Kicking Kawasaki

Dear Freya,

So, we finally acknowledged that angiogram shaped elephant in the room, and on Wednesday 21st February 2018, almost 2 years to the very day that the ‘A’ word was first mentioned to us, you underwent cardiac catheterisation at Leeds General Infirmary.  And you are still here!  Woohoo! I could feel all the fear and worry that has burdened my shoulders for the last few years lift off and evaporate in the waiting room the moment I saw the Cardiologist’s face as he walked towards us, smiling.

Let me tell you about the day so you can read about it when you are old enough to understand what you have been through.  Although I feel that somehow instinctively you know, because you have not stopped squealing “Woohoo!” since we got home!

Our day started at 6am, when I woke to my alarm with a sinking feeling in the pit of my stomach.  ‘A’ day had arrived; a day I had been dreading for so long. Before I woke you, I called the hospital ward to check they were expecting you and that they still had an available bed.  “Yes, we are expecting Freya,” said the nurse, and my heart sank.  I climbed back into bed with Daddy, and cried quietly as I let it sink in.  I know it had to happen, but I was as disappointed that it was going ahead as I had been at it being cancelled a few weeks before.  It was indeed a necessary evil; we wanted you to have it, but we didn’t want you to have it.  But now we just needed to get on with it, and have hope.  I put the angel wing earrings in that our friend had bought me, and although I don’t think I am a religious person, and I am not a Catholic, I will admit I popped the rosary beads that I had bought in The Vatican into my bag!

I woke you up, and after you’d sleepily been to the toilet, I wrapped you up in your dressing gown and put you in the car.  Our overnight things had been packed the night before, as we were told to expect to stay at the hospital.  You weren’t allowed anything to eat or drink after midnight the night before, so we were surprised when your first words weren’t, “I need some brukfust!” like they usually are!  I guess the whole routine was different to you.  As usual, and despite being rudely awakened at 6am, you were happily chattering away in the back of the car, telling us about everything you could see on the journey up, and repeatedly saying, “We love you, don’t we Mummy,” which just made my heart ache.  It struck me as strange that you used the word ‘we’ and I got this odd feeling that somehow we weren’t alone, us three, on that journey.

When we arrived in Leeds, Daddy dropped us off at the doors and went to park the car so that we wouldn’t be late.  Although we visit this hospital regularly for your follow up appointments, I felt my stomach lurch as we walked in.  Usually your appointments are in the Outpatient’s Department, but this time we would be going back to Ward L51 in the Clarendon Wing – the ward where you were admitted when the Children’s Hospital had you transferred up there for specialist cardiac care.  We entered the ward, and right ahead of us was the high dependency unit that you had shared with post-op babies with vertical scars down their chests. I had been so focussed on the procedure itself that I hadn’t considered how it would feel to be back there.

We were ushered into the playroom, where within minutes the Doctor had arrived.  He was in blue scrubs and he spoke in a calm and kind way that put me immediately at ease.  He explained that you would be his first patient of the day, so you would be going down for your procedure at around 9am (it was 7:30am when we arrived).  He gave me a brief outline of what would happen, and explained the risks in such a way that I didn’t feel afraid of them anymore.  He said that both me and your Daddy would be able to go into the room when you were given the general anaesthetic, which made me really happy as the last time you were put to sleep I had to go through it on my own. With every sentence the Doctor spoke, I began to feel stronger.  Daddy then arrived, and the Doctor took the time to repeat the information to him, and I signed the consent forms. He said that if everything went as they expected it to, we should be able to go home that afternoon. He also said that the day needed to be as fun as possible, and that both the hospital staff and us were to concentrate on that, for your sake.  He said, “This doesn’t have to be frightening for Freya, she just needs to think that this is normal.”  I didn’t think it was possible for us to do that, but as you will read, we all pulled it off!

