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...
It has been an emotional week of milestones for this family and today is the icing on the cake.
When Louise was diagnosed in the womb our OB told us that Dandy-Walker was one of the worst conditions we could have hoped for. She probably would never walk or talk. Why would they put her through her needed heart surgeries since her quality of life would be so poor...a useless existence in her opinion. Furthermore, she told us that Louise would be bottom of the list for a heart should she need a transplant...why put good money after bad? The best she could offer was that hopeless trip to UK for an abortion. We had to think of the other children you know.
Shortly afterwards I was speaking to our children's ballet teacher. The founder and head of Ballet Ireland and a Prima Ballerina in her day.She told me we'd get her walking...and dancing...ballet no less. I cried the whole way home, my little girl would never be a ballerina.
The sweet ballerina baby-gro Louise had as a newborn in PICU would rip my heart out every time I put it on her. Every little girl dreams of ballet shoes and tutus. How do you tell your daughter that won't be her?
Today, Louise put on her tiny ballet shoes and smiling from ear to ear tip-toed into her first ballet class...with the very same teacher who had promised me she'd dance. On the way out, her own eyes misting over, she told me she remembered the incident well.
Three and a half years ago God handed me something very unexpected. Something nobody ever expects-a cross so different to the plain old one I was already carrying. The difference was that this cross was ablaze with flame and in the middle of the flame the name of a child.
Nobody will willingly run into a blazing fire but even a stranger will do that for the sake of a child and though I couldn't be called a stranger, the presence of the child compelled me to grab that cross. No sooner had my arms enclosed on that burning wood I realised that God had handed me something else as well-a pen. So finding that pen in my hand I started to write and what has come out of that is this story that I am convinced is not mine but His.
There were times when I was writing this blog that I wondered why I was even writing in detail that left me very exposed. Leaving myself open to criticism, or pity which drags one down, or even worse...praise that can overtake the ego and blind one to the reality that no one is more special than anyone else.
I have just taken a weekend out to be silent, to put things back in perspective, to pray and to think. I have done a lot of thinking and I think I can see some things I didn't see before.
I realise that the cross I have carried this last few years was MEANT to be ablaze because God wanted it to be seen. Why was the pen placed in my hand other than to let the flame take hold. I hadn't written much more than a postcard in all the years since I had left school and suddenly this thing was writing itself. I didn't know whether this was going to be a tale of bereavement or of survival but I did know that one way or another I wanted it to be something that would encourage another family to give their child a chance of life. I will never know till the last day whether any baby is alive because of what I wrote but that doesn't matter. My own embarrassment at my 'public suffering' means nothing to me because I can clearly see that (and I'm unashamed to say this) God wanted me to suffer publicly .
There is something seriously wrong in this world that goes far beyond the 'something rotten in the state of Denmark' and that is the gutter level of value for life and in particular life which is considered imperfect. The hopelessness displayed by the medical profession is like a cancer. Those supposed to nurture life being the very ones initiating the taking of life. I have heard so so so few stories of doctors who encouraged families to welcome their imperfect child as much as the perfect one.
So few.
So few friends, so few relatives who encourage a 'yes' to life.
So this child that was placed into our lives was given a job of sowing hope in a ground that is arid. I didn't plan for her story to take wings but it did. I didn't know she was going to light up a dark path but she did. All I knew was she was mine and my job was to love her...that her story became public is irrelevant to that.
Now I see that something has changed. Louise is alive. She has surpassed all our expectations and dreams. She has been a witness to hope. She is a witness to life and it's immeasurable value. Her job is done.
Since before she was born I have shared her with all of you.
Now I am taking her back.
The cross that was on fire with a flame for all the world to see is now something different. The fire is replaced with glowing coals to be carried by me and John and Louise in private. My future tears will not be shed on this blog but on my husband's shoulder. We are the parents of a little child who has physical and emotional scars which need nurtured in private. Her long term future is uncertain. We don't know what bridges she will face but we will help her over them even if the waters rage beneath. Her life may have limitations but which of us doesn't have limitations? None of us. This we know..she may have half a heart but it lights up the world. We are forever grateful for her.
Of course I will continue to ask for prayers whenever we have appointments or procedures coming up. They will be mostly on Facebook. But overall I think the story that God has wanted me to tell is told and I hope I've done it well.
