Everything changes

I have just returned home from handing back all my equipment to my employer as I embark on this new chapter in my life.  Funny how strange it felt handing back a laptop and a mobile phone that have only been locked away in the garage for the last year anyway.  I guess it felt somewhat symbolic of the final step towards (temporary) detachment from my career.  And just being there in the restaurant, drinking coffee like I used to every morning when I was there, felt quite surreal.  More so, because I had you by my side, and a rice cake in my hand instead of a notepad.  I was very aware, having just finished a year of maternity leave, that under normal circumstances I would have been there alone.  I would have been the one rushing off to that conference call, or heading off to a meeting.  I have no regrets, however. Non, je ne regrette rien.   I consider myself incredibly lucky to have the chance to take some additional time to concentrate on your immediate needs, and to regain some of the time we lost last year.  With your brother and sister I would have missed so much of what I get to see every day with you sweetheart.  Watching you develop and grow is a source of constant amazement for me, and I don’t think I will ever tire of spending time with you.

The effects of the last year were not just felt by me.  I’m sure they were felt by you, but I have no idea how because you can’t tell me yet.  I hope that you don’t remember any of it. That all you remember is how we used to go on the train for days out, or for coffee and cake and to the park.  I won’t ever forget that the backdrop for all of those things was an imposing hospital looming behind us.  But hopefully you will just remember feeding the ducks in that nice park, and you won’t have noticed the tears in my eyes that came because I was saddened by the memories that the park evoked.  One day we will be able to go to Weston Park and sit on a bench and let the warm sunlight wash over us as though it were sent right from Heaven.  We will lie on a blanket and make pictures in the clouds, feed the birds and the ducks and watch the fish in their shady hiding place under the little bridge.  But we will always make our place on the other side of that park so as to spare the mums who, like me last year, watched other families enjoying the summer through the windows of a hospital cubicle.  The nurses thought they were helping me by moving me to a room with a view. The brick wall which was our previous view had been quite oppressive, but no more so than watching mums play with their babies in the shade of the trees.

Back then I thought I did a pretty good job of protecting your brother and sister.  We told them that you had a cold but needed the nurses to help get you better because you were too young for Calpol.  It was a white lie. Quite a big one, but white nevertheless.  And you did test positive for Rhinovirus in that first week, so it seemed like a plausible excuse.  We also kept your siblings from the hospital.  They visited you on your last day at the local hospital (after a week), and then maybe once or twice when we moved to the Children’s Hospital.  We didn’t tell them when you were moved to Leeds, so they didn’t see us when I was in my darkest place.  What I didn’t know was that our attempts to protect them probably made their anxiety worse.  I underestimated Eliza.  She has told me since  that she was very worried that I would be coming home without her baby sister.  She wasn’t stupid; she knew something must be pretty wrong and that a common cold wouldn’t need her mum and sister to be away from them for as long as we were.  So she actually worried more, because she knew it had to be bad for us to want to hide it from her.  And Fin? Well he went off the rails a bit. His behaviour at school hit an all time low, and the effects of last year have only just begun to wear off with a huge amount of support from the school.  He was the baby of the family before you came along, you see.  He was excited about getting a baby sister (although I do think he might have secretly been hoping for a brother to play Minecraft with!), but suddenly not being the baby anymore is quite a lot for any child to get used to.  And then just 7 weeks after we brought you home, just as he was probably getting used to you being around, he lost you and me for a while.  6 weeks.  That’s a long time in a 5-year old’s life.  Because we played down your illness so much, he couldn’t understand why his Mum would leave him.  He thought that I had chosen you over him, and his little head wasn’t quite ready to work that one out.  His self-esteem took a huge knock, and he became very insecure for a while.  No need to feel any guilt about that though, not you or I.  I made the decision to protect them for all the right reasons and I wasn’t ready to tell them that they might lose their little sister.  They didn’t need that worry.  And by the time you read this, you will have seen that none of this had a lasting effect on them; they probably won’t even remember it when you’re old enough to be reading this.

