kawasakidisease

Forever changed…?

Posted on by Bluemama

I often find myself wondering if we could cheat this thing, Peanut.  You know, change the past by putting some distance between you and the place where all of this happened.  Get in the car and drive, just drive, as far away from here as we can get on a tank of diesel.  Far away enough to escape the darkness that threatens to steal the sunshine from our lives.  

 These past few days I have been taunted by intrusive thoughts.  I have a ‘friend’ called Ant* who used to hang around a lot, whispering negative thoughts in my ear whenever I tried to look on the bright side.  It seems these days she’s been inviting a whole bunch of pals round to join in, and they fill every space in thought that I have. Last night, as I laid you down to sleep, I was hit by a thought; “What if this is the last time I put you to bed?”  I don’t even know where it came from, I mean I don’t feel like you are in the danger zone anymore. Do I? To be honest, I don’t really know what I know anymore.  Before the appointment in Doncaster last week, I had reached a point where I felt things were stable, and that we were out of the woods.  Having to relive it all again to a new Doctor has knocked me sideways, and I feel confused and unsure and alone again.

These days my mind is never quiet.  I have to keep myself busy just to drown out the noise.  Today as we drove home from the Children’s Hospital my mind started to wander and I thought about how much I love you.  It’s crazy just how much, sweetheart.  It’s not even healthy.  I mean, of course loving you is a good thing, but when every thought of how much I love you is met with a thought about how devastated I would be if anything were to happen to you… God, I wish I could just love you without the fear.

As that last thought entered my head, I actually shouted aloud to the cloudless grey sky, “DON’T YOU DARE DO IT, DON’T YOU DARE!”  I’m not even sure if I know who I was daring.  God I guess.  I was challenging him, without any clue how I was going to punish him if he chose to ignore my threat.  What could I do?  What power do I have to protect you?  I just wish someone could tell me that things are going to work out, that you will recover completely and this nightmare will become a distant memory.  I don’t want to feel sad every time I think about what you might sound like when you talk, or when you will crawl, or what you will be when you grow up.  I want to be able to think about your future without being checked by the voice in my head that warns me not to tempt fate.

I was jolted out of my thoughts by the beeping of a car horn.  To the impatient lady at the wheel of the Mini behind me: We don’t care much about your beeping horn.  How nice to live in a world that you feel you can rush through.  I personally have no desire to rush through this life.  This precious life.  So beep away, lady, and know that you will not move me today or any other day. Nor shall you induce me to hurry for your convenience, for right now I am sharing the air in my Vauxhall Astra with the most precious thing I have in my life.

There is a quote that does the rounds from time to time about being kind to others, because you never know what troubles people are dealing with.  I will try to remember that, and be more tolerant of others.  Mini lady achieved a whole car’s advantage on me.  Was it worth it to steal a precious moment?

I feel like I am in purgatory.  Stuck in hellish flashbacks of the worst time of our lives, but without the Utopian promise of a life no longer touched by Kawasaki Disease.  I’m not sure how much sadness I can take.  I had you sweet and perfect and just as I made you, for seven weeks.  Seven weeks.  Now tears are streaming down my cheeks as I silently scream, “WHY?!!”  Why, Peanut? Why you? Why us?  Why did they have to ruin everything?  I am afraid that I will never be the same again.

On Friday I will have my first counselling session to try to help me come to terms with what has passed, and what might be in our future.  I have so much I need to say, out loud.  I’m frightened that once I open the floodgates to the emotions inside me, that I will not be able to close them back up.  And I’ll only have an hour.

*reference earlier blogs – ANT = Automatic Negative Thoughts.  “Ant” is the personification of my negative thoughts and feelings

A new perspective

Posted on by Bluemama

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

 

Are we ok?

Posted on by Bluemama

Two of the most common questions I get asked these days are, “Are you ok?” and “Is Freya ok now?”  I’m not sure I know how to answer either of those things, so usually the first question gets a, “Yeah, I’m good thanks.” and the second receives a “Erm, yeah, kind of” response because I’m genuinely not sure what I should say.

Let me try to address the second question.  Is Freya ok?  She is clinically well.  She has no symptoms of any illness, no inflammation, no fevers, no rashes and no pain (as far as I know; she’s too little to tell me).  She feeds well, is growing nicely and has taken to weaning like someone who has just come off a diet.  She rarely cries, and when she does complain it is usually to tell us that she has a dirty nappy, is hungry or tired.  She goes to bed between 5pm and 6pm every evening without exception, and does not make a murmur until she wakes around 7am the following morning.  And when she wakes, she will lay in her cot looking around and cooing to herself quite happily until we greet her with the customary “Good Morning!” and are met with the brightest of smiles which only disappears when she is devouring her bottle!  During her waking hours, she is happy to lie on the floor or on her play mat, or sit in her chair, where she will play until she’s tired or hungry.  If she gets tired she might whimper a little but often falls asleep right where she is playing.  She has a smile for everyone, and she makes it possible for me to smile despite all of our recent troubles.

That said, she has a heart condition as a result of her illness.  Kawasaki Disease is a vasculitis; a response in the body which causes inflammation of every blood vessel, with a particular penchant for the heart.  The coronary arteries supply blood to the heart muscle.  These arteries will become involved in 25% of Kawasaki cases.  Treatment within a 10-day window significantly reduces that risk to around 6%, but for those of you that have followed our story you will know that Freya wasn’t diagnosed until day 13 of her illness and her heart suffered.  There are 3 main arteries that supply the heart; the left coronary artery (LCA), the right coronary artery (RCA) and the left anterior descending (LAD).  At the time of her diagnosis, all 3 of Freya’s coronary arteries had dilated (expanded) to over 5 times the normal size for a baby her age.   The risks associated with that are rupture, (where the artery wall gives out through weakness as a result of the expansion), and thrombosis (where the blood is allowed to clot within this new expanse).  At Freya’s age it was considered inoperable; a graft of the vein in the ankle is taken to replace the coronary arteries, but in an infant the success rate is poor.  Plus, as the baby grows they run the risk of outgrowing the new arteries, resulting in further coronary complications.  All they can do is administer the treatment (in Freya’s case, 2 doses of intravenous immunoglobulin, high dose aspirin, steroids and a single dose of intravenous Infliximab) to stop the inflammation in its tracks, and then regularly monitor the arteries to ensure there  is no further growth.  And hope.  Once that is under control, a child will usually be prescribed with a blood-thinning (anti-platelet) dose of aspirin and, in some cases, an anti-clotting (anticoagulant) agent like Warfarin.  Both of those, in slightly different ways, work to keep the blood flowing through the arteries, and to stop the blood from clotting.  Thrombosis would result in a heart attack.

