Unlucky for some?

Two years ago, on this day, you and I shared something that forever changed us.  It was the day that both our hearts were broken.  Yours by a disease I had never heard of, and mine by the knowledge that your life would never be the same. 

It was Friday 12th June, you were 9 weeks old that day and you had been sick for 13 days.  Despite a cocktail of antipyretics and antibiotics, the fever continued to rage through your tiny body, and each temperature spike would see your heart rate soar to frightening heights.  The settings on the heart monitor had to be set far outside of normal levels or we would never have had any rest from it’s alarm, and the nurses maintained a close eye on you during those first two weeks.  By that time, you had undergone countless blood tests, two lumbar punctures, ultrasounds, x-rays, an MRI and a bone marrow aspiration.  Just a couple of days before this day your haemoglobin levels dropped dangerously low and you had to receive a blood transfusion.  That’s when I promised I would start giving blood as soon as we got out of there; a promise I have kept ever since.  I remember watching the blood travelling through those tubes incredulous that this was happening to us, to you.  I think I must have spent those first two weeks in a state of shock, it seemed so surreal.  How could you be so sick? 

It was after they gave you the blood transfusion that they came to talk to me about a bone marrow aspiration.  They didn’t need to tell me what they were looking for – there’s only one reason they wanted to get at your cells.  After the longest 3 hours of my life, where I was convinced that you were not going to wake up, they called to tell us we could come and collect you from theatre recovery.  Watching you go to sleep from the general anaesthetic was one of the hardest things I had ever had to do, and I hoped with all my heart that I would never have to see that again.  I cannot even begin to explain the relief when I saw you awake again.

The bone marrow test showed that your cells were healthy, and we celebrated that fact.  And yet that meant that you were really sick, and still nobody knew why.

That afternoon, on the 12th day, a Rheumatologist was asked to come and take a look at a rash that had appeared on your limbs.  He said that it was highly likely that you had an infection, and that there was a strong chance that you would get better and we would leave the hospital without any diagnosis; one of those unexplained things that we would eventually forget.  But.  But, he said, we ought to send you for another echocardiogram on your heart just to be sure.  Be sure of what? I didn’t ask.  Why didn’t I ask? I’ll always wonder about that.  In case you picked up on the word ‘another’, yes you are right.  You had already had a check on your heart earlier that week, on day 9, and it showed you had a murmur but nothing to be concerned about.  That echo was to throw them off the scent that the Rheumatologist had picked back up.

And so the day came.  The 12th June.  Day 13.  Unlucky for some.  

I took you with a nurse for your echocardiogram.  You’d had one before and there was nothing to worry about, so I just assumed this one would be the same.  Except it wasn’t.  And nothing has been the same since.  

That echocardiogram showed dilation of your coronary arteries, over five times their normal size.  Coronary aneurysms don’t happen to children.  Not unless they have Kawasaki Disease.  And there it was, on the 13th day of your illness, we finally had a diagnosis.  What a relief! That meant they could treat you, and you would get better, and we would go home and forget all about the worst two weeks of our lives.  Except then I had no idea that the damage wasn’t temporary.  Yes, they could treat the disease, but they could not reverse the damage that it had done to your heart.  We had to face the fact that one of, if not the most important organ in your body, was broken.

You were given a dose of intravenous immunoglobulin (IVIG), which is a blood product made from the antibodies from thousands of human blood donations, high dose aspirin, and methylprednisolone (intravenous steroids).  All the antibiotics were stopped – Kawasaki Disease is not an infection, but a vasculitis which causes inflammation through all the arteries in the body, with a penchant for little hearts.  The Children’s Hospital has every paediatric medical discipline under it’s roof, with the exception of Cardiology, and so they contacted a ‘nearby’ hospital with a paediatric Cardiology unit to ask for advice.  We were told that they had shared your results and that the Cardiac Unit was “not excited.” Apparently that was good news.  It meant that, whilst your heart was affected by the disease, it was not considered worrying enough to require more specialist care, and the children’s hospital continued to monitor your situation.  You had been so sick, and you were so young, that they didn’t take any chances, and you were sent for follow up echoes on Saturday and Sunday morning; no change.  No change is good.  And on Monday morning, you were sent for another.  Except this time, the nearby Cardiologists had reason to become “excited” and preparations were made for your ambulance transfer to the Cardiac High Dependency Unit, 36 miles away – but not before you had received a 2nd dose of IVIG to attempt what the first dose had failed to do.  Within 3 days of your diagnosis, your coronary arteries had dilated further and we would hear the word ‘aneurysms’ for the first time.

I’ve written many times about what happened next, and I hate to bring it all up again sweetheart.  It’s just that, despite my very best attempts to live ‘in the moment’, I must have left the door to this memory slightly ajar.  I didn’t mean to let it in, but it seeped through the cracks and it’s been like watching a scary movie through parted fingers all day.  No matter how hard I’ve tried, I just can’t help but relive those moments.  Today I feel all that pain, all over again.  I drove to Sheffield this evening on an errand, and felt the tears rolling down my cheeks.  I have never seen as many flashing blue lights on the motorway as I did tonight, jeering at me, forcing me to remember, and fleetingly I wondered how easy it might be to make that pain go away.  But then I remembered that you are still here, and it isn’t 2015 anymore.

