Questions I should have asked…

Over the past two years, I have used my writing and my desire to make a difference to raise awareness of this little known illness called Kawasaki Disease.  It all started with a journal that I was given whilst my daughter was in the hospital, a journal whose pages I would fill with thoughts and feelings every night whilst I lay there on a camp bed by my daughter’s side, a journal that would come home from the hospital 50,000 words richer.

Since I began sharing Freya’s Story, I have received many compliments about my writing, or my dedication to the cause. I’ve sometimes been called an inspiration!  And whilst I enjoy those compliments very much, the words and comments leave a bitter-sweet taste because they are compliments I would rather not have been in a position to receive.  At times I feel like a fraud, particularly when people say that without my perseverance, Freya might not have been here today.  Those comments make me feel like a fraud, because in the beginning, in that first week of Freya’s illness, I did nothing.  Nothing.

Freya had just passed the 7-week milestone when she became sick.  Up to that point we had spent some pretty uneventful days at home, where I lapped up the new arrival.  It is no secret to those of you who have read my blog from the beginning, from before Freya was born, that I was not particularly happy when I discovered I was pregnant.  With two children, aged 11 and 6, I already had the family I wanted.  I was approaching 40, and had just obtained my biggest promotion at work.  I couldn’t believe that I had gotten myself into such a mess, and in all honesty the pregnancy severely affected my mental health for some time.  And then over time my feelings settled, and I began to look forward to the arrival of our little girl.  It felt somehow that she was going to change things dramatically, that she was going to change me.  Boy, did I get that right!  I will never forget the moment that I held her for the first time – I wanted to  hold her so tight that I was frightened that I would squash her.  I remember asking the midwife over and over “Am I hurting her? Am I hurting her?” and they laughed and said of course I wasn’t! I had been there twice before, but this was different. Like somehow the journey I had been on through the pregnancy made her arrival so much more poignant.  She was a gift; a gift that I had failed to acknowledge for much of the time that she was growing inside of me, but my love for her once she arrived was more than strong enough to make up for that.  I was smitten.

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As I’ve already mentioned, those early weeks went by pretty uneventfully.  I had to remain in hospital with Freya for a few days after the birth as I had tested positive for Group B Strep during the pregnancy and had to receive intravenous antibiotics during labour to prevent the infection from being transferred to the baby.  Getting those antibiotics in time was a huge source of anxiety for me.  Throughout the pregnancy I had been plagued with thoughts that my baby would not arrive, and when I discovered that I was GBS+ and learned of the associated risks to my baby, I felt I had met her killer.  But once she was here, and we had the all clear to go home, all of those worries disappeared. Having learned the hard way from rushing to be supermum with the other two kids, this time I spent most days laid on the sofa with Freya in my arms, developing a bond like I have never known.  I watched movies as she slept on my chest, and I can remember only the sweet joy I felt from being in her company, just us two.  The first real adventure we went on was a trip to Cannon Hall Farm, a local visitor attraction.  That was on Saturday 30th May; Freya became sick the next day*

*The cause of Kawasaki Disease is still unknown, despite 50 years of research.  There is no evidence that I am aware of, of any connection between Freya’s illness and our visit to the farm, and I wholly believe that the timing was purely coincidental.  That said it took us a long time to return there, mainly because of the pain of the memories of it being our last day with our healthy baby girl.

Freya’s illness initially manifested itself with a loss of appetite.  She awoke in the early hours of the Sunday morning, but she seemed to have a cold and was struggling to take her milk with her nose so snuffly.  I tried for a while, but she just fell asleep with the exertion so I let her sleep until she next awoke.  It was after the refusal of the next feed, that I became concerned; not because I thought she was ill, but because I was worried about her not feeding.  That’s when I noticed her temperature.  It was my intention to give her Paracetamol, but when I saw the bottle said ‘from two months’ the goody-two-shoes in me wouldn’t let me give it to her without seeking medical advice.  So it wasn’t instinct that made me call the doctor – it was my conformity to rules!  Nevertheless, the call was an important one to make and the out of hours doctor said that I should take her to A&E as it was unusual for a baby of her age to have a fever without an underlying reason.  That’s when I remembered the GBS – my worst fears were becoming a reality and the word ‘meningitis’ seeped into my subconscious.

