On why I might once have been angry…

I had a conversation recently with a Paediatrician at our local hospital, who whilst discussing a current medical issue brought up that he was concerned that he had heard that I was angry at the hospital for failing to diagnose my daughter when she became sick in the early Summer of 2015.  He mentioned that I had taken my folder with me to our last visit, and that the staff had commented that I was very angry at the time.  I am not sure when he was referring to, as our recent visit when I took the file was actually a positive experience, and I was impressed by the communication between the three hospitals involved in Freya’s care.  Yes, I took my folder with me, but I take it with me everywhere.  That folder contains the letters from every clinic appointment Freya has had in the last 18 months – Rheumatology, Cardiology, Immunology.  I take it everywhere with me in case a medical professional needed to see the history, and actually it was useful because the hospital hadn’t been copied in on the letters and took copies from me while we were there.

The fact that the doctor brought it up has been niggling me for a little while, so I thought I would try and capture why in this blog post and try to explain why a parent might act in a certain way when their child has been critically ill.

When Freya became sick in May 2015, she was immediately admitted to hospital and her condition very quickly deteriorated.  Within hours of being admitted, she went into septic shock. When the first doctor who saw her decline started to take action, I felt like she was in good hands.  They moved swiftly but calmly as they got her into the High Dependency Unit to administer fluid resuscitation.  I remember the Consultant giving out instructions to the doctor and the nurses in the room, and they responded quickly, without any alarm.  I could feel the tension in the room, and I watched through the bars at the end of the cot in quiet disbelief at what was happening in front of my eyes.  But I watched in awe, not really knowing what they were doing, but feeling that it was right, whatever it was.

For 6 days, Freya was treated with a number of antibiotics.  The main suspect was meningitis, but she was too sick and weak to have a lumbar puncture to diagnose it.  During that first week, Freya displayed a fever that would not abate with the usual paracetamol and ibuprofen combination.  She had sore, red, cracked lips – with hindsight that was a symptom of Kawasaki Disease, but it was attributed to dehydration at that time.  She developed a rash all over her body, but in the first week that would disappear almost as quickly as it came.  Her hands and feet looked puffy and swollen, another symptom of Kawasaki Disease, but with KD being so far from everyone’s minds with her being such a tiny baby, it was put down to swelling at the cannula sites.  Numerous blood tests were taken in an attempt to identify whatever it was that was making Freya so sick, but nothing except the common cold virus (Rhinovirus) would show up in that first week.  That wasn’t enough to make my little girl so very poorly.  A lumbar puncture on day 6 would be inconclusive – elevated white blood cells, but no definitive sign of meningitis.  Perhaps the cocktail of different antibiotics delivered intravenously in that week had dealt with it? So why wasn’t she getting any better?

Apart from one locum doctor who visited Freya in the HDU and suggested we stop all the medication rather than find a new cannula site (I know, right?!), every nurse and doctor that took care of Freya in our local hospital was fantastic.  Not only did they take care of Freya, but they took time to take care of me too.  They treated Freya as if she were one of their own children, and they showed an interest in me, listened to me and showed me nothing but respect.  Some of the faces I can’t even remember, but there are a few that stand out and are etched in my memory, even if I can’t remember all their names.  There were moments too, that I’ll not forget. The time that one of the senior nurses removed all of the equipment from the room so that we could allow my other children to visit without frightening them, that was a highlight.

I hope that the nursing staff know how much I appreciated how they took care of Freya.  If I had any complaints at all they were tiny – it bothered me that the syringe packaging was sometimes left at the end of Freya’s cot.  It didn’t cause anyone any harm, just paper and plastic, but it made me feel bad because her cot wasn’t a dustbin.  The time the heart monitor failed in the night and they replaced it, but left the broken one at the foot of the cot; it was my baby’s bed, and although she took up very little room in it, I didn’t want those things left discarded like it didn’t matter.  And that’s it really, my only criticism.  And I know it might sound petty to share, but I hope that any nurses reading might think about how such a small oversight even when they’re obviously run off their feet, could make a new mother feel when she has too much time to think.

Even though the first hospital failed to reach a confirmed diagnosis, I do not feel anger towards them.  It is easy for me to sit here now and say that Freya’s symptoms were clearly indicative of Kawasaki Disease, but hindsight is indeed a wonderful thing, and I don’t know that they could have done anything differently.  In fact the Paediatrician said that he could understand my anger had Freya been diagnosed within hours of her transfer to the children’s hospital, but it took them a further week to diagnose her, so it was clearly not an easy undertaking.  I said that they had looked for KD on the first day in the new hospital and he said that was right, because it was he who had suggested that was what they should look for.  And now it makes sense why when they told us they were moving Freya, that they mentioned a heart scan.  I thought it was just a routine thing, but clearly they had a suspicion that needed to be confirmed outside of their own local facilities.  And that’s probably my one and only criticism of the doctors of our local hospital –  I wish they had told me what they were considering.  The problem is I never really asked them what they were looking for.  I was naive.  I thought they could just take her blood, plug the results in and hey presto! technology would have the answer.  So I let them do their work, and didn’t ask a thing.  The only question I wanted answering was one that I didn’t have the courage to ask.  I gave that task to my husband; is she going to die?

Had the doctors mentioned Kawasaki Disease, would it have changed anything? Well, you know that is a question that has haunted me since Freya’s diagnosis.  My instincts tell me that I would have read about the disease and asked more questions.  I would have asked the most important question of all, “Why isn’t it Kawasaki Disease?” And when they told me she didn’t have some of the symptoms I would have cried, “Yes! Yes she does, see?!”  They would have reached a diagnosis and given Freya the treatment on day 8, not day 13 when the damage had already been done.  We would have left the hospital, emotionally bruised, but with a child that had been very ill but was now well.  And we would have gone back to our normal lives…

The reality isn’t quite so straightforward.  Would I really have come to the conclusion that the doctors couldn’t, simply by reading what I could find on Google? I have heard of cases that have been diagnosed entirely because of the parent’s knowledge.  Would I have done that? Or would I have read about this obscure disease with it’s stupid motorbike name, laughed and moved on?  I guess even if I had helped them to reach a conclusion, even if they had managed to give her the treatment within that magic 10-day window, Freya was in the category with the highest risk of heart damage – a neonate, female, displaying severe symptoms which we now believe was KDSS.  When she finally got the treatment, she was resistant to both doses, and needed much more aggressive treatment to finally halt the path of the disease.  Maybe she was biologically destined to be one of the unlucky ones?

That said, I wish the doctors had told me about Kawasaki Disease.  It might not have come as so much of a shock when it was finally diagnosed, I might have been better prepared.  The Children’s Hospital decided not to mention it too – had they told me that was what they were looking for when they did the first echocardiogram on day 9 which showed them a reasonably healthy heart (a small murmur) and led them to rule it out as a prime suspect, perhaps I would have reminded them of the symptoms they hadn’t seen for themselves the previous week when we were at the local hospital?  And perhaps I would have had more respect for the doctors in the first week had I known that they suspected it first, but weren’t in a position to confirm it.  That’s why they moved Freya.  They wanted the Children’s Hospital to confirm or deny their hunch.  The Children’s Hospital did an echo and the results lead them to turn their investigations elsewhere.

I felt angry about that for a long time, in fact I’m not sure if a part of me is still angry.  I was initially angry at the local hospital for not getting it in that first week. Let’s face it, when we finally got the diagnosis and learned that Freya’s heart had sustained permanent damage, we were angry at the whole bloody world.  Now I know that the local hospital had KD on their radar, I feel less angry.  I only wished they’d told me.  So how do I feel about the Children’s Hospital?

Much like my experience locally, I met some really fantastic nurses during our stay.  In fact in all three hospitals (Freya would be moved to a Cardiac Unit in another hospital upon diagnosis) we met some of kindest, most caring people I have ever had the pleasure to meet.  I guess that’s why they do the job that they do.  It was a much busier hospital, so there was less time for chit-chat and the nurses seemed run off their feet moving from patient to patient to carry out observations and deliver medication. We were in a private room with Freya so were quite out of the way, which was isolating at times.  There were a couple of issues with medication – an oversight that meant she nearly didn’t get the second half of the drug that was meant to be saving her life and was only pointed out by me when I awoke to see there was no IV going in; that was a big one.  A lot of the time I felt like we were more of a number than we had felt at the previous hospital, but I’ll play Devil’s advocate that they were just so busy.  The majority of the nursing staff were wonderful, especially with Freya, which is what matters.  The nurses that came to talk to me in the night when I lay there crying on the foldaway bed, those that rocked Freya to sleep when I didn’t have the energy or the heart, they’re the ones that have stayed with me since our ordeal.  The actions of the nurse who accompanied us on a transfer to the cardiac unit when the ambulance didn’t arrive to return us until well after her shift will never be forgotten.  She saw it as her absolute duty to care for Freya and made arrangements to ensure that Freya’s medication and observations were carried out in that delay.  She had finished her shift hours before and had her own child to get back to, but all she could think about was keeping Freya safe – it distressed and frustrated her that she was unable to care for her patient, and she did something about it.