And I guess as I sit here tapping away at the keyboard, I have to ask myself whether the number 13 really was unlucky for you.  Because despite the fact that your heart will never be the same again, I have to remind myself that the improvement we have seen has indeed proved them wrong.  In fact, I would go as far as saying that for you, 13 was very lucky indeed.  The decision that your doctors made on that day was one of the decisions that saved your life, I am certain of it.  I cannot even begin to imagine how differently things would have turned out had they not carried out that echo.  And I can be angry and sad for the rest of our days that they didn’t catch it earlier, but that won’t make the pain go away.

Today I will allow myself to feel the grief of loss for your perfect heart again, but tomorrow I shall remind myself of how full my heart is for loving you.  I cannot remain sad, when I am so incredibly lucky to have you here.

I love you Peanut.

image3

 

Best before 31st May…

Two years.  Don’t they go by in a blink.  It is hard to believe that two whole years have passed since that day in May 2015 that we will never forget.  Harder to believe that we are living a life more ‘normal’ than we ever could have imagined possible back then.  Back then it felt as though the bottom had fallen out of my world, and I was shrouded by a blackness that I thought would envelop me for ever. Today, it’s mostly sunny with a tiny chance of rain.  Today life is about as normal as it is ever going to get.  The last two years have been two of the most extraordinary of my entire life.  And you, my dearest Freya, my little “Peanut”, were the single most extraordinary thing of all.  Meeting you was like walking into the sun, and in spite of everything you went through, we went through, the clouds were never allowed to cast too large a shadow because your light burned through them like a flame through silk.

The past few weeks have allowed a melancholy feeling to settle around my shoulders like a familiar, almost comfortable wrap; two hands that placed themselves upon my shoulders, whilst a low voice whispered, “Remember me?”  It was a rhetorical question, of course I remember her.  She is sorrow, and fear, and dread, and grief.  I didn’t consciously awaken her, but the date was drawing near and I guess my subconscious had figured it out before I did.  I had been cleaning out the kitchen cupboards, like you do every now and again when you realise the dust is beginning to settle on the shelves!  I emptied the bottom shelf of one of the cupboards – that’s where we keep all the everyday medicines.  It’s where we keep your medicine, in a little pink sandwich box with Barbie on the lid.  I take that box out of that cupboard every single day to prepare your aspirin. Yet on this day, when I placed the box back in the cupboard I realised that it had gone back in a different way to normal.  I realised because there staring back at me, was the hospital label – we had used that box to store your medicines when you were in the Children’s Hospital.  And there I was, right back in that cubicle behind the nurse’s station, and you were by my side, in your cot, all wires and bandages and looking like someone else’s baby.  Your Auntie popped in just at that moment and for a while I was somewhere else.  When she snapped me out of it, tears came out of nowhere and I shrugged it off as ‘a moment’. What it was, was a flashback.  The first I have had since I was successfully treated for PTSD last year.  Luckily it was short-lived, and I was nothing more than a little shaken afterwards.  I’ve not had one since, though the sound of that musical seahorse going off the other day threatened to bring another.  Instead I considered how the fact that you were playing with it was a good sign that you do not remember like I do.  That is indeed a blessing.

Anyway, enough of that miserable talk! I haven’t written to you in so long, and I have so much to tell you! I want to show you how far we’ve come in the last two years, and let you know how remarkable your life has been so far.  On the 28th May last year, I wrote a blog which I gave the rather despondent title of “Stealing Joy”.  You see, that is how I felt back then.  Kawasaki Disease had come into our lives and stolen everything that was meant to be joyful away from us.  Here is some of what I wrote:

“I want to allow myself to believe all the motivational clichés 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 mourning for normal…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…”

And what I want to tell you now, sweetheart, is that it did come.  Normal came.  And with it came joy.

April 2016 marked the end of my maternity leave, but I wasn’t ready to return to work for many reasons.  For one, there were still issues with your immunity, and the risks connected to you being exposed to chicken pox were too great for me to consider placing you in any kind of childcare.  Heart-wise things were pretty unstable, with lots of unknowns, and much deliberation about whether we should allow the hospital to plough ahead with a procedure that I would prefer to avoid.  At that time the uncertainty filled me with dread and I was struggling to deal with what life had thrown at us.  I was about to receive treatment for PTSD (post-traumatic stress disorder), I had hit a very low point in my life where I felt let down by many people in it, and life itself.  I felt very alone, except for you.  You were the only one who could make me smile.  At that time I was sliding down walls in hospital corridors when an anxiety attack took hold, or crying alone in the early hours of the morning because there was nobody to share my grief or fear with.  When my employer agreed to allow me to take a career break to get back the time we lost, it was like I had been given a new beginning.  I was paired with the most amazing counsellor who helped me deal with what happened to you, and then some. And I began to believe in life again.

Don’t get me wrong, I don’t think I will ever stop being afraid of what lies around the corner.  And I am more afraid of death now than I ever was before.  I think about it a lot, almost daily.  Not in a macabre way, just a reminder that tomorrow isn’t promised.  Sometimes I feel deep sadness at the thought that I might not get to meet your children, my grandchildren.  I just have to try to stay young, so I might get that chance.  I feel a dreadful sorrow when I think about my own mortality.  I am so afraid to leave here, leave this place and my family.  I cannot bear the thought of being parted from it.  But then I guess it is our fear of death that keeps us alive.  If there is one thing I learned from my counselling it is this – that no amount of worrying about the future is going to change it.  I have learned to live in the moment (mostly).  Sometimes the resolve dissolves and I find myself fearful, especially as the seasons change and we head towards the Autumn, and the angiogram that I have such an issue with.   But mostly, we live in the now.  And since I went back to work full-time in April this year, there really isn’t much time to think any further forward than that!

