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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75,000 Reasons to be Thankful…

So Peanut, where do I start?  I guess a good place to start would be to thank you.  Thank you for coming into the world, despite all my efforts to ruin that.  I wasn’t sure that you were welcome in my life when I found out that you were coming along.  Some people might say I ought not to share that, but do you know what? I’m not afraid of my honesty, because I know that you will never, ever feel that you were not wanted.  So I wasn’t sure I was going to love you when you were growing inside me, not in those early months.  But towards the end, our bond began to grow, and when you arrived into the world, a tiny, purple, screeching thing, I knew that the past didn’t matter.  What mattered was that you were here, you were alive (yes, I was afraid of that), and that there was no question in my mind that I loved you at that moment, and that I would continue to love you for the rest of my life.

You were so precious, and I held you so tight I was afraid I would crush you.  But I had been on such a journey to get to that point with you that I had to feel you in my arms, really feel you.  Years ago, when your older sister and brother were born, babies seemed to be whisked off, the moment they arrived, for the all important weighing and measuring.  It seems the health service have learned a thing or two about bonding since then.  I remember saying over and over to the midwife, to your Auntie and your Daddy that were all there with me to share the moment when you burst into that delivery room, “Am I hurting her?”, “Am I holding her too tight?”  And they told me to relax, and I held you for the longest time, drinking every bit of you in.  I felt like I had been truly blessed.  Not in the glib clichéd sense, but actually blessed with a gift.  You were a sign.  A sign that proved to me that I was indeed a lucky person, and all the insecurities, fears and negativity I had held onto for so long were allowed to be set free.

In the early weeks, I took heed of all the advice I had been given and ignored with your siblings.  We didn’t go out of the house for weeks.  We spent our days cuddled up in the cosy corner of the sofa and got to know each other.  When you slept, so did I; the housework could wait.  And I didn’t feel any need to rush out to meet people or go places, because I wasn’t ready to share you with the world yet.  You were all mine, and I all yours.  There would be time for all that fun stuff later.  Had I known what would happen 7 weeks later, might I have done things differently?  No, I don’t think I would have.  In fact I am glad I made the choices that I did, because at least I got to have the perfect you all to myself for 7 blissful weeks.  Before…well you already know what happened next.

I am not going to dwell on the following weeks, on your illness, on your diagnosis or the effects that Kawasaki Disease have had on you.  By the time you read this we will have covered all that.  No, this letter is about thanks, so lets get back to the point shall we.

You truly are a remarkable little girl, Freya.  You have endured so much in your little life, and yet you have managed with a grace that shouldn’t even be possible at your age.  You have more courage than I have seen in people more than 20 years your senior.  Your heart, tiny by comparison, has the ability to love with more fervour than any adult I have met in my 41 years on this Earth.  You touched my heart in some magical way the moment you were born, and you have continued to touch the hearts of everyone who has encountered you since.  You have something special within your soul, that shines out like a beacon through those eyes.  Eyes that have both the power to haunt and to heal me.

You have taken everything your short life has thrown at you in your stride, before you are even able to walk a step! Even when you were critically ill and your eyes pleaded with me to help you, your little mouth worked so hard to form a smile.  And later, as you started to get stronger, you smiled for every nurse and every doctor that crossed your path.  And there were a lot!  You even managed to bowl the phlebotomists over when they came to take your blood.  You would cry for a moment, but once they had taken their fill, that smile would spread across your face like a sunbeam and I would see that you had made someone’s day.  Again.

Thank you for inspiring me to write.  Well, it was your Auntie who gave me the journal and the pen, but you were my muse.  I remember opening that book, and wondering where to start, and then I looked over at you in your cot and I knew you were scared and I had to tell you what was going on.  And so the letters began.  I didn’t give it too much thought after that; the letters just came, at the end of every day when you were sleeping.  I would write in the moonlit room, laid on the parent bed by the side of your cot.  Sometimes I would think about how you would learn all about those days, because I knew you wouldn’t remember them.  Often, it was a chance for me to keep a note of the facts as your story unfolded.  Sometimes, on particularly bad days sweetheart, I would wonder if I would ever get to share your story with you, and I wondered if I knew deep down that I could really be writing for myself.  But let’s not dwell on that, eh?

I knew your story was one that I needed to share.  It came from an intense need to make sure that you had not suffered in vain.  To walk away and do nothing would have made me feel like it was unimportant, and it felt too important for me to do that.  In every moment that I wrote through tears in my journal, I felt that there was another parent like me in a hospital room somewhere with their child; sad, scared and alone.  I felt alone.  I scoured the internet,  I contacted Doctors near and far, and I joined social media support groups searching for answers.  The thing is, you can find the basic answers for the typical cases, but your case was an atypical one in many ways.  Any answers I did get were like gold, and whilst I knew that the information I gathered in relation to your case would not be appropriate to generalise, I knew that I couldn’t hold onto them.

And so Freya’s Story began.  I resurrected this blog, and created your tag.  And then I created a Facebook page that would help me to broaden the audience for awareness.  I had a lot of catching up to do – I had written 40,000 words in that journal if I remember correctly! So I began the task of transferring those journal entries onto my blog, whilst updating on your current situation.  There are many other social media pages who are dedicated to raising awareness and keeping people like me informed on latest news and developments in the world of Kawasaki’s.  The various support group pages were great, but sometimes the updates about children who were continuing to experience health issues years after diagnosis would push me into a very dark place.  Sadly, that is the reality of the disease, I know that.  But I wanted to use your page to show people that there is #lifeafterkawasakis.  Of course the disease continues to blight our life.  You have continued complications with your heart that are still unknown.  In a couple of month’s time you will go into hospital for an invasive procedure to try and get some idea of what is going on.  So far, you look like a miracle kid.  But we need to look deeper to be sure.