Shortly after the Doctor left, a nurse arrived to show us to your bed.  As we were headed towards your bay, the nurse said we just needed to nip into a side room to check your weight and height.  I looked around the room, trying to figure out why it felt familiar.  It looked smaller than I remember it.  More cramped.  And yet the last time you and I were in there, it was full of medical professionals and the ambulance crew were poised in the corridor, waiting to see if you would be taken back to Sheffield.  They were sent away.  I can remember Dr Brown (your Cardiologist) sitting by the side of the bed preparing to do an echocardiogram.  I don’t remember any of the other faces, just that they looked intrigued, serious and concerned.  And I remember I was in such a heightened state of panic as you had got so hot in your car seat strapped to the ambulance gurney and your fever still hadn’t abated after 17 days.  For a moment, it was like I was watching it all again, like a kind of out-of-body experience, but then you needed help to get your back straight against the wall so the nurse could record your height, and I snapped back into the moment.

Within minutes of arriving at your bed, two members of staff came to see you.  One was the Anaesthetist and I can’t remember who the other lady was.  I was surprised at how they addressed you to explain to you what was going to happen.  I remember thinking, “She’s only 2! She can’t understand what you’re saying!” but I realise that they were building trust and forming a relationship so that the experience would be easier for you.  They said they would see you soon for a little sleep, and just in case you didn’t respond well to the breathing mask they put some numbing cream on the back of both hands with a clear plaster to cover them.  Although you giggled at the coldness of the cream, you didn’t like it much and we had to tell you we had the cream on too to convince you to get the other hand done.   They wrapped your hands up in bandages so you looked like a boxer, and the probe on your finger lit up so you looked like ET (you won’t know who that is, but we will watch the movie one day).

When they were done, the Play Nurse arrived!  Her name was Gemma and she was wearing a bright yellow T-shirt.  She had a big bear with her, I can’t remember his name.  He had a cannula in one hand, and a clear plaster on the other, and the nurse was carrying a green mask.  She gave you the bear to play with, talked to you about the cannula and let you play with it, and she gave you the breathing mask to play with too.  Me and Daddy played with the mask as well – we put it over our mouths and pretended to fall asleep, snoring!  You thought that was funny, and you helped put the teddy to sleep too.  After you’d had a chance to play with the teddy, and Gemma had talked to you about your procedure, she asked what you would like to do next.  You chose to do some painting, so all three of us sat at the little table and painted together.  As the time passed by, it didn’t feel like we were waiting for anything at all.  It just felt like you, me and Daddy spending some lovely time together!  Personally, I found the painting quite relaxing, and I couldn’t believe it was 9am already when another nurse came through to collect you.  They were ready for you.

You didn’t want to stop the painting, but we explained that there was somewhere exciting we needed to go, and that you were going to have a little sleep.  You could come back to do some more painting later.  So off you skipped down the corridors, stopping every now and again to look back and ask us which direction we should go in.  We went down in the lift, and walked for ages until we finally reached the Cardiac Catheter Lab, which was through some red double doors.  We went into a room on the left, and could see the Doctor and another member of staff behind some glass.  It looked like the kind of operating theatre that you see on TV hospital dramas, and Daddy and I were surprised by how many people were in the room.   I was expecting to lay you down on the bed like the last time, but instead they asked me to sit down in a chair, with you on my knee facing away from the bed.  I guess when you last went under general anaesthetic you were just a tiny baby, and you were so sick that you really didn’t put up a fight at all.  This time, with you being so much more aware, I guess this was the better way.  When they held the mask over your face, you didn’t protest.  How clever that they introduced you to it earlier on, so now you just accepted it as normal!  They asked you to breathe, and to try and blow up the balloon that was attached to part of the tube.  You started to feel heavier in my arms, and at one point you started to throw yourself about a little.  The anaesthetist said not to worry, that it was the general anaesthetic working and was quite normal.  And then you were asleep, which I knew because you started snoring really loudly!  I’ve never heard you snore like that before, Pickle!  I laid you on the bed, and they said we could give you a kiss goodbye before we had to leave.  Daddy leaned in and gave you a kiss, and then I did too.  I’ll admit that is when I began to cry.  We had been so strong for you, it wouldn’t do any harm to let it out now that you couldn’t see us.  They told us to come back in 30-40 minutes, and we had a feeling it could be one of the longest 40 minutes of our lives.

