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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Unlucky for some?

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

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

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

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

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

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

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

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

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

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

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

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

I love you Peanut.

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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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Return to Cannon Hall Farm

Dear Freya

I must admit the title of this blog post made me smile a little!  Sounds like one of those old stories I read when I was a child, usually involving four or five intrepid children and a dog off on some treacherous adventure.  Today was not quite so dramatic as any of those stories, but it was an adventure for us nonetheless, and I must start by saying thank you for making today wonderful, when it might have been otherwise.

Today we visited a local visitor attraction, Cannon Hall Farm.  It is about a 45-minute drive from home, in Barnsley, South Yorkshire, and is home to lots of animals that you can get close to. At this time of year, in the Spring, the farm gives birth to new life in the form of tiny piglets and little lambs and if you are lucky with the timing you can get to hold a guinea pig and stroke a fluffy rabbit too.  We arrived just after 11am, and you were excited about seeing the ‘aminals’ as soon as we got out of the car.  I’ve visited many times over the years, but this was the first time you were seeing it with your eyes, and it was magical.  I love seeing life through a child’s eyes.  Everything is new and wondrous and those big blue eyes sparkled with excitement at every new thing that you encountered.

We paid our £5 entry – there’s a special offer on at the moment and you were free anyway, so it was most definitely good value for money!  We bought a bag of animal feed too, so you could interact with the animals.  We were presented with stickers to wear to show that we had paid our way, but even though I confidently placed mine on my coat, you were reluctant to allow me to do the same with yours.  See, you have developed this fear of stickers, presumably a mixture of ECG memories and the steady flow of sticker offerings at every medical appointment, but whatever it is those stickers cause you to recoil, and back away like I have some kind of venomous creature in my hands.  I managed to convince you within about half an hour that you had to wear your sticker if you wanted to feed the animals – you may only be 22 months old, but you are  bright as a button and you were really keen to send the food down those chutes.  I took the small victory, and punched the air in my mind.

You were mesmerised by the Meerkats and ran up and down the enclosure as they chased you from the other side of the glass.  Seems they sensed your fun and wanted to join in.  And then we went on to the breeding barns, led by a very eager you running off ahead, giggling “What’s in there?!” as you ran. You adored the pigs and their piglets, and toddled excitedly from barn to barn shouting “Bye bye!” to the animals as we left them.  You weren’t too keen on the tractor cleaning out one of the stalls, but we moved on quickly enough for you to forget about the monstrous roar of it’s engine as it shovelled away all the muck and straw.  You gambled on the blue ferret to win the race, but sadly it had no intention of leaving its starting block and lay there curled up in the warm Spring sunshine, along with green and yellow, while red took the title with ease.  We walked past the birds on our way to the sheep enclosure, where you delighted in the sounds of the lambs, remarkably like new born babies crying for their mummies, until one of the ewes decided to utter a deep, groaning “baaaa” and you ran screaming “Help me, help me mummy” until I scooped you up and headed back towards the centre of the farm!  As we passed the birds again on the way back, I saw the network of tunnels in the children’s play area out of the corner of my eye.  Silly really, that it was the sight of those tunnels that drew an inward gasp and brought a tear to my eye, but I quickly brushed the thought away as we headed back to see the meerkats again – they would bring back the smile in an instant.

We had pretty much exhausted your attention span, and the use of your little legs, by around 1pm so we popped into the restaurant for some lunch.  I ordered a sandwich and chips for us to share and we took a seat in the corner where I could pen you in at the end of a row of benches!  You like to wander, and I needed you to stay put and eat some dinner.  On the table next to us were an elderly ‘couple’ (I could tell by their conversation that they were not married, and later found out that they were both widowed friends).  Both the lady and the gentleman were showing an interest in you.  I mean, at risk of sounding sycophantic, you do draw attention to yourself. Those huge ocean blue eyes, and your little curls, along with this way that you carry yourself that defies your 22-months on this Earth, seem to gather interest everywhere we go.  You are quite simply captivating.  I don’t know what it is that you have, but I guess although it sounds like far too mature a word to use to describe you, I would say that what you have is charisma.  Oodles and oodles of charisma.  Whilst I am most definitely your number one fan, you seem to be gathering a following wherever you go.