When I was approaching my return to work, I became very sad.  The thought of not spending every day with you made my heart-break.  I wasn’t worried for you – I knew you would adapt and that it was time for you to experience new things and to make new friends.  I was worried for me though.  I honestly did not know how I was going to get through it.  We ended the two-year break with a fantastic holiday in Mexico.  We were finally able to fly, having managed to get you caught up on all the routine vaccinations you missed because you were so young when you got sick.  I had some contemplative moments on that holiday – each day that passed took me another day closer to leaving you. But I told myself that rather than think of what I was losing, I should think of what I have gained.  I was given the gift of time; another year to spend with you before I had to return to a ‘normal’ I never thought we would see.   A year to fill with a joy capable of erasing a year of misery.  And I have to tell you, that despite everything, these last two years with you have been extraordinary, because of who you are, and what you have achieved, and what you have done for me.  I am a better person because of you.

And you? Well, what about you?! Let me tell you what you have seen in this last year.  You’ve seen animals and sea-life, you’ve swam in pools and an ocean.  You’ve visited the home of a literary great, and learned where your name came from, my Freya Ellis Belle.  You have made firm friends.  You’ve danced and bounced, and you know your good toes from your naughty toes (thank you Mrs Riley!).  You have inspired a donation of £75,000 from a stranger across the other side of the world, and raised £8,000 from your 1st birthday party.  You’ve sat upon the knee of world leading Kawasaki Disease specialist, Professor Jane Burns.  You’ve been to Christmas markets, and Summer Fayres. You were awarded special recognition for Triumph Over Adversity at the Doncaster Free Press Awards. You have raised awareness of this disease, with tens of thousands of people having seen your face.  Last month over 5,000 people viewed a video of you on Facebook, raising even more awareness.  You have been in Newspapers and on ITV News.  You inspired me to give blood, and register for stem cell donation.  And you inspired me to run so that just this weekend I raised nearly £3,000 running 10km in the Great Manchester Run!  You, or Kawasaki Disease, or a combination of the two have made me stronger, wiser, more compassionate, kinder, more alive than I ever was before, and for that I am thankful.

On the train home from Manchester this weekend, I opened my finisher’s pack to have a look at what was inside.  My runner’s medal, a sick bag (I’ll tell you about that one day), some leaflets, a bar of chocolate.  I look at the wrapper to see what kind of chocolate it was and my eye was drawn to the best before date:

Best before 31st May.  

Perhaps you were best before then.  You were best in terms of being undamaged, your little heart was in tact and you were perfect.  But actually, you are so much bigger than the 7 weeks before you became sick.  You are more than Kawasaki Disease, more than a broken heart.  And I am more than a mum.  I am your mum, and that makes me a very lucky woman indeed.  I became my best after you.

image1 (1)

 

Proving them wrong..?

So today was Freya’s first cardiology follow up of 2017.  In case you don’t remember where we are at from the last visit, I’ll quickly bring you up to speed.  We had a cardiology follow up in September 2016 where I questioned the Cardiologist’s desire to carry our a cardiac catheter angiogram.  At that appointment I was told that I needed to trust the doctor or she would have no choice but to refer Freya to another hospital.  However, shortly after the appointment I received a letter which allayed some of my fears, and so I made an appointment with the Cardiac Intervention team to discuss the procedure.  At that appointment I was made to feel more at ease about the procedure itself, however the doctor said he was not happy to perform it on Freya given she was still so young, and asymptomatic.  I left it with the two of them to argue the case, and eventually it was decided that whilst there was a degree of anxiety about the speed of the remodelling of Freya’s arteries, it was in Freya’s interests to wait a while longer, and she was placed on the waiting list to undergo the procedure in the Autumn of this year (2017).  That was good news for me, as it seemed like a good compromise, although I then felt quite nervous about what could happen in the interim, and gave my consent to wait as long as Freya was reviewed regularly in between.

I believe the idea was for Freya to be seen in 6-months (from the last appointment), however I was keen to evaluate Freya’s current situation before we go to Mexico, so her appointment was made slightly sooner than planned.  And that brings us to today.

Now, at Freya’s last appointment they were unable to obtain an ECG reading as Freya was hysterical and kept pulling the electrodes off (“stickies” as she calls them).  Even when we managed to get her to lay still, she was just too distressed to get a proper reading.  Since then she has developed an acute fear of stickers – all kinds! She wouldn’t even allow us to go near her with a sticker, not even to stick one on her clothes.  So we’ve been working really hard trying to beat that phobia, and we had a breakthrough last week after a visit to Cannon Hall Farm.  I’ve spent the last week chatting to Freya about the hospital visit, talking about the ‘stickies’, where they’ll put them and that she has to be brave and not wriggle or cry.  On the way to the hospital this afternoon she told me and her Dad that she liked hospitals, and chatted away about the stickies and how brave she was going to be (I say ‘chatted’ but it was more like a mantra – “I like hospital, stickies on me, my legs, my tummy, me not cry, me blave.”)  I was hopeful that today’s appointment would be more productive.