The Doctor’s at the Children’s Hospital were always around, either popping in to check on Freya, discuss possibilities.  They always seemed to be busy looking at the files, searching for answers and I don’t doubt that they lost many winks of sleep trying to work out why this beautiful little girl wasn’t getting any better.  I didn’t know until way after her diagnosis that KD had been considered and ruled out.  I wish they had told me at the start.  But again, maybe it wouldn’t have changed anything.  I would like to ask them now why they didn’t think it could be KD, and not just because the first echo was clear – you can’t diagnose KD from an echo.  They believed that Freya’s only symptoms were the fever and a rash.  I believe that her cracked, red lips and the swollen hands and feet were also symptoms – enough symptoms to confirm a diagnosis.  Had they understood more about the disease, might they have also known that whilst KD is very uncommon in young babies, young babies are more likely to suffer from Kawasaki Disease Shock Syndrome? Had they considered that, might they have realised that whilst thrombocytopenia (low platelet count) is not a common symptom in KD (in fact is is usually very high), it is a characteristic of KDSS?  Might they have looked back over the previous week and seen that everything about her was synonymous with a KDSS diagnosis?

Perhaps my expectations of them are too high – they cannot possibly know every single childhood ailment in detail.  There are over 7000 rare diseases in this world and KD is just one of them.  Some doctors won’t ever have seen a case in their career.  They spend most of their career dealing with ‘horses’ and are not on the look out for the odd zebra that might turn up.  Is it only hindsight that allows me to see now what they couldn’t then?  I do know that they have learned from Freya, that she has taught them a lot about the disease.  I do know that they are less likely to make the same ‘mistakes’ again.  Again, I just wish they had told me what they were looking for, and why it couldn’t be Kawasaki Disease.  Because when they ruled it out, for whatever reason, no matter how unusual Freya was, they got it wrong.  They got it wrong, and I will never know if that decision was responsible for the damage that KD did to her little heart.  I cannot change the past, though, and so I have to be thankful for the doctor who came to see her when the rash appeared on day 12 and suggested she be sent for another echo.  That echo showed the damage that confirmed it had to be Kawasaki Disease.  That doctor may just be responsible for saving Freya’s life.

So let me get back to the point of me writing this blog.  I guess I wanted people to understand that I may well have been angry at times, but that my anger does not mean that I do not appreciate everything that all those medical professionals did and have continued to do in taking care of my daughter.  At the time I wanted someone to blame for breaking Freya’s heart. And mine.  I was angry at life.  I was angry at a God that I’m not even sure I believe in.  I know now that no amount of anger or blame was going to change anything, but it is one of the stages of grief.  I could not believe that I had taken my perfectly healthy child to hospital with what looked like a virus, and took her home with a heart condition.  That had to be someone’s fault, didn’t it?  Who knows.  Nothing is going to change.

Do I think that a diagnosis might have been reached sooner? Yes, I do.  Do I wish that they had asked me what I thought, told me about Kawasaki Disease and asked me if I could think of anything that they might have missed? Absolutely.  Do I consider myself lucky that Freya fell ill on a Sunday at 7 weeks old and was therefore too much of a risk to leave to chance? That I didn’t have to face countless appointments with GP’s who might have sent us home with the usual ‘virus’ diagnosis? Yes, yes, and yes – when Freya went into shock, she was absolutely in the right place; what if that had happened at home?  Do I thank my lucky stars that regardless of all that anger and confusion, regardless of whether it was ‘too late’ or not, that the right doctor walked in at the right moment on the right day and helped them to reach the right decision? Yes, a million times yes.

I have found peace with that part of our journey now.  I am just grateful that Freya was strong enough to fight, and that she is here, bold and bright and beautiful.  I used to refer to her as ‘damaged’, ‘broken’.  I don’t see her that way anymore.  The hidden faults within her heart are no longer what defines her for me.  And whilst I can’t change the path that we have been down, I can make a difference to the future of Kawasaki Disease by sharing our experience and never giving up when it comes to raising awareness.

I hope you will continue to support us.

www.bluemama.co.uk          www.facebook.com/freyasstory              Or Tweet @freya_story 

Then and now…

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Freya and The Fairy

One Spring morning,
When Freya was two,
She wandered the garden
For something to do.

While she was looking
At flowers and bees,
She saw something unusual
Amongst the trees.
She decided to look
A bit closer to see
What could be down there?
My! What could it be?!

Young Freya looked down
At the tree stump below
And guess what she spied?
A door, ever so low,
With a tiny gold handle
And a snowbell to ring
She couldn’t help wonder
Who on Earth lived within!

She wanted to knock,
But her hands were too big
So she looked all around her
And picked up a twig.
Gently she used it
To tap on the door,
So surprised when it opened
She fell to the floor!

And what do you think
Stood in front of her eyes?
A tiny young fairy,
Oh! What a surprise!
The fairy looked frightened
To see the huge child,
But she began to feel calmer
Went the girl-giant smiled.

“Hello! I am Freya,
And I live in this house.
I am sorry I scared you,
I was quiet as a mouse.
I discovered your door,
And was excited to see
If there was someone inside
Who’d be friends with me.”

The fairy flew up
Into Freya’s kind hands
And said, “I’d be so happy
If we could be friends.
But the Queen might chastise me
For talking to strangers;
She’ll be worried my new friend
Might put me in danger.”

“Oh no!” exclaimed Freya,
“I would never hurt you.
You can be my little secret,
And I’ll be yours too.”

And so from that day
In the garden they met,
Every bright morning
(Unless it was wet).
Under cover of trees,
They’d sit in the flowers,
Where if mum didn’t call
They’d have chatted for hours.

As the days passed,
The two became close,
As they sat on soft pillows
Made of daisy and rose.
They developed a friendship
That they never betrayed –
Friends they were then,
And friends they remained.

Joanne McBride / 28th November 2016

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Why I Keep Banging the Drum

There’s so much I want to say, I don’t even know where to start.  I guess I am writing this post today to try to help people to understand why I have been banging a metaphorical drum for Kawasaki Disease for more than a year now, and why I am unlikely to stop banging any time soon.  Perhaps if I could make you all understand why, you might forgive me when my blogs and posts pop up in your social media feeds, and maybe you might even help support me along the way.

Kawasaki Disease is a rare, childhood illness with no known cause and no diagnostic test.  Without a known cause, it is impossible to prevent children from being struck by the disease or come up with a test that will confirm a diagnosis within the ‘magic’ timescale for effective treatment.  The disease causes inflammation of all the blood vessels (veins, arteries and capillaries that carry blood through the body’s tissue and organs).  It is believed that there is a population that have a genetic susceptibility to the disease, and within that susceptible population there is another subset where heart damage is more likely to occur. In 25% of cases, the disease reaches a child’s heart and causes irreversible damage.  With treatment, currently high dose aspirin and a product called immunoglobulin, the inflammation can be stopped in its tracks, reducing the impact on the child’s heart, but that is considered most effective if the treatment is administered within 10 days.  And therein lies one of the biggest problems for our children – with many medical professionals having never heard of Kawasaki Disease, it is often diagnosed too late.  Sometimes not at all.  And even when it is, in some countries immunoglobulin is either not available, or just too darned expensive, so many kids go untreated.   

The symptoms of the disease present themselves like many other childhood illnesses, which poses another problem and leads to misdiagnosis in many cases.  Add that to the fact that not all of the symptoms appear at once, and sometimes not at all, and you can see why this is one that doctors are often getting wrong.  Freya was in hospital for 13 days before she received a diagnosis.  She was treated for sepsis and suspected meningitis, underwent countless blood tests and other invasive diagnostic tests, while the doctors scratched their heads and waited for the test results to confirm something.  While they watched and waited, the disease was taking its hold on Freya’s tiny body (she was 7 weeks old), and by the time we reached day 13 it had already taken hold of her heart.

When the coronary arteries become involved, the inflammation can cause areas of the artery to balloon out – that is called an aneurysm.  There are different kinds, but I don’t want to bore you too much with the science.  Coronary aneurysms do not generally occur  (if at all) in children.  They are usually the result of heart disease caused by a build up of fat, cholesterol and other things not usually associated with young children.  In the best case scenario, the aneurysms will reduce over time, and the arteries may even appear normal.  It is worth noting, however, that these arteries will never in fact be normal, and the child will be subject to long-term monitoring and/or medication in order to prevent further complications.  The most dangerous risk associated with coronary aneurysms is rupture, where the wall of the artery cannot take any more stress and bursts –  game over.  Other risks include blood clots, which can lead to heart attack, and narrowing of the arteries as a result of built up layers of blood or scar tissue.  In both cases, narrowing restricts the flow of blood to the heart, which can also cause a heart attack.  Girls who have sustained coronary aneurysms as a result of KD  will have to be monitored by a Cardiologist through pregnancy and childbirth – just one example of how this disease affects the future of a child who has suffered.  Approximately 75% of cases where the heart has become involved will go on to require intervention in later life – a stent, bypass, or a transplant for example.  Kawasaki Disease is indeed the gift that keeps on giving.

Why do I need to tell you that?  To be honest, I don’t think any amount of words could help me to get across the impact Kawasaki Disease has had on my life. I need you to know that this disease exists.  I need you to know the symptoms so you might recognise them in your own child if they become ill (http://www.nhs.uk/conditions/Kawasaki-disease/Pages/Introduction.aspx).  I need you to appreciate that until the cause is found we cannot protect the world’s children from the disease.  I need you to know that this disease is crafty and insidious – in the same way that it creeps into the child’s life, it remains there, barely seen and in many cases sticks around for life.  These children, children like Freya, do not outwardly wear the scars of Kawasaki Disease.  On the outside they look like any other kid.  They laugh, run, play, act up, and mostly they look the picture of health.  I am glad of that, but if I am honest I also find it frustrating, because if Freya looked a bit more like a victim, was weak and fragile and vulnerable, we might get more support in our fight against Kawasaki Disease.  