I am not the same person that I was before I had you.  I might even go as far as saying that you are lucky that this happened to you.  You have had the very best of me this last year, and I dare say  that you will continue to get the best of me for as long as we are both here.  I have not taken a single moment with you for granted, and because of that I think I am more patient with you than I might have been with the others. I am most definitely more intuitive about your needs. Perhaps some of  that is due to age (I was 40 when I had you), or experience with having done it twice before.  But I think most of it is the incredible bond that we have developed by spending so much time together since you came into the world.  Because I am scared of you getting very poorly again (I’ll tell you one day about chicken pox and the medication that you take), I have kept you away from large groups of children in the main.  I don’t take you to baby and toddler groups or play areas where the risk of infection is a constant worry for me.  I don’t wrap you up in cotton wool either – rather than avoid all situations, I tend to weigh up the situation based on my anxieties and I guess you could call it damage limitation, rather than complete avoidance.  That will become easier towards the end of this year (2016) when you have received the MMR, Chicken Pox and Flu vaccines.  Until then, we will stay together in our little bubble where we see just the right amount of people to allow you to build relationships with other children, but not enough for me to worry about you becoming sick.  I know I cannot protect you from everything, but I have to protect you from what I see as a risk, no matter how small that risk might be.  I cannot bear the thought of seeing you that sick again, Peanut.

My social circle is a little smaller than it used to be too.  There are lots of reasons for that. I’ve become very immersed in a Kawabubble over the last year and I don’t have an awful lot of time and energy for much else.  Most of my attention is on you, and getting you through the next challenges that the effects of KD will throw at us.  A lot of my attention has turned to our little family; this experience has made my family much more important to me than I think I allowed it to be before.  It’s also very difficult for me to spend time with people who cannot relate to our situation.  I don’t want constant sympathy, but I want compassion and understanding.  People that think that everything is ok now because I wear a big smile, and you look so amazing, don’t know me very well.  They don’t know how much I still struggle to come to terms with what has happened to you, and what the impact of the disease will have on your future.  70% of children in your situation will have to have invasive treatment later in life – a heart bypass, or a stent perhaps.  100% of children who have suffered coronary aneurysms as a result of KD will suffer myocarditis (inflammation of the heart muscle) which causes degeneration or death of heart muscle cells.  I don’t know how to compute the possibility that my child may show symptoms of myocardial infarction (a heart attack).  Just doesn’t seem real.  Later this year, whether it be through cardiac catheter angiogram or CT angiogram, we should get a closer look at the cause of the remodelling of your coronaries to better understand what the future may hold.  Right now I have to take the cues from your outward appearance and development, and you look mighty fine to me.  It’s very confusing though.  I’m still not sure if there is any chance that you could have complications today, tomorrow, next week.  I guess we have to take each day as it comes.

I don’t want people to think that I am a victim in all of this.  That I don’t want to let go of it.  In fairness, I can’t let go.  Partly because you are still affected by the disease and will be for many years to come. Partly because I feel I have gained a purpose; I can help make things better for other sufferers of the disease, whether it be through raising awareness to help speed up diagnosis, or by providing useful information to other parents going through this.  Kawasaki Disease is now a very big part of my life, of our lives, and it isn’t likely to go away.  Perhaps over time it will become a smaller part of life for all of us, but I don’t think I will ever stop talking about it to anyone who will listen (and a few who don’t!).  It’s too important for that.  I am in contact with parents and grandparents who have seen this illness first hand, some who have suffered the worst consequence of all.  For them, I will always do what I can to help change the future of Kawasaki Disease.

Having a child diagnosed with a rare, or little known, disease is life-changing for parent and child.  And when there is no known cause you never stop asking yourself ‘Why?’.  Why did this happen to you? I hope we find out one day.  I believe the specialists are getting closer – it’s just that research costs money and KD research is severely underfunded.  They believe they have identified the combination of genes that make a child with KD more susceptible to the disease.  And they are pretty certain that they are looking for an infectious/toxic agent that completes the perfect KD storm.  I desire two things; that we can gain a greater understanding of your condition so we might have a better handle on what might be around the corner, and that the mystery of Kawasaki Disease is unravelled in our lifetime so we might gain some closure.  Solving that mystery will also result in a test for the disease so that children might be diagnosed more quickly, and better treatments that further improve the possible outcomes for children with the disease.