Freya has shown remarkable progress, receiving a few “Wow!”‘s along the way, and even a message from Professor Jane Burns (a KD specialist in the States) saying “I expect she is safe and fixing the problem on her own. Well done, Freya!”  The arteries are now only 3 times the size that they should be, and we hope that they will continue to regress to normal size over the coming months/years, or that Freya will grow into them as she grows.  There is a risk as the arteries start to repair themselves, that the scar tissue causes stenosis; a narrowing of the arteries.  This can be treated with surgery in later life (you may have heard of a stent), but if stenosis occurs it can severely affect the function of the heart and is potentially fatal.  We do not yet understand if this is a risk for Freya, nor do we know what the long-term impact this disease will have on her, due to the absence of long-term studies.

And then we have the question of her immunity.  Her immune system was temporarily suppressed by certain medication, such as the steroids and Infliximab.  However, the immunoglobulin contains antibodies from thousands of blood donations and should cover Freya from most routine illnesses (and a few more weird and wonderful ones) for around 11 months from receiving it.  She has had no routine immunisations, and the advice (well, the most reliable advice anyway) that I have received is that she can have the killed vaccines approximately 3 months post-treatment (for Freya that is the 8th October), and any live ones once the IVIG is out of her system (approximately 11 months after treatment; June 2016 in Freya’s case).  For patients taking regular aspirin, there is a risk of developing Reyes’ Syndrome if the patient comes into contact with Chicken Pox or Influenza.  Although the risk of developing Reyes’ is rare, and is more often associated with patients on high doses of aspirin, it is a very nasty illness which affects the brain and can be fatal.  As a result, it has been recommended that Freya be given the Chicken Pox and Flu vaccines, but that she would have to stop taking aspirin for a period of time surrounding the immunisation being administered. That is probably my biggest cause for concern at the moment; how the hell do I protect her from Chicken Pox and Flu until next Summer?

Is she ok? Yeah, I guess she is.  She is not considered to be in immediate danger (from a cardiology perspective).  She will continue to take her daily dose of aspirin, probably for life, and will be monitored into adulthood.  You really wouldn’t know that there was any problem with her heart at all to look at her – she really is a wonderful baby.  But Kawasaki Disease is full of surprises and unknowns and shades of grey, so we just have to take each wonderful day at a time and hope.

Am I ok?  That depends on when you ask me, I guess! Or the circumstances in which I am asked.  I’m not sure that everyone that asks really wants to hear more than a “Yeah, I’m good thanks!” with that Invisalign smile that I’ve perfected over the years. But do you know what, I really am ok.  Ok isn’t brilliant, is it?  Ok isn’t absobloodylutely fabulous. Ok is just ok.  Not good, not bad.  Maybe up, maybe down.  Some people will worry that I may be suffering from depression, particularly since I have some history.  This isn’t that.  I get up every morning with a spring in my step.  I get a shower, make myself presentable to face the world.  I do the school runs, I iron, cook and clean; I function on a day-to-day basis just like any other mum would.  I spend most of my day playing and chatting with Freya (I mean who wouldn’t want to do that?  You’ve seen her, right?!)  We go out when we are organised, we stay home when we’re not.  Apart from staying away from playgroups and nurseries, we are not inhibited in any way.

With the avoidance of the aforementioned child-filled establishments comes a sense of loneliness.  I love spending time with Freya, but my world is mostly just me and her.  We rattle around here and there while I try to make the best of the hours we have together, but I miss having other people to talk to.  I’m an incredibly sociable person, I thrive in a group, and I stumble when I am left alone for too long with my thoughts.  That’s why I try to get out and about, rather than staying indoors where I have too much time to think.  I don’t know how best to describe how I feel.  I feel kind of numb, like every moment I spend out there in the world with Freya is surreal, like I can’t quite believe we are doing normal everyday things where other normal people go to do normal everyday things.  Maybe that is just it.  Maybe this is normal, but that it seems that Freya’s illness has been part of our lives forever so I don’t remember what normal really is.  And then I’m scared to let ‘normal’ in, because what if I forget?  What if I start to take things for granted? What if Kawabloodysaki’s comes up behind us and shouts “Boo!” just when we are least expecting it?

A friend recently suggested I might need some help to get through this.  At the time, I dismissed the concern.  I knew it wasn’t normal to cry as much as I did (still do), or to feel the anxiety that I had (still have), but there wasn’t anything normal about this situation either was there?  So I plodded along, dealing with it in my own way with a watchful eye out for signs that this was becoming something bigger than I could handle.  With each day, and with every bit of good news we receive about Freya, I expected the pain to start to dwindle.  If anything, it has grown.  It’s like now that we are out of the worst, and the adrenalin that kept me going for all those weeks has stopped coursing through my veins, things have finally started to sink in.  What do I feel the most?  Anger, sadness and disbelief.  I am angry that Kawasaki Disease chose my daughter.  I am sad that Kawasaki Disease stole some of what should have been the best of times with my new baby girl.  And I still feel utter disbelief that this happened to us.

I have an appointment with my GP on Wednesday 7th October at 9:30am.  It’s time to get some help.

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The Reality of Kawasaki Disease

Posted on by Bluemama

When the phone rang at around 9:30pm last night and my mother-in-law was on the end of the line telling me to tune into ITV because an episode of Doc Martin was showing a case of Kawasaki Disease, my stomach did a little somersault.  A prime-time TV programme mentioning KD? I could hardly believe it. I switched over a little too late so set the programme to record on ITV+1.  As more and more comments and messages came into the various Kawasaki support group pages on Social Media sites, including Freya’s Story, the adrenalin was pumping.  You could almost feel a collective buzz as parents of children who have suffered at the hands of Kawasaki’s from all over the UK tuned in to witness KD getting a voice.

I couldn’t help but feel a sense of pride in being part of this campaign for raised awareness.  I cannot claim credit for the inclusion in this TV programme, or the coverage in The Daily Mirror last week, but I would like to think that somewhere along the way there will be a link with Freya’s Story and the small bit I have done to help raise Kawasaki’s profile.  I can claim credit of over 300 signatures on the Kawasaki Support Group UK petition (change.org/kawasaki), and I know that a lot of the 500+ people who now follow Freya’s Story on Facebook and Twitter will not have heard about Kawasaki Disease before;

“This evenings episode of Doc Martin includes a case of Kawasaki disease, wouldn’t have known what it was without this page and good coverage to help raise awareness.” (Posted by Facebook user on http://www.facebook.co/freyasstory)

“I saw the Mirror, I have read nearly all of Freya’s story, I have cried for you and smiled at the lovely pictures. It’s amazing though what you have done, I knew you were strong and raising the awareness is fantastic.” (Friend, and follower of Freya’s Story on Facebook)

But I’m not doing this for praise or thanks.  I’m doing it because I don’t want another parent to have to go through what we did, what so many parents have, because of a lack of knowledge and understanding of this disease.

So, what did I really think of last night’s episode of ITV’s Doc Martin?  Initially, I was excited and amazed that Kawasaki Disease had even managed to make in into the public eye in such a dramatic way.  One of the nation’s most prolific TV channels mentioning something much of the country has never even heard about! Fantastic!  And then I watched it.