There are research teams dedicated to finding the cause of the disease, and changing the future of KD forever.  But research costs money.  Doesn’t everything?! But before you switch off because you think I am about to make a plea for your hard-earned cash, let me tell you that this post isn’t about asking you for money.  There are organisations in the world that have the means to change the future of all disease, KD included.  I know that unless you have been personally touched by critical illness, you are unlikely to feel the intense pressure I feel to be part of that change in the world.  I know that there are over 6000 rare diseases in the world, and that Kawasaki Disease is just one of those; why should that be more important than the others? It isn’t more important generally, I get that, but it is more important to me and the millions of parents who have watched this disease change their child forever.  I have contact with parents and grandparents whose own lives were changed forever by Kawasaki Disease when it took the lives of their children.

I am sat here crying with sheer frustration that I cannot change this.  I’m not talking about reversing the clocks for Freya; I can’t change the past.  I’m talking about making enough of a difference to the landscape for KD that it cannot be allowed to take the life of another child because it went unchecked and unnoticed.  When I think about how many more children will suffer as Freya did I feel suffocated, the bile rises up from my stomach like gnarled hands that clench my throat from the inside.

And that is why I share Freya’s Story.  I first started the blog as a hand-written journal whilst we were in the hospital last Summer.  It kept me sane, helped me to remember the details that would help me secure the best possible care for Freya.  It was at times my way of looking forward to an uncertain future, to a time when Freya would read all about adventures she would most likely forget.  At times, I thought she would never get to read it, that she wouldn’t make it.  Her story means that more people know about the disease than they did before.  It has offered hope to parents, and provided information to help others be an advocate for their child.  It has provided parents with an outlet; someone who understands how this feels.  At best, it inspired a gentleman to donate £75,000 to the research program, and Freya’s own birthday party raised £8000 thanks to the huge community spirit that enveloped us at that time.

All of these are reasons why I keep banging the drum, and will continue to do so.  I am sorry if you get frustrated with me clogging up your newsfeed.  I hope you don’t.  I hope you can see that the reason I feel so compelled to put so much effort into raising awareness and supporting the fundraising campaigns, is because if I can’t turn this into something positive, if I can’t make a difference, then this happened to us for nothing.  Nothing.  That just isn’t an option for me.

I have already seen how much of a difference we can make just by sharing our story and raising awareness – the £83,000 raised in Freya’s name is worth double that due to the Macklin Foundation Challenge Grant.  That grant will see a cash injection of $5 million into the research effort, provided we raise half that amount.  That could be enough to solve the mystery of Kawasaki Disease.  Dr Tomisaku Kawasaki is the Japanese paediatrician who first discovered Kawasaki Disease in the late 1960’s.  He is now 90 years old, but is dedicated to the belief that the mystery might be unravelled in his lifetime.  I sure would like to see it solved in mine.  He has written to the Chan-Zuckerberg Initiative (https://chanzuckerberg.com) asking for consideration as part of their $3 billion pledge to rid the world of all disease.  

So here comes the ask….

There is a petition to accompany Dr Kawasaki’s letter, and we’ve got until the 15th November 2016 to get as many signatures on that petition as possible to add some weight to the request.  I would really like all of my friends to sign the petition and share with their friends.  If you don’t want to sign it yourself, please just share the link so that others can be given the choice to do so, it would mean so much.  It takes just a few minutes to sign – open the link, fill in a few details, and hey presto! it’s done.  You won’t get a load of emails in your inbox either – just the one confirming receipt of your signature.  I have 500 friends on Facebook.  If I could get each and every one of them to sign, and they did the same, that could amount to 250,000 signatures on that petition.  For me, it feels like so much could be achieved from so little – you can read the case being put forward, and sign the petition here:

https://www.gopetition.com/petitions/kdresearch.html

You may remember me making a similar request to sign a petition last year.  That petition was started by the UK Kawasaki Disease Support Group as a means to raise awareness of the disease. Your support in that meant so much.  I cannot tell you how much faith I have in this petition from Dr Kawasaki – it literally has the potential to make a real difference in the world, and I for one am proud to say that I might be a tiny part of that.

“Never doubt that a small group of thoughtful, committed citizens can change the world; indeed, it’s the only thing that ever has.”  (Margaret Mead)

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P.S. I mentioned earlier that I wasn’t going to ask for money.  For the purpose of this post that is true, but please know that medical research is grossly under-funded and relies heavily on philanthropy.  So I will continue to do what I can to raise money for the KD Research Collaboration, and will share the information so that you might too if you feel so inclined.  You can donate via the KD Foundation website (US) here – http://www.kdfoundation.org, or via UK charity COSMIC directly or via their fundraising page, below:

http://uk.virginmoneygiving.com/charity-web/charity/displayCharityCampaignPage.action?charityCampaignUrl=KawasakiDiseaseResearch

 

If you love somebody, let them go…?

Dear Freya 

I hadn’t realised just how much I love sharing my life with you until just now. I have a pretty bad chest infection, and I’ve been told to rest as much as possible, and that if I haven’t improved in the next couple of days I may have to be admitted to hospital. So to avoid the latter, I have pretty much been holed up in my bed since 7pm on Monday evening (it’s now Wednesday).

I started to miss you yesterday. Miss your smell, and your beautiful eyes, and your constant babble and chatter. Jeez, I’ve even begun to miss The Little Mermaid! Lying here I feel bereft of the things that I usually take for granted, of the things that I often find tiresome if I’m honest. 

A moment ago you quietly whispered “mama” from your cot. You didn’t need to cry or shout because you know that I am always there for you, and I’ll come when you call me. Your daddy came to get you, and you popped into my room for a minute before he took you down for some lunch. And in the seconds after you left, the tears came spilling out of my face like big fat raindrops. 

Yes, I guess I’m feeling a bit sorry for myself being under the weather and all. But it’s more than that. When you softly called “mama” I was catapulted into a future where you awake in someone else’s care, calling my name into a space where it will not be heard, not by me. And it made me wonder how on Earth I am going to be able to part from you in 6 months’ time when I am due to return to work and pick up where I left off two years before. Two years. It’s a long time to get used to being around somebody, and I must say that I never considered how I would feel at the end of it all when I extended my leave. 

Right now I can’t even think about what I am going to do, so I swat those thoughts away like a pesky fly and try to use the strength I have to will myself back to health so I can propel myself back into our daily, humdrum routine. To think that last week this Groundhog Day kind of life was becoming wearing! That I felt hard done to for not being able to escape it sometimes! And here I am, escaping all of the things that are part of being a wife and mother, and it’s killing me that there’s a load of washing to go on, no food in the fridge, and that the 58,000th viewing of The Little Mermaid is taking place downstairs without me. 

I can honestly say that I have never felt the wrench of an impending return to work like I do now. After two periods of maternity leave, I went back without question to a career that I had not allowed myself to miss, but had been looking forward to returning to by the end of a year. But things with you have been different. Perhaps because you are most definitely my last. Maybe because I am older now, wiser. Possibly because what we went through together last year created a bond that goes beyond emotion; like chemistry. Maybe just because you are like sunshine on a cloudy day. 

So I will rest. I’ll ask for help and accept it with gratitude and grace. I’ll not let this develop into anything nastier than it already is, because I cannot be removed from you. These last couple of days have given me a tiny taste of life ‘without’ you, and a sense of urgency over how we make the next 6-months worth the pain I know I will feel at the end of it. 

I was always so level headed about childcare before you. Now I cannot picture myself dropping you at someone else’s door. What have you done to me, sweet, funny, determined little Freya? I am undone.

Forgotten Fears

It seems like such a long time ago that I ever really felt afraid for you, but today I find myself reunited with feelings I hoped would never feature in my life again.  Looking at you, no-one would understand what there could be to worry about – you are a ‘picture of health’ as they say.  Those rosy cheeks and that sparkle in your blue, blue eyes betray what lies beneath.

For the last year, we have taken the news from every cardiology follow-up as fantastic steps towards you being a miracle in the making.  Each appointment showed further reduction in the aneurysms in your heart, and we celebrated every little move towards what we thought was your recovery from this awful disease.  And then, little by little, the reality dawned that the likelihood that your heart was magically repairing itself from the damage that had been done was slim, if near impossible.  My mind is filled with a constant echo of statements and warnings; babies diagnosed under 6-months are at increased risk of coronary stenosis, 75% of children with giant aneurysms will require intervention later in life, an angiogram will show us if there is any sign of stenosis, look out for signs of angina… the list goes on.