Daddy and I went down to the hospital entrance to get a coffee and some breakfast.  Daddy said he felt bad getting a bacon sandwich when you hadn’t eaten, but I told him it was okay because you didn’t know!  Standing in the Costa queue, I turned to your Dad to ask if he was okay, and he was really upset.  We don’t often see your Daddy cry, but seeing your child get put to sleep is not a nice thing to go through, and it really hit him.  We sat and ate, and I just felt an overwhelming sense of sadness over our situation.  This had never been in any of our plans, and here we were drinking coffee, in a hospital surrounded by Surgeons and Doctors and Nurses on their breaks, while somewhere upstairs your life was in someone else’s hands. I felt helpless, and heartbroken that you were having to go through this at all.

After what felt like 58 million hours, we decided to come back to see if your procedure was over.  My heart jumped up into my throat every time those red double doors opened, but it wasn’t long before your Doctor came through them and walked towards us with a smile and a nod that seemed to stop time.  He sat down beside me and your Daddy and said that the procedure had gone really well, and then he said it.  Your arteries look “pristine”.  That is the exact word he used: pristine.  Now if you don’t know what that means, and for the purpose of illustrating just how amazing this news is, let me share this with you here:


Unspoilt, perfect, immaculate.  Just like you.

We couldn’t believe what we were hearing.  Daddy said “It’s what we wanted, it’s what we wanted!” as the Doctor told us that everything looked normal, and they could see no signs of any damage at all.  Normal!  Do you hear that!  He said he would keep you on the daily aspirin, but I said that your Cardiologist was keen to stop it if there was no need to continue.  He agreed that there was no real evidence either way, so the decision would be as much led by us as by the hospital, and we decided we would wait to see what Dr Brown would like to do.  I said I had not expected to hear such amazing news, and wondered if the fact that you were so young at diagnosis had anything to do with the outcome.  He agreed that babies, and young children, are remarkable healers. He said that you would need to go back in the future for a stress echo, but for now everything was just fine!  You were waking up, and a nurse would come out to collect us when they were ready.

When the nurse came out and lead us through the red doors again, we could hear you screaming.  We entered the recovery room, and there you were, confused and upset and fighting with the staff who were trying to apply pressure to the entry wound to stem the bleeding.  The more you struggled, the harder you made it for them, and you were writhing about on the gurney trying to get away.  It must have been frightening for you, and I am guessing it might have been hurting too.  I’ll admit I wasn’t prepared for the recovery.  I hadn’t expected to find you like that, and both me and Daddy went right into action to try to help you.  You were calling for us, and we were reassuring you that we were there, but you were in such a state.  You asked for a cuddle, so Daddy held you on his knee in a chair whilst the nurse tried to keep the pressure on your groin area, where the catheter tube had been inserted.  Then you started calling for me, and Daddy passed you to me, but you were still very unsettled.  And then the nurse said they had to get you laid down again as you were bleeding out, and I looked down and saw the blood stain spreading across my cream jumper (my favourite jumper might I add!)  Then I panicked, and I cried, and I said “It’s my fault, it’s my fault, I wasn’t holding her right!”, and they told me not to worry, that they expected the bleeding, and it wasn’t the first time it had happened.  In fact they apologised to me for the state of my clothes, but I really didn’t care, sweetheart, a jumper can be replaced.  The Doctor who performed the procedure came back in to help, and eventually the bleeding must have stopped because they called for a porter to come and take you back to the ward.  It was hard to see you so upset, and us not be able to offer much comfort.  Most of the journey back to the ward, you were calm, but then we would stop to open a door, or call a lift, and you would start crying again.  I wonder what was going through your mind.  I reminded you of the CBBC Get Well Soon video we watched where Dr Ranj was singing about a bed with wheels, and you smiled a little at that.

