Going home should have been something to celebrate, but instead I felt scared and alone.  The pressure I felt from the responsibility of preparing Freya’s medication was immense.  She left the hospital on aspirin, lansoprasole (to protect her tummy from the medication), steroids (prednisolone), amoxycillin and fluconazole (they identified Group G Strep infection and oral thrush a few days before we were discharged).  Those in tablet form had to be dispersed in a certain amount of water, and then a specific dose drawn up the syringe to be given to Freya.  The best part about giving the medication was how well Freya handled it.  She sucks on each syringe like I’m giving her a drink!  Now we only have the aspirin and lansoprasole to deal with.

The night before I had left a number of messages via phone and e-mail with the Cardiology team, asking for an urgent conversation with our Cardiology Consultant.  I was consumed by a fear that we were running out of time, and that every minute that passed without adequate treatment for her coronary complications was a step closer to losing our daughter.  I felt sick with worry, and so confused about what I should do.  I mean, this kind of thing doesn’t happen to real people does it!  It happens in soaps and in movies!  Or it happens to someone else.

I had made contact with the KD specialist in the South West of England, and he had concurred with the specialist in Seattle that Freya should be offered an anticoagulant as well as the aspirin she was receiving.  You see, where there are coronary complications the key is preventing the blood from clotting in the artery.  Aspirin keeps the blood thin and flowing nicely, whereas drugs like Warfarin work by preventing the blood from clotting altogether.  That’s why people receiving Warfarin therapy have to be careful with certain activities and are restricted from doing things like contact sports.  I was in no rush to give Freya Warfarin, I mean it’s primary use was as rat poison back in the day!  And I’d heard it was a nightmare drug to administer because you have to be weighed and checked and have blood samples taken on a regular basis.  But I didn’t care about the origins of the drug, or the inconvenience.  If it was what was needed to save my child’s life, then so be it.  The Professor offered to see Freya in person.  I thanked him for his offer, but felt that I ought to obtain a full explanation from our Cardiologist before I made any decisions about how to act.  I gave the Cardiology team 24 hours to put me in contact with the Cardiology Consultant and advised them that I would be taking Freya to Bristol if I did not hear anything by the Wednesday morning.  I was so angry that my first day at home with Freya was overshadowed by this issue, and felt terrible for spending most of that day on the phone or reading and sending e-mails.  But I was doing it for her and it was important that I fight for her.  The US doctor had made me feel like we didn’t have a moment to spare, and I spent those 2 days mostly pacing the house and becoming more and more anxious.  I felt that Freya would die before we had our next follow-up appointment (we all know that wasn’t to be the case, but at the time the fear was very real).

Eventually I received a call from our Cardiologist.  I picked up a pen and paper, and scribbled down everything she said, whether it made sense or not.  She explained that there would be a risk of gastrointestinal bleeding from Warfarin when the patient is taking steroids.  She said that she had individualised the risk for Freya, and that she had considered Warfarin to be inappropriate at that time.  She did say that this might change, but not until the steroids were finished, but that the risks and benefits would change anyway as Freya moved out of the acute stage and further away from the inflammation of the Kawasaki Disease.  She said that there are a number of calculations that can be used to determine a ‘z score’ and that each calculation had resulted in a different number in Freya’s case.  One number had suggested the necessity for an anticoagulant, but another didn’t.  She made a judgement based on what she had seen of Freya.  Then I got the science bit about flow dynamics and the physics of blood flow; that with Freya’s case she was more concerned about lamina flow (not sure if that’s the right term but it’s what I wrote down!) which is apparently the speed of the blood cells moving down the artery.  She said that we should expect further testing in Freya’s future – CT, MRI, Echo, Exercise Testing and angiograms.  All in all she believed in the treatment she was providing, and she did a really good job of convincing me too.  I was considering asking for a second opinion, but that’s not as easy as it sounds and there is a formal process to follow.  Instead I said that when we were at Sheffield, they acknowledged that they were unsure about how to treat Freya and had sought counsel from other specialists in the UK, and I asked if she could do the same.  She agreed to seek advice from the other Cardiology units across the UK, and said she would bring Freya’s cardiology appointment forward by a week so we didn’t have so long to wait to see what was going on.  I still had a niggling doubt about why other experts gave different advice, but felt that I should trust those who were taking care of my daughter, particularly as they had seen Freya’s heart first hand and the others were simply going on the information that I sent them.