Finally, even though the huge flaming cross has been replaced by my private one, I'm looking down and seeing that the pen is still there. I will continue to write over at Raindrops On My Head. My hands have been burnt a few times by what I have needed to write but on the whole I hope it's an uplifting read. Please join me there. You might even find Louise there from time to time.
For the last three and a half years the cloud of Fontan has been hanging over us, we always knew it was inevitable and really I never looked much further than that. Now that it's over and we're getting back to normal I have been looking into life beyond Fontan and the long term implications for Louise. I have really only had one friend online who is a 'heart-mom' and her son has a different condition to Louise's. Last week I joined a FB page just for Tricuspid Atresia. It has opened my eyes, and in particular this conversation with a girl who was one of the recipients of the earliest Fontans. It was in response to a post by a pregnant mom whose child has just been diagnosed with TA.
Because I can't get the words this girl has written out of my mind and her testimony to her parents, I asked her permission to post them here. I think it shows the steadfast determination of those young parents who refused to give up...Please read this..
Wendy~ I'm not a parent, but a person with a feisty human spirit living with the single ventricle condition T.A. I'm 39 years old, born in 1973 when the Fontan surgery was only merely in its infancy. I wasn't diagnosed until I was 3 months old. My parents were 19 years old at the time and devastated to be told that I wouldn't live, but my mum never gave in and never stopped searching for the best treatment.
I had my first surgery a waterstun shunt at 18 months old. The medical professionals involved in my care did not think I would make it out of theatre alive...but a day after surgery I was bouncing up and down in the cot...Palliative was always the word emphasised for this surgery. By the time I got to 5 I started to deteriorate and again, my mum wouldn't take no for answer and continued to push for better treatment. When I was around 7 and very ill, by chance my cardiologist at this time had been to a paediatric cardiology conference. There he met Professor Somerville and Professor Magdi Yacoub...The most internationally renowned cardiac surgeon and congenital heart consultant in the UK at this time. I was referred from there on in to the care of Professor Somerville and in November 1982 when I was 8 years old I had my Fontan surgery performed by Professor Sir Magdi Yacoub.
Since then I have NEVER looked back. I worked hard at school, did well academically and went onto study at university and developed a professional career. My Fontan first began to fail in the 1990's and I managed to have intervention to keep it working for longer. In 2006 I learnt that its failing further and discussions have ensued regarding my future. I declined conversion Fontan surgery (converting my old style Fontan to the newer version ie TCPC) due to the high risk factors involved. I've been told that heart transplantation is highly unlikely in my case, but I keep on ticking and living life to the full, grateful for all that I have had and continue to have ; ) I am at a crossroads in my life with regards to continuing my career, but I know that I have done everything possible in my life so far and achieved what was beyond many peoples expectations. I hope that my story will inspire you.
If there is one piece of advice I can offer I would say encourage your daughter to reach for the moon and back and more. She will learn to live within her limitations, but make the most out of her life also. My mum has done all this and more for me throughout my life and I wouldn't be the person I am today if she hadn't : ) I am a great believer that hope and human determination prevail and the sky well and truly is the limit! There is an organisation in the UK called Little Hearts Matter. They support families with children with single ventricle heart conditions (T.A, P.A, HLHS, HRHS, Double inlet left ventricle, Double outlet right ventricle). You may wish to check out their website. Equally in the UK the Somerville Foundation supports adults with all types of congenital heart disease. My own story is published in issue 72 (most recent newsletter). In the US and Canada there is the Adult Congenital Heart Association who again support adults with all types of congenital heart disease. Hope some of this information helps. Take care.
Also just to say there are others out there just like me!! ; ) So please don't feel alone...
Me~Wendy, it has certainly helped ME! While we were looking toward Louise's three surgeries, I never looked to much beyond that. Now that she has just last month completed all three, for the first week the cloud on my horizon was lifted. But it has been starting to gather again as I've been pondering her future and her lifelong expectations. I think it is a foolish train of thought, medicine never goes backwards, it only goes forward and that is what all of us need to focus on. I recently discovered Little Hearts Matter and their teen website and it is so positive and full of hope. We were given no hope to clutch at during my pregnancy (apart from our cardiologist) but Louise has shown that negative doctors are discounting the strength of the human spirit the determination of parental love and the power of prayer.
