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

Pyjamas

The postman just delivered a package.  It wouldn't fit in our postbox so he rang the door bell.  'Oh Thanks...it's pyjamas!' I told him.  I often tell the postman what's in the packages like 'Oh thanks..it's Santa stuff!" or 'Oh thanks...new wellies!!' I know if I was a postman I'd find it infuriating never knowing what's in the packages that light up people's faces.  I'm sure he thinks I'm a bit zany but invariably, being Irish, there follows a discussion about weather...predictions of rain...or snow...or the scandal of climate change..you know...that thing that was supposed to give Ireland a Mediterranean climate and instead just gave us more rain.  Oh well.

Well anyway I'm sure he's wondering what's with all these pyjamas.  I'll tell you what.  I've been buying them.  For Louise.

All the children's pyjamas in this family are usually bought in Penneys, and then probably in the sales.  Cheap, cheerful and comfy.  Every year Santa brings an unusual new pair or a nightie, in fact it's now a custom and pyjamas appear on every Christmas list.

But this little girl is being treated to a new supply of extra special pyjamas.

You may remember before Louise was born the effort I went to to find a little blanket that was  just right for a sick little baby.  I know now why I made such a project out of that and I'm fully aware that that is why I've been trawling the internet for the prettiest soft, cotton, button front pyjamas.  It was, and is something I can do.  So much else is out of my hands.  There is nothing I can do to force the phone to ring, or to make sure the surgeon doesn't have a headache that day, or that a microscopic germ won't make it's way into a drain wound.  I have no control over those things.  So I'll do the things I can do. I can prepare her pillow-slips and the lovely old fashioned soft sheets her 95 year-old Great-Grandma gave us for her little hospital bed so that it will be as cosy as her own bed.   I won't be able to take away her pain or her discomfort, but I can make sure that when she's given her anaesthetic her I'll be holding her and that when she wakes up, I'll be there and she won't know I've ever left her alone.  I can control that when she's ready to be held, I'll be ready too.  And I can make sure that when her new pyjamas arrive in the post I'll wash the factory starch out of them so that when she's ready to wear them they'll be soft and familiar and smell like home.  I can do those things. So I will.

I can make sure the folks in Heaven are fully aware of what's going on and what I want from them.   Of course God's will is what I want but I can tell you I'm trying my best to instruct request that His will is that my little girl will fly through this ordeal and get on with living a full and not-breathless life.  I can't wait for that.  I know she has some strong advocates up there, not least her Grandma and her siblings watching out for her.

 I know she has one strong guy standing watch night and day.  For those of you who categorise Angels along with Easter Bunny and the Tooth Fairy, think again...they are mentioned no less than 273 times in the Bible, both Old and New Testaments.  Jesus Himself said 'See that you do not despise even one of these little ones, for I tell you their angels continually look upon the face of My Father who is in Heaven'  That's pretty powerful!!  

I remember a while ago listening to a talk  by Ven. Archbishop Fulton Sheen about angels.  He was talking about the modern (wrong) depiction of angels as cutesy children with wings.  Or even worse today...erotically clad women. Whatever one thinks of that, I for one prefer the strong fearless beings described in the bible carrying their swords...the Greek word used is nothing short of Rhomphaia....Oh Wow!  No ineffective weaponry here, Rhomphaia refers to a huge and deadly double-edged sword such as this guy has...now that's who I hope is watching over Louise.

Bacteria BEGONE!!!!

Well, in the meantime...Thursday today...waiting for the phone to ring.  They'll ring us on a Thursday to go in on Monday for pre-op, home Monday evening to go in on Wednesday for surgery.

Tick...Tock...Tick...Tock...

Just hanging in.

Pray for us.

Jennifer xx

Surgeon Visit...Full Steam Ahead.

Before I start I just want to remind you what good hands we're entrusting our little girl to. I have a policy of not naming doctors or nurses in this blog but if you check this link he's the professor who's not photographed in surgical scrubs.  He is considered one of the best cardiac surgeons in the world and though he knows it, he is in no way unapproachable.  He has the exact air of confidence in his ability that any parent entrusting the life of their very loved child would wish to see in a surgeon.  That on top of the fact that Louise is part of only 15% of children worldwide who receive the lifesaving cardiac surgeries they require makes me feel very humble and thankful.

