Saturday, September 7, 2013

Ballet Dreams

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. 

Saturday, April 6, 2013

On The Shoulders Of Giants

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's his story, it is beautiful.

Friday, March 22, 2013

3rd Birthday & A Hospital Sojourn.

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.'

Jennifer xx

Friday, March 15, 2013

The (un)Wonderful World Of Warfarin

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'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 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 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.

Jennifer xx

Monday, March 4, 2013

How To Avoid Taking Yucky Medicine

And if they do succeed in getting it into you all you have to do is vomit it all up...every time.

Sunday, March 3, 2013

Louise Is Not A Mistake

Yesterday was a very difficult day, to have gone from so well one day to so poorly the next was a big disappointment. John stayed over with her last night. He was very eager to for the reason that for the last two operations Louise was breasted so there was no option of John having a chance to stay. To be honest I was happy to get a full night's sleep after my one hour the night before.

In the morning I text'd John to see how Louise was and how was the night.It seems poor John had the same experience asI had the night previously. I'm not sure whether he even got a chance to get into his sleeping bag! Louise wanted him to hold her hand and stroke her all night. Her temperature was still spiking each time her medicine wore off and he had to witness the sorrowful sight of re-doing the failed bloods from yesterday-he hated that.

Today Louise has turned a corner, her temperature is still spiking but she managed, with a great deal of self-pity, to go for a little walk out to the playroom, a much shorter walk than her 'Shufflin'' expedition. She's very nervous as the painful drains are still in, we're hoping her fluids reduce enough to get at least one of them removed tomorrow. She sat out in the chair for a good while though she's very protective of being moved even an inch.

Her sisters really gave her a boost today, they spent several hours entertaining and playing with her. Her mood and demeanour is much improved.

Because her x-ray came back clear, the cardio-thoracic registrar decided to remove Louise's wound dressing to rule it out as the source of the fever. Lo and behold...there was the icky patch at the very bottom of the incision. It's very near the site of the drains which have been in for this will be the fifth night and also the pacing wires which are also still there, both leading to the very inside of her body. So she is now on a very strong antibiotic. So today was better than yesterday and hopefully tomorrow will be better again.

Last night and this morning while I was at home I have been thinking a lot about the meaning of suffering and in particular the sufferings of children. When an adult suffers it's bad but we expect adults to suffer somewhat. People age and also most of us don't care for our bodies as well as we could...we exercise sporadically, we love fats and sugars and so on. But a child suffering?? What can we make of it? It goes against our comfort zone. Why should a child suffer? I have thought over this long and hard since Louise was diagnosed. We know suffering is a mystery we will never understand. It's part of being human, even the healthiest of us have stood on a Lego. If you stop and concentrate on your body right I guarantee some bit of is is sore or aching or uncomfortable. We don't even notice it any more. But back to a child particular a child that was conceived with suffering as part of their lot. Nobody could say they deserve it, innocence personified carrying a cross that would daunt Mr Universe. Why? Why is that? We recoil from the thought, and I always did as well. You know, drive past this hospital, spare a thought then forget it at the next traffic light. I'm not saying I didn't care but it wasn't MY life and it's hard to go there.

I know I've said this before and many of you will be aware that the thing I find most difficult to put behind me is not Louise's problems but the fact that someone suggested we kill her. Nobody ever wanted to kill any of my other children but it was taken as almost expected and the responsible thing to do to take Louise's life because she wasn't 'right'. In fact I have been accused on Twitter of cruelty because of Louise's sufferings over her operations and procedures. In other words, better dead than suffer even something that will ultimately help her. When I was here when Louise was a tiny baby a woman in the queue for x-ray told me she definitely would have aborted Louise rather than have a disabled child. When I got over the shock of someone saying that about a baby sitting in front of them I realised that it demonstrated the way our culture has such a fear of imperfection and suffering is something to be avoided at all cost, even to the point of taking life.