Wendy~Hi Jennifer Kehoe. I think you are right in that looking beyond the 3 stage surgeries is difficult in today's terms and yes medicine has moved forward significantly so since I was born. However, as you can see in my generation, we had no staged surgeries planned and we did not even know what would happen month to month, never mind planning the next surgical phase. All we had was sheer human determination, strong, supportive and loving parents and a wing and a prayer so to speak.... and boy did we hope and pray that we would find a cardiologist who knew and had the insight to know what they were doing. I found that and more when I got to 8, but I was virtually dying on both occasions of surgery before it got to this stage as few really only had the expertise to treat me.
My first surgery was merely 'palliative' to prolong my life, but nothing was known beyond this and by the time I got to my Fontan I had very little healthyish childhood as I was so poorly and significantly cyanosed. I owe my life to Professor Somerville and Professor Yacoub and although they were pretty much grappling in the dark as the Fontan surgery was pioneering at this time, I and my parents trusted and believed in what they were doing. As for my generation now, yes medical advancements have moved on, but sadly for most of my generation we have not and will likely not benefit from this progression. The staged Fontan surgeries will serve and has served better the generations of now and those to come and childhood is near to normal, but as for us 'oldies' we are back to our initial prognosis in many ways...being the pioneers once more as to where to go from here, of which quite frankly appears limited in terms of our options.
But above all I am thankful that my life has contributed to making things significantly better for the younger generation to enjoy their childhood and young adult years as healthy as possible. You see all the times I have been asked to contribute to medical research for single ventricle conditions via tests (some very invasive like having a 24 hour catheter inserted into the neck area to measure pressures) I realise that every time I see a single ventricle child running or riding a bike, my contribution and many others of my era, have not been in vain. I wish you and your family well on this journey ahead, its peaks and troughs all the way....but worth the ride all the same!! ; )
And just as I always say 'life as a single ventricle GUCH goes on'...... : )
Take care. x
Me~Wow! Wendy, we have a lot to be grateful to you for. Wendy~Hi Jennifer Yes its been tough for our generation but i think i probably speak for many of us, if not all when i say we take comfort, hope and reassurance that things are improved for today's children and young people and their families. I went to the little hearts matter open day recently and i was so inspired by all the young people and families i met. Today you are a better informed group, with greater support networks now than we were and i think its amazing! Its only in more recent years that i have met others with single ventricle conditions of my generation and i truly value it. But i also think its crucial that the oldies and the youngsters learn from each other in order to truly move the care for all single ventricles forward in the future ; ) Wishing you and your family hope; happiness and a love of life. X
The bottom line is nobody, least of all us, knows what Louise's future holds. We know she will get to adulthood. Beyond that we are dependent on the research which is now taking place. We are thankful to the families who faced an unknown future and have hewn the furrow in which Louise travels.
Thank you, Wendy, for allowing me to post this.
And to honour those families and the children who lived through Fontan in it's earliest forms, can I ask all of you to consider registering for organ donation. The criteria is different in every country and I will post the links for the different countries shortly but today perhaps discuss it with your families.
The award-winning pianist Paul Cardall was born with Tricuspid Atresia...here's his story, it is beautiful.
Louise had a lovely birthday. We decided it would be understated as she's still in recuperation and not really up to a big event. None the less we had plenty of visitors and Louise was definitely princess of the day.
Dancing with cousin Amelia ♥
(what do you think of Isabelle's Slayer t-shirt?
John ordered himself a child size by mistake!)
I got this idea from Pinterest.
Present from Granda
John drops the children to school most days giving himself enough time to pop home for a little chat before he goes to work. It was during this chat two days after Louise's birthday we began to discuss her blueness. I have been noticing that she is bluer now than she was before the operation. John thought so too so we agreed I should ring Louise's specialist cardiac nurse just to ask whether this was expected. I know the Fontan operation is not one that completely kicks in straight away as it is a totally different circulation system for her body including the liver, lungs and kidneys to adjust to so perhaps the blue tone is normal. The nurse told me to come up and she would check her oxygen saturation. A few hours later, seeing Louise's reading of 73 when before the operation it was 85 the nurse said she'd ring the cardiologist who immediately decided to admit her overnight for a thorough check out. Here I was with no bag, no purse as I couldn't find it going out the door and stocked up with nothing except two €20 notes I'd swiped from Laura's confirmation money.