It was lucky really that our Cardiologist made the call that she did because of her concerns about gastrointestinal bleeding.  Before we had our conversation that morning (Wednesday 1st July 2015, Day 32) I had changed two nappies that both had specks of blood in them.  I remembered this happening once before in the hospital and the doctors hadn’t seemed too concerned about it so I decided it wasn’t anything to worry about.  However, the next dirty nappy (Freya was having about 15 poos a day!) had a significant amount of blood in it.  I contacted the Rheumatology Nurses in Sheffield to ask what I should do and they asked me to take Freya in.  So, less than 48 hours after our discharge we found ourselves back in the car and on our way back up the motorway to the hospital.

Wednesday 1st July 2015, 22:00pm

“I’m afraid to tell you that we are back at Sheffield Children’s Hospital.  I’ve not written since Monday because Monday night we brought you home…We didn’t do very much. We cuddled a lot! You ‘played’ on your teddy bear playmat – you hated it before all this started and now you love it.  Those beautiful blue eyes of yours, deep as the ocean, bulged with amazement at all the new things you saw.  Yesterday you went in the garden for the first time.  You laid on a blanket in the shade of the playhouse and laughed at the washing blowing on the line.  I could see that you could hear the birds singing in the trees.  It was wonderful sweetie.  Simple things; you and me.”

The blood in Freya’s nappies would get worse as the evening went on, and into the following morning.  I had been advised to save the nappies so the medical staff could take a look.  By the morning I had a collection of dirty nappies in our cubicle so I asked the nurse if they needed to take them to look at them.  As I said, Freya always had a lot of dirty nappies so I was glad when she took them away!  The following day we were visited by the Gastroenterology team. The consultant said that they had looked at Freya’s nappies and saw nothing too concerning.  I was really surprised; the last few nappies had been full of blood.  I expressed my surprise and from what the consultant said in reply I knew they hadn’t seen all the nappies.  Lucky then that I had taken photographs of the contents of every one and was able to show them.  As soon as the consultant saw the photographs she said “now that changes everything” and thanked me for having the foresight to take photographs.  The nurses had thrown the nappies in the bin and they had not seen them.  Taking photographs had become a bit of a habit for me.  Before the diagnosis was reached, Freya’s rash would come and go and you could guarantee that by the time the consultant came to see her it would have gone.  So I took photographs of any visible changes in Freya’s condition, which helped the medical staff see the things they otherwise would have missed.

The Gastroenterologist believed that the bleeding was likely to be caused by the aspirin and said that it would need to be stopped.  I jumped in, “I think you should discuss that with LGI; aspirin is the only thing that is preventing her from having a heart attack, I think they will have something to say about you stopping it.” They were not aware of the full situation and agreed they would discuss with our Consultant and the Cardiologist.  Not surprisingly, the Cardiologist advised strongly against it.  Instead Freya was sent for an ultrasound of her abdomen and the dieticians prescribed a mild baby milk formula which is non-dairy, called Neocate.  The Rheumatology Consultant informed me that he would be weaning Freya off the steroids sooner than planned, and that should we need to take any further action for the bleeding (an endoscopy for example) he was prepared to stop the aspirin temporarily as it would remain in her system for long enough to create a window of opportunity for treatment of this new concern.  After less than a day on the new milk, the bleeding stopped.  The ultrasound of Freya’s abdomen showed an abnormality in the position of the arteries in her bowel.  The gastro team mentioned something called malrotation and said that Freya would have a Barium Swallow Test in a few days. Just when I though there were no more tests or procedures left for her to go through.