What were the positives?  Well, you get to understand the symptoms of the disease.  The little girl has a temperature, a red tongue and a rash on her body.  They allude to the fact that she may have peeling skin on her hands when a little boy remonstrates about having to hold the sick child’s hand on a school outing.  The child eventually collapses, and the show’s ‘hero’ appears.  After one look at the child, he makes a rather pompous statement, which went something like “this child hasn’t been eating sweets, she has Kawasaki Disease”.  And just like that, after seconds of seeing the child, he makes his diagnosis and drives the child at high-speed (she may die within 30 minutes apparently) to get her the treatment she needs to avoid the risks of a coronary artery aneurysm.  Amazingly the medication is waiting for them on arrival at the hospital and the child receives the treatment.  In terms of the programme, this whole thing took 3 minutes.  Remarkable.

So why the long face? It didn’t take long for the celebratory feelings to wear off once I let what I had just watched sink in.  Part of me still wants to believe that no matter how the issue was covered, any coverage is good for raising awareness of a disease that most haven’t heard about.  But (you could see that but coming couldn’t you!) it was scripted in such a glib and blasé manner that I wouldn’t be surprised if the message was lost on most viewers.  I suspect many won’t even remember the name of it today, let alone the symptoms.  And if they did remember it, I do not believe that anyone who hasn’t experienced KD first hand will have one iota of an idea about how serious, or how scary this disease is.

I know that the ITV would argue that Doc Martin is a medical comedy drama series; it does not profess to deliver messages in a serious manner (that can be left for the likes of Panorama and Casualty who gave KD some airtime about 20 years ago, in the mid-90’s).  We can be grateful that such a rare disease has even made it on the ITV radar, I guess.  But when you’ve watched your child battle with this disease, you can’t help but be left with a sour taste in your mouth after watching the episode.  In fairness to the programme, however, I am not a regular viewer so am unfamiliar with the usual format and scripting, and I know that they are likely to come under attack from victims of all manner of illnesses that they cover; they’re never going to please everyone.

If you watched the programme, and you think that Kawasaki Disease is a funny sounding illness, maybe even fictitious, then please know that it is very real, and is the leading cause of acquired heart disease in children.  If you think that it is easy to diagnose on first look at a few symptoms, please know that is not the case and often the symptoms come and go, disappearing just when you needed them to be seen.  In fact in many cases, not all the symptoms are present.  If you agree with Doc Martin’s assessment of the child’s parent being negligent by not noticing the signs, then please know that most medical professionals in our country have never heard of Kawasaki Disease, and many mis-diagnose the illness, sometimes with catastrophic results.  If you think that the treatment is readily available, please know that intravenous immunoglobulin is in short supply, and it is expensive.  It’s a product derived from human blood; we’ve probably all seen the marketing campaigns from various blood donation schemes recently – there is not a bottomless pit full of blood that we can all tap into at any time.  And if you think that all cases of Kawasaki Disease have a happy ending, you’ve just got to be a part of the support groups on social media sites to know that isn’t the case.  There are children currently fighting for their lives, and many that have lost their fight altogether.

I am disappointed in the programme, not only for its flippant handling of the illness, and think they could have done more to make it clear that this is a sinister disease that needs some real focus.  Many programmes include messages in the credits to support people who may have been a victim to the contents of an episode.  I think the ITV could have at the very least put up a public message about Kawasaki Disease, making it clear that this is very real, and even just supplying a link to NHS Choices for further information.

In order to reach a diagnosis, I had to watch a 7-week old Freya battle constant temperatures above 39 degrees for over 2 weeks.  Her heart rate was dangerously high.  She was resuscitated in a high dependency unit after her body started to shut down.  She had cannulae in every site possible in her tiny body, delivering a cocktail of various potent antibiotics, ibuprofen and paracetamol, and she was being fed through a tube.   She had blood taken every day for a period of 6 weeks, sometimes twice a day.  She had an MRI scan, ultrasounds, x-rays, ECG’s and echocardiograms.  She had 2 lumbar punctures, one under general anaesthetic which they performed at the same time as performing a bone marrow aspiration to test for Leukaemia.  She received a blood transfusion when her haemoglobin levels dropped dangerously low.  And she has received intravenous immunoglobulin – a product that is derived from thousands of blood donors – as well as a drug that shuts off the protein that is instrumental in the body’s fight against cancerous cells.  She is not even 6 months old yet.

Sounds shocking to read it all like that, doesn’t it? See all the tests that she underwent, just to try and work out what this illness was.  Kawasaki Disease threatened to take her life.  If the Rheumatology Consultant that came so see her on Day 12 of her illness had not suggested performing another echo “just as a precaution” the damage to her heart would have remained undetected, and her coronary arteries would have continued to dilate further and further until perhaps they wouldn’t have been able to stretch any more.  That means rupture.  Rupture means sudden, unavoidable, unpreventable death.

Now think about what you just read about Freya, and imagine now that her case is not the worst by far.  Yes she had a severe presentation of the disease, and her life was in very grave danger.  But as I type there are parents fighting against doctors won’t entertain a KD diagnosis, parents whose precious children have been in hospital for weeks struggling to fight off the disease, parents who still co-sleep with their children for fear of losing them, and worst of all there are parents sitting at home wondering how they will ever recover from the death of their child.

I cry every day.  I cry when I hold Freya in my arms and am overwhelmed with how much I love her.  I cry because I can’t shake the images in my head of how close we came to losing her.  I cry when I see other people happy, without a care in the world and no appreciation of just how lucky they are.  I cry because I am still heartbroken that this happened to my baby girl, before we had even had a chance to get to know her properly as Freya, just Freya.  I cry because I am so scared about the future.  I cry because I daren’t take Freya to baby groups for fear of her contracting chicken pox or the flu (as they can develop into another rare and potentially fatal disease, Reyes’ Syndrome) and I cry from guilt that she is missing out on a massive part of her development as a result.  When I visit the children’s ward at the local hospital where this all started, I have panic attacks.  Even this week, when I thought I’d be ok and felt no anxiety approaching the hospital, I was suddenly struck by uncontrollable sobs, and I couldn’t breathe.  I cry because I am grieving for the loss of my child’s health – she was perfect when she was born; Kawasaki stole that from me far too soon.  I cry because I feel the pain of every parent who is going through this, or something similar.  And I cry with frustration that more isn’t being done to improve the advice and support that is currently being offered by the medical profession.  Chances are I cry a lot because I sleep for less than 4 hours a night; not because I have a young baby – she sleeps through the night – but because I am permanently researching the illness or related issues, or communicating with parents and doctors on the other side of the world.  I cry because I am devastated, because I am sad, because I am frightened, because I am exhausted, because I am frustrated.  I cry because I cannot forget the journey we have been on, and am haunted by the playback of the last few months, which is constantly on repeat.

A quest for peace

Posted on by Bluemama

Today I accepted that I might need some support to move on from this thing. I don’t mean forget, I mean how could I ever forget what we’ve been through? And I would never want to shut KD out of my life completely, because I feel like I can be of use somehow in raising awareness of this elusive disease.  But I do need to work out how to process it all.