At the hospital, they checked Freya over and could see that she was working pretty hard to breathe (you could see her little tummy moving up and down instead of her chest), and the fever was soaring.  I remember telling them about the positive GBS result, and that she had been born within such a short time of the antibiotics being administered.  We went up to the children’s observation unit with her where we held her practically naked body as she slept in our arms.  I remember having to obtain a wee sample, which involves catching a wee in a kidney dish – no mean feat with a tiny baby, and we celebrated when we managed it!  What happened next is a bit of a blur, but I think because of her symptoms, and my warnings about the GBS, plus the standard protocol to treat a feverish infant for meningitis as a precaution, they decided to admit her to a ward and give her intravenous antibiotics and antipyretics.

I know I have told you all that already in previous blogs, but I’m telling it here to illustrate the point  that this wasn’t because of my instincts.  We were here because of the instincts of the medical professionals that this tiny baby was just too precious to send home.  

If you’ve read my blogs you will also know that after the medication had been administered I laid Freya down in the hospital cot and tried to get some rest in the chair in the corner of the room.  You might recall that as I laid her down, I could smell that she had dirtied her nappy, and you’ll remember that I chose to ignore it, to allow my exhausted baby to sleep without disturbing her again.  You’ll also know that I hadn’t sat back down in the chair for any more than a few minutes before I decided I could not let her sleep in a dirty nappy, and rose from my seat to change her.  She would sleep better if she was clean.  Was it instinct that got me out of that chair?  No, I don’t think so.  I think it was common decency and a conscience.  Whatever it was, it was the single most important decision that was made, because as I changed her nappy I noticed that her body was covered in a rash.  I hadn’t noticed that Freya was unresponsive when I changed her nappy.  She was sleeping, but I was pretty adept at changing nappies without waking the baby – I was like a ninja!  I can’t remember if she felt different. I called the nurse, the nurse called the doctor, and from that moment I was introduced to a whole spectrum of feelings that I will never forget and never want to endure again.  I can’t remember much at all, except the way the atmosphere in that room changed when the doctor and also by then a consultant, uttered the words “shutting down” and started to wheel my baby out of the room.

Two years on I can still see myself standing in that corridor in utter shock.  It was like something from a TV hospital drama.  It wasn’t real.  I called my husband to tell him he needed to come to the hospital.  I think it was around 6am.  I had this sinking feeling that my baby was dying right there in that room, and I remember how alone I felt. I walked slowly into the high dependency unit where they were working on my tiny little girl, pumping her with fluid boluses to resuscitate her.  I stood at the foot of the cot, my hands gripped around the bars, and just watched in silence as the doctors calmly gave orders to the nurses to hand them this, and pass them that.  It was how I imagine an out-of-body experience to feel.  Even now, I have to wipe away the tears at that memory.

Clearly they managed to stabilise Freya, but she was incredibly sick.  They continued to keep meningitis at the top of the list, particularly as she had gone into septic shock.  A number of different antibiotics were being delivered to her tiny veins through cannula sites in every place they could get one.  We would remain in the HDU for 3 days, where Freya was considered stable but no better. The temperatures continued to soar, she was tachycardic (elevated heart rate), and she was being fed through a nasal NG tube.  On the Wednesday (day 4) she was considered stable enough to move back onto the ward.  All I remember about those days was how quickly I settled into a routine of getting up and showering in our en suite cubicle, before Freya would wake.  I would try and sleep when she did, but I was too distraught to harbour much sleep.  I chatted with the nurses as they came and went, and it felt like we had been there for weeks.  I ate when family brought me food, but I rarely left the room.  Sometimes at night, my best friend would come with reinforcements and entice me out for a break and some food.  It was during those meals that I aired my fears to my friend; what if she dies?