My whole perspective on life has changed.  Things I was afraid of before no longer occupy any space in my mind.  I no longer sweat the small stuff.  I have a lot more compassion for others – through our journey I have come into contact with so many parents who have suffered challenges with their children, KD or otherwise.  Before this, I was ignorant to all that – it was happening to someone else and that didn’t matter to me.  Now it does.  The only fear I have now is of losing you, or losing any of the people that I love dearly.  I will not allow people to hurt me anymore, because nothing can come close to the pain I have felt over the last year with you.  None of that matters.  Yes, everything has changed. Our lives are different than they would have been if KD hadn’t crept in.  But it’s not all bad. It’s different, but not bad.

Sometimes I am scared, often I am sad.  I worry a lot about the future for our little family.  But above all that, you will grow up knowing that you are loved and cherished more than you could ever fathom.  You are a special little girl who will achieve great things one day, and I will be there by your side with every step you take towards greatness.

I love you, Peanut. 

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The Gift that Keeps on Giving…

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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To Immunise or not to Immunise…

…That is the Kawaquestion!

If you’ve been following Freya’s Story through my blog or on Facebook (www.facebook.com.freyasstory), then you’ll know that one of the hurdles I have been trying to get over since her diagnosis and treatment for Kawasaki Disease is the question of when to immunise.  When is the important question here – it is not a question of ‘if’ for me.  Freya is my 3rd child; I had the older two vaccinated in line with the standard UK immunisation schedules without hesitation.  They both had the (then controversial) MMR vaccination that was vilified for years, for those claims to be later contradicted by the very doctor who made them.  I am not an antivaxer (yes, that is a word that is banded around various mum networks).  I can’t say I’m a provaxer either to be fair; in all things medical I am a conformist.  I trust the NHS and the doctors to have made the right decisions.  Well I used to anyway.

Now before I go on to share all the information I have in relation to this subject, there are a couple of things you should know.  First of all, the information I have received is contradictory to say the least.  I’ve even had different information from the same doctor, just on a different day.  And therein lies one of the biggest issues, and the key reason why we should be campaigning for standard protocols for treatment and care of Kawasaki patients.  But that’s a whole other blog.  Secondly, I take no responsibility for the decisions you may make in relation to this issue.  I am not a medical professional; I am just a mum that wants to do the right thing for her child.  I could not protect my baby from Kawasaki Disease; I have to be able to protect her from the other nasties, if I can.  I will share the information I have and where I’ve found it.  You also need to understand that Freya’s case is an individual one.  We certainly haven’t found a doctor in the UK who has received a patient as young as Freya where Kawasaki’s is concerned.  Generally children are older (commonly between 6 months and 5 years) and have therefore already begun their immunisation journey.  Infants diagnosed with this disease have often already had at least the first tranche of jabs (leading some to believe the vaccine to have been the trigger that KD needed to start it’s evil – a theory that I don’t discount, in fact I find it a very interesting theory with some validity and would not be surprised if it is confirmed as one of the triggers in the not too distant future).  Indeed, some of the vaccines state an increased risk of Kawasaki Disease in the patient information – information we often don’t read before we give consent for the needle to go in.  Again, I do not want to be a scaremonger, and it is absolutely not my intention to send cats amongst pigeons.  I’ve already said I am a provaxer.  I am pro-choice in all things – but I believe in those choices being informed if you are someone like me who needs to know it all (and often thinks she does, lol!).  

Freya was 7 weeks old when she became sick.  She was hospitalised on the very first day that she showed a single symptom (fever) and spent a number of days on a paediatric HDU, several weeks in a Children’s Hospital, and a week on a Cardiac HDU.  We hadn’t yet seen a health visitor, and the appointment for her first set of immunisations (8 weeks) has just come through.  I guess due to her condition, and later the treatment she received, it wasn’t possible to start the process of vaccinating with Freya whilst she was in the hospital.  She was 7 weeks old when she went in, and 13 weeks when she came out, so she was already behind schedule.  She received a blood transfusion, intravenous immunoglobulin (a blood product) and intravenous and oral steroids.  All of these have an impact on the immune system, rendering vaccination difficult for a certain period of time.  And I can’t be more specific than that, because that is one of the biggest areas of contradiction I have come across when researching this subject, and the reason why now, at nearly 10 months old, Freya has not had a single vaccination.