Freya’s Story is about more than the disease though.  It is about a special little girl, who has the ability to inspire a mum to write; I always knew I had words in me, I was just lacking the inspiration.  It’s about a pair of eyes that have the power to lock with the reader’s through a screen and implore them to read your story.  It’s about flying in the face of adversity, seizing opportunities, loving life and having hope.  It’s a celebration of a little girl who will not allow some nasty bastard illness (sorry for the language but I get a bit angry at KD sometimes) to stand in her way.  You are not the Kawasaki Kid.  You are my marvel, and you surprise me every day.  I hope as you read this, all grown and proud of whatever you have achieved in your life, that you still cannot see the scars that KD left you with.  It is an invisible illness that shows itself for a while, then skulks back off into the shadows where it belongs.

I knew I had done the right thing as soon as the messages started coming in.  Ok, so Freya’s Story hasn’t exactly gone viral – let’s face it, Kawasaki Disease doesn’t have the same amount of clout as Meningitis, say, but it needs putting on the map, and you and I will help to put it there.  And anyway, if even just one parent feels less alone, or one child receives a swift diagnosis or the right treatment after reading our blog, then we achieved what we set out to.  We’ve had parents sending messages of hope, parents asking questions about medication, treatment, immunisations, all kinds of stuff.  I’ve had to be careful with my responses; I don’t have a medical degree (although I do sometimes feel like I have one in KD), so I have mainly signposted parents to useful social media pages, internet links, research papers, support groups and the like.  I have given words of comfort when they’ve been asked of me.  I’ve kept people up to date with how you are doing when they’ve contacted me to ask how you are.   We’ve been credited with helping parents get the treatment that they needed for their child, and helping some people through some lonely times.  I am sure there have been some doctors across the country muttering “Who is this Freya’s mother?!” But I have never claimed to know it all. I only know about you really.  But at least I have been able to provide information that has helped to inform discussion and provide a line of questioning that might previously have been more difficult for a parent to navigate.  Thank you for inspiring me to do that.

You might not thank me for it, but I am sacrificing your 1st Birthday to raise funds for Kawasaki Disease research.  Ever since I made contact with the Professor heading the research after we sent off our swabs for genetic testing, I knew I had to do something.  I had asked the Professor to show me a tangible offering for parents like me to donate to.  Something that would show us how we could contribute to the amazing opportunity that had been granted to Professor Jane Burns in the States.  He cemented the offering in a 2-page document with a link to the COSMIC Kawasaki Disease Research Fund campaign on the Virgin Money Giving site, and I shared it with the Kawasaki community back in November last year.  Once I had a willing recipient, I could concentrate on bringing in some funds – no matter how small our contribution might be, the Professor had assured me it would be worthwhile and gratefully received.  And so the idea of turning your birthday party into a fundraiser was born.  I’ll tell you another time about those details, but for now, I want to thank you for forgiving me for giving up your birthday for Kawasaki Disease.  I have promised that I will not steal any more birthdays from you.

There are so many people to thank for their contribution to your birthday party, and it’s not for another 6 weeks! I will make sure I cover that when I blog about your event.  From local businesses donating prizes to entertainers offering free services, we’ve had a huge amount of support.  The local press have followed your story since we first approached them to help us raise awareness after you came home.  We’ve had cupcakes sold in your honour, and cash donations have started to hit the Virgin Money page.

And then there was this one thing, that started with a Tweet.  

Twitter and I are kind of new friends.  I set up your Twitter account not really knowing what I was going to do with it.  I still don’t really, but I dabble here and there and started to share your blogs when I’d worked out how to!  I mainly use it to hound celebrities in an attempt to increase the reach of your story and shine the spotlight on Kawasaki Disease.  I have had some successes; some of the key KD and Rare Disease organisations follow Freya’s Story.  We’ve had retweets from some celebrity Doctors, like Dr Miriam Stoppard and Dr David Bull.  One of the stars of TOWIE retweeted once, and we’ve had a couple from actresses and directors.  We set out on a bit of a challenge with a Kawadad from the other end of the country, (you know who you are!) but he has had more success than we have, lol!  I have a lot to learn about Twitter it would seem.

But, somewhere, on one sleepless night during their own Kawasaki ordeal, it would seem that the right person stumbled across your story.  They saw that we were raising money for the Kawasaki Research Collaboration between Imperial College London and Rady Children’s Hospital in San Diego.  And that person did a remarkable thing;

“…managed to get…some funding for research. 75 thousand pounds…”

Even though I’ve had that information for a couple of days now, I still struggle to let that sink in.  I think my response was something like “Are you serious? How is that even possible?!” which was met with a reply about the world not being all bad.

I received an e-mail from the charity too, but I still didn’t quite believe it Peanut.  How could we have inspired such a huge donation?   But it is true.  And if the donation can be counted towards the collaboration pot, it will be doubled. Meaning that single investment in research is worth £150,000!!

“…you may want to thank someone who raised awareness about the KD research thru her twitter activity: I found you guys late at night reading the posts from a @freya_story” 

Thank you for giving me a story to tell.  Whilst I would have given anything to have prevented you from going through this, I can take comfort in the fact that because this happened to you, great things might happen.  No amount of raising of awareness or funds will change what happened to our family 8 months ago.  The emotional scars will take a long time, perhaps forever, to heal.  What I hope it will do, what every parent who has experienced this with their child hopes, is that the research being undertaken now will change the future of Kawasaki Disease and the lives that it lays claim to.

I’m going to leave you with these lovely words, received from Ilsen Cafer (Fundraising Co-ordinator for COSMIC – Children of St Mary’s Intensive Care);

…You deserve to be very, very proud.  I can’t imagine how tough a time it must have been for you and your family when Freya was diagnosed, but your special little girl, her story and your courage have helped lots of parents, as well as inspired a donation which will make a difference to the lives of Kawasaki patients around the world.  Never underestimate the impact of your story, you’re doing a fantastic job!” 

“…Please tell Freya in her letter that she is our little COSMIC star and that she should be very proud that aged only 1 she has inspired so much more than most people ever do!”