We were soon up on the ward again.  They told us you wouldn’t be able to eat for a while, particularly as vomiting can be a side effect of the anaesthetic, but that you may want to have some sips of water because your throat may be sore.  We had to get you to lie flat for a number of hours – how do you manage that with a toddler! But actually you were pretty whacked out by the experience, and you did lay there with your favourite teddy, the teddy that had stayed with you in the theatre when they did the angiogram.  I budged you over on the bed, and snuggled up beside you so we could read books, which I held above our heads until my arms ached!  You kept sending Daddy back and forth to the library to fetch new books, and he kept going because it was the least we could do to keep you happy after what you had been through.   My advice to a parent going through this with their child to pick lightweight books! You wouldn’t have wanted to hold the book yourself – you were too busy sucking your thumb on one hand, and fiddling with Pink Teddy’s ears with the other! You had a probe attached to your foot to monitor your heart rate and oxygen levels.  I watched the numbers on the machine, and noticed the stark contrast between the monitor then, and the constantly alarming monitors of 2015.  My, how far you have come!


I guess it was around 11am by the time we had got back to the ward, and you still hadn’t eaten anything since 6pm the night before.  You were getting hungry, but it was too early to eat so we told you that your lunch was cooking, and distracted you with more books.  Whenever a nurse walked by you would shout out, “Doctor! I’m starving!”  Soon enough it was lunchtime, and being a paediatric cardiac ward the offering was jacket potato and baked beans, the suggestion of which was met with a rather indignant response from you, and a request of “Have you got any cheese sandwiches?”  Despite not usually serving that, the staff went off and came back with a plate of cheese sandwiches, a yoghurt and a banana that they had made to look like a dolphin.  They all made such a fuss of you, and all the staff said you were very cute.  You even made a friend – 8 year old Emily who was waiting for open heart surgery.  Once you had eaten and you were able to sit up, it was like you hadn’t been through anything at all.  You were back to being your usual self, a cheeky little chatterbox, charming everyone that you met!  The rest of the afternoon was pretty uneventful, and because you could sit up you were able to open the gift that ‘La La Lis’ had sent with us, with treats and activities inside, which kept you busy for a while.

The Doctor came to see us again in the afternoon, to say that he was happy for the nurses to discharge you when they were ready.  You had recovered well, and the only sign of what you had been through was the nasty bruise in your groin that w

ill probably be there for some time.  He repeated that everything had gone really well, and that your heart looked perfect.  He mentioned the stress echo again, and we asked how old you would need to be for that – he said at least 8 years old.  I asked about CT Calcium testing as I know that research has shown that the presence of calcium in the arteries can indicate risk of further complications, e.g. heart disease.  This test is recommended 10 years post-diagnosis, as calcium can take years to build up.  The Doctor did mention the ‘D’ word (discharge), but I quickly jumped in to say that I would expect you to be followed up for life, and he didn’t argue.


And so, around 5pm, after a long but strangely fun day, we were told we could go home.  I asked Daddy to go and get the car, and I would meet him around the front of the hospital; there was something we had to do…

On Friday 18th June 2015, as we were leaving the Cardiac unit to return to the Children’s Hospital following your week on the HDU, I made a visit to the hospital chapel.  Your Auntie had mentioned that she wanted to leave a prayer before we left, but we were discharged before she got the chance.  So I decided to do it for her.  The day before that we had been told there was a chance we could lose you, so I knew what I wanted to write on my prayer slip.  Dear God, Prove them wrong.

On the 22nd February 2016, your Cardiologist told us that they wanted to do an angiogram, and the start of a worrying two years began.  That night, I wrote a blog, where I shared a photograph of the prayer slip along with these words:

“Somewhere in there…there is a tiny voice of hope…She sounds how I imagine Freya to sound in a year or so.  I see those eyes through the fog too.  They are looking at me, huge and wide, and they are saying “I’m going to be ok, Mummy.”  This kid is miraculous.  She is made of something stronger than you and I were made of.  Maybe, just maybe, she is going to prove them all wrong…”

And on the 21st February 2018, after we had faced the elephant in the room, and survived, you and I went to the chapel together.  I took a blank prayer slip from the table and wrote what I needed to say.  And then we walked up to the altar and both of us said out loud, “Thank you.”  And we meant it from the bottom of our perfect hearts.

