To Fontan and Beyond

I'll write this as fast as I can because John and I are just home for a little while as it's quiet time in PICU where Louise has found herself with not much warning.  It all happened on Tuesday when we got a phone call at about 3.15 pm to ask could we be in the hospital for 6 due to a cancellation and Louise's operation would be Wednesday, which was yesterday.  Of course we agreed and following a rushed packing of bags and showers and baths off we went to face Louise's biggest challenge so far.

First I'll tell you that our cardiologist told us on Monday to be under no illusions as to the extent of this operation...it's about as big as they get and much bigger than Louise's last two.  The recovery is much more complicated with lots of pitfalls, any of which Louise could fall into...including one...we won't even go there...

So Louise went down at 1.40 pm and after two hours to put in her central line and one and a half hours to get her onto bypass (I'm positive these problems which she has had every time are due to her PHACES Syndrome and the anaesthetist agreed it was probably so.) her surgery went as planned and we got to speak to the surgeon at 8.15 pm while the rest of the team were finishing the operation, sewing her up etc.  He's happy with how everything went and even went so far as to say her lung pressures which are VITAL are the best he has seen for years.

The specialist nurse spoke to us then and went into all the details and the plan for the next while.  The next week is going to be very very hard for Louise  as they are going to have to push her to get up and about faster than any other operation as it is important for the Fontan to kick in.  In fact they often ask the parents to leave for a while as the physiotherapists need to get her to work hard which can be heartbreaking for the parents.

We were told it'd be another half an hour, just long enough for us to go to the hospital chapel and spend some time where it's important.

Then at 10.15 pm the anaesthetist (did you know they are also the ICU consultants, it's their job to keep these children alive..oh wow!) rang and said he'd bring us in to see her.  He looked wrecked and laughed at the difficulties she had presented...all in a day's work I guessed.  Those guys deserve every penny they get.

Our little treasure was out for the count though the nurse told us she had woken just to say she wanted her Mammy.  She was aware of us on and and off and the rest of the time away with the  fairies.

Today she is much more awake.  She had a good first half of the night and then spent the rest of the night and the morning vomiting.  She's a poor miserable little pet...just wants a big drink and can only have a few sips.  She has never been refused a drink in her life before (that she remembers) so that can't be easy for her.  She's on a bit of support oxygen just with the nasal prongs but she's not in any pain.

 The surgeon came around and said he's very happy with her...her lung pressures are continuing to be excellent.  She won't get any of her drains out today as she's still producing a lot of fluid.  I have to bring up her slippers this afternoon as they are going to try and get her up for a walk.  Then she can get back into bed with her own home-scented sheets and pillowcases.

All the same, when we came, all the ICU staff knew all her sisters' and Peter's name and that it was John's birthday...Louise had told them!! I didn't even know she really knew that herself!  So even half doped on morphine she's with it! That's our girl!!

So...of course...continue to pray for our little trooper.  The surgeon's job is more or less finished...hers is just beginning.  And also for her siblings.  They're missing her and it can't not be stressful for them.

Jennifer xx

The Naughty Ones Need The Most Prayers

I came upon this picture and couldn't but think of Louise.

 My little imp is such a bag of contradictions. There's no way you could call her 'bold' (in the Irish understanding of the word), she is not bad tempered or defiant but she is definitely 'naughty'. All I have to do is turn my back and she has a sink flooded  "I'm just cleanin' the 'ink Mammy!", a whole jar of spice has been shaken over the dinner simmering on the hob "I'm just elpin' you bake Mammy!" or she has released the gerbils from their cage "I'm just lettin' dem out Mammy!" And yet she is such a gentle Mammy to her dollies and to her imaginary friend, her cousin Amelia, who is also her real-life friend, who is given every treat and toy. She invents entire scenes and stories with her sisters' Sylvanian families while they are at school. She is so delighted when the children come home but she's not adverse to using her strong personality, but only with the two next to her in age.