So echo, ECG, X-ray, more bloods..she has developed such a terror of bloods that she literally took to her heels when her name was called and ran as though escaping a fire. She managed to almost reach the end of the corridor before I caught her! Finally up to A&E to wait for a bed on the ward and to be admitted whereupon the triage nurse put her onto oxygen as she was so surprised at her low sats. Then up to the ward to begin her 24 hour heart trace.
Anyway to cut the story short, everything to do with the operation is fine, thank God. They figured her medicine prescriptions were all too high for her and drying her out excessively, and would also explain her backward step in toilet training. Her INR (warfarin related blood thinning levels) are all over the place with no pattern emerging so far. To explain the blueness, the fenestration they made in her heart as a release option is completely open, which is neither good nor bad. In some children the fenestration isn't used by the body but hers is being completely used meaning she is still getting a lot of de-oxygenated blood circulating in her body. This explains how she is blue but not breathless like before. At the moment he is happy enough to allow her sats to be in the low to mid 70s and he'll review it later. The hole will either close naturally or they will surgically close it in time.
Well it was just a blip and I'm thankful nothing sinister has shown up. It's just an indication that Louise will never run a marathon but then again...lets find out what other amazing things she'll be able to do. We love her to bits, blue, pink or striped.
For the immediate future our plan for Louise is to get her psychologically back on an even keel. To be honest I think she's totally traumatised. She's clingy, she's afraid to let me go anywhere, she's fractious and her fuse has shortened by about 90% and she had her first ever screaming episode on the floor of TKMaxx which we visited with the younger ones to get her some birthday presents...that is a completely new turn of events, her nappy training is out the window and her fear of blood tests and medicines is now deep seated. None of this surprises me and I think it will take a little while for her to get back to her old self. She really has been through a physical, emotional and psychological war and has come out alive but battle weary and with scars which will heal with the medicine of the love of her entire extended family.
This morning Louise was helping me to put away the groceries. As we were putting some caster and icing sugar into the cupboard I told her it was for baking for Easter. And because I think no child is too young to learn to love God I said
'You know what Easter is? Some people hurt poor Jesus and he was very very sore...and you know what? He made Himself better and now He's ALIVE..and that's EASTER!!'
Her face lit up and with wide open eyes she asked me
'And did I helped Him?
Did I help make Dee-zis better?'
I was able to reply without a moment's hesitation and without a trace of a lie..
'YES Louise!! You definitely helped Him. You helped make Jesus better.'
Firstly apologies to anybody who follows Louise's story only on this blog. I have updated things on Facebook but I'm sorry I left you hanging in the air wondering whether she's doing well or not. Truth is this is the first time I've sat down in a week. Louise's hospital stay dragged on longer than we'd hoped because of her vomiting problems. The doctors tried and tried to discharge her in time for Laura's Confirmation on Saturday, and twice we got as far as almost making it to the door before having to unpack and kip down for another night. It got to the stage that John was wondering whether the vomiting was actually caused by stress as it always seemed to coincide with a doctor walking into the room. When you think of it, she was in there almost two weeks and in that time nothing good happened to her. The only good thing from her point of view was when the physiotherapist walked her down to the hospital shop with some money tucked in the pocket of her dressing gown to buy a 10c lollipop. That and when my sister arrived in with a bag full of pick-me-up activities which took her mind off her predicament for a while.
In the end, Louise was too sick to discharge or to leave with my sister and so John ended up missing Laura's Confirmation ceremony. We didn't really do much to mark Laura's special occasion but we'll make up for it.
Well we got her home that evening and it was like bringing home a new baby we were all so excited.
Since coming home it's been a mixed bag emotions. Louise's operation seems to have been a great success but when I wrote that the surgeon's job was finished and Louise's had just begun...I was right.