Well anyway, here's how we got on.  John, Louise and I went to the appointment.  We didn't tell Louise it was a doctor or that it was hospital as she's starting to recoil from the idea.  The surgeon's rooms are in a suite in a different part of the city and there's no give-away medical clues apart from a model of the human heart sitting on his desk.  To be honest, we came and went and I'd say Louise had no idea the appointment had anything to do with her at all as there's no examinations or scans involved.

He just asked us how Louise was and we explained her breathlessness on exertion which he was already aware of.  He said that's exactly expected now that her lower body and limbs are bigger than her upper body meaning that the level of de-oxygenated blood that's circulating in her system is greatly increased causing her tiredness and panting.

 First he warned us that in a child that's well, in order for him to subject her to such a big operation and all the possible complications, he has to feel it's justified.  He feels it's justified. So if we're happy to consent, she's ready for surgery.  Obviously we said we're happy to proceed.

This is what he's going to do:



The only difference in this animation is that Louise has hypoplastic RIGHT heart because of her tricuspid atresia rather than LEFT heart.  This is good from Louise's point of view as the left side is a naturally stronger pump.  He mentioned the little fenestration he'll make in the top of the heart which should give her a better outcome as explained in the video. That should either close itself eventually or need a small operation (not open-heart) to close it surgically at a later date.

Now he also mentioned the original pulmonary artery band which was placed in her first operation about 200 years ago.  If you haven't ever read that post, I personally think it's the one which most captures Louise's story...read it here.  Well up to this, the band has been doing a vital job of protecting her lungs.  It will no longer be required so he'll remove it.  The pulmonary artery exiting the heart will no longer be doing anything so I think he said he's going to just close that off from the heart to the site of the Gore-Tex shunt.

I'm writing all this partly to try and get it clear in my own mind too, it's not for the purpose of sounding technical.  The cardiologist could probably write a whole post on his attempts at explaining the structure of the heart to me.  I think he's given up at this stage though he seems to think I can read echo cardiographs as he's always pointing out this and that and this looks great and so on...I just nod and say 'Ooh...yes...' so as not to look too dopey!

After the operation, which is 5 hours, she'll be in ICU for a few days and then on the ward for however long the (very painful) three drains need to be kept in.  They are vitally important as fluid or blood can cause congestion or infection.

She'll be in hospital from anything from a week post op (best case) to a month and a half (not so good). Of course I'll stay with her.  John wants to take his turn too this time, before it wasn't an option as she was breastfed. Last time she was discharged a week after surgery so lets hope for that.

 Louise will be on warfarin for up to a year which is a bit troublesome but the less troublesome medications haven't been tested on children so not used on them

Then he talked about the timing of the operation.  He wants her to be 15 kilos which I'm pretty sure she is or very close to since I've just had to pass on most of her lovely clothes which I thought would last till spring  to her gorgeous cousin. She's had a recent growing spurt, though I can see when she loses her baby fat she's going to be a slim little girl. I must weigh her tomorrow.  We indicated that our preference would be earlier rather than later.  He agreed with that because he said he prefers the children to be younger so that it will be less traumatic for them and there's some hope they won't remember it.  He doesn't realise he's dealing with little miss elephant memory.

So basically we're on phone watch.  I suppose the hospital will contact us.  I know they have the play therapy and the psychological preparation.  I don't know how long that takes.  I've noticed hospitals like CHOP and Boston have those preparations too so I guess we should go along with it, though John's opinion is that her best psychological preparation will come from her own family.  I tend to think so too.

Then ( and this is why I'm happy John came because I wouldn't have had the neck to ask this question) John asked him what was Louise's long term prognosis.

He said she should live many many years on foot of this operation, grow up, have her own children...

WHAT???

When Louise was tiny I was told that she'd be very ill advised to ever become pregnant!!

Now one of the world's best surgeons has said the opposite! I asked him again...would there be any implications if she ever was to become pregnant?  He replied that one of his patients recently had a baby BEFORE her Fontan at the age of 32 and managed very well, so with the Fontan already in place it would be even better and they'd get her through.

I am absolutely delighted.

So now, I'm setting up a comfortable chair beside the phone...this might take a while...tick..tock...

BTW, if anyone would like to donate me a free iPad to keep me company in hospital feel free :-)

I don't think I need to remind you that prayers got us through before...we'll be grateful of them again.  As you can see this isn't minor surgery.