I have spent the last 3 months or so immersed in the world of Kawasaki Disease; at least the world that I can access through medical journals and the Internet, tirelessly reading, learning and challenging. And that’s all great, but it only serves to distract me from the fact that I am still a mother, and I’m a mother with a fracture in my heart.

I am surviving on very little sleep. I stay up until the early hours of the morning, sometimes researching, other times just thinking. And thinking. And thinking.  I stare at photographs of Freya and I feel grief, and then I feel guilt because she’s still here isn’t she. I’m not sure how long it takes for sleep to take me when I finally do go to bed, it feels like I am laid awake all night. I hear every sound that Freya makes, from the familiar sound of her breathing or when she does that little grunt under her breath, to all the unfamiliar sounds that strike fear into my very soul; what was that? Did something happen??  And then there’s the silence, when I lie awake holding my breath, waiting for her to make a sound, any sound, to reassure me that she’s still ok. For those seconds I lie frozen, wanting to reach across and touch her to be sure, but too afraid. It’s irrational I know. The risks she faced at the beginning of this journey are no longer a source of constant worry. I know every new mum experiences degrees  of worry about losing their child. I don’t have the monopoly on those fears. But I feel sick with anxiety from the thought of losing her.  Thoughts are not facts, Joanne.

I think on the whole I have handled this thing pretty well. In all honesty I’m surprised I haven’t cracked up completely. I think there must be a strength inside all of us that we never find out until we are faced with something big.  But you can’t be strong always. I thought that by writing my blog I would exorcise a few ghosts, but it doesn’t seem to be working as I had expected.  Maybe because the blog doesn’t answer me, so my words are left hanging in the air like a dense fog.

Most days I’m good. I’m not a neurotic parent, watching over Freya 24/7.  I don’t put her up in her room for naps like I did with my other children, allowing her to fall asleep on her playmat when she’s tired herself out. To be fair, though, she’s so content she can be playing one minute and sleeping the next. But I do take her up to bed in the evening, and she sleeps in her cot in our room from 6pm until 7am the following morning, later if I let her.  Of course either me or my husband tiptoe upstairs at regular intervals throughout the evening, but that’s normal. I get on with the housework, or I hang up the rubber gloves and play; depends how clingy Freya is feeling at the time. We get out and about, I enjoy little trips out with her.  We went to York on the train the other day to see my niece who has just started at University there. I am my most content when I’m mooching about, pushing my little Peanut in her pram. But every now and again, more frequently these days, I am aware of a dark shadow following me around and I am transported back in time to those weeks in the hospital. Every day I relive a part of our journey and the tears fall silently down my cheeks, sometimes completely unprompted, other times triggered by a tender moment with Freya, a conversation, something on TV.  Tonight I watched a programme where a 3-month old baby was put to sleep for surgery, and the tears appeared within a nano-second. It’s still so raw.

I thought I could handle it alone. Of course I was going to be feeling like I do after this ordeal. But I thought that every appointment that brought good news would take the pain away. But it doesn’t and I so want to celebrate Freya’s triumphs over Kawasaki Disease.  I need to package the last few months up into an easily accessible box and store it away in the corner of my mind where the unpleasant boyfriends and school bullies live. But right now I’m on a ride that I can’t get off. I have to find some peace with KD; need to accept that no amount of hating this disease and the uncertainty and lack of concrete information that goes with it is going to change the fact that it bulldozed its way into our perfect little life and left its mark on my precious girl.

Kawasaki and I are not friends, nor will we ever be. All I can hope is that over the coming weeks, with whatever avenue I choose to help me get out of this kawahell, I can focus more and more on a bright future than on the dark and dismal past of the last few months. Here’s to a day when I might look at Freya without being haunted by memories I wish I could forget.

A productive day and a Superbaby

Posted on by Bluemama

So today has been a Kawasaki day; I’ve spent most of it either conducting research, replying to e-mails or producing information slides.  Not quite what you can call getting back to normal.  But needs must, and if I didn’t spend as much time reading and learning as I do, then I would be at the mercy of our medical profession who are great, but can’t seem to agree on the right treatment or after-care for this disease.  It makes me so angry that I don’t feel I can rely on the information I get because at every turn there is something that will contradict the last piece of advice you were given.  Just when you feel like you are getting somewhere, another nugget will drop in your lap and you’re right back to square one.

So, what did I achieve today?  Well I’ve been battling for a while over how to catch Freya up with the immunisation schedule.  Because she was only 7 weeks old when she went into hospital and has since received treatment that affects the immune system, she has not had any of the 8, 12 or 16 week vaccinations.  About a month after Freya was discharged from the Children’s Hospital, we were advised that she could be given any of the killed vaccines, but no live ones.  I was not given any indication of what was live and what was killed, so had to turn to the internet yet again.  I called our Health Visitor to ask for clarity and they confirmed that all of the routine 8, 12 and 16 week vaccinations are killed (or inactive) with the exception of Rotavirus which is live.  The flu spray is also a live vaccine, but this isn’t routinely offered to young babies; the flu vaccination is inactive however, should Freya be required to have it.

I still felt uncomfortable for some reason.  I guess when you’ve been through an experience like this and realise that the medical professionals don’t always know everything they need to, you are a bit less likely to take the first bit of advice you get.  So I debated and procrastinated until Freya’s next Rheumatology follow-up appointment.  I raised the subject again and our consultant said she was fine to get caught up with all of them, as all the standard vaccines are inactive.  I corrected the doctor and advised him that Rotavirus is now included and is indeed a live vaccine.  You might gasp in horror that a doctor didn’t know this simple piece of information, but what I have learned from our experience in the hospital is that when you specialise in a certain area you really can only be expected to retain the knowledge for that specialism.  Outside of that there are other specialists for different disciplines, and if that fails…well, there’s always Google!

The decision was reached that Freya could have any inactive vaccines immediately, but that we needed to wait for 3 months post-steroid treatment before giving her any live vaccines due to her immune response being suppressed.  I asked about whether Freya should have the chicken pox vaccine because of the risk of a pretty nasty (and potentially fatal) illness called Reyes’ Syndrome which has been known to occur when a child taking aspirin contracts chicken pox (and influenza, I’ve found out today).  The response was that the chicken pox vaccine is only given to children considered at high risk of catching chicken pox.  I replied that whilst Freya might not be high risk for catching chicken pox, she was at risk of developing Reyes’ Syndrome as a result of being on aspirin.   Right now I can protect her from chicken pox to a certain extent, but next year if she has to go into childcare how do I protect her then?  The doctor agreed to take the question away and come back to me with a response.  That response came by way of a letter, confirming that following consultation with Immunology it was agreed that it would be prudent for Freya to have the chicken pox vaccine, it should not be given sooner than 3 months post-KD treatment and we could go ahead and book her in with our GP.  I also e-mailed one of the consultants that looked after Freya in Leeds, who responded to say that there had been little research in this area and as such I should make a judgement call.  Still I felt uneasy.