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Freya’s CRP levels (the markers in her blood that show infection/inflammation) were dropping slightly day by day.  When she went in her CRP was over 340 (normal is under 7).  I took the downward trend as a good sign, but hadn’t considered that even as it was dropping it was worryingly high.  To give you some context, Freya wasn’t released until her CRP dropped below 7, which would be 6 weeks after she was admitted.  I don’t know what I thought was happening.  I think I believed that she had meningitis, and that she was on the right medication to treat her for that.  She needed a lumbar puncture to be sure, but was too sick to undergo the procedure until the Friday (day 6).  They took blood samples every single day, but none of them grew any cultures.  Around the middle of the week, a nurse came in and said “You know they’ve found it don’t you?! They’ve found rhinovirus!”  I remember feeling so silly! I had taken my daughter to the hospital with a cold!  I think I must have been in shock, because I actually believed they would say “Mrs McBride, Freya has a cold, you can take her home!”  Even with the heart monitor constantly alarming as her heart rate soared, even as her temperature continued to spike, even when she was unable to feed on her own, with cannulae in her ankles, her wrists, her head! Even when she stared at me vacantly from that cot, and I yearned for cuddles that I couldn’t give for fear of dislodging the wires and tubes.  What was going through my head? It was like I really believed they would just switch all that off and let me take her home.  And yet, deep down I knew that something wasn’t right.  I still had an overwhelming fear that she was going to die.  But I’ve been told all my life that I am a neghead, a catastrophic thinker, so I batted that away as irrational and ignored my instincts, even though the doctor’s faces betrayed their fear.  I remember remarking to the consultant that Freya had rhinovirus, hoping that he would confirm how stupid I had been to worry.  But his response was “Yes she does, but rhinovirus doesn’t make a baby this sick.” I think that’s when I finally allowed myself to believe what my gut was telling me; that Freya was gravely ill.

The lumbar puncture would come back negative.  Elevated white blood cells gave them reason to believe she may well have had meningitis, but that the medication had already begun to work, but the test was inconclusive.  I remember how scared I was when they told me to hold her after the procedure.  They said I had to keep her flat – I was petrified that I would make a move that could cause her a serious injury.  Every test she endured during that week she had me by her side.  All but the lumbar puncture.  For that, the nurses asked me to stay away, and to be there for the cuddles afterward.  I took myself far away from the treatment room, but I knew my baby’s cries when I heard them, as they bent her tiny body into the position necessary to carry out the test.  That would be the first of the three lumbar punctures they carried out, but the other two were done under general anaesthetic in theatre so again I wasn’t present.

It was on day 7 that I told my husband how afraid I was that the doctors weren’t telling me something.  I said I could see the concern in their faces, eyes that betrayed their calm words.  It was then that I asked my husband to meet with the consultant.  He had to do it, as I needed him to ask them a question that I couldn’t bear to hear the answer to. I needed to know if they thought there was a possibility that she could die.  That is when she told my husband that on a scale of 1-10, with 11 being dead, Freya had been a 10.  My instincts had been right, and I just didn’t have the faith to believe in them. The following day, Freya would be transferred to the specialist children’s hospital as the local hospital had exhausted its investigative scope.  I do recall the consultant saying that if it were his child, and they had been sick for 8 days without diagnosis, he would be demanding answers.  He said that the children’s hospital would be able to scan Freya’s brain, and do an echocardiogram on her heart.  Still no alarm bells rang for me – belts and braces, I thought.

It is worth stating that in that first week, the only notable symptoms were a fever that would not abate with the usual antipyretics (Paracetamol/Ibuprofen), and a rash.  Freya’s lips were very red, sore and cracked, but this considered a symptom of dehydration with her being tube fed.  I had commented that her ankles and wrists looked swollen, but it was attributed to cannula damage, particularly as the cannulas were constantly having to be replaced due to failure.  The staff did not give me any cause for concern, and I didn’t therefore consider that all of these were possible symptoms of some strange disease.  Why would I? I mean that kind of stuff happens to other people doesn’t it!

It was in that first week that I should have asked Dr Google’s advice.  The fact that I didn’t  will haunt me forever.  Do you know what the top result is if I put this question in my search engine…

Question:  “My baby has a fever, a rash and cracked lips.”

Answer: Kawasaki Disease – Symptoms – NHS

I didn’t ask Dr Google though, and I have no idea why not.  Trust?  Complacency? Ignorance? Perhaps all three, and other reasons too.  I simply had no reason, or point of reference, to lead me to believe that I would need to look it up myself! We were in the hospital, surrounded by doctors and nurses with many years training and experience.  You don’t ask Dr Google, you sit back and watch and wait and let the professionals do their job.  You are in the right place, and they will make your child better. That’s how it works.  Perhaps I had the instinct, but didn’t know what to do with it?  Not once did the thought enter my head that my child could have something seriously wrong with them that the doctors were missing.  Not once.  I’ve literally driven myself crazy for two years asking myself why I didn’t see it.  Why I didn’t ask the right questions.  I’ll never get that answer, and will have to live with the consequences of that.