Jennifer xx

Nurturing The Crew Of This Ship

This evening I was putting the children to bed.  The Christmas break is over and school is back tomorrow so I was spending a little time in pillow talk with each child to reassure them that fun is ongoing.  We can go on outings other Sundays if they ensure they have no homework left to do, it's not long until mid-term, nobody likes going back to school and so on...I was expecting huffing, puffing and complaining. I was expecting 'I forgot I had homework', 'I didn't practice piano', 'I never bought the book I need'... What I wasn't expecting was a breakdown.

You have to remember that when Louise was born the children were all three years younger than they are now.  They had never experienced anything like what we all went through before.  In a lot of ways and from my questions to them since, a lot of what was going on seemed to go completely over their heads...well the younger ones anyway.  Her first operation was on a baby they'd only had to themselves for four days, they barely knew her.  The second operation was a surreal experience I don't think any of us would even believe happened only for this blog.  It's different now.

The specialist cardiac nurse told me recently that we'll find this time worse.  I didn't hear her to be honest, it went in one ear and got lost somewhere.  I remembered it this evening as I was sitting on the side of a bed holding a sobbing 10 year old.  You see, Louise isn't a generic baby any more, cute and cuddly and smelling of Heaven.  She's Louise, around whom her siblings world revolves.

Louise has this quirky little way of being soothed to sleep.  She loves someone, anyone, to stroke her lower leg or arm or tummy very very gently with their fingertips.  It's like an off button when she's tired. Her eyes roll luxuriously in her head and she's OUT.  This evening, after a trip to the zoo she was ready for sleep but as usual fighting it.  She shares a double bottom bunk with her 10 year old sister, Isabelle.  Incidentally, poor Isabelle briefly had the bedroom to herself when Peter was finally moved into his own room.  She went from being like a contented dormouse entwined with her sibling asleep and safe to resembling a poor lost soul floating noiselessly around the house each night hoping someone would take her in.  The thing was...nobody wanted her, their beds have grown too small now...or they have all grown too big, there's not really room for two in most of the beds any more.

It was the final incentive to get Louise out of our room.  There was no real medical reason she needed to be in with us...the reasons were unashamedly sentimental.  The fact is...and I say this with some genuine sadness...the likelihood of another child in this family is pretty slim.  Time has caught up on us.  The memory of the longed for twins we lost still brings a tear to my eye..but then we couldn't have had Louise.  We'll meet them in Heaven and this time there'll be no tears goodbye...them and the rest of our children who got there before us...little lives that were but a brief whisper and the world doesn't even know they passed through.  We do though, and so does God and that's all that matters.

Well anyway, Isabelle was delighted to have a new cuddly little bundle to snuggle with and in the mornings when we go in, just as it was with Peter, it's hard to tell where one child ends and the other child begins.  Like two little animals hibernating in a nest.

Tonight Louise asked Isabelle to stroke her tummy while she was going to sleep.  I don't know if you're aware of quite the mess Louise's tummy is, cardiac scars, drain wounds, stitch marks...it triggered questions in Isabelle, how do they reach her heart...how do they break her bones...what's the machine like...how long will she be in hospital...she could go no further.  Clearly I answered her questons on a 'need to know' level of detail and with the gentleness due to a child teetering at the edge of a major life event.  I don't know whether it's been building up or whether it's a new realisation that her precious precious precious little sister is going to be gone from her but this little tween had a heart wrenching breakdown.  And I realised that while the pregnancy and the early days were very much about me as well as Louise...this time I have to put on my big girl pants and focus on the children who know Louise so so much more than they did before.  They're older than last time and they know much more.  The fact that Louise keeps saying 'I don't love no 'opital ' isn't much help to them.  I guess it's not for nothing the cardiac department in the children's hospital has dedicated psychology staff, not just for the child heading into surgery but they are also freely available for the siblings.

I think we'll manage to carry everyone through but I'm prepared that my little women and of course Peter might need a few handkerchiefs and a safe shoulder of comfort before we're through.

Just so you know...it's all triggered by the fact that we're seeing the surgeon on Friday.  We'll get an indication of what timeframe he's thinking of.  The cardiologist has said in the next 3-6 months.  I'll update when we know more.

The cardiologist has also contacted the neurologist and the dermatologist for follow-ups re the new findings in Louise's brain scan.

So...continued prayers are requested and very much appreciated.