Thursday 2nd July 2015, 12:26pm

“I am so worried about you. You are so small and you have been through such a lot.  When is it all going to end?  I feel a huge sense of loss.  I still have you, but I have lost your health.  The heart you were born with is broken and may never be the same again.  You will be on medication for many years and hospitals will be a big part of our lives.  This is the new normal for us.  We won’t know for some time what impact this will have on your life.  What limitations might be placed upon you, or us.  Can we travel…? Can we even fly with you?  Will you be able to do anything you want to do as you grow up?  

...You most certainly have been sent into this world to shake things up little lady.  Our lives may never be the same again, but our lives are richer for having you in it.”

After the follow-up echo on the previous day the doctors in Sheffield indicated they were pretty happy with Freya’s progress, and it seemed they were ready to let us have some time at home.  That afternoon, waiting for a decision on the day release for the weekend, I reflected on my time with Freya in the hospital.  Whilst the whole situation had been terrifying and devastating, one thing I did get from being in hospital with my daughter was an immeasurable bond and an intense understanding of every move and signal she made.  I became so in tune with her over those weeks from being in such close proximity all the time, that I can say I worked out what she needs from me much more quickly than I ever did with my other children.  Whenever I am away from her I feel there is an empty space somewhere near my heart, like we are inextricably linked through our experience.  I know that sounds daft; we are mother and daughter, of course we are linked.  But this feels different somehow.

Friday 26th June 2015, 16:05pm

“Well it has to be said that every cloud has a silver lining and the upshot of this month in hospital is being able to watch you grow and develop.  I mean really watch you.  Being with you 24/7 means I haven’t missed a single change.  Not only…the physical changes like your hair growing or you putting on weight (today you were 9lb 15oz), or that you’ve grown out of some of your clothes….Whilst you’ve been in here with me you have hit a major development leap, and I’ve noticed you taking much more notice of the world around you in the last couple of weeks.  Such a shame then that the world according to you has been this hospital room.  When we go outside [once Freya’s condition had stabilised they would let me take her out for short walks], your eyes open so wide I think they are going to pop out of your head!  If you could talk you would be saying “Wow!”

You had started to find your hands just before…this adventure and had begun to suck your thumb.  Unfortunately the nurses then stole those fingers away from you by putting a cannula in each hand and bandaging them up.  But now you are free from all the cannulae and just as you should be.  You have more control over you movements and like to grab hold of things like my finger, or even the wires of the medical equipment (you tried to take over an echocardiogram from the sonographer yesterday).  You…like the feel of material…I’ve made a little muslin nest in your cot to make you feel more secure and you like to hold a piece up near your ear.  Earlier you were gripping the label as you fell asleep…  

…Other times you hold onto the neck of your top, or mine if we are having a cuddle like we are now.

I’ve noticed that you rub your head when you are tired, and if you are trying to get to sleep but wriggle about a lot you have usually wet your nappy.  You like the idea of a dummy, but…you suck on it so frantically it actually stops you from going to sleep.  You like to be cuddled, but when you get too warm and just want to be put down you moan a lot!

…You are such a smiley, happy girl too (unless you are hungry and then we all know about it, haha!)  You wake up at about 6am and cry a little, but as soon as you see me your smile lights up your face, and warms up my heart.  

You like music…This morning we did a bit of dancing and singing to various tunes on my iPhone.  It would appear that you love Ed Sheeran, don’t mind a bit of Bruno Mars, but are really not keen on One Direction!

[The Rheumatology Consultant] has said that we can go home over the weekend as long as we come back at teatime and stay the night here.  I can’t wait to see you back at home tomorrow, even if it is just for the day.  Your eyes are going to pop out when you see our house properly for the first time, and when you get a look at your beautiful bedroom with the paper butterflies flying up the wall…

…It’s time to get you home sweetheart, we’ve been here long enough.”

