My little girl is having major major heart surgery next Wed. Can I be so bold as to ask my Twitter friends to send some prayers her way? Tks

On 23rd November 2009 the routine ultrasound scan of our 20week old unborn daughter showed up some serious anomalies. Shortly afterward I began this blog so that no matter what happened our little baby would have an impact however short or long her life may be...this is Louise Mary's story...

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.

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.