Thanks,

Jennifer x

Brain Scan Report.

I don't know how long it's been since Louise was diagnosed with PHACE(S) Syndrome but she finally had her MRI/MRA on Wednesday just gone at the same time as she had her heart scan. The rigmarole of cancelled scans, changed dates and so on is water under the bridge and I won't complain now about that.

Having Toast & Juice & Feeling Very Wobbly

The cardiologist looked at the heart scan straight away and came down to see me while Louise was having her toast and was still pretty dozy after the general anaesthetic.  He's very pleased whit how everything is looking, her PA band is holding up great and doing it's protective job very well.  Her Glenn is looking good too.  Everything as well as can be expected.  Her blood oxygen level is good for a Glenn child and her only symptom really is her breathing.  He said he doesn't want to put her into surgery (which I was expecting since her last visit to be after Christmas) until she is seen by pulmonary to rule out any asthma or lung issues which might be accounting for her breathing.  He asked the nurse to ring them and ask could they come down and see her while she was there.

The answer came back that they were too busy and we'd have to make an appointment...and there were no appointments available for THREE MONTHS!!!!

So from building myself up for surgery after Christmas, God knows when it will be now.  I was fit to be tied on the way home and so deflated but on the good side, if her surgery is delayed, the beautiful new state of the art children's cardiac unit will be completed (this summer) in time for us to benefit from it and I won't have to sleep on a thin freezing rubber mattress on the floor beside Louise's cot.

That was the heart.

Thursday John rang to see were the brain scan results ready yet. No, they weren't.

Tick...Tock...Tick...Tock...

Friday...I answered the phone to John's number.

When your husband starts a conversation with 'Now, before I say anything..it's not as bad as it could have been...'

You don't breathe a sigh of relief.

He proceeded to read out what I later learned was half of what was written down.

Rather than try and explain what I don't know myself, here's what it says:

'Blah...Blah...Blah....etc

There is a large extra axial fluid collection within the posterior fossa, predominantly right-sided, which is shown to communicate with an enlarged distorted fourth ventricle.  There appears to be complete agenesis of the cerebellar vermis, with significant hypoplasia of the right cerebellar hemisphere.  The posterior fossa appears slightly enlarged, with a mild elevation of the tentorium.  No supratentorial abnormality is identified.  Findings are consistent with a Dandy-Walker Malformation, which appears unchanged since the previous examination.

Regarding the vascular anatomy, the right cartoid artery is significantly smaller than the left.  The right internal cartoid artery is significantly smaller than the left and the anterior and middle cerebral branches are also smaller.  I do not see convincing continuity between the right posterior communicating artery and the posterior cerebral artery.  (Oh, and great news...)   The vertebral-basilar arteries appear normal.'


The bottom line is we really have no idea what the short or long term significance of this is.  Thanks be to God, and I sincerely believe it is thanks to Him, there are no aneurisyms  present which was our greatest fear. So that's good.  I guess we'll need to see a vascular specialist and a neuro-surgeon as well as the birth-mark department which diagnosed her in the first place to see what the course of action will be.  On a very superficial level (and I can always find something superficial to worry about in the midst of great drama), I hope we don't have to go to a different hospital as I suspect because there is ZERO parking available around the one I think it might be and I can visualise me and Louise taking the bus in the city traffic to her appointments.

I was sitting beside John examining the fax today and commented to him that there was no mention of the rest of the scan, only the brain.

 'Oh, that'll be two more separate reports.  I've ordered them and hopefully they'll be ready on Monday'.

What are they going to show up? I don't know.

For anyone wondering why I've put that whole report in this post and that I should be more...well...private, firstly, a lot of my family and close friends are interested and secondly, I'll be posting this in the PHACE Syndrome group page. This is such a rare condition, every bit of medical information is important and of interest to other families, and I'm also hoping some of them might be able to impart some of their knowledge and experience onto me.  If you have any, please leave it as a comment.  This is new to all of us.

In the meantime, I'm delighted to say Louise as ever is a little rascal.  As I write this she is on the floor with my paper cutter (you can't cut yourself on it :-)) cutting my Christmas card making supplies in half.

Oh, and this week she decided to go from full time nappies to fully daytime trained in one day.  I'm very very proud of my little girl.

And did I mention...we love her to bits. ♥

Finger Painting

How To Keep A Heart Baby Warm At Night ♥

Jennifer xx