess once hope has finished feeding, I'll crack on and do my best whilst my heart is not so heavy.

Much love
Xxx

I feel like I'm falling

Me again. 2nd depressing blog in one day. But this is my only release. I want to scream really loud. Bang things. Cry. But I can't. I can't even talk to anyone because I don't want Jordan and Max to hear anything. So I'm here. Tapping away on my iPhone. Hope is at the milk bar (ie me) as usual. Jordan is on the laptop. Chris and max have gone out to get trainers and lunch for max tomorrow. (my lil max is off to London tomorrow by himself. Chris couldn't get the time off work, and Swindon Town can hardly play without their keeper, so he's going with the centre of excellence director).
On the surface, everything seems normal. But it's not!

I feel like I am grieving. Again. But inside. I feel like my legs could give way. Not sure how long I can keep up...will just keep trying for the sake of the kids.

We visited my gran today. She'll be 80 this year. I didn't tell her anything. As much as possible I've avoided sharing bad news about HOpe with her.
But she knew.
She was saying how perfect her skin is. The softest ever.
So bonny
Such blue eyes
That she's worried about Hope.
That she doesn't want to upset me
But ....
That I should just enjoy her
Because we don't know how long she's got

:0(

I keep thinking about that. I mean we don't know it's M.E.L.A.S yet... That's what Chris keeps saying.
But, as far as I can see, and my understanding is this...
Her body is not functioning properly
The acid will build up in her organs
And fail :0(
How long? Years? I fooking hope so. But how many? 5? That's not enough. Nor is 10. Or 30.
I shouldn't be thinking of this. I don't want to


I keep hoping they are wrong. But it's not going to be nothing.

Thank you all for the lovely messages by the way

Feeling proper sorry for myself ;0(

Me and Hope are still in bed after a rather sleepless night. I don't seem to be able to muster the energy to get up and face another day. All seems pretty pointless

Somewhere on Hope's blog there's a sticky that says 'everything happens for a reason...' think I'll remove that. How can there be a reason?

Was it not bad enough that I had a baby boy who died?
And of all the days in the year, he died on Christmas day!
Was that not bad enough?

Then to concieve, and dream of Hope
And them find problems with her brain.
I thought ventriculomegaly and cerebellar hypoplasia were pretty bad.
We were told she may never walk or talk already.
So the eplilespsy...
Well I thought it couldn't get any worse
How wrong
What did I do to deserve this?
Why my babies?


Dr CH did tell me not to go looking it up. Not until we know more. But I'm not the kind of person that can wait. My motto is 'knowledge is power'. But actually that's crap! I am powerless. I can only be there to love her and witness what I fear most.

I hope so much they are wrong.

And my poor big babies. Poor dolly and moo. They've had so much sadness already. How do I tell them? Do I tell them? No is the answer. I will protect them for as long as possible.

So, I put on that brave face and carry on. Wear the familar mask. Smile. Laugh.
Enjoy

Xxx

I am numb.
Still don't think it's suck in.
I think it's worse than I could have imagined

We've been at the hospital all afternoon. Jenn, holly, jocalyn ( other blog mummies of babies with similar problems)u r the only ones who know how my head feels at the moment. Exhaustion. Information overload. Detatched? Headache etc

Today hope has had:
Blood tests. Oh she sobbed. It took 45 minutes of trying different veins and squeezing b they got all they needed. Urine samples taken
An ultrasound to check her liver, kidney spleen and heart
An ECG to check her heart

Oh and to top that, next week she has to have a lumbar puncture :0(

Why r they checking her organs u may wonder. So did I. Wasn't it enough she had all those problems in her brain?

Her previous blood test showed high levels of lactic acid in her blood. Anything under 2 is considered normal. But one would expect it to b under 0.5 in an infant.
Hopes was (and reconfirmed today) 5.75
This acid, will build up
In her organs. Eventually.

So. She has a rare metabolic disorder

Every cell in her body is wrong

Not as it should b

Her body is not functioning properly

There is no cure

Only treatment

It will take 2 weeks before we get the actual name of the syndrome. But dr ch thinks it's 1 of 4. All of which are fairly similar. It's bad news. He suspects M.E.L.A.S syndrome. I'm hoping
The other things he suspects r much nicer. And that it's not this

Here r some bits of info copy and pasted from net

MELAS is a condition that affects many of the body's systems, particularly the brain and nervous system (encephalo-) and muscles (myopathy). In most cases, the signs and symptoms of this disorder appear in childhood following a period of normal development.[3] Early symptoms may include muscle weakness and pain, recurrent headaches, loss of appetite, vomiting, and seizures. Most affected individuals experience stroke-like episodes beginning before age 40. These episodes often involve temporary muscle weakness on one side of the body (hemiparesis), altered consciousness, vision abnormalities, seizures, and severe headaches resembling migraines. Repeated stroke-like episodes can progressively damage the brain, leading to vision loss, problems with movement, and a loss of intellectual function (dementia).