Others say how could God allow a child to suffer? Children become ill and children are conceived with anomalies so therefore there is no God or if there is one He's cruel. My many many hours of pondering this over three and a half years and even more intensely on days like yesterday have led me to a very very different perspective.

I think there is real purpose in the lives of children who carry a heavy cross. A lot of you will have seen the film The Passion Of The Christ. It's brutal and stark. I sit on the fence as to whether and by whom the movie should be watched. But I remember the scene that touched me by far the most in the movie, and this was years before Louise came along. It was the scene when Simon of Cyrene helped Jesus carry the cross, albeit under duress. At first he was reluctant even resentful and angry but as time passed he carried the weight if that ugly reality with more enthusiasm. We hear the stories from the bible so often they become routine to us. But look at Simon...the ONLY person in the history of mankind who physically carried the actual cross of Christ. What's with that? As we know Jesus did nothing for no reason.

Who was Simon? Nobody as far as we know. Who am I? Who is Louise? Less than nobody in some medical mindsets. But not in here where I'm sitting she was somebody from day 1, here she was Louise. Here her life had and has value. Here they treat children with the most 'grotesque' of anomalies ( here I'm talking from the world's view, most definitely not mine) as someone important, and real and due the same love and care that is due to any child. Nobody here ever said kill her, no, they asked us her name and from there on she was Louise, not an anomaly or an inconvenience or a drain on resources.

Back to Simon...a random man who never thought it would happen to him. Carrying the cross and finding it changed him forever for the better. Sometimes, and I've seen it time and again, looking back on a bad situation people say it was the best thing that happened to them...put life in perspective..etc etc. But sometimes I think it takes more than our own suffering to do that. Sometimes it takes the suffering of innocence to stare us in the face to wake us up.

Yesterday and this morning and on other low days in Louise's journey I have faced suffering of innocence head on. What does it do to you to do that. It strips you bare of every single thing in this world that is superfluous or superficial. It leaves you with nothing except the core. It is stark and raw and the pain is something else but you can see it by it's name.


What has Louise done to deserve this? Nothing! Is her suffering the pointless act of a cruel God? Most definitely not. Before I formed you in the womb I knew you...I have you CARVED in the palm of my hand. CARVED...that's pretty drastic. Not written on with a sharpie or not even tattooed. Carved implies a deep cut. Why would you do that if you didn't care? Louise didn't ask for or earn this cross but look what she has done by carrying it. The very fact that you are reading these jumbled thoughts means she has done something to you.

My very close friend had a little son whose prognosis was very poor. Some of his 'anomalies' overlapped with Louise's. He passed through this world for one year and two weeks. A speck in the world's eye. A life if no value some might think. And yet those of us who walked his path know nothing could be further than the truth. This little boy opened the eyes of thousands right across this planet. People loved him in a way they couldn't understand, we're almost taken aback for what they felt for this boy they didn't really 'know', whose family they will never meet and it has forever marked their lives in a way few people mark the lives of anyone. I don't think I am being an overly proud or boastful mother to say I think the sufferings of the little child asleep with exhaustion in front of me right now with drains and monitors and depending on an O2 boost to keep her sats from crashing has done the same. Why are you crying about Louise? Because you know she doesn't deserve it and you know that we complain all the time about nothing. It puts us to our knees, it makes us hug our healthy children with a closeness we'd never have thought of. You know, I used to care what people thought of me. To know your child is sick and that somebody else's prayers might help soon cured me of that. Of course I still care about reputation but you know what I mean.

God doesn't make mistakes. He doesn't and He didn't. Louise is not a mistake. She's not an anomaly, she's not a problem. Louise and the children I've come to know through her are not the problem. They're the antidote.

So take that antidote and thank God for sick children. It's an honour and a privilege I could never have imagined to have a suffering child in my life. I need her, you need her and the world needs her. She is Simon and God picked her.

And now I'm going to publish this without reading it or I know I'll delete the whole thing.

J xx