Since then I have had many conversations with the Health Visitor, and spent countless hours online reading everything I could about immune response and immunisations.  Some of what I read scared me; for instance, a child on aspirin should not have the chicken pox vaccine due to the risk of Reyes’ Syndrome.  Good job I didn’t take Freya right on down to the Dr’s surgery to inject a little dose of the pox into her.  In fairness, I had already decided that I would catch up the inactive vaccines, but leave the live ones until next year when the need for them would be more pertinent.  I had even made the appointment, but after receiving the “make a judgement call” e-mail, I decided to do just that and cancelled the appointment.  One thing I have learned about this KD journey is to trust your gut instincts.  Mine were pretty spot on all through this illness, and even when I tried to convince myself I was wrong, I was over-reacting, my gut won out.  I just couldn’t shake an uneasiness that would creep up on me every time I thought about vaccinations.  I needed to do more research.

As well as research, I’ve kind of gone on a one-woman crusade for Kawasaki Research this last week or so!  I’ve bombarded people on Facebook with please to sign the support group petition.  I’ve just spent a couple of hours reproducing one of the support group’s guide for parents as they didn’t have a soft copy to share.  One morning I woke up feeling so cross that I couldn’t get the answers I needed and angry that parents are basically having to fend for themselves, that I penned an e-mail to our local MP (Ed Miliband) and contacted the local press!  I received a holding letter from the House of Commons this morning, and the local newspaper would like to run a story on Freya to help me raise awareness.

As a last-ditch attempt to get some clarity on Freya’s immunisations, I e-mailed 2 Kawasaki experts; one in the UK and one over in San Diego, California, and yesterday I received the answers that I needed.  Although I have yet to discuss their responses with our doctors, the new information which states we should wait longer before giving Freya any vaccinations at all.  I’m not going to include all the details here as I’ve posted the slides I have pulled together on http://www.facebook.com/freyasstory.  I feel like a weight has been lifted and that I now fully understand the reasons why the immunisation question is complicated.

It’s a bitter-sweet journey with Kawasaki Disease, perhaps with any childhood illness.  I’ve said before that one of the hardest things about KD is the lack of consistent information, and with there being no UK protocol for standard diagnosis and treatment the advice you get differs depending on who you speak to on what day of the week.  That’s why the petition is so important (sorry, can’t help a plug when I get the opportunity!)  Just as you feel that things are getting back on an even keel, something else comes up and bites you in the arse and you’re back in the dark place you thought you’d escaped long since.  And then I remember that Freya is only 5 months old, and it was just 8 weeks or so ago that we returned home after her ordeal.

Today was a day like that, down one minute and up the next.  One of the reasons was the communication between me and the specialist in America.  And she isn’t just any specialist – she’s known as a world leader in Kawasaki Research, having dedicated the best part of the last 20 years or so to trying to find the cause of this disease.  We were to-ing and fro-ing with different questions, facts about Freya’s medication, etc when we got onto the subject of coronary artery aneurysms and anti-coagulation therapy when I mentioned that it would appear that Freya didn’t have a typical balloon-shaped aneurysm, but that her arteries were significantly dilated throughout the whole artery (well, the bit they can see from an echo anyway).  I knew that had meant things were pretty scary for Freya at their worst, but assumed that now the arteries were regressing that she was no longer at risk.  I’d asked the professor if she thought that it was in any way remarkable that Freya’s arteries had regressed so far so quickly:

“Many young [patients] have rapid remodelling of the arteries, so not surprising that Freya did well.  Important to remember that these are still damaged arteries and she will need life-long monitoring…The long segment dilated arteries are actually at greater risk of thrombosis. See attached.  You might want to share this with your cardiologist.”  And the hits just keep on coming.

I haven’t read the document she sent me. I’ve printed it for reading another day when my head is ready to get away from immunology and back to cardiology.  As long as I read it before the next cardiology follow-up in November, we’ll be ok.  Sometimes I wonder if I ought to do a medical degree!

But, like I said earlier, a life with KD is bitter-sweet, and I am glad that today I get to leave my blog on a high note.  After I received the above response, I replied to the doctor to ask if she felt that under the circumstances it was appropriate for Freya to be treated solely with aspirin and asked for an opinion on anticoagulant therapy.  This was her reply:

“…does not qualify for systemic anticoagulant now but did at start.  We would treat with dual anti platelet agents…I suspect she is safe and fixing the problem on her own.  Well done, Freya!”

That is an e-mail I won’t forget in a while, because in just a couple of brief sentences a doctor over the other side of the world who has never met Freya or seen the details of her case, managed to sum up just how remarkable Freya is.  She has had to deal with more in her short time here than some of us will ever have to deal with in our lifetime.   She barely cries, she takes to every new experience like she’s been here and done it all before.  She is calm and content, and absolutely beautiful.  She is my little Peanut, and she is a Superbaby…

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Out of the mouths of babes

Posted on by Bluemama

Sometimes I forget that Freya’s diagnosis didn’t just happen to her, and me.  It’s like I’m caught up in this kawabubble where my thoughts and feelings about what happened to her are all-consuming, and I have to remind myself that I am not the only person affected by Kawasaki Disease. It sent my whole world into an uncomfortable darkness; a darkness that enveloped not only me, but my husband and children and the wider family.

I think I did a pretty good job of protecting my children from what was happening to their baby sister.  They had already gone through a massive change in their environment when we brought Freya into the world, and suddenly Eliza (11) wasn’t the only girl and Finlay (6) wasn’t the baby anymore.  They handled the introduction of a fifth family member pretty well, and with just the right amount of balance.  Let’s face it, newborn babies can be pretty boring and when both kids realised that there was very little they could do to interact they soon got bored and went about their own business; Eliza up in her room listening to music and making make-over videos, and Finlay with his head stuck in Minecraft on my iPad.  They showed a little interest, but their mum was constantly feeding or changing the baby so there wasn’t much for them to do.  That said, it must have come as a huge shock to them when after just 7 weeks with a new baby sister in the home, she was taken from them, and with her went their mum.

When Freya became ill on Sunday 31st May 2015, we played it down and explained that because Freya was so young we weren’t able to give her any medicine, so we had to take her to the doctors to see what they could do about her temperature.  We said she had a cold, and in all honesty we thought that might be just what she had.  I don’t remember what time we were finally admitted to the children’s ward but I imagine the kids were in bed by the time their Dad got home so he was spared any explanations after a stressful and upsetting afternoon in A&E.  I’m not sure what was said to them the following morning, but Gavin had to leave them before they awoke as I had called him at around 6am to tell him he needed to come to the hospital.  Freya had taken a turn for the worst and had been rushed to the high dependency unit.  Luckily we have a great support network and it just so happened that my mum had come to stay with us for a visit so there was no need to wake the kids when my husband left.  We continued with the story that Freya had a bad cold, but that the medicine she needed had to be given to her at the doctors. I remember my daughter asking where Freya was going to sleep at the Doctors, so I guess then we told her that we really meant she was in the hospital.