That weekend we managed to get Freya home both days.  By the time all the paperwork had been done it was early afternoon before we got home.  But those few hours with her back where she belonged were great.  When Freya was in the hospital, people would keep saying “She’s in the right place”.  If they didn’t know then how much that statement made me want to punch them in the nose they will now!! The hospital was never going to be the right place for her.  Home was where she belonged.

After an uneventful weekend, the doctors decided on Monday 29th June, 30 days since Freya first became ill, that we could take her home.  We would have to return with her twice a week to repeat blood tests and review her progress, but we didn’t care!  That morning I felt like I could tackle anything, and that our journey was finally over.  But as the day went on, hanging around waiting for all the necessaries to take place for our release, the reality of going home begun to sunk in.  As much as we wanted to escape the confines of the hospital, I had underestimated the security I felt within those walls.

Monday 29th June 2015, 16:20pm

“We are nearing the end of your in-patient adventure here at Sheffield Children’s Hospital sweetie.  The doctors have all been to see you for a prod and a poke, and all are happy for us to go home.  Your blood results continue to be positive with CRP, ESR and your platelet count all heading in the right direction or in normal range.  We are all packed up and ready to go as soon as they come with your medicines for home.  You are fast asleep in the cot for the last time!

It isn’t the end though sweetpea.  It’s the end of a chapter I guess, but it feels like we are just setting off on a new path.  I wonder how long your life will be plagued by hospital visits and tests, or if we will ever receive the all clear to live a normal life.  But who wants normal, eh?  Normal is boring!  You weren’t destined to be normal, nor should you be.  Here’s to living an atypical kind of life.

I have a feeling we will have many hurdles to leap over in the coming weeks, and your journey is going to be a long one.  We have yet to challenge the treatment for your heart…We’ve received some really valuable information from a KD specialist in Bristol that is going to help us begin that battle this week.  I cannot protect you from the condition you have been left with, but I can fight to ensure you are receiving the right treatment to give you the best possible chance.

I am trying so hard to focus on each day at a time, and I’m doing pretty well mostly.  But lurking in the back of my mind, forcing a tightness in my chest and a fluttering in my stomach, is the thought of losing you.  I’m trying to bury those thoughts way down deep because they threaten to destroy my hope and my faith and me along with them.  Not a minute goes by where a tear does not prick at the corner of my eye, or my stomach doesn’t do a tiny somersault [every time I look at you].  I can only hope that over time those feelings become less and less.

Today we should be celebrating going home.  Usually you leave a hospital fit and well after receiving the appropriate treatment. I feel like a lamb being thrown to the slaughter to a life of joy tarnished by fear.  Every day I will grow to love you more and more, and every day the fear of losing you will grow too.  There is a scream at the back of my throat threatening to come out, and I bite my tongue to stop the tears because I know if I…let them fall they will not stop.

Kawasaki and me are not friends.”

Two things happened that afternoon that would send me back to the dark places where KD hid.  Bored sat waiting for the nurses to bring Freya’s medication, I was browsing through Facebook, just flicking through the News Feed half-heartedly.  And there it was, a picture of a little boy that had been posted in one of the Kawasaki Support Group pages.  His name was Max, and the post was from his parents asking to ‘pay it forward’.  He had had KD, and it was the anniversary of his death.  I felt a familiar fear envelop me and I sat and cried at the reminder that this disease had the power to take a life.

As I was getting into my car to drive us home, I heard the familiar ping of an e-mail notification and opened it to read it before I set off.  It was an e-mail from the KD specialist in Seattle.  We had been exchanging e-mails, and I had kept him informed about the response that I received from the specialist here in the UK.  He wrote:

“You never told me your daughter’s first name.  One last piece of important advice.  Management of KD patients requires a substantial bit of trust between parent and cardiologist.  She is in the danger period, as clots tend to form in the first month or so after acute KD…My British friends tell me that Bristol is a bit of a trip from Sheffield, but I have patients drive all the time several hours to see me as a KD specialist.  I think your daughter deserves the trip to Bristol to see someone who is confident and not negative towards anticoagulation in infants with KD” Monday 29th June 2015, 18:16

I think I nearly threw up. I was already feeling uneasy about the treatment Freya was receiving (or not), and here I had a KD specialist urging me to put my daughter in the car and drive her to Bristol.  It was all I could do not to program the Sat Nav there and then.  I drove home in a daze, confused and numb.  When I got home I fell apart, and I can remember sobbing to my husband, “She’s going to die Gavin, and I don’t think I can bear it.”