When John heard Louise would be going on warfarin his heart sank. It didn't bother me much as I thought 'shur half the country's on warfarin, including my own father, can't be too hard.' When the two phlebotomists commiserated when I told them she's be on warfarin I began to get a bit more nervous and when the specialist nurse sat down with me to explain it all in detail, including the necessary mollycoddling she will need if playing outdoors, I realised the imminent future was going to be none too easy. In the week Louise has been on this medication she has had nothing but constant hospital blood tests..some even daily. Apart from the drive to Dublin and the time waiting in the blood clinic and the fear that she is developing at the sight or mention of the hospital...it's getting more and more difficult to find an intact vein in her skinny little arms. It is heart wrenching to witness it, not because she screams the house down but because she's so stoic and sorrowful with huge tears rolling down her face, it would break a heart of stone. Actually it's struck home to me the horror of adults who deliberately hurt little children...it truly is a sin which cries out to Heaven for vengeance. No child should be hurt . Sadly some children need to be hurt for the good it will bring about but to indulge temper on an innocent child, deserving of nothing but love is just chilling.
Warfarin is such a troublesome drug and there is no alternative available for children. The dose is different for each person and it takes a while to get it right. So far the slightest adjustments of Louise's doses have flung her INR levels to dangerously too high or dangerously too low. In fact when they rang us last night we figured they were going to admit her to hospital had we given her a dose at all yesterday her level was so high. I think they can give IV vitamin K to counteract it.
Today is the first day her reading has been anywhere near as hoped so we'll see what happens over the next few days. Also we have just got her handheld finger-prick blood testing machine which will make things a little easier. Of course, warfarin is a blood thinner and so bleeding takes that bit longer to stop...our cute little lady has discovered the art of 'blood painting' after her last finger-prick test...only Louise!! At least it distracted her from her distress, maybe I'll have the blank sheet of paper ready for her bribe next time.
Our other problem is that although up to this, Louise has been so compliant with taking her medicines, she has now developed a terror of every medication except for one familiar one she was taking before the operation. Every dose has become a battle and last night when she saw it coming she started retching, God love her. Practically none of her medicines are actually designed for children and are all very 'mediciney'. We could pop a sweet into her mouth but we're also supposed to be caring for her teeth as a cavity in a child with CHD can cause a fatal heart infection!
This picture doesn't even feature her blue warfarin!
On top of everything else, because of the two strong diuretics Louise needs to take to place some of the heart's workload onto her kidneys, she's back in nappies full time after being completely toilet trained. This was under doctor's advice, and rightly so. The only thing is it's embarrassing for a child to have so many accidents, to miss the toilet and to need nappy changes more often even than a small baby. Her little bum is sore too probably due to my own carelessness with putting on sudocrem because I've never needed it before with an older baby. There's a lot of learning to be done by all of us I suppose.
So though the 'worst' is over and that cloud which has been on our horizon for the last three years is gone...we're not quite there yet. My eyes have been opened to what the lives of parents (including many who read this blog) must be like who have constant therapy appointments of every sort with their children. I have found this time around very humbling from a lot of different perspectives. I'm once again humbled at the sight of my little girl facing and managing a cross that most of us would run from at full speed. I'm humbled by my inability to meet the needs of all my children all the time. I'm humbled by the knowledge that certain of my children have silently co-suffered with Louise. I'm overwhelmed by the goodness of others and the goodwill we have been at the receiving end of...you will never know how comments on Facebook, Mass cards, prayers, gifts and messages have helped us all through.
And lastly, something so beautiful I have to show you...
It's bigger than it looks in the picture, it covers Louise easily and it's so soft.
Every heart child that passes through Paediatric Intensive Care Unit in Ireland receives a hand crafted blanket made by the old ladies of the country. Louise got hers with a little name pinned to it...Winnie. (I have the rest of her name and where she's from). Winnie...Winifred..from a little village in the West of Ireland. Nobody under 80 in Ireland is called Winifred. It was only last night I read with tears rolling down my face the page with the information about these ladies and what they do. It originated when Irish children had to go to America for heart surgery. The blankets were sent with them for comfort from home. They continue it because of the realisation that heart children still need comfort. Winifred, thank you, thank you!! I love this precious blanket and we will treasure and care for it with the same love you put into making it for my little battle weary soldier.