It seems that I have so many people to thank, and so much to be thankful for, despite this terrible experience that we have all been through at the hands of this disease.  Most of all though, I have to thank you for being you.  You have got us through this.  You.

And then there’s one person whom I would like to be able to thank 75,000 times, and that is one very cool Italian dad who lives in Hong Kong.  Thank you from the bottom of our hearts.

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To Immunise or not to Immunise…

…That is the Kawaquestion!

If you’ve been following Freya’s Story through my blog or on Facebook (www.facebook.com.freyasstory), then you’ll know that one of the hurdles I have been trying to get over since her diagnosis and treatment for Kawasaki Disease is the question of when to immunise.  When is the important question here – it is not a question of ‘if’ for me.  Freya is my 3rd child; I had the older two vaccinated in line with the standard UK immunisation schedules without hesitation.  They both had the (then controversial) MMR vaccination that was vilified for years, for those claims to be later contradicted by the very doctor who made them.  I am not an antivaxer (yes, that is a word that is banded around various mum networks).  I can’t say I’m a provaxer either to be fair; in all things medical I am a conformist.  I trust the NHS and the doctors to have made the right decisions.  Well I used to anyway.

Now before I go on to share all the information I have in relation to this subject, there are a couple of things you should know.  First of all, the information I have received is contradictory to say the least.  I’ve even had different information from the same doctor, just on a different day.  And therein lies one of the biggest issues, and the key reason why we should be campaigning for standard protocols for treatment and care of Kawasaki patients.  But that’s a whole other blog.  Secondly, I take no responsibility for the decisions you may make in relation to this issue.  I am not a medical professional; I am just a mum that wants to do the right thing for her child.  I could not protect my baby from Kawasaki Disease; I have to be able to protect her from the other nasties, if I can.  I will share the information I have and where I’ve found it.  You also need to understand that Freya’s case is an individual one.  We certainly haven’t found a doctor in the UK who has received a patient as young as Freya where Kawasaki’s is concerned.  Generally children are older (commonly between 6 months and 5 years) and have therefore already begun their immunisation journey.  Infants diagnosed with this disease have often already had at least the first tranche of jabs (leading some to believe the vaccine to have been the trigger that KD needed to start it’s evil – a theory that I don’t discount, in fact I find it a very interesting theory with some validity and would not be surprised if it is confirmed as one of the triggers in the not too distant future).  Indeed, some of the vaccines state an increased risk of Kawasaki Disease in the patient information – information we often don’t read before we give consent for the needle to go in.  Again, I do not want to be a scaremonger, and it is absolutely not my intention to send cats amongst pigeons.  I’ve already said I am a provaxer.  I am pro-choice in all things – but I believe in those choices being informed if you are someone like me who needs to know it all (and often thinks she does, lol!).  

Freya was 7 weeks old when she became sick.  She was hospitalised on the very first day that she showed a single symptom (fever) and spent a number of days on a paediatric HDU, several weeks in a Children’s Hospital, and a week on a Cardiac HDU.  We hadn’t yet seen a health visitor, and the appointment for her first set of immunisations (8 weeks) has just come through.  I guess due to her condition, and later the treatment she received, it wasn’t possible to start the process of vaccinating with Freya whilst she was in the hospital.  She was 7 weeks old when she went in, and 13 weeks when she came out, so she was already behind schedule.  She received a blood transfusion, intravenous immunoglobulin (a blood product) and intravenous and oral steroids.  All of these have an impact on the immune system, rendering vaccination difficult for a certain period of time.  And I can’t be more specific than that, because that is one of the biggest areas of contradiction I have come across when researching this subject, and the reason why now, at nearly 10 months old, Freya has not had a single vaccination.

Some might wonder why that is an issue.  Plenty of parents (those antivaxers) make the decision not to immunise their children against some or even all of the diseases that the immunisation schedules seek to protect them from.  There are children who are unable to receive the immunisations because of poor, weakened or non-existent immunity.  We vaccinate the many to protect the few; herd immunity.  I could trust that everyone else has been protected, so Freya will be ok among the herd.  But, what if…  And then there is chicken pox – we don’t routinely vaccinate for that here in the UK (they do in the States).  If you Google “chicken pox and aspirin” you will find one big reason why aspirin and kids don’t mix; Reye’s Syndrome, a rare but potentially fatal illness linked to the fever associated with Chicken Pox and Influenza in children taking aspirin.  Ok, so the link has been found where high doses of aspirin are taken, and not yet with the low anti-platelet doses that a KD kid is prescribed, but nonetheless it’s a nasty illness and one I don’t want to take any chances with.  Freya has been through enough.

So what happens if you have a child who has not been immunised, and you are concerned about them coming into contact with what others would consider pretty innocuous illnesses?  Let me tell you what happens with us.  Freya has not been to a baby group; not baby massage, or baby music, or baby yoga.  Hell, she hasn’t even been to a playgroup, and when my older two get invited to a kids party, Freya stays at home.  Don’t get me wrong; we have not become social recluses.  Instead I apply my common sense to the activities we will undertake.  I know there might be someone in the coffee shop who has the flu.  Why is that kid in the shopping centre not at school? Could they have chicken pox?  I can’t hide us away from the whole world, but I can reduce the risk of her coming into contact with these nasty germs and bugs.  I call it damage limitation. She has one or two little friends that she sees from time to time, but only when they are well.  And don’t forget she has an older brother and sister who are exposed to all manner of things at school every day – we don’t make them wear a mask!  It is a source of sadness for me though.  Freya is such a happy, sociable child, and I worry about the effect this lack of exposure to her peers might have on her development.  I want her to laugh and play with other children, to learn how to share, to grow bonds and discover new things.  Instead there is a lot of singing and dancing going on in this house, which isn’t a bad thing (unless you’ve heard my singing!!). And with a bit of luck we can catch up with those immunisations soon and provide what we believe to be a little protective bubble around our child before it is too late for her to start over.  The knock-on effect of KD will be huge – when I return to work, she will have to go to some form of childcare.  She will be over a year old, and will never have been with anyone else other than her close family.  That’s going to be a tough one for both of us to handle…

