awareness

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.

 

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.

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…

Freya’s Story (15)

Posted on by Bluemama

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

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

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

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

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

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

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

That night I wrote in my journal:

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

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

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

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