When we arrived we were sent straight for an ECG.  Sitting in the corridor, one of the nurses that had looked after Freya before came and had a chat with us.  She made a fuss of Freya, and after a while asked if she would like her to do her ECG for her.  Freya said she did, so the ‘nurse’ (not sure what her actual job title is) went and asked the other staff if she could do this one as Freya is very nervous and she knew how to handle her.  She decided to place the electrodes on her arms and legs only, leaving her torso free.  Despite her best efforts, Freya still screamed at them to stop.  I managed to calm her down (by telling her that Paw Patrol would be on the ECG screen any minute!) for just long enough to get a reading, although I gather that it wasn’t a full reading with it only being on her limbs.  Freya then refused to be weighed or measured so we had to leave that part too.

We went into the Cardiologists room almost straight away, and she asked how Freya had been.  We updated her on the chicken pox vaccinations, which are now complete.  The episodes of single fever spikes in the early hours of the mornings (can be associated with angina) had ceased, and Freya was no longer having periods of suddenly lying still on the ground, so it must have just been a phase she was going through.  The doctor tried to do an echocardiogram but Freya resisted (for the first time so far on this journey), and the Cardiologist wasn’t able to keep her still for long enough to get a look at her coronary arteries, let alone measure them.  She kept fighting to get away, shouting “Stop it, stop it, please help me!” which was so upsetting to watch.  She did however get a look at the heart chamber and could see that the heart was functioning normally – this was also confirmed by the ECG results, and to be fair, heart function has never been a concern in Freya’s case. As long as Freya’s blood can keep flowing nicely through the arteries everyone is happy, and that’s what the daily dose of aspirin is there for.

I must admit I felt more than a little uneasy about not being able to get a close look at Freya’s arteries.  I mean, it’s the arteries that are the issue and I really wanted to be sure that there had been no further narrowing from her last appointment.  The doctor didn’t seem to be too concerned however so we sat down for Q&A before setting off home.

So, what did we learn? I am going to bullet point it to make some sense of it all:

  • The Cardiologist referred to the latest paper on the long-term treatment guidelines for Kawasaki Disease, and said that what she had read had given her some reassurance that the speed of remodelling in Freya’s case is not as unusual as she first thought.  I think she may have been referring to the latest JACC review which I actually sent her after our last appointment (I can forward it to anyone who would like it – you can contact me through the awareness page www.facebook.com/freyasstory).  Her initial anxiety was that with Freya’s aneurysms remodelling so fast it might be a sign of stenosis occurring.  However having read the paper, she established that actually it is common for remodelling to occur within the first 2 years, particularly in cases where children are treated more aggressively than the standard treatment, as in Freya’s case.
  • Freya was treated with the standard IVIG and high-dose aspirin at the time of diagnosis, with the addition of intravenous steroids.  Resistant to two doses of IVIG she was then given another drug called Infliximab.  This is not widely given in the UK, although there is a strong case for it’s use, particularly in severe or high-risk cases.  As a very young baby, Freya was in the high-risk category for heart damage, and also presented with a severe case of the disease.  The hospital had to apply to NHS England for permission to administer Infliximab to Freya – I still have a copy of the letter granting that permission.  Freya’s Cardiologist believes that the Infliximab was instrumental in Freya’s cardiac recovery from the disease and I have to say that I agree.  I think Infliximab saved Freya’s life.
  • I asked about our impending long-haul flight, and we were reassured that there should be no concern.  We should make sure we don’t forget Freya’s aspirin, and should keep her hydrated on the flight, but that was the only caution. Oh, that and that we had to remember to send the doctor a postcard 😉
  • I also asked about cholesterol testing, as there has been some information circulated about undergoing the test one year post-diagnosis.  We were advised that it may be standard in cases fitting the ‘normal’ age bracket for KD, but that Freya is too young for a low-cholesterol diet and would not be treated with statins for high cholesterol as her brain is still developing.  I guess that’s where we have to trust the individualisation of our children’s cases compared to the standard protocols.
  • I have also read that aspirin is best taken at night to reduce the risk of cardiac events during the nighttime hours.  Freya has always had hers in the morning, but with her soon going to be with a childminder, I wanted to switch it so I can give it to her before bed.  This is not a problem, and the anti-platelet effects of the drug will remain long enough for the longer delay between doses when I make the switch.
  • The last thing I asked was in relation to specific care plans/patient specific protocols required for the childminder, for example.  The doctor said that as long as the childminder knew what to look out for – becoming pale, sweating and quiet – there were no other ‘special’ arrangements to be made.  I expressed my concern that in the event of a potential cardiac issue, were an ambulance called it would take Freya to our local hospital which is not a PCI centre.  The doctor is going to provide me with a letter outlining the care Freya should receive in such an event, and advised that in coronary cases, Freya would be better transported to a local hospital for immediate care than risk the journey to Leeds.  Once stabilised, she could be transferred by the EMBRACE medical transport service to the cardiac unit if necessary.

We agreed that Freya will be seen again in 6-months, and that although they feel less anxious about the progress so far, they will still carry out the angiogram later this year.  I agree that it is prudent to take a closer look to be sure we know the full picture.  I also explained that whilst in the past I have questioned the steps they wanted to take, it was never from a place of distrust but only from making informed choices about what was best for Freya.  Even though I was the one who wanted to delay the procedure, I have still had to live with the fear that I may have made the wrong choice, and something terrible could happen without warning.  Overall it was a very positive visit today.