We also left a note on the prayer tree for the research team

Our journey with Kawasaki Disease is not over, we have merely reached a pretty big break in the road.  The distance between the follow up checks will become greater, perhaps 5-yearly from now, and we will have a number of years to wait before the further tests (stress ECG/echo, Calcium CT test and perfusion scanning) are undertaken to ascertain the risk of long-term effects on your heart.  Although the remodelling process can be excessive and lead to narrowing (stenosis) at a later stage, we remain cautiously optimistic for your future.  We know you are at greater risk of heart disease and will do our best to protect it, as the doctors have advised; healthy eating, no smoking, and get you into an activity now that will keep you physically fit into adulthood – cycling, running, swimming or dancing.  Oh yes, there will be lots of dancing!

I e-mailed a number of the Kawasaki Disease specialists who have been on your KD journey with you from afar, including Professor Jane Burns (World Leading Kawasaki Disease Specialist).  Whilst they all celebrated our news, the cautionary notes were still clear.  “Make sure she has ongoing and long term follow up and that no one is tempted to discharge her!” was the note from one of the specialists, and another said “While I hope the later narrowing will not occur – it is important to be aware that it’s possible and arrange long term follow up.”  Jane didn’t need to say any of that, as she knows me too well, and knows that I will never stop advocating for you and the disease, so this is how she received your wonderful news:


You can count on it Jane!  Whether we like it or not, we are part of a wider family now, and we will never stop campaigning to raise awareness of this disease.  Freya’s 3rd birthday party fundraiser is on the horizon, and we can only hope that whatever amount we raise can make a difference to the research programme.  For now though, our family can focus on that ‘normal life’, although I am not sure I am satisfied with ‘normal’.  No, for us I want an extraordinary life, and the hope that we have gained from seeing that sometimes you can believe in miracles might help us do just that.




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!


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.


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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The Gift that Keeps on Giving…

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

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

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

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

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


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

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

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

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

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

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

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

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

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

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

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

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


Because life, at best, is bitter-sweet

It has been a little over 8 months since Freya was diagnosed with Kawasaki Disease following her hospital admittance on the 31st May 2015.  She was 7-weeks old.  Infants under 1 year are hit particularly hard by the illness, and are at greater risk of heart involvement.  The magic window for treatment that has the highest odds for reducing the risk of coronary artery dilation and/or aneurysms is considered to be 10 days.  Freya was diagnosed on day 13 when an echocardiogram showed significant dilation to her tiny coronary arteries, and she was transferred by ambulance to a specialist paediatric cardiac high dependency unit (HDU) in another hospital.

Since her diagnosis, Freya has had numerous ECG and echocardiograms performed at follow up appointments. Some of those appointments have shown no change, but two of those appointments showed some remarkable improvement that managed to incite a “Wow!” from her Cardiologist.  In September we were advised that her right coronary artery (RCA) had improved to within normal parameters (around 2mm), leaving just the left coronary artery (LCA) and left anterior descending (LAD) with reduced, but significant, dilation.  The aortic valve had ceased leaking some time before.

I remember at the time asking the cardiologist (who wasn’t our usual one due to a mix up in dates), whether the swift improvement was cause for concern in relation to longer-term issues, such as stenosis (narrowing of the coronary arteries), but he had said that there was no evidence to suggest that was the case.  And so I made my peace (kind of) with the condition of Freya’s heart, and accepted that things were improving and that her prognosis looked good, regardless of how scary it all still was.