Most people with MELAS have a buildup of lactic acid in their bodies, a condition called lactic acidosis. Increased acidity in the blood can lead to vomiting, abdominal pain, extreme tiredness (fatigue), muscle weakness, and difficulty breathing. Less commonly, people with MELAS may experience involuntary muscle spasms (myoclonus), impaired muscle coordination (ataxia), hearing loss, heart and kidney problems, diabetes, and hormonal imbalances.



There is no known treatment for the underlying disease, which is progressive and fatal. Patients are managed according to what areas of the body are affected at a particular time. Enzymes, amino acids, antioxidants and vitamins have been used, but there have been no consistent successes reported.

WhAt else is there to say?

:0( my baby

Xxx

Preparing myself for bad news? :0$

I've just had a phone call from Hope's lovely neurologist (dr Ch). He wants to see her. Tomorrow! He said he has some results from some blood tests he wants to discuss. Plus do some more tests. Boo hoo, I hate blood tests. Plus he wants a urine sample. So it may be a long afternoon :0(

He asked how Hope was doing too. I told him that I had upped the meds again today (that's 800mg now) and that the seizures were still creeping up, and hope is having between 30-40 spasms a day. He said that's too much still, so I think we may end up discussing changing the meds a bit earlier than originally planned.

Oh god, I am so scared. I was shaking after the call, but now I just want to cry :0( but can't because I don't want Jordan and max to worry .

Earlier today we had the leader and a playworker round from koalas (the special needs playgroup). They were both lovely and I'm looking forward to starting In September. It will be nice to meet parents in similar situations and their extra special babies and children.

They asked me whether I had made a claim for disability living allowance and carers' allowance. To which i replied I hadn't. I guess that's because I don't want to have to. I'm still hoping I suppose that she'll all of a sudden be fine. That she won't be entitled to it. But who am I kidding? Only myself! And since my maternity money ends this month, and there's no way I can return to work yet, the extra money will help. It's just I don't want to be eligible for it :0( either of them; disability allowance because it means hope is disabled, and carers' allowance, because I am not her carer. I'm just her mummy

Oh, for an ordinary life ... :0(

Xxx

Disappointed :0(

Not with honk of course! But I said a week ago or so ( I think) that we had a photographer round to try and take some photos if her royal honkeness. So far 2 photo shoots have been fruitless; they are unable to capture her true beauty, so I hoped this lady might have had more success.
Now usually, I slightly dread viewing photos taken by professionals. You know they are gonna cost you a bomb! How to choose when there are sooo many pictures you love. Not so :0(
There was really only one good photo. Well that's not true. There were some beautiful ones of her sleeping, and of her lil tootsies. It's just I really want that perfect picture; her smiling and looking in The right direction. So we haven't spent a bomb. In fact we just claimed our complementary picture. (will add to blog when it comes). The lady has kindly offered to try again in a few months. She knew herself she hadn't really captured Hope.
Oh well, just another one of those things I
Previously took for granted. Nevermind.

On a more positive note, H is now tolerating her meds and is back to her noisy smiley self today. She seemed to have lost her appetite too the last couple of days, but i now realise that that was probably also to do with the meds increAse and the sleepiness .

We've had a lovely day today meeting with some old friends at Coate water. We had a picnic and beautiful sunshine!

Thanks for reading
Love always
Xxx

Lazy blogger

Sorry, I'm slacking. I blame the summer holidays. We're soooo busy everyday, then some evenings, plus I am trying to finish my uni course, and am pooped!

Last night hope came along to weight watchers, and our first stitch n bitch group. She was of course the only baby there, but everyone seemed to understand why I am so over protective of her and won't even leave her with her daddy.

The seizures are still here, so I've upped the meds yet again. She's now on 700mg per day. Once again, she's zonked. Chris is currently trying to wake her, but she's not in the mood for playtime :0( hopefully she'll be back to herself more tomorrow.

The honkeyplonk blog hit 2000 visitors today. That's a lot. Especially when I have no idea where these 2000 hits came from. Ok, so I know a few people who follow...but 40+ visitors today already. Would love to know who you all are, so if u r reading, please say hello so I know u have been :0) and thanks for following.