On the Tuesday, Eliza was going on a school residential trip to Northumberland for 4 days, which in some ways was good as it relieved some of the pressure for us for a few days.  Finlay hadn’t asked any questions and was unlikely to, but Eliza is more astute and we couldn’t have kidded her for long.  I knew I had to present a brave face for her to be able to go off happily on her school trip and not worry about her little sister. We were not allowed contact with her while she was away, so again we were able to avoid any questions.  I remember she was quite upset on the Monday as she was worried about packing her bags without my help, and I had promised to see her off the next morning.  I nipped home with a heavy heart on the Monday evening to help her pack, and raced back to the hospital.  On the Tuesday morning, one of the family came over to sit with Freya in the HDU so that I could leave to be home with Eliza as she prepared for her trip, then my husband and I took her to school and waited to see her off on the coach. Every inch of me wanted to be back in the hospital with Freya. At that point her condition was pretty critical and I couldn’t shake the feeling that if something happened when I was away from her I would never forgive myself.  And so I went through the motions of being a caring mum, excitedly waving off my child on a school trip.  I remember staying as far away from the other parents as I could; I wasn’t strong enough to engage in conversation.  At that point no-one even knew that Freya was poorly.  People kept coming over to us and asking us how Freya was, how we were settling in with a new baby, were we getting any sleep; the usual newborn banter.  One person asked where she was and my husband replied that she was with his mum (not a lie), and they responded with “Don’t be getting too used to that!”  If only they had known, at that point the notion of getting used to a life without her seemed very probable.

I had to move into the crowd of parents once my daughter was on the coach so that she could see me and I could wave her off.  One mum gave me a little nudge and asked if I was ok.  I tried to shrug it off, but she pushed again.  She knew that I had suffered with post-natal depression with my first child and was worried that I might be struggling again.  And then the tears came, and I told her that Freya was in hospital being treated for suspected bacterial meningitis.  Another mum touched me on the shoulder and kindly said to turn around, as Eliza was looking out of the window, so I wiped my tears, painted on the biggest smile I could muster and waved and blew kisses like I should.  When Eliza returned from the trip days later she made fun of me; “And you said you weren’t going to miss me, you were the only mum that was crying, haha!”  I’ve never corrected her; I was happy for her to keep that illusion.

I didn’t see either of the children for the rest of that week.  Finlay didn’t ask any questions, other than the odd “When’s mum coming home?” or “Where’s the iPad?” but his behaviour at school took a bit of a nosedive.  Fin can be a challenge at the best of times, but it was the first week back at school after the holiday and he was clearly unsettled.  On the Sunday (Day 8) we felt it was time to let the kids come and visit their baby sister in the hospital.  By then the cannula in Freya’s head had been removed, which made her look a little less scary, and the Ward Sister had been doing everything she could to help us try to get Freya back to feeding normally, and off the IV fluids.   I had been home on the Saturday night and left my husband in the hospital; I needed a good night’s rest.  On the way over to the hospital I tried my best to prepare the kids for what they were about to see.  I asked Fin to tell me how he thought Freya might look, and he said she would look poorly, and pale and probably tired.  I described the room to them, and explained that every room was fully equipped for all eventualities, and not all of the equipment was there for Freya.  When the children arrived, Freya still had cannulae in her wrists, and a feeding tube in her nose, but we played it down, focussing Fin on how cool it must be not to have to bother to eat and just get food poured down a tube straight into your tummy. I don’t remember Finlay having much to say when he saw Freya.  He had spied the play area opposite our cubicle and was distracted by the Playstation.  I was glad for the distraction.  Eliza seemed more affected by the situation, and sat quietly with her grandparents on the sofa most of the time.  It was that morning that a consultant came to see us to advise of the transfer to Sheffield. He said we had been there long enough without a diagnosis and had it been his child he would not be satisfied.  Our efforts to play the situation down were scuppered when a nurse entered the room to tell us that an ambulance was on its way.  I remember seeing Eliza’s face full of panic and confusion.  Why were we going in an ambulance? Why were we moving to another hospital?  I reassured her that we weren’t allowed to drive Freya ourselves whilst she was an inpatient, and that we were moving because Sheffield had all the medicines there that Freya needed.  She seemed satisfied.  Looking back, I think I underestimated her.

We kept the visits from the children to a minimum over the 6 weeks we were in the hospital.  They visited less than a handful of times in total; once in Doncaster and a few times in Sheffield.  They didn’t come to Leeds.  Once my mother in law came over and sat with Freya for the afternoon whilst my husband and I took the kids to the museum across the road from Sheffield Children’s Hospital.  It was our attempt at a bit of normality, and it worked if only for a short while.  Whenever they visited, Eliza would become upset when it was time for her to go.  She’d ask if she could stay with us, but that wasn’t possible or appropriate.  It had been a pretty significant time for Eliza; she had recently had her SATS, it was her junior school graduation and she worried I wouldn’t be there (I was), and she would be starting a whole new chapter at Secondary School after the Summer Holidays.  This was a lot for her sensitive soul to deal with on top of all that.

All in all, I think we did a pretty good job of protecting the kids from the seriousness of the situation.  We didn’t feel that either of them needed to be frightened for their sister’s life.  Although Finlay didn’t say much to us about it, other than “Is she better now?” which we answered in the affirmative, I was told by one of his teachers that he had been quite excited one Friday at the thought of me coming home, saying “My mummy is coming home tomorrow because she wants to spend some time with me now.”  Funny how a 6-year old mind works; in his small view of the world his mummy had 3 children, but had chosen to spend her time with another and not him.  His behaviour settled down once we were back home, and things started to return to something close to ‘normal’.

It was a tough Summer holidays for both kids really.  With Freya’s immunity shot to pieces, we couldn’t do any of the things we might normally have done, or the things we had planned and promised to do.  There were times when Eliza would seem frustrated that we couldn’t go on holiday or have friends round like we might have done.  But she’s not a spoilt kid, so I know she didn’t mean any malice.  I had never asked Eliza how she felt about the situation; I guess I didn’t want to put her on the spot in case she hadn’t felt anything much at all.  I saw a glimpse of how this has affected her the day before she started her new school.  She came downstairs to say goodnight and I noticed a friendship bracelet on her wrist.  I told her she would need to remove it as the school had a strict policy on jewellery.  She couldn’t remove it, she said, it was a wishing bracelet.  A friend had bought it for her for her birthday a couple of weeks before.  You have to tie it on and make a wish, and only when it has unravelled will the wish come true.  We argued for a little while and she ran upstairs crying.  Then it hit me. I followed her up and said “So come on, tell me.  You’ve either wished for a puppy or it’s something to do with Freya.”  Tears rolled down her cheeks as she told me that she had wished for Freya to get better, and if she took the bracelet off her wish wouldn’t come true.  I said “Freya is better”, and she answered “No, I mean really better.” (by this time she was aware of the complications with Freya’s heart, but only to the extent of “the illness has made Freya’s heart a bit poorly”).  I held her in my arms and told her that no wish was going to make Freya better.  She was getting better every day, and would continue to get better because she was receiving the right medication, regular follow-up and had a family that loved her.  I said that if anything were to happen to Freya it would have nothing to do with that bracelet, and managed to pull it off over Eliza’s hand in tact.