Something shifted this weekend following the receipt of the letter from your last Cardiology appointment.  At that appointment the Cardiologist measured your coronaries with an echocardiogram.  I noticed the figures 0.18 at the bottom of the screen – that is 1.8mm.  The cardiologist said “About the same [as last time]” and I remember thinking, “No, that’s smaller still…”  That said, the outcome was still the same, and your Cardiologist continued to urge us to proceed with the advised angiogram.  In the time since that appointment I had some e-mail exchange with a reputable Cardiologist in the Kawasaki world which gave me some comfort, and I have also spoken with the Cardiac Nurses who have agreed to make an appointment for me to meet with the consultant who will perform the angiogram to ensure I am completely comfortable with what you will go through when the time comes.  And then at the end of last week the letter came.  Encouragingly, your Doctor has discussed your case with a Paediatric Cardiologist in London, whose name I immediately recognised as one of the speakers at the KD Symposium in London earlier this year.  I was pleased that your doctor had taken this step, and it restored my faith a little.   Well a lot, really.  The letter also gave a better explanation than I received verbally at your appointment, and I now understand that an angiogram is really the only solution because of your size.  Although I still feel some degree of trepidation about the procedure, I have reconciled myself to the idea that this really is necessary for us to understand how this disease will continue to affect you in your future.

It was niggling me that the measurement was described as ‘the same’ at your appointment, but my memory was telling me something different. So I fished out your medical file (yes, I have a very organised file that is getting pretty thick now!) to have a look through the previous letters.  I was right – the previous appointment recorded a measurement for your left coronary artery (LCA) as 2.7mm.  So rather than being ‘the same’, the artery has actually remodelled by almost another millimetre, which is a reduction of a third of the size that it was just three months prior!  I don’t know if you can work out where my thoughts are going with this, but let me tell you, it has got me really concerned.

My initial concern is obviously the speed of remodelling.  OK, so we don’t know what is causing that – could it be layers of blood clot (thrombus) that is causing a narrowing of the arteries, or scar tissue forming on the artery wall as the aneurysms repair?  Since your KD journey began, I have plotted the measurements on a graph since the beginning (this will come as no surprise to those that know me personally!).  It’s been a bit hit and miss, because not all the measurements are documented in the letters (sometimes we get all three – left, right, and left anterior descending) sometimes only some, sometimes none.  And I haven’t always managed to jot the numbers down from the bottom of the screen during the appointments.  But one measurement that I have been able to capture consistently is that of your LCA, which was the most severely affected by Kawasaki Disease.

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What the graph shows me is that your LCA is now 65% more narrow than it was at it’s worst, and the pattern of remodelling has been as follows:-June (diagnosis) to July 2015             15% reduction

July to August 2015                               32% reduction

August to September 2015                 10% increase (anomaly?)

September to November 2015           6% reduction

November ’15 to May 2016                 13% reduction

May to September 2016                       33% reduction

Now I’m not statistician, but these numbers have got me worrying about the rate of remodelling.  How long it could be before that 1.8mm results in 100% stenosis?  And if your arteries become completely occluded, preventing the blood from flowing through your heart, there is no other outcome but a heart attack.  Is that even possible?!  I can’t even…

So now I’m thinking all sorts – why didn’t the Cardiologist mention this difference at the last appointment? Is she not as worried as me?  She said the measurements were the same, but they clearly are not.  And if you are now on a waiting list for months before the investigation can be carried out, I am worried that you could suffer a heart attack before we get the chance to understand what is going on. I mean, can that happen? What is the likelihood?  It’s possible, but is it probable? 

I’ve left a message with the Cardiac Nurses – I really need to speak to them to air my concerns and get some answers. I feel, whether logical or not, that we are just waiting for your heart to fail.  And what just dawned on me last night as I was over-thinking this whole situation is that we have never considered when the worst could happen.  We were told to look out for signs of angina – the only sign we were made aware of was if you were to suddenly stop playing for example.  But last night as I marvelled at this amazing little girl who sleeps through the night, always has, the thought entered my head; what if it happens when you are asleep…?  I mean, if I were a statistician I would be able to calculate the probability of a heart attack happening at a certain point.  You sleep between 14 and 17 hours per day!  That means that you are asleep for up to 70% of any given day…

So now I have a new fear, and I cannot shake it. It kept me awake last night and brought tears silently streaming as I lay there struggling to get to sleep.  It caused me to jump out of bed on more than one occasion to check the monitor was switched on, and it meant that I woke this morning with that old feeling of dread until I heard the first murmur from your room.  When you were diagnosed with KD and we were told that your heart was significantly damaged by the disease, I feared every day that I would wake to find you taken from us.  I was so afraid of finding you gone that I was frozen to the bed until I heard even just the slightest movement from the baby monitor.  Every bit of me wanted to get out of bed and check you were breathing, but I just couldn’t physically move because the fear kept me pinned down.  How would I know? I mean are there any signs? You often cry out in your sleep, maybe just once, and then you drift back off to sleep.  Is that what it would be like? If you were sleeping, and me and Daddy were fast asleep, would there be anything to hear?

I have read that the signs of heart failure in a child could include the following:-

  • excessive sweating
  • extreme tiredness and fatigue
  • poor feeding
  • rapid heartbeat
  • rapid breathing
  • shortness of breath
  • chest pain
  • a blue tinge to the skin

Would you let us know about any of those if you were sleeping? Would we hear? Would we know you were in trouble?  You think about emergency procedures in the event of a heart attack – call 999, get an ambulance, perform CPR… But if it happened in your sleep and we didn’t know, you could be left for hours before we realised, and by then it would be too late.

I wish I didn’t have such a vivid imagination. My mind plays out these scenes like a movie in my head, and I can’t shake it.  I have ‘seen’ it – the moment I find you gone.  I have ‘felt’ how I didn’t want to live anymore in a world without you in it.  I am angry at the world, angry at this insidious disease that crept up on you and broke your heart.  And I am afraid beyond words of what might be around the corner.  All parents worry about the fate of their children.  Hopefully most see the death of their child as an unlikely threat, and it features just for a nanosecond of each day.  To know that there is a possibility (regardless of the probability) of losing your child is a feeling I cannot describe adequately to anyone who hasn’t experienced something similar.  It’s like standing on the edge of a cliff, a dark and indeterminate cavern beneath, knowing that just one breath of wind could change the course of your life forever.  Sometimes I can barely breathe for fear that the slightest movement could unbalance me.

You have many people rooting for you – hoping, wishing, praying.  Some believe you are going to change the KD history books.  Others say you are a fighter, an inspiration and a miracle.  I’m too superstitious to believe in any of that for fear of jinxing your progress.  There are some that have so much faith in God that they ‘know’ you will be ok.  I worry that if there is a God his plan could be for the world to learn from a tragedy – your tragedy.  I just don’t know anymore, Peanut.  All I know is that right now I find myself out of control yet again at the hands of this disease.  I am still in utter disbelief when I consider the events of the last year, and often think about bundling you in the car and running far, far away from all this.  Pretend it never happened.  But there isn’t just you to think about, and I have responsibilities and relationships to consider.  And sometimes, no matter how hard it is, I just have to accept that I am indeed a grown up.

I don’t want to be a grown up anymore 😦 

 

A Day of Ups and Downs…

…or that time the Cardiologist ruined our day

Dear Freya

What a day we had yesterday! The time had come around for your cardiology follow-up appointment in Leeds.  I can’t believe it has been three months already since the last one! At that appointment, your doctor told us that they want to perform an angiogram to get a closer look at your heart to understand what is causing the rapid remodelling of your coronary arteries.  I bought us three months’ breathing space little Peanut, but it just flew so quick, and here we are again.

Your appointment wasn’t until 15:40pm.  I figured we could sit around at home all day agonising about the impending appointment, or we could make a day of it.  So Daddy dropped us off near the train station, and we hopped on a train to Leeds at around 10am, not before sharing a cinnamon swirl from the coffee shop on the platform.  You were such a good girl on the train.  We managed to get a seat with space for the pram (very unusual! We usually have to resort to standing room only, even if we manage to get into the wheelchair carriage because often ignorant, able bodied people sit in those seats).  It isn’t a long journey to Leeds, so it felt like we were there in no time.  Luckily a nice gentleman helped me to get the pram off the train, and we were on our way!

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I thought we would have a little ‘mooch’ around the shops for a bit, then grab some lunch before your appointment.  I had arranged to meet with a friend – a fellow Kawamum – and her little boy.  You remember Tom – we meet for playdates sometimes.  Well he had an appointment at the hospital just after yours, so we thought it would be nice to catch up.  We went directly to the Trinity Centre – there is a ladies shop there called Mango that I like very much (despite being told by a young girl on holiday once that she found it a “bit mumsy” – ouch!!).  As we were heading towards the lifts, a woman with a clipboard and a gentleman with a video camera approached us.  The lady said that they were filming a TV commercial (I think for the Trinity Centre), that they were looking for certain types of people, and I was exactly what they were looking for.  She asked if I would be interested in leaving my details, and I said “Go on then!”  So she wrote my name, my age and my number on a card and I had to hold it for the cameraman whilst he recorded and took some stills.  The shoot is on the 13th September – I said I would have you with me, and they were happy for you to come too.  So we just wait now, and see.  I really don’t mind which way it goes – it was nice to be stopped and asked, and it was fun saying yes.