Let me go back to the beginning of Freya’s immunisation journey.  While we were in the hospital with Freya we were advised that she shouldn’t have the immunisations, first because of her condition, and secondly because of the medication.  A lady used to pop her head in our cubicle door every other day with an immunisation leaflet and I repeated often that we had been advised Freya would have to be left to catch up.  I was promised an individualised immunisation schedule to leave the hospital with; it never materialised.  I made a number of calls to the hospital to enquire about when I could start the process, and to the Health Visiting team to ask them to stop the weekly postal reminders telling me what I already knew; Freya had not received her first immunisations.  Eventually I got the call from the hospital that advised I could give her any killed (inactive) vaccinations immediately (she was discharged one month after treatment for KD), but that I should wait 3 months post steroid treatment for any live ones.

I did a bit of research, because I was convinced there should also be a timescale for receiving vaccinations after a blood transfusion.  All avenues confirmed that vaccinations should not be given until 6 months after a transfusion.  Not to mention the varying timescales quoted for giving vaccinations after IVIG!  But all my hospital ever referred to was the steroid treatment, I guess because that is an immuno-suppressant.  But what of the other things? Had they forgotten what she had been given? I felt uneasy with the advice, so I sat on it for a while.

At a follow-up appointment with Rheumatology, I asked about whether Freya should be vaccinated against Chicken Pox (Varicella).  The doctor said that in the UK we only vaccinate children who are at high risk of contracting the disease.  I said that whilst Freya wasn’t at higher risk of contracting chicken pox, the potential consequences of her contracting the disease whilst taking aspirin could be catastrophic.  He agreed to take the issue away, and on the 19th August 2015 I received a letter confirming that they believed it was “reasonable for Freya to receive vaccination against Chicken Pox, both in relation to the issue regarding ongoing treatment with aspirin and the small degree of risk of developing [RS] and also the small risks that Freya may require treatment with immunosuppression therapy in the future…”.   Freya had been weaned off steroid therapy a lot more quickly than originally planned when the gastrointestinal bleeding started. From memory, it was the 8th July 2015 when she took her last oral prednisolone; that meant she could have the live vaccines, including CP, anytime after the 8th October 2015; “…it is important that Freya does not receive this until at least 3 months following…the last day Freya received the steroid treatment…”.  I still felt uneasy, and decided to wait until our next follow-up appointment to discuss.  It was at that appointment that I shared all the research that I had done, and got a referral to Immunology.  I had to wait until yesterday for that appointment.

I wonder if now might be a good time to share what I have learned, from the internet (reliable sources, like the NHS and the American Heart Association) and from medical professionals from London to California…

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From the list above, all vaccines are inactive with the exception of the following live vaccines – Rotavirus, MMR, Children’s Flu vaccine (note that for the children’s flu vaccine, the nasal spray is live, however there is an injectable inactive flu virus available).

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These are just a few examples of the responses I received from medical professionals, and I can say that my internet research has thrown up much the same; anything from 3 months to 12 months.  Helpful, right!  My gut feeling was telling me I should err on the side of caution, and follow the advice of the US doctor who is considered the leading Kawasaki Specialist in the World – she calls herself a ‘Kawasakiologist’ and I believe she deserves the title.  Our GP agreed that Freya qualified for the injectable (inactive) flu vaccine.  My decision was made.  Well, kind of.  I had decided I wanted to get Freya caught up on the inactive vaccines immediately, then discuss the issue of the live ones with our Immunologist.  Chances were, by the time we got any decisions we would be nearing that 12 month mark anyway, so it was a win-win.  But our ever cautious GP decided that if we were speaking to an Immunologist anyway, there was no harm in waiting for that meeting before we started the immunisation process, and he felt happier to wait for clear direction from her.  So, again we would have to wait.

So yesterday we had that appointment with the Immunologist.  I was all geared up to go in and cover all my questions, but unfortunately an hour’s wait with a baby that has decided that a high pitched squeal is the best means of communication made the whole process a little trickier than I had anticipated!  The doctor was the Infectious Diseases Consultant who treated Freya when she arrived at the Children’s Hospital on day 8 of her illness.  That was nice, as I had the opportunity to thank her for her compassion, support and tireless determination to get to the root of Freya’s condition (especially as I was quite a difficult ‘customer’ who asked lots and lots of questions, and even accused them of breaking Freya’s heart at one point!)  She seemed genuinely pleased to see Freya looking so well.  She received the news that we had seen some improvement in her coronaries since the acute stage of the illness with a broad smile, and she was happy that Freya has not had anything more than one little cough and cold since her hospitalisation.  I expected her to call me a muppet for being a cautious and over-protective mother, but instead she said she felt I was doing the right things, which was reassuring.

I came away from the appointment with a solution, individualised for Freya.  I still don’t have a definitive answer about the timescales related to immunising after IVIG.  But then why would I?  The fact that I can quote at least 5 different timescales from medical papers and journals across the world tells me a lot of them are guessing. I’ve read that medics aren’t completely sure why IVIG even works in the case of KD – they are still trying to understand what causes the illness in the first place, so I guess it would be near impossible to understand why the treatment works. It just does (in most cases).  The Immunologist told me that they deal with children who have compromised immunity as a result of a bone marrow transplant, for example.  Those children are given immunisations  in line with the hospital guidelines and they do not wait 12 months.  That said, she agreed that they had learned more than they previously knew about KD from Freya.  Presenting at just 7 weeks old, and having had no previous immunisations, she is a bit of an unusual case.  So they have agreed to follow a similar process to that of a bone marrow transplant patient.

What are they going to do?