It's so funny because I remember when I was pregnant with her I prayed and prayed she would have a choleric temperament as opposed to a calm and peaceful phlegmatic. I figured that if she was going to face challenges in life a tough can-do personality would stand by her. Well that prayer was answered all right. She is a pleasant loving and endearing child. A shrinking violet...she is not!!

A few days ago she was feeling tired and crotchety and hit out at Peter, she announced to me 'Now I have go in the tor-ner!!' I have never used the corner a a discipline, I've nothing against it, I just don't use it. I haven't a clue where she got the idea but off to the tor-ner she marched. I think it was to pre-empt any punishment, she wouldn't give you the satisfaction!!

I love it! My father has 20 grandchildren and he has always said he loves to see children with a bit of fire as it stands by them in life. My little girl has fire!! She LIVES life!!!

Meanwhile...we wait...please join this naughty girl in prayer.

Jennifer xx

Neurology Visit

I'm just back from Louise's neurology visit and this is going to be a very short post because Wednesday is our busy busy day when our only chance of food is the slow cooker, which I haven't put on today so I'll have to hurry and rustle up something.

The Neurologist had a look at Louise's MRA reading and said it confirms the diagnosis of PHACES Syndrome. No more question mark over that. He hasn't seen her actual MRA yet and he's going to study that and have a meeting about Louise in the near future.  The one new thing he mentioned is that he wants to follow her up for yet another unheard of disease...Moyamoya Disease.  Moyamoya is Japanese for 'puff of smoke', not because the people who have it are snuffed out like a puff of smoke (though they can be) but because the MRA once it progresses looks like a puff of smoke in the brain.

(A little Japanese version of Louise.)

Basically it presents exactly the same as Louise's MRA but as opposed to a congenital malformation which doesn't change, it is progressive and can be very dangerous.  It seems the only way it can be diagnosed is repeat MRAs to look for further thinning of the cranial arteries.

Here's the link to more detailed information about it which I haven't read yet.

On the other hand, if she doesn't have Moyamoya he said he wouldn't be overly worried about the malformations but we definitely need to keep an eye on them because PHACES is so newly named and rare that they aren't quite sure yet whether the malformations or aneurysms are congenital and non changing and present at birth or whether they can develop at a later stage.  It looks like regular MRAs are on the agenda for Louise, which I'm quite happy about from a mother's point of view as opposed to any medical reason.  Once she's a bit bigger she won't need general anaesthetic for them and they are non-invasive and safe.

He said he isn't really an expert so far in PHACES but he is going to study it now in time for the meeting.

Then he said he'd do the bloods today for Louise thyroid function which is also a part of PHACES.

Oh poor little girl, we promised her this morning there'd be no bloods today.

While we were waiting in phlebotomy John rang and asked me to mention to them about her going on warfarin and was there anything we could do to lessen the trauma of multiple blood tests.  When I told the two phlebotomists  both of them said at the same time 'Aw...God love her!!'.  It seems that for the first few months her tests could be two or three times a week.  Her hands and feet are always cold, though maybe that will change after her operation, so they said even if it's high summer, put on her coat and two pairs of gloves and maybe even those chemical hand warmers you can get in camping shops (or LIDL) to maximise her veins because they'll get harder to get as she has more bloods done.

I said that maybe that would be worse than the operation and they both said it probably is. Job's comforter eh?

Poor poor poor little girl.

Poor little girl.

♥

Well then I said I'd bring her to the shop and get her a prize.

They wouldn't take my €50 note so she had to hand back her chocolate buttons.

Poor little girl.

So I wonder will they ring tomorrow to call her for surgery.

This is a song I used to listen to when I was pregnant with one of the babies who didn't make it...a little hero who did her best.  I never realised how much I'd be calling on all those babies for support...they're coming up trumps.  I have no doubt hey're rooting for their little sis.  I know she'll do great.


Jennifer xx