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We wandered along to Mango where I had a browse through the clothes – you moaned as soon as you saw what kind of shop it was, so I bribed you with a biscuit.  You were great while I tried on a few things, and I walked out with a bit of a dent in the bank balance (oops! Those savings are not going to last me much longer on this career break!)  From there we headed down to Harvey Nicholls (don’t judge me! It was only because I know they have a MAC counter and I wanted to buy a specific lipstick).  I picked up a few things, and joined the Harvey Nicholls Rewards club – don’t think I’ll be earning many points in there, haha!   All the while, you were chattering away in your pram, shouting “Hiya!” at everyone who passed by (we need to have a word about stranger danger soon).  Outside the sun was shining – that was a turn up for the books, as the forecast had suggested rain all day.

From the shops, we took a walk down to the bar where your cousin works.  She’s just graduated from University this year, and is now out in the big wide world.  She was pleased to see you – it was very quiet in the bar.  We ordered some lunch, and your cousin joined us while we ate.

Shortly after that I got a call from my friend, the Kawamum; she had arrived, so we walked to Millennium Square to meet her.  You and Tom had a lovely time watching the waterfall and running around the square chasing the pigeons.  I chatted to his mum about the latest with you two, and before I knew it your Daddy was on the phone – he had arrived at the hospital, it was time to go.

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When we arrived at the hospital, we were immediately sent for an ECG – that’s normal, don’t worry! We always get that out of the way first.  Problem is you hadn’t had a sleep earlier in the day because you refuse to sleep anywhere but in your cot, so you were a bit grouchy by the time we went in.  The lady tried to put the stickers on you, but you weren’t having any of it.  You were crying, and screaming, and pulling the stickers off.  Another lady came into the room who was really surprised by your reaction, as she has done lots of your ECG’s in the past and you have always been such a good girl.  We managed to calm you down in my arms, and the lady attached the wires to the stickers (not technical terms, I know!), but no sooner had we tried to lay you down again were you crying and screaming and ripping the wires off.  They decided that you were too distraught now to continue; they didn’t want to put you off for life, but also the reading from your heart would not have ben a normal one with you so worked up.  I was worried about us missing something, but they reassured me that the echocardiogram would show up anything we needed to see. I hoped you would be more settled for that.

We went back to the waiting room and you were called in to be weighed and measured.  As soon as you walked into the room you started crying and screaming again.  You have been weighed in there so many times in your little life – it’s just a room with a table, a changing mat and some weighing scales.  But for some reason, yesterday it seemed much more sinister to you, and you were just too tired to take it.  We did managed to get you weighed and measured though, as you were able to stand up on the big girl scales instead of having to lie down on the table.  We went back out to the waiting room where we were told that the cardiologist was running a bit late, so you played for a while until we were called.  A baby was crying in the corner, and you looked ever so concerned and kept saying “Ahhh”.  And in the next breath you were pulling the steering wheel off the push along car and driving it into the wall.  You are a monkey!

Your doctor finally called us in, and we went into her room.  We updated her with your current aspirin dosage and told her that you have been displaying some unusual behaviour.  Specifically, you have started randomly lying down on the floor after some activity and just staying there staring for a while before we give you a nudge and you get up.  That might not sound particularly alarming, but the cardiologist had told us to look out for signs of angina, and when we asked her what they would be, this was the only symptom she could describe for a child as young as you who is not able to verbalise any pain they might be feeling.  We then went on to do the echo.  Because of your reaction to the ECG I had sent Daddy down to the shop to get something that might bribe you to lie still and let the consultant do her work.  He returned with chocolate buttons, which we slipped into your mouth one by one until she was finished!  You need to know that your brother and sister didn’t get chocolate until they were at least 5 years old, so I apologise in advance for any resentment that might build up over the coming years! 

The Cardiologist seemed pretty pleased with what she could see on the echo.  I noticed that the smallest measurement of your coronary arteries was now 1.8mm. The doctor said that it was about the same as last time.  I think they have actually shrunk a little further than before, but we are talking in fractions of mm so I guess it’s neither here nor there.  Once she had finished, there was a pause.  And then she looked at me and said “I want to do the angiogram.” Just like that.  I don’t know what I was expecting really – she had made her point clear at the last appointment, so why did I expect it to change?  Hope, I guess.  I told her that I have recently met with a world leading KD specialist from the US and attended a KD Symposium in London with esteemed professionals from the Kawasaki world.  I mentioned that a number of professionals had advised me that a cardiac catheter angiogram was not the most suitable procedure as it is invasive, and that there are other procedures that would be more appropriate for Freya, namely a CT angiogram.  Our doctor disagrees.  She feels that the resolution from a CT angiogram is not sufficient to show us what she is looking for – evidence of thrombus (layering of clotted blood inside the arteries) or stenosis (build up of scar tissue).  She dismissed some of the advice I have been given by the KD specialists because “they are not Cardiologists”, which is fair enough if not a little short-sighted.

After a few questions, I asked “Do we have a choice?” And her reply came as a shock.  She said that her advice is to perform a cardiac catheter angiogram, that it is the standard protocol for the hospital in relation to the aftercare of KD children, and that if I do not trust her advice then she would have real difficulty in continuing to be responsible for Freya’s care, giving her no option but to refer us to another hospital.  Wow! My way or the highway! And that’s where we are today – sent away with a decision to make.  And it feels like Hobson’s Choice.  Either we go ahead with the advice we have been given, against the advice of professionals who have seen more cases of this disease than our doctor will ever see, or start all over again with an unknown quantity – better the devil you know? Or is the grass greener?  I have no idea what we should do.  All I know is that I have seen this in my nightmares.  I have watched you go to sleep, left you in the hands of a surgeon, and waited.  And I have heard the words, “We are very sorry Mrs McBride, but the catheter caused a spasm in the coronary arteries, triggering a heart attack, and we sadly lost your daughter.”

I know in the scheme of things, this procedure is pretty insignificant to a cardiac unit.  The  little babies in the waiting room with that telltale scar down the centre of their chests is enough to give you some perspective over that.  But you are my baby, and I am scared.    Depending on how the procedure goes, you will be under general anaesthesia for at least an hour.  They will insert a thin tube (catheter) into your groin (most likely entry point, although this can differ) and feed it up into your heart to release contrast dye which will help them to see the insides of your coronary arteries.  That’s a pretty big deal for me and it doesn’t come without risks.

As I write this, I feel sick in my stomach, and my eyes are pooling with tears.  I cannot believe that we are going to have to hand you over to a surgeon again, watch you go to sleep again, and put our trust in strangers to get this right again.  Your last experience was deemed as necessary without alternative (you had a bone marrow aspiration to test your cells for Leukaemia before your diagnosis with Kawasaki Disease).  Although it was traumatic for us, we felt confident that it was necessary.  I don’t know why, but I just can’t seem to shake this gut feeling that this procedure is wrong.  Maybe I am just scared.  Who wouldn’t be.

All I can hope is that you are reading this, and laughing at how silly I was to be so worried.  “Jeez,mum! I’ve had twenty of these things now! I can’t believe you were so stressed that first time around!”  

We need to get a closer look to see what’s going on in there sweetpea. I just wish there was another way.

You can also follow Freya’s Story on Facebook and Twitter:

http://www.facebook.com/freyasstory and @freya_story 

Everything changes

I have just returned home from handing back all my equipment to my employer as I embark on this new chapter in my life.  Funny how strange it felt handing back a laptop and a mobile phone that have only been locked away in the garage for the last year anyway.  I guess it felt somewhat symbolic of the final step towards (temporary) detachment from my career.  And just being there in the restaurant, drinking coffee like I used to every morning when I was there, felt quite surreal.  More so, because I had you by my side, and a rice cake in my hand instead of a notepad.  I was very aware, having just finished a year of maternity leave, that under normal circumstances I would have been there alone.  I would have been the one rushing off to that conference call, or heading off to a meeting.  I have no regrets, however. Non, je ne regrette rien.   I consider myself incredibly lucky to have the chance to take some additional time to concentrate on your immediate needs, and to regain some of the time we lost last year.  With your brother and sister I would have missed so much of what I get to see every day with you sweetheart.  Watching you develop and grow is a source of constant amazement for me, and I don’t think I will ever tire of spending time with you.

The effects of the last year were not just felt by me.  I’m sure they were felt by you, but I have no idea how because you can’t tell me yet.  I hope that you don’t remember any of it. That all you remember is how we used to go on the train for days out, or for coffee and cake and to the park.  I won’t ever forget that the backdrop for all of those things was an imposing hospital looming behind us.  But hopefully you will just remember feeding the ducks in that nice park, and you won’t have noticed the tears in my eyes that came because I was saddened by the memories that the park evoked.  One day we will be able to go to Weston Park and sit on a bench and let the warm sunlight wash over us as though it were sent right from Heaven.  We will lie on a blanket and make pictures in the clouds, feed the birds and the ducks and watch the fish in their shady hiding place under the little bridge.  But we will always make our place on the other side of that park so as to spare the mums who, like me last year, watched other families enjoying the summer through the windows of a hospital cubicle.  The nurses thought they were helping me by moving me to a room with a view. The brick wall which was our previous view had been quite oppressive, but no more so than watching mums play with their babies in the shade of the trees.