Anyway, to get back to the point of our ‘adventure’.  After a number of musings and comments from the couple next to me, the gentleman remarked at how clever you are – he had been observing you when you were let down from the table and noticed how considered every move you made was.  He said you seemed to be well aware of everything and everyone around you, and he said “She’ll go far that one.” He said you seem ‘lively’ and I laughed and said “Oh yes! She keeps me on my toes!” and the lady replied, “Yes, but better to have them like that,  than in hospital poorly.”  I could have brushed over that comment, but instead I remarked that you had indeed had your fair share of that, and of course this sparked some interest in your situation.  “In fact, the last time we were here was the day before she became sick.”

And there it was; the reason for our visit.  We have not been back to Cannon Hall Farm since that day before our lives were changed forever.  Saturday 30th May 2015; you were 7 weeks old and it was our first outing as a family.  I don’t know why we haven’t returned to visit the farm really.  There is absolutely no suggestion that your illness was linked to the farm, and whilst the cause is unknown so it could never be completely ruled out, it is more likely to be just a coincidence and bad timing.  I think I just didn’t want to undo the memory of that perfect moment in our history.  I mentioned earlier that the sight of those tunnels was the only thing that triggered an emotional response (though I had been working hard to suppress the emotions I could feel crushing my chest all the morning).  I guess it’s because we spent a while there whilst your brother and sister got themselves lost in the maze of tunnels.  I think I fed you as we sat on a bench and watched the children playing in the early Summer sunshine, and congratulated myself on this perfect life we had created.  And up to that moment, Peanut, it really had been perfect.  You were perfect.

That day in 2015 at Cannon Hall Farm marked the end of perfect for us, or so it seemed.  It feels so unfair that things changed so early on in your life, before we had a chance to make any more perfect memories.  I guess I’ve wanted to preserve the memory of that day, hold on to the precious moment that is captured in sepia images in my mind.  It has been a huge flag on a timeline, marking the change from perfection to imperfection, and giving me something to grieve, to pine for.  But do you want to know something, Freya? What I realised today is that there was really no need for me to hold that moment on a pedestal as something to be quite so treasured.  Because, with the exception of 6 long weeks in a hospital cubicle and all the trauma of 2015, every single moment with you is something to be treasured.  Yes, I felt sadness wash over me when I remembered how carefree and wonderful our lives seemed on that day at the farm, but when I sat and thought about it I realised that without what happened to you, without Kawasaki Disease and it’s gifts (yes, you detect sarcasm) then we wouldn’t have experienced the wonder of today.  Without Kawasaki Disease, I would have returned to work after a year on maternity leave, and our moments would have been reduced to the same precious hours on weekends that your siblings were lucky to grab in between the mountains of washing and ironing.  Because of Kawasaki Disease, you now have a broken heart and an uncertain future.  But because of Kawasaki Disease, I now have a heart that is capable of feeling more than it ever had before.  It showed me a glimpse of what it might feel to lose someone I loved, and made sure that I will never take you, or anyone else I love, for granted.

Of course, I wish that we had never been introduced to this insidious disease.  I wish that you hadn’t been so little and therefore susceptible to the very worst that the disease had to offer.  I wish we didn’t have to face a future with hope, but no guarantees.  Our lives would have been so different without it, but I am not sure if our lives would have been better.  Is that wrong? I don’t know.  I just know that what happened to you in 2015 created an opportunity for me to be a better version of the me I had once been.

Today, as I saw the wave of sadness cross that lady’s eyes when I told her that you had a damaged heart, it struck me at how terribly unfortunate your circumstances must seem to someone on the outside, even if, as the lady said, “You wouldn’t know to look at her!”  But to us, this is just our normal.  Today I realised that what we have is not an altered you or a changed you.  The you we had for 7 weeks at the start of your life with us was wonderful, but it was not a patch on what was yet to come.  You have given us 22 months of extraordinary, and I cannot even begin to put into words how grateful I am to you for today, and all the days just like it, both in our past and in our future.

Whatever it is that radiates from within your little body, it takes my breath away and  I love you with all my heart.

An Uncertain Future

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

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

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

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

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

So what are my fears for Freya’s future?  

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

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

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

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

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

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

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

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

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

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

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

Amy said:

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

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

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

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

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On why I might once have been angry…

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

I hope you will continue to support us.

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

Then and now…

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Forgotten Fears

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

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

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

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

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

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

July to August 2015                               32% reduction

August to September 2015                 10% increase (anomaly?)

September to November 2015           6% reduction

November ’15 to May 2016                 13% reduction

May to September 2016                       33% reduction

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

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

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

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

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

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

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

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

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

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

 

A Day of Ups and Downs…

…or that time the Cardiologist ruined our day

Dear Freya

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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