On Friday last week, I sent an e-mail to our local press to ask them for their support in promoting the change.org petition.  A reporter called me that afternoon and interviewed me over the phone as they are interested in running a story about Freya.  During the conversation the reporter said, “You said you have other children?  How have they coped with all of this?” and I answered that they had been very level-headed about it and did not seem affected by it at all.  And then I realised that I couldn’t really say that for sure as I had never asked them.  Well, I don’t think I would ask Fin, but I wondered if I should ask Eliza.  So after the interview, I put the phone down and asked Eliza how she had felt when her sister was in the hospital.

“I knew something wasn’t right, I mean she was in there for ages and she had all those tubes and machines and things.  And you were there with her all the time.  She must have been really poorly.  And then I…I can’t say this to you…”  I urged her to go on.  “I thought only one of you would be coming home.”

I don’t know if how I handled this was right or wrong.  Is there a right or wrong way to deal with something like this?  Or do you just do whatever feels right in your heart at the time?  I do not regret keeping the majority of Freya’s story from my children.  What I do regret is underestimating the impact that losing their mum and their baby sister for 6 weeks had on them, and not giving my daughter credit for her intuition and intelligence.  Had I known, I would have invested more time with her during mine and Freya’s ‘incarceration’.  We did have one special day – graduation dress shopping and lunch at a nearby shopping centre one Sunday.  And whilst practically every inch of me yearned to be back with my Peanut, this smile on this face was worth every second I was away from the hospital…

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What’s in a Petition…?

Posted on by Bluemama

If Freya could talk, she would be saying a huge THANK YOU to the 39 people who signed the petition for me today (not to mention the 150 that had prior to today’s plea).  My e-mail has been pinging notifications at me all day, and each one has made me smile.  But, there are still less than 2,500 signatures in support of the petition, and that makes me sad.

To a lot of people, a petition is generally someone trying to stir up some kind of trouble.  I know I’ve always steered clear of them in the past, leaving them to the militant types that like to wear a sandwich board and march up and down the precinct yelling at passers-by about some political view or another.  The word rouses all kinds of images of do-gooders, activists and trouble-makers with extreme views.  But what does the word ‘petition’ actually mean?  Well, the dictionary definition is that a petition is “a formal written request, typically one signed by many people, appealing to authority in respect of a particular cause.”  And that is exactly what the Kawasaki Support Group UK’s petition is – it is an appeal to the NHS to raise the profile of Kawasaki Disease and inject some much-needed funding into research that will support the diagnosis, treatment and after-care of this terrible, yet little-known disease.

So, in case you are reading this and haven’t signed, or at least shared, the petition I thought I’d give you a few of my reasons for so energetically encouraging your support (otherwise known as boring you with endless posts with the petition link!).

First of all, I would like to clear up a question around the origin of the petition.  It isn’t mine, and it wasn’t my idea.  It was the brainchild of one of the founders of the Kawasaki Support Group UK who had a light-bulb moment one evening and thought it was time to try a new tactic to raise awareness and drive support.  The KSSG might sound like an elaborate and vast organisation, but it is not.  It was created by 3 mothers after their children suffered from Kawasaki Disease many years ago, when information was less readily available than it is today (and that’s saying something). I think it was even before the internet was born, so hats off to the founders for getting where they did.  It is a voluntary organisation dedicated to supporting and informing parents of sufferers (and indeed sufferers themselves) of this sinister disease.  Whilst I have led my own one woman research mission into this disease to make sure I was adequately advocating for Freya, I will not forget the support this group offered me in my darkest days.  This is the least I can do to pay that forward.

Why I am sad about the numbers of people supporting the petition is because I know how many supporters the KSSG have, and how many members their Facebook support pages have too.  And if every one of them were to sign, and share, and ask their friends to sign, there would be hundreds of thousands of signatures on that petition, instead of 2000.  Since Freya became ill, I have felt completely helpless and out of control.  The feeling that your role as protector has been snatched away from you, and the realisation that you cannot protect your child no matter how hard you try, has been emotionally debilitating.  But then this petition came along, and I suddenly felt a sense of purpose.  I felt like there was something tangible I could do that could help give Kawasaki Disease a voice.  It may be rare, but it is the world’s leading cause of acquired heart disease in children.  That has to count for something doesn’t it?

In case you are reading my blog for the first time, and have not been with me on my journey with Freya, let me tell you a few things about why it is imperative that we drive for a common protocol for handling Kawasaki Disease.