Back then I thought I did a pretty good job of protecting your brother and sister.  We told them that you had a cold but needed the nurses to help get you better because you were too young for Calpol.  It was a white lie. Quite a big one, but white nevertheless.  And you did test positive for Rhinovirus in that first week, so it seemed like a plausible excuse.  We also kept your siblings from the hospital.  They visited you on your last day at the local hospital (after a week), and then maybe once or twice when we moved to the Children’s Hospital.  We didn’t tell them when you were moved to Leeds, so they didn’t see us when I was in my darkest place.  What I didn’t know was that our attempts to protect them probably made their anxiety worse.  I underestimated Eliza.  She has told me since  that she was very worried that I would be coming home without her baby sister.  She wasn’t stupid; she knew something must be pretty wrong and that a common cold wouldn’t need her mum and sister to be away from them for as long as we were.  So she actually worried more, because she knew it had to be bad for us to want to hide it from her.  And Fin? Well he went off the rails a bit. His behaviour at school hit an all time low, and the effects of last year have only just begun to wear off with a huge amount of support from the school.  He was the baby of the family before you came along, you see.  He was excited about getting a baby sister (although I do think he might have secretly been hoping for a brother to play Minecraft with!), but suddenly not being the baby anymore is quite a lot for any child to get used to.  And then just 7 weeks after we brought you home, just as he was probably getting used to you being around, he lost you and me for a while.  6 weeks.  That’s a long time in a 5-year old’s life.  Because we played down your illness so much, he couldn’t understand why his Mum would leave him.  He thought that I had chosen you over him, and his little head wasn’t quite ready to work that one out.  His self-esteem took a huge knock, and he became very insecure for a while.  No need to feel any guilt about that though, not you or I.  I made the decision to protect them for all the right reasons and I wasn’t ready to tell them that they might lose their little sister.  They didn’t need that worry.  And by the time you read this, you will have seen that none of this had a lasting effect on them; they probably won’t even remember it when you’re old enough to be reading this.

I am not the same person that I was before I had you.  I might even go as far as saying that you are lucky that this happened to you.  You have had the very best of me this last year, and I dare say  that you will continue to get the best of me for as long as we are both here.  I have not taken a single moment with you for granted, and because of that I think I am more patient with you than I might have been with the others. I am most definitely more intuitive about your needs. Perhaps some of  that is due to age (I was 40 when I had you), or experience with having done it twice before.  But I think most of it is the incredible bond that we have developed by spending so much time together since you came into the world.  Because I am scared of you getting very poorly again (I’ll tell you one day about chicken pox and the medication that you take), I have kept you away from large groups of children in the main.  I don’t take you to baby and toddler groups or play areas where the risk of infection is a constant worry for me.  I don’t wrap you up in cotton wool either – rather than avoid all situations, I tend to weigh up the situation based on my anxieties and I guess you could call it damage limitation, rather than complete avoidance.  That will become easier towards the end of this year (2016) when you have received the MMR, Chicken Pox and Flu vaccines.  Until then, we will stay together in our little bubble where we see just the right amount of people to allow you to build relationships with other children, but not enough for me to worry about you becoming sick.  I know I cannot protect you from everything, but I have to protect you from what I see as a risk, no matter how small that risk might be.  I cannot bear the thought of seeing you that sick again, Peanut.

My social circle is a little smaller than it used to be too.  There are lots of reasons for that. I’ve become very immersed in a Kawabubble over the last year and I don’t have an awful lot of time and energy for much else.  Most of my attention is on you, and getting you through the next challenges that the effects of KD will throw at us.  A lot of my attention has turned to our little family; this experience has made my family much more important to me than I think I allowed it to be before.  It’s also very difficult for me to spend time with people who cannot relate to our situation.  I don’t want constant sympathy, but I want compassion and understanding.  People that think that everything is ok now because I wear a big smile, and you look so amazing, don’t know me very well.  They don’t know how much I still struggle to come to terms with what has happened to you, and what the impact of the disease will have on your future.  70% of children in your situation will have to have invasive treatment later in life – a heart bypass, or a stent perhaps.  100% of children who have suffered coronary aneurysms as a result of KD will suffer myocarditis (inflammation of the heart muscle) which causes degeneration or death of heart muscle cells.  I don’t know how to compute the possibility that my child may show symptoms of myocardial infarction (a heart attack).  Just doesn’t seem real.  Later this year, whether it be through cardiac catheter angiogram or CT angiogram, we should get a closer look at the cause of the remodelling of your coronaries to better understand what the future may hold.  Right now I have to take the cues from your outward appearance and development, and you look mighty fine to me.  It’s very confusing though.  I’m still not sure if there is any chance that you could have complications today, tomorrow, next week.  I guess we have to take each day as it comes.

I don’t want people to think that I am a victim in all of this.  That I don’t want to let go of it.  In fairness, I can’t let go.  Partly because you are still affected by the disease and will be for many years to come. Partly because I feel I have gained a purpose; I can help make things better for other sufferers of the disease, whether it be through raising awareness to help speed up diagnosis, or by providing useful information to other parents going through this.  Kawasaki Disease is now a very big part of my life, of our lives, and it isn’t likely to go away.  Perhaps over time it will become a smaller part of life for all of us, but I don’t think I will ever stop talking about it to anyone who will listen (and a few who don’t!).  It’s too important for that.  I am in contact with parents and grandparents who have seen this illness first hand, some who have suffered the worst consequence of all.  For them, I will always do what I can to help change the future of Kawasaki Disease.

Having a child diagnosed with a rare, or little known, disease is life-changing for parent and child.  And when there is no known cause you never stop asking yourself ‘Why?’.  Why did this happen to you? I hope we find out one day.  I believe the specialists are getting closer – it’s just that research costs money and KD research is severely underfunded.  They believe they have identified the combination of genes that make a child with KD more susceptible to the disease.  And they are pretty certain that they are looking for an infectious/toxic agent that completes the perfect KD storm.  I desire two things; that we can gain a greater understanding of your condition so we might have a better handle on what might be around the corner, and that the mystery of Kawasaki Disease is unravelled in our lifetime so we might gain some closure.  Solving that mystery will also result in a test for the disease so that children might be diagnosed more quickly, and better treatments that further improve the possible outcomes for children with the disease.

My whole perspective on life has changed.  Things I was afraid of before no longer occupy any space in my mind.  I no longer sweat the small stuff.  I have a lot more compassion for others – through our journey I have come into contact with so many parents who have suffered challenges with their children, KD or otherwise.  Before this, I was ignorant to all that – it was happening to someone else and that didn’t matter to me.  Now it does.  The only fear I have now is of losing you, or losing any of the people that I love dearly.  I will not allow people to hurt me anymore, because nothing can come close to the pain I have felt over the last year with you.  None of that matters.  Yes, everything has changed. Our lives are different than they would have been if KD hadn’t crept in.  But it’s not all bad. It’s different, but not bad.

Sometimes I am scared, often I am sad.  I worry a lot about the future for our little family.  But above all that, you will grow up knowing that you are loved and cherished more than you could ever fathom.  You are a special little girl who will achieve great things one day, and I will be there by your side with every step you take towards greatness.

I love you, Peanut. 

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The World keeps Turning

What’s in a date? According to the dictionary, a date is “the day of the month or year as specified by a number.” It’s that simple.  And yet dates mean so much to so many people.  Every day of every year marks something significant in somebody’s life; a birth, a death, a wedding, a break-up, the anniversary of good and bad events that have occurred in a lifetime.

For us, today brings mixed emotions.  It would have been almost impossible not to face today with some sadness at the memories of this day last year when we watched our 7-week old daughter slip away from us with the fear that we may not ever get her back.  It is the day she became critically ill, and marks the beginning of our journey with Kawasaki Disease; Freya’s Story.   It feels somewhat surreal to be sat here today, putting the washing on, clearing up the aftermath of the children’s lunch, normal everyday things that a parent has to get on with.  A year ago today our lives were changed forever.  Changed in so many ways, some bad some good.  How did last year change me for the worse? Well, for one it altered my perspective on this thing we call life.  We all believe we have the right to live our lives how we choose, and rarely stop to consider the consequences of the actions that we take.  Being faced with the possibility that your child might actually die turns your entire belief system on it’s head.  I had never considered my own mortality before then – why would you? You just coast through this life taking it for granted that it is yours for as long as you want it.  But there are much stronger forces at work than any of us are able to prepare ourselves for, or protect ourselves against.  People get sick.  Kids get sick.  This world is full of illnesses and diseases that affect everyone else, and we read stories about them in the News or on Social Media, and think “poor them”, but we rarely stop to consider that the trials we see other people facing from a distance may land themselves on our own doorsteps one day.

I had never felt so much anxiety about the brevity and uncertainty of life before the events of last year.  Now I find myself living a confused life, where on the one hand I wish to grasp it with everything I have whilst I have it – make memories, move mountains – and on the other hand I face it with a fear of the unknown, and a desire to stop time so that we might be able to let go of that fear, just for one day.  Wherever I walk I see beauty in everything, magnified like I’m seeing through the wondrous eyes of a child, but out of the corner of my eye I can always see a shadow lurking and I imagine that the Grim Reaper is sharing every moment with us.  I am reminded of that movie, Final Destination; you can’t cheat death, can you?