But (yep, there’s always a but with me isn’t there!), I can’t help but still feel a little uneasy that what was decided today was not based on what we could actually see (with the exception of the heart function being ok).  For all I know, Freya’s arteries could have remodelled even more than the last appointment and now be heading dangerously towards narrowing. I guess that is a possibility, but we have made decisions today based on probability.  The doctor thinks it is improbable that Freya is in any real or immediate danger.  Hopefully the aggressive treatment Freya received in the acute stages of the disease have set her up for a bright future.  There are still many unknowns, some of which we won’t get answers to until much later in Freya’s life.  For now, perhaps it is time to have a little faith

For some reason as we reached the exit level of the hospital, we turned the wrong way out of the lifts.  It wasn’t a big problem as you can get out either way.  This way, though, took us past the Brotherton Wing – the old part of the hospital which houses the parent accommodation where I had to stay, alone and far away from Freya when she was being taken care of in the cardiac high dependency unit.  As I walked past the entrance to the old wing, I couldn’t help but feel a deep sense of sadness at the memory.  And then I remember the chapel that I used to pass on that long, lonely walk to my room every night when I couldn’t keep my eyes open any longer.  I don’t consider myself a religious person, but not wanting to leave anything to chance, I had entered the chapel the day after being told there was a chance we could lose our daughter.  It as the day Freya was taken  back to the Children’s Hospital, and as a parting gesture I had taken a slip of paper from the table inside the doorway of the chapel and wrote on it, “Dear God, prove them wrong.”    I am beginning to think He might just have been listening.

IMG_5289

Living with a Broken Heart

I have written many blog posts over the last two years, sharing our journey with Kawasaki Disease, but it struck me this morning that I have never really been able to articulate how living with this disease day-to-day feels for a parent.  It is a feeling I have become so accustomed to now, that it almost feels normal, and that is why I think I find it hard to describe.  My goal today is to try, so that I might share with you a glimpse into how our world changed in June 2015.

In a nutshell, Kawasaki Disease is a form of vasculitis, causing inflammation in the medium to large blood vessels when it strikes.  It is generally contained to children, mainly affecting those aged between 2 and 5 years old, though not exclusively (my daughter was 7 weeks old).  It is characterised by a number of symptomspersistent fever, red eyes, rash, swollen extremities, sore/cracked lips or strawberry-type tongue, and swollen lymph glands in the neck.  With no known cause, there is no diagnostic test to differentiate Kawasaki Disease from other similar childhood diseases (Scarlet Fever looks very similar and is a common misdiagnosis), and therefore a diagnosis is reached based on presentation of the afore mentioned symptoms.  Given that these symptoms don’t always show up at once, and sometimes not all of them are present, Kawasaki Disease can be a tricky illness to diagnose quickly.  And the speed of diagnosis is important, as research has shown that treatment within 10 days of onset of symptoms can dramatically reduce the risk of heart damage in affected children.  You see, 75% of children who get the disease will recover without long-term complications, whilst the remainder will sustain permanent damage to their heart if not treated (this reduces to around 5% with timely treatment).  Sadly, this means that no matter how good the doctors are at diagnosing the disease, 5% of children who get Kawasaki Disease will suffer heart damage that will remain with them for life.

Whilst mortality rates for KD are considered low, at around 1% of cases, I’ve always felt that the statistic gives unreasonable hope to those of us with children whose hearts were damaged.  I would be willing to bet that any deaths resulting from Kawasaki Disease are exclusively related to heart damage.  So if there are 100 cases of Kawasaki Disease, the statistics say that 1 of those children will die.  But when you consider that, with timely treatment, just 5 of those 100 children would suffer permanent heart damage, and you factor in the 1 child who will lose their fight, suddenly the numbers seem very different – especially if your child is one of the 5%.  Suddenly that 1%, when factored into the heart population alone, becomes 20%.  Now, whilst those numbers give the fear some perspective, I don’t want to cause unnecessary alarm for those of you living with a child with a broken Kawasaki heart – another bet I would place is that the majority, if not all, of the deaths resulting from Kawasaki Disease occur from misdiagnosis, or no diagnosis at all.  Like the little girl who was diagnosed with rhinovirus and died 6 weeks later when her heart failed her.

So, I guess that sheds light on a couple of things:

  1. My daughter is one of the 5%.  Unfortunately at just 7 weeks old when she became sick, she was in the high risk category for heart damage.
  2. It took 13 days to diagnose Freya, and therefore treat her (outside of the ‘magic’ window).  Add that to the high risk factor,  and she didn’t really stand a chance.
  3. Under the circumstances, it is pretty normal for me to feel afraid.

Thanks to Kawasaki Disease, my now 22-month old daughter Freya has a heart condition.  She was born with a healthy heart, and after just 7 weeks in the world, and 13 days with a mystery illness, Kawasaki Disease broke it.