Some might wonder why that is an issue.  Plenty of parents (those antivaxers) make the decision not to immunise their children against some or even all of the diseases that the immunisation schedules seek to protect them from.  There are children who are unable to receive the immunisations because of poor, weakened or non-existent immunity.  We vaccinate the many to protect the few; herd immunity.  I could trust that everyone else has been protected, so Freya will be ok among the herd.  But, what if…  And then there is chicken pox – we don’t routinely vaccinate for that here in the UK (they do in the States).  If you Google “chicken pox and aspirin” you will find one big reason why aspirin and kids don’t mix; Reye’s Syndrome, a rare but potentially fatal illness linked to the fever associated with Chicken Pox and Influenza in children taking aspirin.  Ok, so the link has been found where high doses of aspirin are taken, and not yet with the low anti-platelet doses that a KD kid is prescribed, but nonetheless it’s a nasty illness and one I don’t want to take any chances with.  Freya has been through enough.

So what happens if you have a child who has not been immunised, and you are concerned about them coming into contact with what others would consider pretty innocuous illnesses?  Let me tell you what happens with us.  Freya has not been to a baby group; not baby massage, or baby music, or baby yoga.  Hell, she hasn’t even been to a playgroup, and when my older two get invited to a kids party, Freya stays at home.  Don’t get me wrong; we have not become social recluses.  Instead I apply my common sense to the activities we will undertake.  I know there might be someone in the coffee shop who has the flu.  Why is that kid in the shopping centre not at school? Could they have chicken pox?  I can’t hide us away from the whole world, but I can reduce the risk of her coming into contact with these nasty germs and bugs.  I call it damage limitation. She has one or two little friends that she sees from time to time, but only when they are well.  And don’t forget she has an older brother and sister who are exposed to all manner of things at school every day – we don’t make them wear a mask!  It is a source of sadness for me though.  Freya is such a happy, sociable child, and I worry about the effect this lack of exposure to her peers might have on her development.  I want her to laugh and play with other children, to learn how to share, to grow bonds and discover new things.  Instead there is a lot of singing and dancing going on in this house, which isn’t a bad thing (unless you’ve heard my singing!!). And with a bit of luck we can catch up with those immunisations soon and provide what we believe to be a little protective bubble around our child before it is too late for her to start over.  The knock-on effect of KD will be huge – when I return to work, she will have to go to some form of childcare.  She will be over a year old, and will never have been with anyone else other than her close family.  That’s going to be a tough one for both of us to handle…

Let me go back to the beginning of Freya’s immunisation journey.  While we were in the hospital with Freya we were advised that she shouldn’t have the immunisations, first because of her condition, and secondly because of the medication.  A lady used to pop her head in our cubicle door every other day with an immunisation leaflet and I repeated often that we had been advised Freya would have to be left to catch up.  I was promised an individualised immunisation schedule to leave the hospital with; it never materialised.  I made a number of calls to the hospital to enquire about when I could start the process, and to the Health Visiting team to ask them to stop the weekly postal reminders telling me what I already knew; Freya had not received her first immunisations.  Eventually I got the call from the hospital that advised I could give her any killed (inactive) vaccinations immediately (she was discharged one month after treatment for KD), but that I should wait 3 months post steroid treatment for any live ones.

I did a bit of research, because I was convinced there should also be a timescale for receiving vaccinations after a blood transfusion.  All avenues confirmed that vaccinations should not be given until 6 months after a transfusion.  Not to mention the varying timescales quoted for giving vaccinations after IVIG!  But all my hospital ever referred to was the steroid treatment, I guess because that is an immuno-suppressant.  But what of the other things? Had they forgotten what she had been given? I felt uneasy with the advice, so I sat on it for a while.

At a follow-up appointment with Rheumatology, I asked about whether Freya should be vaccinated against Chicken Pox (Varicella).  The doctor said that in the UK we only vaccinate children who are at high risk of contracting the disease.  I said that whilst Freya wasn’t at higher risk of contracting chicken pox, the potential consequences of her contracting the disease whilst taking aspirin could be catastrophic.  He agreed to take the issue away, and on the 19th August 2015 I received a letter confirming that they believed it was “reasonable for Freya to receive vaccination against Chicken Pox, both in relation to the issue regarding ongoing treatment with aspirin and the small degree of risk of developing [RS] and also the small risks that Freya may require treatment with immunosuppression therapy in the future…”.   Freya had been weaned off steroid therapy a lot more quickly than originally planned when the gastrointestinal bleeding started. From memory, it was the 8th July 2015 when she took her last oral prednisolone; that meant she could have the live vaccines, including CP, anytime after the 8th October 2015; “…it is important that Freya does not receive this until at least 3 months following…the last day Freya received the steroid treatment…”.  I still felt uneasy, and decided to wait until our next follow-up appointment to discuss.  It was at that appointment that I shared all the research that I had done, and got a referral to Immunology.  I had to wait until yesterday for that appointment.