But, the 31st May is just a date.  It doesn’t really matter what the date was, Freya got sick.  It doesn’t matter what date we got our diagnosis, Freya had Kawasaki Disease.  It doesn’t matter what date they told us her heart was damaged, Freya’s heart suffered.  Even so, it is hard not to see these dates as milestones, those first hurdles you have to get over after any loss.  And I know I should be grateful that we didn’t have to suffer true loss, but this is still part of a grieving process, and I do grieve for the loss of that perfectly healthy, normal little girl that I was holding in my arms on the 30th May 2015 and all of the 50 days before it.  Some would say I am ungrateful, that I should get over it already.  I don’t blame them if they haven’t been through something like this.  And it isn’t like I don’t want to let it go.  Of course I do – that’s why I see a counsellor every week to help me with the trauma of the last year (as well as a few other troublesome things).  If anyone thinks that I want to be stuck in this perpetual limbo between sadness and joy, that I get any gratification from feeling tears prick at my eyes every time my youngest daughter shows me just how special she is, is mistaken.  I want to be able to move forward, forget the last year, make plans for the wonderful future that I promised myself for my family.  But KD doesn’t let you do that.  It doesn’t just leave, like a cold, or a sore throat; KD leaves permanent scars, physically and metaphorically.

I will allow myself to feel what I feel on these dates, these first milestones.  The day of her diagnosis (12th June) is likely to bring up a lot of the same emotions.  Hopefully I will be better prepared and can plan to do something that will change the memory of that day.  I had hoped to do something today that would give me a new memory of the 31st May, but the awful weather and the restrictions I place upon Freya’s indoor activities (I don’t want her in an indoor play area, during half term, in chicken pox season!) put a stop to that notion. So instead I have tried to distract myself with the children and the chores.  But I sat for a moment and watched the raindrops run down the window, an image that my eyes immediately chose to mimic, and it has left me feeling wretched.  Out there new memories are being made and new anniversaries created and the world just keeps on turning.  And I keep breathing in and out, and whispering to my heart, “It will get better one day.”

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Stealing Joy

A year ago, on this very Saturday (though it was the 30th May), we had our last day of ‘normal’.  My parents had visited from Kent to see their new granddaughter.  Freya was 7 weeks old and we went on our first family day out since Freya was born, to a local farm.  The sun was shining; it was a glorious day that told us that summer was on the horizon.  The unexpected baby we had thought would ruin everything was proving to be our best ‘mishap’ to date. We were smugly happy with our perfect little life. We watched our older children play in the sunshine, whilst their baby sister slept in her pram, and it was like we had our own little slice of heaven right there.

That evening my husband and I went out for dinner with my parents, and my mother-in-law sat home with the children.  It was a pleasant evening, and when we returned home there was nothing to report, other than the fact that Freya hadn’t wanted all of her bedtime bottle.  It was unusual, but not exactly a serious situation; perhaps she was too tired, too full, or maybe there were some teeth on the way.

The next morning Freya would wake for her morning feed, around 6am.  Except she was so snuffly and bunged up that she just couldn’t take the bottle and gave up pretty quickly.  I was concerned, because she had gone for so long without a feed through the night (I remember celebrating our first proper night’s sleep), but I put it down to the fact that her nose was just too blocked up to let her feed.  At around 9am, I made another bottle and tried again, but just as before she couldn’t manage to take the feed, and fell asleep in my arms from the effort.  “She’ll feed when she’s hungry,” I told myself, and put her down in her bouncy chair.  A couple of hours passed, and I began to get quite fretful that she hadn’t fed since the previous day, so I decided to wake her and try again.   It was about 11am.  When I went to get her out of the chair, I noticed how hot she felt and so I took her temperature with a digital thermometer.  38.3F.  She had a fever.  I remained calm, I mean kids do get sick, and she has an older brother and sister who come into contact with all kinds of germs at school.  She had been snuffly for a couple of days; she had probably picked up a bit of a cold.  So I sent my husband to Mothercare to buy a medicine dispensing dummy (if you haven’t seen these, they are fantastic for getting medicine into a little baby).  Meanwhile I hunted out the Calpol. From 2 months.  That’s what it says on the front of the box.  Many would have given the medicine; I mean, she was only a week away from 2 months, what harm could it do? But for some reason, I felt like I couldn’t take that chance.  Unsure what to do, I called the out of hours GP service (it was a Sunday, remember).

The doctor called us back pretty quickly, and asked me to explain what symptoms Freya had.  She wasn’t feeding.  She was sleepy (but she was a newborn baby, and sleep was pretty much all she did).  She had a fever.  And come to think of it, her little tummy was moving up and down quite a bit – she was working hard at breathing.  The doctor said we should take her to A&E, as it was unusual for such a young baby to have a fever for no reason.  And that is what we did.

I’m guessing it was around 3pm in the afternoon by the time we were seen at the local hospital.   I don’t remember what was said by the nurse/doctor that checked Freya over.  I remember telling them that I had tested positive for Group B Strep during the pregnancy, and that although I was treated with IV antibiotics during labour, it was pretty tight timing wise.  I was on high alert for late onset GBS infection in Freya, and I knew that if she had contracted GBS during labour, there was a chance that she could develop meningitis.  They did too.  So they sent us up to the children’s observation unit with a view to keeping her in overnight for observation.

That part is all a bit of a blur too.  I remember sitting in the waiting area, Freya, burning up, lying across my knee.  She was laid on some paper towels whilst my husband and I attempted to catch a wee in a kidney dish. We went into a small room, and a doctor came to check her over.  I can’t remember what he said, or why he felt she should be admitted to the Children’s Ward.  I don’t remember when they put the IV antibiotics in.  I just remember that’s what happened.  We were admitted to a private room, and my husband went home to our older kids.  I sat in a chair in the corner of the room with Freya sleeping in my arms.  She was connected by the IV to a machine that dispensed the medicine, and every time I moved the machine would set off alarming, and the nurses would come in.  I think I stayed there until the medicine had all been delivered to Freya’s tiny, hot little body, and then I decided we both needed to get some proper sleep.  By this time, it was quite late.  It may even have been the early hours of the morning.  The nurses came in every hour to complete their observations, and then I put Freya in the cot so she might be cooler, and I might manage some sleep in the chair.

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As I laid her down, I noticed that all familiar smell – she had a dirty nappy.  “The poor thing just needs to sleep, I’ll not disturb her” I told myself,  and I went back to my chair in the corner.  I’m not sure how long I sat there trying to get to sleep before my conscience got the better of me and I decided that I could not leave her in a dirty nappy. Disturb her or not, I needed to change her.  And it was as I lifted her vest that I noticed a pin-prick rash over her torso that I could just make out in the dim light.  I think it was about 5am, because it was starting to get light outside and I could hear the birds singing outside of our window.  Were my eyes playing tricks on me in the half light?  I was so tired.  I turned on the light and went to get a better look, but still in the fluorescent lights I couldn’t work out what I was seeing.  I walked over to the window and opened the curtains, and in that light I could see that this wasn’t an illusion.  Freya was covered in a rash.  I called the nurse who came to have a look, and we both spent so much time scrutinising that rash that I can’t remember to this day if it blanched under a glass or not.  The nurse called the on duty doctor, and by the time he arrived (which wasn’t long at all) the rash had spread down Freya’s legs and across her arms.

It was in that moment, watching the doctor silently complete his review of my baby, that I noticed the atmosphere change.  They called a consultant who joined the doctor in his silent inspection. It was very calm, but the air felt thick. I thought Freya was sleeping.  In fact she was unresponsive.  I remember hearing words like “shutting down”, and the doctors started to wheel Freya in her cot into another room.  It was the High Dependency Unit (HDU).  They delivered fluid boluses to Freya by stealth.  At 6am I called my husband “Gavin, I think you need to come quick.”

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If you’ve followed Freya’s Story, you will know what happened next.  You’ll know that she became stable, but did not get any better for the next week, despite numerous antibiotics.  Her fever would not come down with antipyretics, and when her fever spiked (above 40 at times), so her heart rate would soar, and her oxygen levels would plummet.  She was very sick, yet it would take 13 days, lots of medication and countless tests before a diagnosis of atypical Kawasaki Disease would be reached.  Three hospitals would be responsible for her care during a six week incarceration (!) which began on the 31st May 2015.

I want to allow myself to believe all the motivational cliches about things happening for a reason, about how far we have come, about how lucky we are to have Freya here in our lives and how we should count our blessings.  But today that all feels like bullshit.  Today I find myself in mourning for normal.  I want to go back to that day at Cannon Hall Farm, where everything was blissful and beautiful and picture-book perfect, and somehow re-write history.  Today marks the anniversary of the last day we knew what normal was.  I wish that day were Groundhog Day and we could live it over and over and over so that the 31st May would never come.

I don’t want to wallow in the misery of the last year, believe me.  I want to be thankful for what we have, and cherish every moment.  I want to believe that this happened to us for a reason, that we will all be better people for it, that I will look back on all this one day and laugh at how wrapped up I was.  I will allow that to come, I’m sure (and the counsellor will give me a good kick up the arse if I don’t!), but for now I am allowing myself some wallow-time.  I have every right to feel sorrow, and to mourn the loss of the life I planned to have.  I have every right to feel angry that Kawasaki Disease came into my daughter’s life and robbed her of her health so soon after she arrived in this world.  I have every right to feel angry that the world keeps turning, and that people are moving on even though I seem to be stuck in this perpetual Kawahell.  Today, I hate Kawasaki Disease.  It broke my baby’s heart, and it broke mine too.

Most of all, I hate that this disease took away some of the joy that I ought to feel from moments like this…

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The Gift that Keeps on Giving…

“Mum, remember when you thought you might not get to see me grow up? Bet you feel silly now?!”