Looking back I don’t think it was the diagnosis that changed our lives.  Hearing that the doctors had actually reached a diagnosis after two weeks of tests and various failing treatments, was a huge relief.  Even when they told me that it was the damage to Freya’s heart that provided them with the conclusion, I didn’t realise they were talking about something that would stay with her forever.  I presumed the damage to her heart was a symptom, one that would go away with the treatment.  I was wrong about that.  A week after celebrating Freya’s diagnosis I would be sitting in a room with a Cardiologist and a nurse as they told me that if my daughter’s coronary arteries (which had dilated to over 5 times the size of a normal baby) ruptured, she would die.  They could not prevent it, and they would not be able to save her.  That is what changed our lives.  Until then I had never considered the possibility of losing a child.  Facing that possibility head on, with no sugar coating, rocked the very foundations on which I had built my perfect, indestructible life.  Of course, nothing is promised, there are no guarantees, life is fragile (and all those other clichés you see on Social Media memes), but I really didn’t think any of that applied to me. Stuff always happens to other people, doesn’t it…

20 months post-diagnosis, and I think I can dare to say that we are in a pretty good place (frantically touches wood).  Freya’s coronary arteries showed significant improvement in the months following her diagnosis.  Her heart function appears normal, her coronaries have remodelled to within ‘normal’ parameters and she shows no signs or symptoms of anything troubling going on in there.  Good news, right? I guess that’s how we take it for now.  The thing is though, that dealing with Freya’s heart is one thing, but add in the complications that come with ‘rare’ (KD affects just 8:100,000 children in the UK each year) and you find that ahead of you is a lot of uncertainty, confusion and contradiction.

There are a number of long-term complications from Kawasaki Disease.  We often hear the words ‘normal’ from Freya’s cardiologist, we are also told by world leading specialists in this disease that Freya’s heart will never be ‘normal’.  We are told to celebrate remodelling, but warned of possible stenosis (narrowing of the previously dilated arteries).  We are told stenosis will take decades to occur, but read stories of emergency heart bypass surgery within months of diagnosis.  We are told to live a normal life, not to worry, but research shows that 80% of children who suffer significant damage to their hearts as a result of KD will require surgical intervention later in life.  Our doctors talk about what they see and know, but do not give confidence that they are familiar with all of the ways in which KD alters the child’s heart.  I won’t even pretend to fully comprehend all the possible risks associated with Freya’s heart.  I read about fibroblasts, myocarditis, and damage to the heart muscle, but I can’t begin to understand what those things mean in relation to Freya’s heart.  No matter how much I have learned and think I know about this disease, I didn’t spend years in school learning about human hearts.

When I allow myself to think positively about the prognosis for Freya, I believe she has a good chance of living a normal life.  But then I worry that I am becoming complacent.  Freya’s doctors have shared that they have a degree of anxiety over Freya’s condition, and need to take a closer look.  They’ve postponed that closer look until later in the year, and I worry about what changes might be occurring inside her heart in the interim.  Only a few days to wait for some reassurance on that score (Freya’s next cardiology follow-up is on Monday 13th March).

When I consider that there is a possibility (though perhaps not a probability) of Freya suffering a heart attack, for example, I find myself suffocated by the thought. It’s like there is no end to the suffocation because you never know when something like that will strike. And I know that can be said for anyone, but the worry is more tangible when you are faced with a known condition with associated risks. When I wake in the morning, I lay paralysed in my bed until I hear something through the baby monitor that will defy my worst fears – a rustle of a sheet, a snort or a murmur from my sleeping girl. Not until I hear a sound do I let out the breath that I’ve been holding since I opened my eyes.  I’m sure every mother could say the same, we all worry about our children don’t we.

Aside from my concerns for Freya’s future, the uncertainty that came with our daughter’s heart condition has caused me to question my own mortality too. I was never afraid of death before, I don’t think I gave it any thought. Now I am terrified. Until just now I thought it was just because I love my family so much that I am afraid of leaving them behind and missing out on what is yet to come. But actually I have come to realise that I am not afraid of dying, per se, I am afraid of dying too young. I have had to work so tirelessly, stay so strong, fight so hard to keep on the front foot with our daughter’s condition, that I fear for her future if I am taken from her too soon on her journey with this disease.  I am afraid that she will enter into adulthood not realising how important it is that she keeps her heart healthy, or how she may need to have a cardiologist on hand in labour if she ever has children of her own.  I worry that others will forget about all of this, because they can forget.  There are no visible reminders of what this disease did to Freya, so it wouldn’t be hard to put KD firmly in the past.  Me; I’ll never forget. The last 2 years are etched in my memory so deep that I can play the entire journey in my mind as if I were watching a video recording.  And my overactive imagination has managed to convert my fears into a DVD that plays for me over and over – I have ‘seen’ moments where life ended, clearly enough to feel the loss as if it were real.  Perhaps the intense fear that I have about losing my daughter is one of the lasting gifts that KD gave me?  Perhaps it has helped me to treasure what we have more than I may have done without it?

I think I would have been able to handle the effects of Kawasaki Disease better had it been more commonly known.  The rarity of the disease means that we don’t really know what the future holds.  We can guess, we can use the limited amount of information that is available from adult KD studies, we can trust that Freya’s doctors understand what they are dealing with.  We can have faith that it will all work out, that Freya is a little fighter,  a tough cookie, a miracle.