They have taken blood samples from Freya in order to test her body’s immunity status.  They will be testing Freya’s immunoglobulin levels, and lymphocytes (T-cells).  Depending on those results (and I am sorry, I didn’t ask what result they would be expecting or what a low or high result might indicate, or whether there was a chance they might just be reading ‘borrowed’ antibodies as a result of the circulating IVIG – I will ask that when we discuss results), they will start the routine inactive vaccines and the injectible (inactive) flu vaccine.  After a period of time following those (I think she said 6 weeks), Freya’s immunoglobulins will be tested again to check her immune response to those vaccines.  Assuming they get the right result (whatever that might be), they will then progress to live vaccines, including chicken pox.  If we get moving quickly, Freya may even make it in time to have the MMR vaccination at precisely the right time.  I have some decisions to make about Rotavirus (which I believe is time-specific so she may already be considered too late) and Meningitis B, as both have stated increased risks of contracting Kawasaki Disease in the patient information.  I know it is incredibly rare for KD to reoccur, but show me one parent of a kid with a rare condition who finds that word reassuring.

Yes, I am still confused as to what the right answer is about timescales for vaccinations post treatment Kawasaki Disease.  But, I think I have a great solution for me, for Freya.  Rather than guess based on the varied guidelines out there, we are going to take a look at Freya as an individual.  Actually take a look inside and see what is going on, and make the decisions based on what she tells us.  The Immunologist has always said that Freya showed them the path to a diagnosis, and that they learned so much from her.  Seems she is going to teach them something new.  And as much as I probably do their heads in with my questions and powerpoint presentations, I do know that they respect me for it and that they are luckily not too arrogant as to discount this mum’s research and opinions.  She actually said it was helpful and told Freya that she is as well as she is because of her “amazing mum”. I don’t know about that, but in a sea of negativity and low moments, I’ll take that right now.

Whatever I learn from this experience, I will share.  Every Kawasaki case is different, no matter how similar some of the stories might seem.  No wonder it is a tricky one to spot, diagnose, treat and understand.  I hope in my lifetime they find the cause so I can finally understand why this happened to us, to my little Peanut.

Our Rheumatologist said that the subject of immunisations in these cases is “contentious” and currently being debated.  Another said that “guidance is not clear as there are no trials/studies to guide us for this exact situation…” and “…is a balance involving unknowns…”  He gave his opinion and advice, stating that “…there are other equally ‘correct’ versions too!”  No shit!

I’ll leave you with a few of the comments that I received from KD specialists here and in the US, which might help to dispel a few myths relating to IVIG and immunity.

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And in case you can’t read that, I have pasted below:

“The reason not to give live vaccines after IVIG treatment (has nothing to do with steroids) is that your daughter has received antibodies poled from 10,000 adult blood donors who have antibodies against the viruses in the live vaccines. Therefore, the live viruses are inactivated by the circulating IVIG that is still detectable 11 months after administration…it is not dangerous to give the live vaccines…it’s just that they will be inactivated and the body will not make a lasting response. All killed vaccines are fine to give once your child is past the acute phase. 3 months sounds like a good period to wait. All live viruses vaccines should be delayed one year after IVIG.”

“We don’t usually stop aspirin when we give chicken pox vaccine for our [patients] with serious aneurysms. The risk of Reyes’ Syndrome was associated with higher doses of aspirin and not the very low dose used for the anti-platelet effect in KD patients with aneurysms. There is no data to support the notion that low dose aspirin is a risk. That being said, Reyes’ Syndrome [is] terrible and it should be easy to stop aspirin for 2 weeks. Clopidogrel can be substituted for aspirin during that time.”

“Your daughter’s immunity is not compromised by KD. It can be compromised by steroids, but only while actually taking the steroids. Your daughter’s immunity has been boosted by the IVIG she received so she is quite protected from all routine infections for the next year. There is no evidence that children with KD have immune defects that make them more susceptible to routine infectious agents.”

“…The issue of when to vaccinate children who have received…IVIG…arises because the antibodies present in the IVIG may prevent an adequate response to live vaccines. This is less clear in the case of vaccines that do not contain live organisms. The second issue is that steroids may diminish immune response and also make live vaccines dangerous. If Freya has been off steroids for 3 months she can go ahead with all the killed vaccines…”

“…The IVIG may prevent live vaccines working for over 6 months, and may interfere with MMR vaccine working. However MMR is not needed until after 11 months of age so unless there is a local measles outbreak she does not need MMR until the normal age…”

“Rotavirus vaccine is supposed to be given before 24 weeks of age which Freya may be beyond; it is probably not worth giving it at this stage…”

“…Varicella vaccine [chicken pox] should be given but as both steroids and IVIG may prevent it working I would suggest waiting at least 6 months from the IVIG and steroids before giving it…”

 

 

A new perspective

These last few months have been some of the toughest I have ever had to face, and I have experienced a whole plethora of emotions from sadness to anger, and every feeling in between.  I have even experienced grief, which was a particularly hard thing for me to deal with as it has been well over 20 years since I lost someone dear to me.  And I know that Freya is still here, very much alive, but I grieved in anticipation of the possibility of loss, and I grieve still for the loss of her healthy heart.

Kawasaki Disease doesn’t rule my life quite as much as it has done over the last few months.  I still dedicate a lot of time and effort to making sure Freya is receiving the best possible care, but mainly to satisfy myself that I understand the care she is getting because that’s the way I am.  I’ve never been one to accept something just because someone tells me that’s how it is.  I like to be fully informed, and I like to question the ins and outs until I am comfortable that information and decisions are accurate because I know that people don’t always get it right.  Not even me, haha! No, Kawasaki Disease has started to take a back seat to normality as we get on with being a family again.