  1. There is no known cause.  There are suggestions that there is some genetic predisposition and a viral or environmental trigger, but research has been undertaken that suggests that the winds, stagnant water, carpet cleaners and even soy consumption could be factors that determine whether your child is unlucky enough to develop the symptoms of KD, none of which has reached a definitive conclusion.  You can have no warning that it is coming.
  2. There is no diagnostic test.  Without a cause (or ‘aetiology’ to use the correct term), the medical profession cannot develop a test.  As a result many cases are diagnosed too late to prevent coronary complications, or worse still cases go undiagnosed altogether and result in the worst possible outcome.  The lack of a diagnostic test, combined with a sneaky disease that likes to present itself as many other childhood illnesses do (meningitis, scarlet fever, for example) means that children suffer for days with soaring temperatures, septic shock, pain and discomfort, not to mention the daily blood samples that are taken, day or night, whilst the Doctors and Specialists try to ascertain what is wrong with them.  They’ll test for everything they can think of, and then test for the things they didn’t.  They will subject children (and babies as young as little Freya) to a series of medical procedures – lumbar punctures, MRI scans, echocardiogram, bone marrow aspiration (in Freya’s case), ultrasounds, x-rays, to name but a few – and still no answer.  The child will often receive a cocktail of broad spectrum antibiotics in the hope that whatever it is will be zapped by one or other of them.  They might even have a blood transfusion like Freya.  And that’s before a diagnosis, when the real heavy drugs are introduced.
  3. There is no standard UK or International protocol for the treatment of Kawasaki Disease.  Once diagnosed, it is pretty standard to administer a dose of IVIG (intravenous immunoglobulin).  It’s a blood product, made up of antibodies taken from over 1000 blood donors.  KD is a vasculitis – it causes inflammation in all of the blood vessels in the little child’s body, until it eventually reaches the coronary arteries (they’re the ones in your heart), causing varying degrees of damage.  That damage is responsible for the ongoing health issues suffered many years after diagnosis for some children.  For others, that damage is responsible for a life being taken far too soon.  After IVIG, it all becomes a bit more sketchy.  Some are left at that.  Some go on to have a further dose, others have even more.  Some receive steroid treatment.  Once it has been ascertained that there is heart involvement, most (if not all) children will be placed on blood thinners (like aspirin).  Where the coronary dilation is at its’ most severe, some are given anticoagulant therapy – but which one is used varies from county to county, and country to country.  Heparin? Warfarin? There are many different views.  And then you will find a specialist who has an opinion about the use of a ‘new’ drug that might not have been widely used in the treatment of KD.  Freya was one of those cases, receiving permission from NHS England for Infliximab to be given due to the severe presentation of the illness in her specific case.  Because of this inconsistency, parents are forced into their own routes of research, and the fear and panic that is born from all the “why didn’t my child get x”, “why did we only get x doses” and similar concerns, is all-consuming.  It makes you feel out of control, and you genuinely fear that your child could die because they may not be receiving the appropriate treatment.
  4. People just haven’t heard of Kawasaki Disease.  I mean, had you?  I know I hadn’t.  And because I hadn’t heard of it, when we received Freya’s diagnosis I was almost relieved. I mean, how bad could it be?  It wasn’t one of the ‘biggies’ like cancer, meningitis, leukaemia… And then a week later you are taken to a room by a Cardiologist who tells you that there is a risk that your baby girl’s coronary arteries could rupture and there is nothing they can do to prevent it, and nothing they could do to save her if it happened.  Don’t get me wrong, I don’t want to frighten people or cause widespread panic that Kawasaki Disease is out to get our children.  Let us not forget how rare it is – affecting around 8:100,000 children in the UK each year.  But, had I known about this disease and the symptoms; had I known that it should be on the medical radar if a child has had a fever, not controlled by antipyretic drugs, for 5 days or more, perhaps I would have asked the question, jogged a memory, sparked a new line of enquiry.  Had KD been on the radar in Freya’s case, maybe the doctors would have looked at that rash a little closer, not so easily dismissed those red, cracked lips as signs of dehydration, not ignored those swollen hands and feet.  When the diagnosis was finally reached (as a result of an echocardiogram showing significantly dilated arteries) it was like all the puzzle pieces suddenly fell into place.  Hindsight is indeed a wonderful thing.
  5. The future treatment and care of Kawasaki victims is unclear.  There is debate over how often a child who has suffered with Kawasaki Disease should be followed-up post-diagnosis.  What if there was no coronary involvement?  Should they be discharged and forgotten about, assuming nothing will change?  What if there was coronary involvement, but improvement or complete reversal has been seen? Do we celebrate their discharge and pray things remain that way?  And what should a long-term treatment plan look like? How regularly should a child be seen, and for how long? What are the success criteria for a full medical discharge? We have been told that Freya will be seen regularly until her coronary arteries have normalised.  Then she will continue to have annual cardiology check-ups with ECG, echo, MRI tests being undertaken, as well as exercise stress testing as she gets older (she can’t even sit up by herself at the moment, so no exertion just yet!).  And if by the age of 15 she has shown no concerning symptoms and the test results are normal, she will be completely discharged, only to be re-admitted should she develop any signs of coronary issues.
  6. The information available about the immunity of these children is confusing.  Kawasaki Disease is a syndrome that kicks off an auto-immune response in the patient’s body.  Basically instead of fighting off whatever virus might have entered the child’s body, the body turns on itself, attacking all the healthy cells.  The drugs that are given have an impact on the child’s immune system.  I won’t even try to explain why or how here because although I have carried out lots of research, I still can’t quite work this one out.  If I could, I guess there wouldn’t be a #6 on this list would there!  Freya was only 7 weeks old when she became ill, and as a result she missed all of her routine immunisations (at 8, 12 and 16 weeks).  The amount of conflicting information I have had in the last couple of months has meant that at 23 weeks old, my daughter remains unprotected from many illnesses and is relying purely on herd immunity – basically we are hoping all of your children have been immunised so she won’t come into contact with any nasties.  We’ve been told to give her the killed vaccines but not the live ones.  She can have the live ones 3 months after she finished her treatment.  Or can she?  Because in the US the advice is to wait 11 months after IVIG.  The blood transfusion Freya received will impact this also.  And then there’s chicken pox.  When my other two children were young, I would take them and rub them all over a kid with chicken pox to be sure they got it when they were young.  With Freya it is different.  There’s something about taking aspirin and contracting chicken pox that can lead to another rare illness called Reyes Syndrome.  It is a disease that affects the brain, and it can be fatal.  We’ve been told that we shouldn’t be concerned as Reyes is rare.  Hmm, pardon me for not being too comforted by the word ‘rare’ these days.  Kawasaki Disease is rare; it still attacked my kid.  Now we are being advised by the consultants to have Freya immunised against chicken pox.  Next month.  I’ve read that chicken pox vaccine should not be administered until 11 months after KD, and that it shouldn’t be administered whilst the recipient is taking aspirin.  Confused?  Join the club.

I’m guessing if you asked other parents, or members of the support group, or even the support group founders themselves, they could multiply this list threefold, more maybe, but I’ve rambled on enough.

I hope that this goes some way to explaining why the change.org petition is so important to me.  Amidst all of the fear, sadness, confusion, frustration and a whole plethora of emotions I could go on to list that go with your child being diagnosed with this devastating illness, there is this one chance to fight for all of the children that have been lost to this illness, and give current and future cases a chance at receiving the attention they deserve.  Parents should not have to spend hour after relentless hour trawling through the internet, reading medical journals, making contact with specialists all over the world just to try to understand their child’s illness and make sure they are being treated well enough to survive it. At times I felt like Freya was a ticking time-bomb and I was racing against time in a fight for her life.  Call that dramatic if you will, but there is no poetic license at play here.  At less than 5 months old, my daughter has already been through what most  of us won’t have to face in our entire lifetime, and I have learned more from this little girl than anyone else has ever succeeded in teaching me.

So please, make an allowance for my constant pleas to like and share Freya’s Story or to sign and share the petition.

“Never doubt that a small group of thoughtful, committed people can change the world. Indeed, it is the only thing that ever has.” ~Margaret Meade

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You can follow Freya’s Story where I share this blog, as well as updates on her progress and life ‘after’ Kawasaki’s, by visiting http://www.facebook.com/freyasstory 😊

And if you would like to view the petition, sign and/or share, please click on the link below:

https://www.change.org/…/nhs-clinical-reference-group…

Change.org Petition to make a better future for Kawasaki Disease Survivors

Posted on by Bluemama

“With your signature, we can ensure that every Kawasaki child is given the same chance at recovery the support and care they and their families so desperately need.”

Freya suffered for nearly 2 weeks before a diagnosis was reached, and it was too late to save her heart from the damage that Kawasaki Disease had done.

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Please take a minute to participate.  Sign.  Share.  Simple.

 https://www.change.org/…/nhs-clinical-reference-group…