They say that you shouldn’t wish for your children to grow up, that you should will them to slow down because in the blink of an eye your once tiny baby is all big and independent and doing things you never thought possible.  Well, in a way Peanut that is true.  But as bad as it may sound, there’s a huge part of me wanting you to hurry up! Hurry up and talk, hurry up and walk, hurry up and grow big and strong so that I can be sure to have had the chance to see it all in case it is taken away from me.  I’m scared.  Scared that I won’t get the chance to know you like I should. Scared that Kawasaki Disease has not finished with you just yet.

Today we took you to the hospital for your cardiology follow-up appointment.  You were last seen three months ago, where the cardiologist remarked that the speed of which your coronary arteries have been remodelling is concerning, and they would like to carry out an angiogram to take a closer look.  I knew she was referring to possible stenosis; that the reduction in the internal diameter of your coronaries might not be healthy, and is more likely to be the result of a build up of scar tissue or layered blood clots that have effected  the change.  But I have read that stenosis can take decades to cause any ill effects, and as such I felt it would be prudent to give you more time.  More time to grow, so that less invasive procedures might tell them what they want to know.  Apparently an MRI or CT scan isn’t as effective in giving a true picture in such a young child, and an angiogram is the only option that will show them what is really going on in that little heart of yours.  I asked the opinion of 3 experts, all of whom concurred with my view that there would be no harm in waiting, perhaps even up to a year, and carrying out a less invasive procedure.  I put that question to your cardiologist, but she seemed pretty keen on pushing forward and continued to voice a preference for carrying out an angiogram a year post-diagnosis; June.

I decided to wait until your next appointment to understand more fully why the consultant was so eager to press on.  That appointment was today.

We arrived at the hospital just in time for your appointment, and were sent straight down the corridor for an ECG.  It took the cardiographer 20 minutes to get a reading from your heart because you were wriggling so much! We had to bribe you with ‘sweets’ (little fruit things that we call sweets because they’re as close as we will let you get to confectionary at your young age!) to get you to sit still for long enough! You’re not as easy a patient as you were last year, this time pulling off the electrodes and yanking on the wires, but he got there in the end!  Nothing was said about the result, and I am guessing that means that, as usual, the ECG showed normal heart function.  Next it was time to weigh and measure you (you weigh 8.46kg and are 74cm in height), and then you were called into the examination room.

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It wasn’t your cardiologist that called us in; it was a registrar.  She introduced herself and asked if we had any concerns since the last appointment.  We had none.  She then asked us to take you over to the bed so she could perform an echocardiogram, and I asked if we were going to see your doctor today.  She said that we would, and that she was just with another patient.  I guess they were helping each other out so they might all finish the day at a reasonable time.

Surprisingly, you laid quite still for the echo.  Okay, so you had to be given a probe of your own because you do like to take matters into your own hands, but she got there (with the help of a bottle of milk!).  She didn’t say much, but as usual I clocked the numbers appearing on the bottom of the screen and was able to catch that your LCA (left coronary artery – the one that has caused the biggest worry on this journey so far) measured 2.8mm.  Was that bigger than the last time? I seem to remember it being closer to 2mm, but what’s a fraction of a mm between friends, eh?  Your consultant then entered the room, and continued the appointment with the registrar present.

She looked at the screen, and remarked that your RCA (right coronary artery) looked almost normal.  She seemed happy with function and blood flow.  It seemed that she was about to suggest a routine follow-up appointment and gave the impression that it would be a longer period next time, but the registrar mentioned that an angiogram was mentioned at the last appointment. Ah yes, remarked the consultant. I reminded her that she was considering the procedure because she was concerned about the extensive remodelling that your arteries have undergone in such a short space of time.  Ah yes, she remarked, and commented that at worst, the diameter had measured 8mm.  I corrected her.  Unless I missed something along the way, the largest measurement was 5.2mm.  Whilst small in comparison to some children affected by this disease, they were more than 5 times normal size and in a baby as young as you were was considered significant and cause for serious concern.

I told the consultant that I had a few questions, and pulled out my notebook (I know! I do like to make notes!)  The first question I asked was why she felt it so important to carry out a risky, invasive procedure now? What benefit could it have? What was her thought process, and what were her concerns?  I had thought that she might concede with a “perhaps we ought to wait” kind of statement.  But instead she told me that she thinks it is highly likely that the remodelling has come about as a result of stenosis – anything other than that would be nothing short of a miracle.  I asked if there wasn’t a chance that the remodelling has come about as a result of the aggressive treatment you received to stop the disease in it’s tracks (you received steroids and a dose of Infliximab when two doses of IVIG proved ineffective).  She agreed that was possible, but didn’t seem convinced.  An MRI/CT would be effective in providing a better picture of aneurysms, but in a child as young as you they would not be as effective in showing stenosis.  I asked what would be gained by doing it now? Stenosis can take decades to develop before the arteries might close enough to prevent blood flow.  And if the angiogram did show evidence of stenosis, how would that change the treatment plan? And then she used words that I had not considered would be used in your lifetime. Stent.  Bypass surgery.

I guess I knew what her concerns were before I asked them.  I mean, she isn’t likely to put you through an unnecessary procedure, is she! I told her that I would prefer to wait, but that if she felt that waiting would put you at risk I would trust her judgement.  She said that she would really like to get a look, and it dawned on me that you are quite the case of interest.  One of the youngest cases to be handled, aggressively treated, showing almost too-good-to-be-true recovery.  Yes, of course they would like to get a look.  I said I appreciated the medical interest in understanding what has been happening with your heart, but that you are not a specimen, you are my baby.  She reluctantly agreed to see you again in three months, and noted that whilst there was a great deal to be learned from you, that she would not consider the procedure for medical knowledge alone.   She agreed to discuss your case with at the next MDT meeting (multi-disciplinary team) with a view to negotiating with me further then.  She has already discussed your case with the surgeon, and they too believe it would be prudent to do an angiogram sooner rather than later.  That said, she still agreed to give us another 3 months (which will end up being more if the waiting list is as long as I expect), on the strict proviso that if we see any evidence of angina we are to contact her.

Let me take a moment to tell you about the symptoms of angina;

  • Chest pain or discomfort (not sure how you will tell me about that!)
  • Pain in your arms, neck, shoulder or back accompanying chest pain (ditto!)
  • Nausea (won’t generally know that one until you actually throw up!)
  • Fatigue (hmm, should I be concerned that you sleep through the night?)
  • Shortness of breath (you don’t get above a fast crawl yet, and I’ve not seen you panting!)
  • Sweating (nope)
  • Dizziness (again, how would I know?)

So apparently the only way I might be able to tell if you are suffering with angina would be if you are playing one minute, and then go quiet, and maybe glaze over for a moment.  This could be interesting :/

It would seem we are not yet out of the woods, my darling.  Just when I thought that there could be a future where KD becomes nothing but a distant memory, here it is threatening to place a dark cloud over the years to come.  The only thing I can hold onto is hope, but that is so hard when I feel like I am fighting a losing battle with this disease.  I have no control over it, none.  It came, it messed you up real bad, and it just won’t leave us alone.  I know there is a chance that they could do the angiogram and discover that you are indeed the miracle I had started to believe in.  But I also fear the worst.  And I know that there are far more complicated procedures happening to very sick babies, with huge success, every single minute of every single day.  But they are not happening to my baby.  And I so want to bury my head in the sand like an ostrich and pretend that this isn’t our life, that this isn’t your life.  Today for the very first time I saw ahead of me just how complicated your life might be, and it has made me incredibly sad.

And because with this disease the hits just seem to keep on coming, I returned home to a voicemail from your GP.  We were given the green light to crack on with catching you up on the routine immunisations, and also later given the go ahead to give you the MMR vaccine.  It was unclear, however, what timing/order the Immunologist felt these should be given in.  You’ve had the 8 and 12 week immunisations now, and I was hoping that we might be able to intersperse them with the MMR, Chicken Pox and Flu vaccines rather than wait any longer than necessary.  This family needs a holiday far, far away from here.

Sadly, the advice was more disappointing than I had anticipated.  The instruction has been to wait until after the final routine vaccinations have been given (around June).  Because you are a unique case, they are not comfortable in progressing to the remaining vaccines until 3 months after that.  So at best, you will get the MMR in September.  No mention has been made of the Chicken Pox or Flu vaccines, but I can only guess they will want to wait longer for those too.  I just wanted us to do some normal stuff, Freya.  Mix with other kids, in places where normal kids go.  Jump on a plane to somewhere warm where we can forget all about this for a time.  I am sick of being restricted by my fear, but I cannot knowingly expose you to illnesses that have the ability to take more from you than KD already has.  I’m talking about Reye’s.  And yes, I know it’s rare, and it hasn’t yet been linked to low doses of aspirin, but it’s a risk I cannot bring myself to take.  What were the odds of you getting KD at 7 weeks old?? Exactly.

I’m sorry, my tone is getting quite unpleasant isn’t it? And I am sorry for burdening you with this worry.  Almost a year ago I asked a God I don’t believe in to prove the doctors wrong.  If he’s listening, this is his chance to show me what he can do.  I can only hope that if you are reading this, it means that you have already proven yourself to be the miracle that we all hope you will be.  I don’t want to live in a world without you in it.

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