One of the things that has niggled me the most is the notion that the hospitals took too long to diagnose Freya’s condition.  I have at times been angry and judgemental about the fact that they were unable to reach a diagnosis within the ‘safe’ window, and that it was too late by the time they worked it out.  After our stay in Leeds, where I had the worst day of my life (reference Freya’s Story (11)), I returned to Sheffield Children’s Hospital angry and bitter.  I felt that Freya’s doctors had failed to prepare me for what was about to unfold, and accused them of sending me to Leeds like a lamb to the slaughter.  I had not imagined that my worst fears were about to become a reality; up to that point I believed that my negative thinking was going to prove unfounded.  Both the Rheumatology Consultant and the Immunologist came to see me when they heard that I had questions, and I asked them pointedly “Thirteen days?! Why did it take you so long?”  I remember thinking that I could read the hurt in their eyes, and could see that they were genuinely upset that I had questioned them.  I do believe that they worked incredibly hard to do the best they could for Freya, and that they were almost as saddened by the outcome as I was when the echocardiogram confirmed that she had indeed been fighting KD.

It is easy when you are in a particularly black moment to want to hit out at those around you, and I very quickly forgot what I had seen from them in the days that Freya was in their care. Looking back now, it was really no time at all.  Sheffield reached a diagnosis within 5 days of receiving Freya onto the ward, and during those 5 days I could see that they worked tirelessly to try to fathom out what was making my baby so sick.  And they were brilliant at keeping me informed. I had not expected to see the Consultants very often during our stay, but there wasn’t a day that passed (well, weekends maybe) that we didn’t see one or more of them, sometimes more than once.  The Immunology Consultant was one of the most warm women I have had the pleasure to meet, and I always felt that Freya had touched her heart in some way.  She would come and sit next to me on the fold away bed, sometimes holding my hand when she had to discuss something difficult,  like the bone marrow procedure.  The Rheumatology Consultant and the SHO that we often saw would come into our cubicle and talk us through their thought processes and plans, and I always felt that it was a collaborative process.

Amidst all the researching and fighting of the past 4 months, I have always felt that there was still a reason to be thankful.  I have to remember that I took Freya to A&E at the very first sign of a temperature; she had no other symptoms of KD and would continue to hide those from the doctors for a number of days.  It seemed like a long time in comparison to other KD stories, but most of those didn’t take their child to the hospital on day 1.  There was so much luck involved in Freya’s story – had she been a week older, I would have begun to treat her fever with paracetamol at home instead of taking her to the hospital.  Had she developed a fever one day later, on the Monday, I would have taken her to my GP and probably would have been sent home with “just a virus”.  Had the Immunologist not asked for an opinion from Rheumatology, Freya would never have had that second echo.  Had Freya not developed that angry rash on day 11, the Rheumatologist would never have been called.  It’s like the doctor said, in the end “Freya showed [them] the way.”

Last week, at Freya’s follow-up appointment in Sheffield, I thanked the doctor for everything they did for her.  I said that whilst I would probably always wonder if there was anything they could have done to diagnose her sooner and whether her heart would have suffered if they had, I had nothing but gratitude for them for the way they aggressively treated the condition once they reached a conclusion.  They were as relentless as the disease itself, and had entered a battle that they were determined to win.  And they did win.  Because even though Freya has suffered complications with an unknown longevity, they stopped Kawasaki Disease in its tracks when it was intent on causing further damage.  I know that they did more than was necessary, carrying out daily echocardiograms even though the likelihood of a day-to-day change was small.   They often said that they thought some of what they were doing was “overkill”, but I know they did that because they wanted a positive outcome for Freya.

It has taken me a long time to stop trying to find a door to place my blame at.  That blame landed well and truly at my own front door in the beginning, and there is still part of me that wishes I could have made her strong enough to have avoided this disease.  I often feel like I failed her in that respect.  I don’t feel the need to blame so much anymore, and whilst it has been a slow process I do believe I have found my peace with this situation sooner than most.  Don’t get me wrong, I still believe there is more to be done to make the diagnosis of Kawasaki Disease more successful.  I still think it is a travesty that this disease can have such a huge impact on a child, and yet parents and medical professionals alike are unaware of its existence.  I still think that it is appalling that mums like me have to spend so much time and energy reading documents that may as well be written in Swahili, and trying to separate the wheat from the chaff of internet searches.  Let’s face it, mums have plenty of other things to be getting on with.  But, I do feel that I have a stronger appreciation of the medical profession and as a result I understand why our children are so difficult to diagnose.  So what brought about this catharsis?  It was in fact a letter that was delivered to me via Ed Miliband MP, whom I wrote to airing my frustrations as a parent of a child with this rare disease.

When I received the letter yesterday, I was anxious to pour over its contents.  On first reading, however, I was angered and frustrated.  He had written to the Secretary of State for Health (Jeremy Hunt) asking for his response to my concerns, and enclosed a copy of the response he received.  It appeared to be, on the face of it, a justification of my perceived failings of the NHS, “…challenge for GPs..”, “…6000 rare diseases..”, “…not possible to be an expert in all of them..” In all honesty, when I read the letter yesterday I was all fired up to write a pretty curt reply.  And then I remembered something that an old manager once told me; if you are angry, don’t respond, sleep on it and respond later.  So I read the letter again a few hours later and it was like some of the clouds that occupy my mind began to disperse.

What the Rt Hon Jeremy Hunt MP was actually saying was that we have to appreciate that with over 6000 rare diseases to consider, and particularly with Kawasaki Disease affecting only around 8 in 100,000 children each year, it is unreasonable to expect every GP to immediately diagnose a child presenting symptoms.  You think that doctors are trained to handle every medical eventuality, but it is like anything in life – no-one knows everything about everything.  Why, even in my field I would say that customer service advisors cannot be expected to answer every customer query that reaches their ear.  All we can do is equip them with the tools to provide the best possible solution.  Sure, there will be common questions that arise that even the worst advisor could answer with their eyes closed, but for every 100 simple questions that come along, there will always be one, a little more obscure, that needs closer attention, perhaps even a second opinion.  Why couldn’t I apply the same common sense to the medical profession?  I guess because they are dealing in life and death, so you expect more from them to know everything.  But how many times have you been to the doctor when they’ve had to use Google, or refer to a medical handbook?  Doctors are incredibly clever people; they are not geniuses with greater memory capacity than you or I.