I wonder if now might be a good time to share what I have learned, from the internet (reliable sources, like the NHS and the American Heart Association) and from medical professionals from London to California…

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From the list above, all vaccines are inactive with the exception of the following live vaccines – Rotavirus, MMR, Children’s Flu vaccine (note that for the children’s flu vaccine, the nasal spray is live, however there is an injectable inactive flu virus available).

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These are just a few examples of the responses I received from medical professionals, and I can say that my internet research has thrown up much the same; anything from 3 months to 12 months.  Helpful, right!  My gut feeling was telling me I should err on the side of caution, and follow the advice of the US doctor who is considered the leading Kawasaki Specialist in the World – she calls herself a ‘Kawasakiologist’ and I believe she deserves the title.  Our GP agreed that Freya qualified for the injectable (inactive) flu vaccine.  My decision was made.  Well, kind of.  I had decided I wanted to get Freya caught up on the inactive vaccines immediately, then discuss the issue of the live ones with our Immunologist.  Chances were, by the time we got any decisions we would be nearing that 12 month mark anyway, so it was a win-win.  But our ever cautious GP decided that if we were speaking to an Immunologist anyway, there was no harm in waiting for that meeting before we started the immunisation process, and he felt happier to wait for clear direction from her.  So, again we would have to wait.

So yesterday we had that appointment with the Immunologist.  I was all geared up to go in and cover all my questions, but unfortunately an hour’s wait with a baby that has decided that a high pitched squeal is the best means of communication made the whole process a little trickier than I had anticipated!  The doctor was the Infectious Diseases Consultant who treated Freya when she arrived at the Children’s Hospital on day 8 of her illness.  That was nice, as I had the opportunity to thank her for her compassion, support and tireless determination to get to the root of Freya’s condition (especially as I was quite a difficult ‘customer’ who asked lots and lots of questions, and even accused them of breaking Freya’s heart at one point!)  She seemed genuinely pleased to see Freya looking so well.  She received the news that we had seen some improvement in her coronaries since the acute stage of the illness with a broad smile, and she was happy that Freya has not had anything more than one little cough and cold since her hospitalisation.  I expected her to call me a muppet for being a cautious and over-protective mother, but instead she said she felt I was doing the right things, which was reassuring.

I came away from the appointment with a solution, individualised for Freya.  I still don’t have a definitive answer about the timescales related to immunising after IVIG.  But then why would I?  The fact that I can quote at least 5 different timescales from medical papers and journals across the world tells me a lot of them are guessing. I’ve read that medics aren’t completely sure why IVIG even works in the case of KD – they are still trying to understand what causes the illness in the first place, so I guess it would be near impossible to understand why the treatment works. It just does (in most cases).  The Immunologist told me that they deal with children who have compromised immunity as a result of a bone marrow transplant, for example.  Those children are given immunisations  in line with the hospital guidelines and they do not wait 12 months.  That said, she agreed that they had learned more than they previously knew about KD from Freya.  Presenting at just 7 weeks old, and having had no previous immunisations, she is a bit of an unusual case.  So they have agreed to follow a similar process to that of a bone marrow transplant patient.

What are they going to do?

They have taken blood samples from Freya in order to test her body’s immunity status.  They will be testing Freya’s immunoglobulin levels, and lymphocytes (T-cells).  Depending on those results (and I am sorry, I didn’t ask what result they would be expecting or what a low or high result might indicate, or whether there was a chance they might just be reading ‘borrowed’ antibodies as a result of the circulating IVIG – I will ask that when we discuss results), they will start the routine inactive vaccines and the injectible (inactive) flu vaccine.  After a period of time following those (I think she said 6 weeks), Freya’s immunoglobulins will be tested again to check her immune response to those vaccines.  Assuming they get the right result (whatever that might be), they will then progress to live vaccines, including chicken pox.  If we get moving quickly, Freya may even make it in time to have the MMR vaccination at precisely the right time.  I have some decisions to make about Rotavirus (which I believe is time-specific so she may already be considered too late) and Meningitis B, as both have stated increased risks of contracting Kawasaki Disease in the patient information.  I know it is incredibly rare for KD to reoccur, but show me one parent of a kid with a rare condition who finds that word reassuring.