Perhaps if I’d read about this weird thing called Kawasaki Disease, I’d have associated the lips and the swollen extremities as symptoms and made them see?  Perhaps when the consultant mentioned a heart scan, the penny would have dropped and I would have asked him, “Do you think she has Kawasaki Disease?”  Perhaps if I had, they would have talked to me about it, and we would have agreed that she met the brief, and not for just an incomplete case as was finally diagnosed on day 13 of her illness.  No, she had all the symptoms they needed for a complete diagnosis.  My old boss once described me as being like “a terrier after the postman.”  When I get a bee in my bonnet, I rarely let it drop.  Had the KD seed been planted, I know I would have done so much research in those twilight hours when I dared not go to sleep that I would have made the connection.  I know that when they did an echo on day 9 that showed no damage that I would have told them that didn’t mean she didn’t have it! 75% of children with KD don’t sustain any heart damage, and you can’t use a clear echo to rule the disease out!  I know when the new rash appeared, that I would have recognised it from pictures I would have seen plastered all over the internet! There’s no mistaking it.  I might even have known that young infants are more likely to suffer large and giant coronary aneurysms, that they are more likely to be resistant to standard treatment (IVIG) and that they are more likely to get a severe case of the disease that results in a dangerously low platelet count (thrombocytopenia), not the usually high platelet count that is characteristic of Kawasaki Disease.  Had they decided to be open with me about their fears or suspicions, had they told me that KD was the prime suspect, I would have made them give her the treatment and not allowed them to rule it out. Which is what they did. They ruled it out on day 9, and she went through further tests including an MRI and a bone marrow aspiration in theatre, both of which failed to give them an alternative.  I wonder how it felt to see her coronary aneurysms when they showed up on an echo on day 13….

Now that sounds like I am blaming the hospitals.  Maybe I am? I don’t know.  I do know that it wasn’t negligence on their part.  They worked so hard to find the answers, and were thrown off the scent by this insidious disease.  I know how important it was to them that Freya get well again.  They know I was angry, in fact they told me how upset they were that I was angry with them because they felt that they did everything they could.  I won’t lie.  I will always feel angry that they were looking for it and still allowed themselves to be misled.  Could they have known more about the disease?  Enough to give them the conviction to go with a diagnosis regardless of that first echo?  I know they had to make certain decisions, and that the treatment for KD could have proven catastrophic had it had turned out to be Leukaemia as they had also suspected.  And no amount of blame will change where we are.  What I am thankful to them for is how they treated the illness once the diagnosis was confirmed.  Their aggressive treatment of the disease and their over-cautious approach to monitoring from that moment was fundamental to her recovery, and I truly believe that their actions from that point saved her life.  I will always question the decisions made by both hospitals leading up to Freya’s diagnosis, perhaps because it would feel easier if I had someone to blame, but their actions afterward more than make up for any misjudgement.

Perhaps it’s one of the reasons why I can never forget those early days in the hospital?  Perhaps it’s the main reason why I do what I do to raise awareness of the disease?  I cannot bear the thought of another parent feeling the regret that I do over questions I should have asked…

Whatever the reason, I know that I will always wonder if I could have helped my daughter get an earlier diagnosis and more timely treatment.  I will always wonder if I could have stopped her heart from breaking.  I can’t change what happened to her, but perhaps I can swallow some of that guilt and grief if I can change it for someone else.  So know the symptoms! Let our story show you how important it is to advocate for your child.  Ask questions – direct ones!  What do you think could be wrong with my child? Make them tell you what they are thinking!  And when you get some idea, and you think your child is displaying symptoms that give you even the remotest possibility of this disease, then ask them this…

“Tell me why it ISN’T Kawasaki Disease!”

It could be the most important question you ever ask.

 

 

If you would like to follow Freya’s progress, you can like and follow her story on Facebook (www.facebook.com/freyastory), Twitter (@freya_story) and Instagram (@freyas_story).  

For more information about Kawasaki Disease, please visit the NHS Choices website: www.nhs.uk/conditions/kawasaki-disease/pages/symptoms.aspx

If you know of anyone who is dealing with this disease and would like support, they can contact the UK Support Group through their website www.kssg.org.uk, or visit the KD Foundation (US) www.kdfoundation.org

To donate to Kawasaki Disease Research, please visit my fundraising page: www.virginmoneygiving.com/joannemcbride – all funds raised go directly to the COSMIC Kawasaki Disease Research Fund, registered charity number 1043697)

You can also visit the UK Foundation for Kawasaki Disease, Societi, to see the work they are doing to change the face of this disease: www.societi.org.uk

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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.

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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.

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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.

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