The MP went on to provide information about the steps that have been taken to support medical practitioners in the diagnosis of rare diseases.  One of the resources he drew my attention to is a number of videos that have been produced by Health Education England, “in collaboration with the Department of Health, rare disease charities, patient groups and clinicians”.  One of the videos is called “Rare Disease: The GP’s Role” and has helped to give me some grounding in this whole thing (you can view it at https://www.genomicseducation.hee.nhs.uk/resources/videos).  The NHS appreciate the challenges surrounding the diagnosis and treatment of rare diseases, like Kawasaki’s.  Yes, more needs to be done to inject funds into research projects, but who am I to say that Kawasaki Disease needs more attention than the other 5999 rare diseases in the mix?  I know I run the risk of sparking political debate about government spending and the like, but I don’t claim to understand that in any detail, and I am sure that the solution isn’t quite as easy as we all might think.  And if being a Doctor was easy, we could all be one, couldn’t we?  I already feel like I’ve qualified with a degree in KD!

The videos that I watched with interest discuss genomics, a discipline in medicine concerned with DNA mapping (in simple terms!) There are currently 11 genomic medicine centres in the UK who are part of the 100,000 Genomes Project whose role it is to participate in “sequencing 100000 DNA codes of patients, leading to better, earlier diagnosis and personalised care, for cancer, rare diseases and infectious diseases” (https://www.genomicsengland.co.uk).  I was pleased to see Imperial College London on that list, and proud that my family has played our part in the study to understand the genetic determinants of Kawasaki Disease.  I was pleased to hear, in the letter, that “The Rare Diseases Advisory Group…is considering a proposed way forward on an approach to earlier diagnosis, which will be developed with the medical Royal Colleges.”  So, work is being undertaken behind the scenes to try to deal with this disease more effectively, and a Professor leading one of these research projects in London recently told me himself that they were about to publish a paper which proposes a diagnostic test for Kawasaki Disease.

As well as this, the MP went on to tell me about the NICE Guidelines that are in place for the treatment of children presenting persistent fevers (“Feverish illness in children: Assessment and initial management in children younger than 5 years”) which “provides advice in relation to the diagnosis of Kawasaki Disease, including details of the main symptoms…”  This information can be found on the website https://www.nice.org.uk.  I followed the flowcharts on the website and it was like watching Freya’s journey played out in a diagram! The doctors weren’t pissing in the wind (pardon the expression), guessing, or coming up with miraculous discoveries.  They were following an NHS guideline for diagnosis and treatment, and by a process of elimination were finally able to reach a diagnosis for my daughter.  Given the red herrings that Freya threw at them along the way, I am not surprised at all by the route they took, and I have a new appreciation for the process that they had to go through.  Perhaps this is common sense, and you are all saying “Well, what did you expect?!” But you know, I really didn’t expect anything other than to take my child to the hospital, for them to look at her, tell me what was wrong, treat her and send her home.  Well.  100% better. And not broken.

So what has changed for  me?  I no longer feel the need to blame anyone for what Freya went through, or for her current condition.   I feel that my respect for the medical profession in general has been restored; they can’t know everything, especially about a disease that many have probably not seen before.  I feel like I have reached a better understanding of the process that all the medical staff went through to diagnose my child.  Don’t get me wrong, there were a few ‘blips’ along the way, and I have no doubt that there will have been something that could have been done differently somewhere, but I don’t think I believe that a different course of action would have changed anything for us anymore.  Freya would still have Kawasaki Disease, she would still have received treatment and sadly because of the nature of the disease she was one of those children who was unfortunate enough to suffer heart involvement.

And what remains the same?  My determination to raise awareness of Kawasaki Disease.  My wish to see increased funding for research into the disease, whether from the NHS or public fundraising.  My commitment to use whatever spare time I have to produce information about the disease where I think it will help other parents in my position.  My passion to research and share what I can, and my appetite to challenge our Doctors to answer the unanswered questions that parents have about the disease and related issues.

I never thought I would use the word ‘lucky’ to describe our experience, but do you know what? We are lucky.  Because so many other stories have not panned out the way ours did.  Yes, Freya was critically ill, and yes she has a heart condition that will continue to worry us for an undeterminable amount of time.  I will continue to suffer anxiety when Freya screams out in her sleep like she did at 6am this morning and my first thought was is this it? And I will always wish that Kawasaki’s could have passed Freya by.  But right now, a mum is holding her child’s hand as they are put to sleep for heart surgery.  A GP is sending a child home because all they have is a virus.  A child is being pumped full of drugs that are not going to work, and will allow the disease to hide a little longer.  Right now, somewhere in the world, a parent is grieving the loss of their child to Kawasaki Disease.

 

Freya’s Story (15)

That night (Tuesday 23rd June) I began researching in earnest.  I had spent a fair amount of time and energy researching Kawasaki Disease and certain medical terms up to that point, but mainly to educate myself on the subject so I understood what the doctors were referring to.  One of the doctors commended me on the interest I had shown and the research I had undertaken, adding that they never came into our room without all the information to hand as they knew that I would ask questions like “What were her last CRP levels?”  CRP (C-reactive protein) is a marker of inflammation in the body that is measured from a blood sample.  I had some understanding about CRP levels from a period of time when I had an illness and my CRP levels were elevated – normal is below 7, and mine had risen to over 140.  When Freya was at her worst, the CRP levels in her blood had reached over 300.  She wouldn’t be allowed to leave the hospital until her CRP was within normal levels, so that, along with other markers was to be the success criteria for her release. Freya’s blood would be taken every day, mostly from a heel prick, but sometimes from a vein.