Her last appointment in our local hospital was a disaster.  We were kept waiting for nearly 3 hours with no explanation before finally being seen by a consultant who felt under-qualified to deal with Freya’s case.  Nevertheless, she performed an echo, the results of which suggested there had been no change since the previous echo.  No change is good news in these cases – at least things hadn’t got any worse.  Had she told me there was some improvement, however, I would not have believed it, and immediately contacted the original hospital to request that we be moved back under the previous cardiologist.  Sometimes convenience isn’t the best option; I would travel 10 times the distance for Freya to be treated by someone that I trust and respect.

Today found us back in Leeds, back with the Cardiologist that first treated Freya, and has taken pretty good care of her ever since.  She is the same Cardiologist who took me in a room and explained the risks of Freya’s condition back in June; the same Cardiologist who I went to battle with over not giving Freya Warfarin despite the treatment protocols suggesting that was what she should have.  She made a strong case, one strong enough for me to put my trust in her.  It was the hardest decision I have ever had to make, but it appears to have been the right one.  Freya has been thriving since her diagnosis, she appears to show no adverse affects, no night terrors, no evidence of joint pain, none of the things I see mentioned in many social media forums.

So come on then, tell us the news….

Ok, so today Freya had an ECG and an echo.  As usual, the ECG showed that heart function is all good.  During the echo, the Doctor remarked that Freya’s heart function was “Beautiful!”  She measured the arteries at their widest points and remarked that she could see two aneurysms on the RCA.  I was surprised by this, as previously we had been advised that the RCA had normalised, and I wondered aloud whether as the artery had remodeled, could any ‘true’ aneurysms now be more noticeable (Freya’s arteries were significantly dilated all the way along, without the typical balloon shape aneurysm you expect to see when you use the ‘a’ word).  The doctor went in to look again, and could not get the same image, or any image to show the aneurysms she believed she had seen.  Perhaps it was a bad angle?  Maybe Freya wriggled too much for her to get the same view?  I’m not sure, but the Doctor concluded that all three coronary arteries (the RCA, LCA and LAD) appeared to have remodeled to within normal parameters!  Wow! That’s cause for celebration!

During the acute stage of the illness, we were advised not to expect to see any improvement at all until Freya was at least one year old.  To have seen her arteries reduce to near-normal levels in just 8 months is remarkable!  She truly is a miracle.  Or maybe this is just the case with tiny babies?  Sure, they are hit hard by the illness and more of their little hearts suffer, but their organs are still developing and perhaps they just compensate for the damage and work harder to repair it?  Who knows? Research is still very much in it’s infancy (thus my passion for fundraising for this cause).

We sat down, and I asked my husband to dress Freya whilst I asked the questions I had prepared before I went in.  These are the questions I wanted to get answers for:-

  • What do you consider to be the current risks?
  • What are the exploratory steps we can expect for Freya?
  • When would those next steps take place, i.e. at what age?
  • What is the view on Reye’s Syndrome, and what are the suggestions in relation to aspirin during the Chicken Pox vaccination period?

Before I had the chance to ask my questions, the Doctor started to answer the first.  She exclaimed that the improvement was indeed remarkable, but that the speed at which Freya’s arteries had remodeled was unexpected and gave her cause for concern.  She knew that I understand that an echo can only see approximately 3mm of the length of the coronaries, and explained that she was concerned about what was happening beyond what she could see.  She aired the same concern that I had raised back in the Summer when I told another Cardiologist that I was worried that the swift remodeling could result in higher risk of stenosis.  Sometimes I wonder if I am bloody psychic!  She said that she would be happier if she could take a closer look at Freya’s heart to satisfy herself that the whole picture was as good as what she could see.

So, what’s the good news?

Ok, so the good news is that Freya’s heart has shown remarkable improvement, basically remodeling itself to where it should be, or would have been under normal circumstances.  If the next steps prove that the bigger picture is the same, the Cardiologist feels she would be comfortable to stop the aspirin.  That means that Freya would continue to be monitored into adulthood, that further tests (exercise stress testing, MRI, etc) would continue to take place during that period of monitoring, but that she would be treatment free.  Fantastic! That would be almost ‘normal’.  Wouldn’t it?  Wouldn’t that just be normal?