People tell me I should stop worrying.  They tell me that no amount of worrying will change the future.  Some tell me that the future is out of my hands, that ‘someone’ already has a plan for us and we should put our faith in ‘Him’. Some say that any one of us could get run over by a bus tomorrow or struck by illness.  They are all right of course.  Except accepting that something could happen, and knowing that something might happen, are two completely different things.  The fact is that the most important organ in my child’s body is faulty, and she only has one of them.   I am finding it easier to deal with that with each day that passes.  I find it easier to be optimistic about the future, even if only cautiously so.  But I won’t ever be completely free of the overwhelming sadness that this disease brought to my doorstep, and I will never stop being afraid of what this disease might yet have in store for Freya .

Kawasaki Disease didn’t just break Freya’s heart.  It broke mine too.

IMG_1117

An Uncertain Future

I guess we all have one of those don’t we – not many of us know what lies ahead of us.  Some know what might be in their path, but nobody knows exactly how or when those things will happen. That’s why it is important to live life, not take things for granted, and do your very best to be the best you can be. And all the other clichés about living life to the full that might seem flippant, but are actually really true.

But how many people lose sleep worrying about what might lie ahead in our futures? People with anxiety, sure.  Anxiety makes you worry about almost everything.  I imagine the majority of people think about their future, but worrying about what might happen next year, in five years, in twenty years… I don’t think that is common.  Amongst the rare disease community uncertain futures are a given.  Many people living with rare diseases don’t have a diagnosis for their ailments and spend years searching for answers and fighting for treatment.  Some have a diagnosis, even treatment, but have no idea how their lives might take shape with the disease they are living with.  Kawasaki Disease is one of those.

Take Freya.  I think most people think that she is okay now.  She had this disease that made her really sick, but she got treatment and now she is at home living a ‘normal’ life and looks perfectly healthy, like any other kid her age.  And in the most part that would be true, except for the ‘normal’ part.  You see, if you weren’t already aware from my previous blog posts, Freya was one of the 6% of children with Kawasaki Disease who sustained damage to her heart despite treatment.  The disease causes inflammation of the small to medium arteries throughout the child’s body, and if treated quickly enough heart damage can be prevented.  In those cases where treatment is delayed, due to misdiagnosis (or a lack of diagnosis altogether) Kawasaki Disease is allowed to continue on its destructive path and cause damage that may never be reversed.  Once an aneurysm forms, the child’s heart will never be ‘normal’ again, even if the coronary arteries remodel and aneurysms regress to within otherwise normal parameters.  This leads to complications throughout the child’s life and into adulthood, which is why long-term care and life-long follow up are so vitally important.

The good news in Freya’s case is that her arteries have remodelled quite dramatically from when she was at her most sick.  There are no obvious aneurysms any more, and the arteries that had expanded to over 5 times the normal size for a baby of her age, have now regressed to what would be considered normal in a child that hadn’t been touched by heart disease.  The bad news is that the extent of the remodelling has her Cardiologist feeling a bit anxious.  They are concerned with what might be causing the narrowing of her arteries, and whether that narrowing might continue to progress until Freya’s arteries are too narrow to allow the flow of blood.  When blood can’t get around your heart, that’s when a heart attack occurs.  Sure, Freya takes aspirin every day to try and help keep the blood flowing, but nothing can get through an occluded artery.

That said, do I think that she is at immediate or short-term risk of a heart attack? Hesitantly I say ‘No’.  I say it hesitantly because whilst I have this pretty good feeling that Freya might just be some kind of miracle success story, my experience with this disease is not to let your guard down.  Complacency has no place where this disease is concerned.  Let’s say that I hope that she is not at immediate risk.  We will find out more in the Autumn when Freya will undergo the cardiac catheter angiogram that she was meant to be having last year.  It has been delayed to allow her more time to grow, so that she might be stronger to outweigh some of the risks associated with the procedure.  The rapid remodelling of her arteries has led me in the meantime to insist that Freya receives regular follow up between now and then so that we don’t miss something while we wait.  An echocardiogram won’t show what is happening with the arterial walls, but you can see the diameter of the arteries (up to a point – an echo can only see about 3mm into the artery) so you can get a view of whether they are continuing to narrow.

So what are my fears for Freya’s future?  

Of course I am anxious about the angiogram, but I’ll worry about that more when the time comes around.  I am confident in the ability of the intervention cardiologist, and am happy that it is a relatively low risk procedure.  I’m not too excited about her going under a general anaesthetic – I’ve seen that once before and it felt like I was watching my baby die.  I would prefer never to see that again, but that’s the reality and I will always be there by her side when she goes to sleep, no matter how hard I find it.

I am anxious about what is going on in that heart.  I’d love to have a window to look through so we could check in on it every now and again.  We assume from outward appearances and general health that nothing too sinister is going on, but the fact is we just don’t know.  I’ve read many stories from other KD parents who have experienced dramatic turns of events with their children, resulting in the need for heart surgery.  Sadly, some children have lost their lives to this disease – mostly those that were not diagnosed in time, or at all, but often because the changes in their hearts happened quickly and in spite of regular follow up and treatment.  Nothing is promised.  Do I spend every day worrying that Freya will have a heart attack? No.  Does my heart leap into my throat when I wake in the night and can’t hear her breathing on the baby monitor? Every time.

I am worried about what the angiogram might find, and whether there is any likelihood of Freya needing surgery in the future – around 80% of children who suffer giant coronary aneurysms will require intervention later in life, whether it be a stent or bypass surgery, for example.

I am worried about whether Freya’s diet is sufficiently ‘heart-healthy’ to protect her heart from disease.