This time my research was different.  Not only did I need to understand the issues surrounding Freya’s heart, but I needed to find out what Freya’s chances were.  So I took to the internet and searched for anything related to Kawasaki’s and acquired heart disease.  I felt desperate to find a story that resembled Freya’s that had a happy ending.  I can recall the feeling of despair building as I searched for positive stories with no luck.  I did come across one story that seemed to resemble Freya’s pretty closely – same age, same delay in diagnosis, almost identical issues with the heart.  Before I reached the end I realised that what I was reading was an autopsy report.  I decided to stop looking.  I found a couple of support groups on Facebook and joined, spending hours reading story after story from parents across the globe.  I posted questions and a brief synopsis of our situation and although I couldn’t find a case exactly like ours, I got a lot of comfort from reading stories about babies who had been through what Freya had and had grown up to be happy and healthy.

During my internet search, I came across the Kawasaki Disease research centre at Seattle Children’s Hospital in the United States.  I found a ‘contact us’ section and decided to send a message to ask if they had any stories like ours that they could share.  You see, I had asked my consultant if they had any, but they were not aware of another baby as young as Freya that they could use as a comparison.  In fairness, it’s difficult to provide any real prognosis of recovery with a disease that has only been around for 40 years or so as that there have been no long-term studies that can help to predict the future.  To my surprise, I received an e-mail from the Dr who leads the Seattle KD research centre.   This was his response:

“…I’m sorry about the delayed diagnosis and treatment.  But it sounds like she’s getting good care now.  Unfortunately most children under 6 months old develop narrowing or stenosis in their coronaries.  It’s going to be scary for a while.  I’m sure you are giving twice daily injections of Lovenox. But there is certainly hope.  I follow 3 children with huge aneurysms under 6 months which resolved completely within 2 years.  I have many others living pretty much normal lives with some exercise restrictions and on aspirin and blood thinners…So far, we have had no deaths and only one heart attack, who totally recovered and is now doing well.”  Wednesday 24th June, 00:19

It was scant information, but it was positive news, and a direct response was more than I could have hoped for.  I read it a couple of times.  “I am sure you are giving twice daily injections of Lovenox”…no we weren’t.  I replied to say that the use of Lovenox hadn’t been mentioned and explained the treatment Freya had received.  He replied almost immediately;

“Standard of care in US and UK for large aneurysm includes anti-coagulation or a blood thinner in addition to aspirin.  That means Lovenox …or low dose heparin if the aneurysm is very large near 4mm in a baby your daughter’s age.  Sometimes warfarin is used but doesn’t work well in small babies…”  Wednesday 24th June, 00:38

Freya’s aneurysm measured 5.2mm.  My heart was racing.  I felt the panic building; my daughter wasn’t receiving the treatment necessary to prevent a heart attack!  The Dr asked a few more questions about where we were being cared for, etc, and then responded again to tell me that I needed to phone our cardiologist first thing in the morning.  He said that I could use his name, that they could look him up.  He told me that in the US they do not use the 8mm+ criteria for a giant aneurysm in small babies stating that it is relative to body size, and that “…5mm in a 2 month old needs anticoagulation asap.”

That night I wrote in my journal:

“…I do feel that this disease will take you from the world one day.  Hopefully not until you are 90 years old, and the rest of us are long gone.  Because of that I want to make sure we make the most of whatever life we have been given, and if we end up having 50 wonderful years together then what an amazing life we will have had…I don’t know why my eye is drawn to the stained glass window of the chapel just outside the window to our room, but God bless you tonight my little Peanut.  I pray He keeps you safe.”

IMG_5381

The following day (Thursday 25th June, Day 26) we would travel back to Leeds for Freya’s first cardiology follow up since we received the worst news.  The echo showed no change from the previous scans which was good news, but I left with an uneasy feeling in my gut. I’d tried to ascertain what the likelihood was of an undesirable outcome.  I remember asking if they could tell me how many young babies they had treated with Kawasaki Disease and how many of those had a favourable outcome.  The Dr said that they didn’t really see babies as young as Freya with KD.  So I asked if they could forget the KD and tell me how many babies they had treated with coronary aneurysms, and she replied that babies don’t get coronary aneurysms unless they have had KD.  I wasn’t going to get any answers, because there weren’t any answers to give.  Even the nurse that travelled with us in the ambulance said that it wasn’t a very reassuring visit.  The journey back to Sheffield was a sombre one.  I saw one of the on-duty Dr’s that evening and she said I was entitled to ask for a second opinion, so that was what I resolved to do.

…”I don’t want to cause a fuss or seem like I am being over-dramatic, but I think I need to trust my gut instincts on this one.  I’m not losing you without a fight my darling.”

Later that night I received an unexpected e-mail from the Dr in America, stating his concern for the advice we were receiving.  He had also asked the opinion of a KD specialist in Boston and she agreed.  They mentioned something called a ‘z score’ which is apparently used to ascertain the correct treatment for coronary aneurysms in a small baby.  The Dr had calculated Freya’s based on the information that I gave him.  He said that Freya’s ‘z score’ was 16.9, and was well above the range at which US specialists would treat with Lovenox.  He attached a paper from Great Ormond Street which details the UK advice for the treatment of Kawasaki Disease, and pointed out the section where our own advice states to treat a z score higher than 7 with warfarin; “Take the paper with you and good luck.”  I felt sick.  That niggling feeling in my gut that everyone tells me is me being negative, was right on the money.  Later I would receive another e-mail.  The Seattle Dr had queried the rheumatologist whose name was mentioned on the Great Ormond Street paper and asked her to recommend a Cardiology Consultant for Freya.  24 hours later he sent me the name of a Professor of Cardiology in the South West of England who had been involved in the writing of the GOSH paper.  I searched online for his contact details, and immediately e-mailed him.  I could feel the adrenalin rushing through my veins.  I had information, expert medical opinions from people who have dealt with more cases than we have here in the UK, I had the official guidelines for treatment of Freya’s condition, and now I had a lead to someone who was within our reach who might just know more about this disease than anyone.  All I had to do was sit back and hope for a reply.