Yes, I am still confused as to what the right answer is about timescales for vaccinations post treatment Kawasaki Disease.  But, I think I have a great solution for me, for Freya.  Rather than guess based on the varied guidelines out there, we are going to take a look at Freya as an individual.  Actually take a look inside and see what is going on, and make the decisions based on what she tells us.  The Immunologist has always said that Freya showed them the path to a diagnosis, and that they learned so much from her.  Seems she is going to teach them something new.  And as much as I probably do their heads in with my questions and powerpoint presentations, I do know that they respect me for it and that they are luckily not too arrogant as to discount this mum’s research and opinions.  She actually said it was helpful and told Freya that she is as well as she is because of her “amazing mum”. I don’t know about that, but in a sea of negativity and low moments, I’ll take that right now.

Whatever I learn from this experience, I will share.  Every Kawasaki case is different, no matter how similar some of the stories might seem.  No wonder it is a tricky one to spot, diagnose, treat and understand.  I hope in my lifetime they find the cause so I can finally understand why this happened to us, to my little Peanut.

Our Rheumatologist said that the subject of immunisations in these cases is “contentious” and currently being debated.  Another said that “guidance is not clear as there are no trials/studies to guide us for this exact situation…” and “…is a balance involving unknowns…”  He gave his opinion and advice, stating that “…there are other equally ‘correct’ versions too!”  No shit!

I’ll leave you with a few of the comments that I received from KD specialists here and in the US, which might help to dispel a few myths relating to IVIG and immunity.

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And in case you can’t read that, I have pasted below:

“The reason not to give live vaccines after IVIG treatment (has nothing to do with steroids) is that your daughter has received antibodies poled from 10,000 adult blood donors who have antibodies against the viruses in the live vaccines. Therefore, the live viruses are inactivated by the circulating IVIG that is still detectable 11 months after administration…it is not dangerous to give the live vaccines…it’s just that they will be inactivated and the body will not make a lasting response. All killed vaccines are fine to give once your child is past the acute phase. 3 months sounds like a good period to wait. All live viruses vaccines should be delayed one year after IVIG.”

“We don’t usually stop aspirin when we give chicken pox vaccine for our [patients] with serious aneurysms. The risk of Reyes’ Syndrome was associated with higher doses of aspirin and not the very low dose used for the anti-platelet effect in KD patients with aneurysms. There is no data to support the notion that low dose aspirin is a risk. That being said, Reyes’ Syndrome [is] terrible and it should be easy to stop aspirin for 2 weeks. Clopidogrel can be substituted for aspirin during that time.”

“Your daughter’s immunity is not compromised by KD. It can be compromised by steroids, but only while actually taking the steroids. Your daughter’s immunity has been boosted by the IVIG she received so she is quite protected from all routine infections for the next year. There is no evidence that children with KD have immune defects that make them more susceptible to routine infectious agents.”

“…The issue of when to vaccinate children who have received…IVIG…arises because the antibodies present in the IVIG may prevent an adequate response to live vaccines. This is less clear in the case of vaccines that do not contain live organisms. The second issue is that steroids may diminish immune response and also make live vaccines dangerous. If Freya has been off steroids for 3 months she can go ahead with all the killed vaccines…”

“…The IVIG may prevent live vaccines working for over 6 months, and may interfere with MMR vaccine working. However MMR is not needed until after 11 months of age so unless there is a local measles outbreak she does not need MMR until the normal age…”

“Rotavirus vaccine is supposed to be given before 24 weeks of age which Freya may be beyond; it is probably not worth giving it at this stage…”

“…Varicella vaccine [chicken pox] should be given but as both steroids and IVIG may prevent it working I would suggest waiting at least 6 months from the IVIG and steroids before giving it…”

 

 

A productive day and a Superbaby

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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