Yeah, so that’s sweet isn’t it?  What’s there to feel bitter about?

Alright, alright, I know what you are thinking!  For months I’ve suffered mentally over the prognosis for Freya and the fear that comes with having a child with a heart condition.  I should be unconditionally happy, right?  It’s just that the way they want to find out what is going on with the bigger picture is to carry out an angiogram.

“An angiogram is a test that’s used to find out more about your heart. It can help to show if blood vessels called coronary arteries, which supply blood to your heart, are narrowed or blocked. If they are, it can show where and how severely they are affected. It can also see how well your heart is pumping blood.”

Whilst a CT or MRI are preferable in a child as young as Freya because they are non-invasive procedures, an angiogram is considered (by our Doctor) superior to a cardiac CT or MRI scan in that it provides a clearer image where the arteries concerned are very small, like in a young child.  The procedure involves inserting a catheter (a thin tube) into an artery in the groin or wrist and guiding the catheter to the coronary arteries where dye is inserted and a number of x-ray images are then taken.  Freya would need to be sedated, likely to go under general anaesthetic.  Not only is it invasive, but it is a procedure where the risks are greater the younger the child.

It is considered pretty standard practice to carry out an angiogram, or other exploratory test, a year after diagnosis with KD.  However, an angiogram is not performed in very young children without careful consideration.  We were told that the catheter that is used is kind of a one size fits all; they will use the same sized tube to enter Freya’s tiny arteries that they would use on an adult.  We were also advised that young babies’ arteries are quite sticky, and have been known to grasp onto the catheter,  causing the artery to spasm.  This can cause a heart attack.  The Cardiologist said that the hospital will be reluctant to carry out the procedure on Freya at such a young age (she will be 14 months old when she is a year post-diagnosis), but said that she feels that Freya is a special case and that it is important that this step be taken.  She says she will convince them to do the procedure.  I replied, “Thank you…I think.  I mean, on the one hand you are keen to move things forward and a clear result could end with us stopping the aspirin.  But you are expediting a potentially risky procedure and I am not sure how I feel about that.”

But I do trust her.  I think.  Didn’t stop me from e-mailing the world’s leading Kawasaki Disease expert for her opinion though…

And that’s where I am right now.  I’ve written this down to provide an update, but mainly to try and work out how I feel about it all.  I’m not sure, I feel quite numb.  So I am just going to dump all the crappy thoughts that are running through my head right now, and hopefully the answers will come to me over the coming days or weeks.  Excuse the rantings of a confused mind…

  • Here we go again, as soon as I let my guard down and ignore my instincts, someone goes and throws a curveball!
  • I knew this was going to happen!
  • I knew that there was a chance that the fast improvement was too good to be true.
  • What if I lose her after all?
  • What if I can’t bear to watch her go under general anaesthetic again? It was like watching her die on that table.
  • What if she doesn’t wake up from the anaesthetic?
  • What if she has a heart attack?
  • What if she dies?
  • What if she dies because of the procedure and they tell me everything was ok anyway?
  • What if there are signs of stenosis? They can’t operate on her this young anyway.
  • What if there are no signs of stenosis, but it happens when they’re not looking and she dies?
  • What if we say no to the procedure? How much potential danger would we be putting her  in?

I think that’s about all I can figure out from the fog right now.  Somewhere in there, though, there is a tiny voice of hope.  ‘Ant’ is doing her best to drown her out, but I hear her.  She sounds how I imagine Freya to sound in a year or so.  I see those eyes through the fog too.  They are looking at me, huge and wide, and they are saying “I’m going to be ok, Mummy.”  This kid is miraculous.  She is made of something stronger than you and I were made of.  Maybe, just maybe, she is going to prove them all wrong.  Maybe, (and I don’t consider myself religious so this just came as a huge surprise to me, hitting me like a juggernaut and reducing me to sobs), just maybe, someone heard my prayer….