What’s in a Petition…?

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…

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