Promise to b a better blogger soon. Must get some pics on too

Xxx

Consultant appointment today

We met with the lovely dr ch again today. All good news really; he's still pleased with her progress, though hopes for her to become completely seizure free- which we aren't, despite recently upping her doses of vigabatrin.
Mind you, her weight has increased significantly (6.15kg/ 13lbs 9oz) so therefore she needs more. She was on 500mg per day, but is now allowed as much as 900mg, so tonight I'm increasing to 600mg and hopefully that will sort her- at least for a while. I hate upping doses :0(
Her MRI has been reviewed (again). And she does have a corpus callosum! Though only a rudimentary one. But still that's better than nothing at all.

Her head circumference is below the 0.4th centile, which is a concern to me, but he's not as worried, nor surprised considering the brain anomilies.

We had to go for blood tests after. HORRIFIC is the only way to describe this. It never gets easier. They always try and get blood from her tiny veins in her hands, are never successful, upset her (and me) a great deal, and then resort getting it from her feet, which is equally difficult and timely.

Anyway, just a quick update. ... Hardly seem to get any chance at moment with chris and kids being off

Xxx

Oh, had photographer cone to house earlier to take pics. Was nice and hopefully got some nice pics. Having photos taken is something one takes for granted, but when your baby can barely see, it's difficult to capture their attention and get them looking in the right direction.

Xxx

Camping madness

O
M
G
It's dark, cold, and very windy. And we are in a tent in a field! Argggghhhh. Camping with a 6 month old. Crazy? Must be!

We've been fairly lucky so far, in that we've only had a couple of showers. We've had a nice day; Jordan and max did zip wiring, max did climbing, we've had a pub dinner, then made animals out of our bodies (h,j,m and me) for the kids caberet competion, played on the park.


Hope is loving the sea air, the loud noises from the pub and being out in her pram.

But now.... J and m have freaked themselves out tellin ghost stories, hope is screaming and not taking her meds, and our neighbours are playing robbie Williams a bit too loud for bedtime. And we've gotta sleep on the floor!!!!! On airbeds of course, but still.

Happy camping :o)
Xxx
Will catch up with you when we get home (2moro morning maybe? Lol)

Sleepy Honk

The seizures came back. Though we were lucky enough to experience some seizure free days not long ago, they've gradually been creeping back.
Yesterday me n da honk were in asda, when she had a prolonged seizure lasting about 20 minutes. I just held her, and missed the fact that even a shopping trip isn't normal. She had a few more spasms yesterday evening too, so when the evil seizure monster reared it's ugly head this morning , I decided it was no longer a glitch- we needed to up the meds.
Hope has managed on 4.5 ml of vigabatrin twice daily for weeks now, but we had the go ahead to give as much as 6mls. This morning I gave her 5mls. And don't I know it. She's slept all day. Waking (just about) for feeds. I miss her on days like this. I miss her sparkly blue eyes and loud shouts and funny laughs. I hope she's more herself tomorrow. Today
I am reminded of how powerful these meds are. Just an extra half a ml can have such an effect.

We've been back to Borders closing down sale. I scoured the shelves for more special needs parenting books. I found another 2. My collection is expanding. They do help though.
Nearly finished blue sky July. A beautiful book!

After that, I really really need to get back to uni books. I've really slacked recently. So whilst my fellow students are enjoying a summer break, i'll be playing catch up. But I need to do it. I want to. I'm enjoying the course- inclusion. Gives me a different perspective on things. I'm sure it'll come in handy in the future. Perhaps if I decide I want H to go to a mainstream school?
Xxx

Not much to report

We're back home from our jollies.
Hope ate banana today- loved it of course. No idea how I'll ever get her onto 3 meals a day. 2 fit in nicely, but the day doesn't seem long enough for 3 meals yet.
I think the seizures are creeping back up. I haven't been counting because we've been away, but will keep a closer eye, and if need be call her neurologist about upping the dose of vigabatrin.

I have continued reading blue sky July, and now feel greatful that Hope is infact much less disabled than lil joe- though some of her (the mother) thoughts and feelings and experiences are similar to mine.

I noticed today how well Hope's body responds to her massage. Just simple things; the way her toes curl as I rub her soles, the way she seems to now help me....seemingly pushing into the strokes. We were lucky enough to recieve a course of baby massage at home by a nursery nurse. I was keen because I knew the benefits, especially for special needs babies. We did it religiously, daily for the first few months, but now only 3-4 times a week. I must try and get it back into our daily routine!
Her legs are getting so chubby now, I cannot even fit one hand around the top of her thigh, yet she used to be sooooooo skinny! Bones with covering really.

That's all- v quick update. We're all snuggled up on sofa watching harry potter. I think the tapping from my iPhone may be slightly annoying everyone xxx