Louise didn't get on so well today. After her amazing effort yesterday she sat out in the special little armchair for a few hours where she dozily watched the Wiggles until she fell into a sound sleep. That was during quiet time so I went home for a while. While I was there PICU 1 (High Dependency) rang to say they were going to sedate her to remove the mid-line drain, the biggest of the three. When that was removed she could go to the ward. The wards are much more specialised now so children can spend less time in PICU. So we waited a while and went on up with the three eldest children, the younger two going to a friend's house.
When she spotted her big sisters Louise burst out crying with huge tears. I think she was just overwhelmed with her situation. Over the next few hours she basked in their attention and they even got a brief smile. I hoped that it was the lift that would boost her recovery.
Unfortunately, the night didn't go so well. She vomited up everything she had managed to keep down. It was quite a task to clean all her wires and drains. While the nurses were changing her bed I managed to get my first cuddle with her. She just snuggled in so pathetically like a little broken bird. As we were moving her back into bed I managed to accidentally tug at one of her drains...she screamed so loudly. My stomach flipped in the way it does when you see a child hurt. To be honest I was completely traumatised to have caused her extra suffering.
On the good side here is that as I was stroking her back I was completely amazed at the realisation that there was no roughness...the gritty whoosh that was her heartbeat is GONE!!!!
Later in the night, due to the medications she had, she completely saturated her nappy (she's more or less out of nappies at home) as well as the bed. Another bed change but they managed to do it without lifting her right out. I don't think I got too much sleep as she wanted me to hold her, and stay close. She slept more or less all night but with frequent wake-ups to call me. By morning I was feebly trying to convince myself I was asleep while at the same time reaching up to stroke her hand. From about 6 am I noticed that her tight swollen little hands were feeling hot. Sure enough by morning she was running a temperature.
So of course that meant sending urine and bloods for culture They can't be taken from her central line because of something they put in there which would contaminate the sample. She has barely a vein left that's available for bloods and the sample needed to be 6mls x 2. The doctor tried and tried but fell short of the required amount from the only veins he could find...in the instep of her foot, she cried and cried telling me it was sore...and when he had gotten the last drop he could, she had the good manners to thank him. Dis-heartedly he sent off the samples hoping it would be enough. They phoned down this evening apologising that no...there wasn't enough...they have to try again in the morning.
So all day Louise was listless and miserable. The physiotherapists came down hoping to be able to walk her to the playroom to continue her therapy. One look at her told them she wasn't up to it. They even decided against trying her in the little armchair. All she managed was to blow a few bubbles through a straw in a cup of water...this is important to release the secretions in her lungs. After a few puffs she fell asleep. They'll try again tomorrow. In the afternoon I found an iPhone app where you blow out candles and we did that as much as we could with her.. Her only lift was looking at a little video her cousin sent her wishing her well.
John came up with all the children except Rebecca who had gone with her friend present shopping for Louise. The Tinker-bell balloon she had so eagerly anticipated got barely a glance. As the nurses were changing her sheets yet again after a little (or big) accident, she sat on John's knee. I think it was just the secure arms she needed. She dozed on and off. The good thing was that in that upright position her oxygen saturation levels rose to the low 90s which is great.
John is staying with her tonight. I think that will help her because when I'm there she's clingy and won't let me even stand up without worrying I'm going...which makes using the bathroom a bit difficult. She doesn't do that so much with John and so is more relaxed. Her temperature has risen a bit again but luckily the cardio-thoracic registrar came around tonight and said she's not worried about the fever, it's not too high and is typical enough. She told John they could remove a drain tomorrow then looked at the volumes and said no it won't be coming out after all. Seems this drainage is complicated with Fontan. It's interesting how many nurses and doctors have said...'well she is a Fontan'.
So Louise still has some way to go. I'm at home and heading up for a good night's sleep. I'll be back to staying up in the hospital tomorrow. She has come so far, I know she'll conquer this final mountain. I'm praying the prayer of the two Andes Survivors who tramped off into the mountains to look for help not knowing whether they were walking in the right direction or further into the Andes...'Lord, make it difficult...but don't make it impossible...'
Tomorrow is another day. I think it will be better.
Please continue to entrust Louise to God's loving care.
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.
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.
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.
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.
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.
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.