I think about when Freya might decide to have children of her own.  Seems like a long way off to be worrying about it, but the truth is that she will most likely need to have a cardiologist involved in her ante-natal care.  There have been cases of heart attacks during labour where the mother had KD in childhood – there’s a whole medical paper dedicated to this subject (yes, I have a copy!).  I even worry about whether I will still be here when she enters that stage in her life and whether she will remember that she needs to consider her medical history.

And this one you might think of as really strange, but I worry about them telling me everything is ok, and that she can stop the medication and live a normal life, with KD firmly in her past.  You would think that should be something I would hope for, and be pleased about.  But, first of all, I will never forget the words of Professor Jane Burns, World Leading Kawasaki Disease specialist, that once a child’s heart has been damaged by KD it is never considered healed, or normal again.  So if  a doctor tells me everything is normal, and tries to send us on our way, then I will be putting up a pretty big fight!  Freya, and all the other children like her, need to be monitored for the rest of their lives.  Rady Children’s Hospital recommend obtaining a CT calcium score 10 years post-diagnosis to help identify and prevent further complications, for example.  It may just be a check-up once a year to make sure there have been no changes over time, but it is the peace of mind that we all need to ensure that there are no hidden issues lurking that might cause a serious problem later on.  Complacency is a killer.

“Oh Jo, you’re so dramatic!” I hear you say.  But you need to understand that I read stories, comments and messages every single day from someone who has been touched by Kawasaki Disease and suffered terrible consequences and devastating loss.  Every time I feel like I will never make a difference where KD is concerned, I remind myself of those stories, or I stumble across a new member of the Kawasaki community and the fire in my belly is reignited.  It is one such story that got me thinking again about the future, and reminded me that although I can hope for positive change in Freya’s condition, I must never allow my own complacency, or that of the medical profession, to create a false sense of security.  The story is that of Lisa Connelly. Her sister Amy has given me permission to share it in the hope that it might help to raise awareness of this disease and the devastating consequences when we get it wrong.

Lisa Connelly was diagnosed with Kawasaki Disease at the age of three.  By age five she had been given the all clear, following an angiogram that showed no persistent damage to her coronary arteries.  I can only imagine the elation of the family as they put KD firmly in the past, and watched Lisa to grow and live a normal, heathy life.  She was very active growing up, and was an avid runner.  She had a beautiful son, Levi, and was very much loved by her family and friends.  Described as “the kind of person you want to be like”, she had a heart of gold and was the first to come to your aid if you were in need.

Out of the blue, on the 25th November 2015, 37-year old Lisa had symptoms of a heart attack. The hospital performed an echocardiogram and Lisa underwent a stress test, both of which cleared her of any issues. Four days later, on November 29th 2015, Lisa passed away as a result of a massive heart attack.

The pathology report on Lisa’s heart showed that her coronary arteries had muscle cell tissue build up on the arterial walls, which caused restriction of blood flow to her heart, thus causing a heart attack.  The family had no idea that Lisa would ever have to deal with Kawasaki disease again, and even after the heart attack they did not consider it might be linked to an illness that she had when she was a child.

Since Lisa’s passing, her sister Amy has done everything she can to raise funds and awareness to prevent another tragedy like this.  Professor Jane Burns has said that conducting a CT calcium test earlier in Lisa’s life may have prevented further complications from occurring.  It was from a Facebook post sharing a medical paper which discusses the importance of a CT Calcium Score that I found Amy, and I am honoured that she has allowed me to share her family’s story in my blog.

Amy said:

“My sister was truly a beautiful light in this world. My sister had a great smile and loved to see others smile. She helped so many people while she was living and we know she continues to help even today.  Our hope is that somehow she helps in the research efforts of KD. So, my prayer is that your blog post reaches someone that takes the information and gets the proper treatment they need to live a long, healthy and hopefully happy life. We’re making beauty from ashes…My mom and dad’s grief has been almost unbearable this past year. And the hardest part for my mom is that the cause of the heart attack was from KD.” 

As well as raising awareness of the devastating consequences of this disease, Lisa’s family have made sure that her legacy lives on, and donated some of her heart tissue samples to Dr. Jane Burns and Dr. Anne Rowley, leading KD experts in the US. Lisa’s organs have also helped to save the lives of four people, and the family are in touch with the gentleman who received her liver.

I know how important it has been to me to make sure that Freya didn’t suffer this awful disease in vain.  We still have our daughter here with us, and thanks to the awareness that Amy is continuing to raise about the potential long-term and hidden consequences of Kawasaki Disease, we can hope that Freya will long outlive us.  I don’t know if I would have had the strength to fight had the unimaginable happened in the Summer of 2015.  As we were starting to get our lives back on track that year, a family was being torn apart by the loss of a truly beautiful person.  I join Amy in their hope that this story might reinforce the need to ensure that Kawasaki Disease survivors are given appropriate attention from the medical profession throughout their lives, and shines a bright light on the insidious nature of Kawasaki Disease.

Writing this, I felt my heart break all over again.  This is Lisa.

5508921_300x300

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…

IMG_521315826706_10209362733123114_2981185137876170344_n

 

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)

unknown-1

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

 

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.

fullsizerender-2

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!

IMG_7835

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.

IMG_7836.JPG

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.

This slideshow requires JavaScript.

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. 

IMG_6210