Freya has been back to Sheffield Children’s Hospital this morning for a follow-up appointment with the Rheumatology Consultant.  He was really happy with how Freya is doing and took one last blood test to see if all her results are back to normal.  We are not expecting any surprises but the last time her blood was tested a few weeks ago, the ESR levels in her blood were still not normal and the doctor would like to see that where it should be.  Had fun trying to catch a wee sample in a pot!  How is it that if I take Freya’s nappy off at home, she pees immediately if I haven’t got a clean nappy at the ready.  Half an hour with her nappy off with me staring at her bits and bobs waiting for any sign of a wee, and I was nearly ready for giving up!  We got there in the end though, wee caught, tested and all ok!

The drugs that Freya was given have suppressed her immune system.  IVIG (intravenous immunoglobulin) uses antibodies as a means of regulating the body’s normal immune response, steroids reduce inflammation and reduce the activity of the immune system and Infliximab is a chimeric monoclonal antibody (artificially developed in mice) that switches off the protein TNF-a which is a protein involved in systemic inflammation.  As a result, she has not been able to have any of her routine vaccinations, missing her 8 week immunisations whilst she was in the hospital receiving treatment means she has a lot of catching up to do and in the meantime we have to be careful about what/who we expose her to, so it’s been a bit of a dull school holidays so far.  Luckily Fin is obsessed with Minecraft, and Eliza is out with her friends most of the time (she’s gone camping with a friend and her family for a few days actually, so that’s been great). I was concerned that I was being over-cautious though and had a good chat with Freya’s consultant about the immunity issue so I could be sure I wasn’t forcing my family to miss out on life unnecessarily.   He was happy with the precautions I have taken.  We tend to limit any outings to things that are outdoors.  If friends want to visit, we ask if they are well and if their children are well.  We also make sure the children are up to date with their immunisations.  We know chicken pox can be dangerous if you are taking aspirin, so Freya needs to stay well away from anyone who has, or may have been in contact with chicken pox.  If I’ve needed to do some shopping (2 kids birthdays in the Summer holidays make that a necessity) I’ve avoided indoor shopping malls.  We shouldn’t fly until Freya is up to date with her immunisations, so no holidays abroad for a while.  The doctor confirmed that Freya can have any killed vaccines now, but mustn’t have any live vaccines until she has been clear of the steroids for 3 months.  Rotavirus and MMR are the only live vaccines we currently give babies in the UK.  Some babies will also be immunised against flu which is in the form of a spray; that’s a live one too so Freya can only have the flu vaccine in injection form.  He said the further away from the acute stage of the illness and the medication we get, the less worrying all this will be.  But he did say it was time we started living a ‘normal’ life.

Normal.  Let that sink in for a minute.  Yes, I guess it is normal for us now.  It’s a new normal, but it’s normal nonetheless.  It has been 8 weeks since we discovered that our baby girl had suffered some damage to her heart as a result of the Kawasaki’s.  Thinking back to those days, back to the first weeks of her illness when she was unrecognisable to us, back to being told our daughter could die…well I don’t even know what to say.  Even typing now I am shaking my head in disbelief.  Did all that really happen to us?  If someone had told me back then that one day very soon I would feel like everything was ‘normal’ I would have laughed in that person’s face.  If someone had told me that one day very soon I would be able to look at my daughter without feeling immeasurable sadness, I would have stared in utter disbelief.  And yet here I am, 8 weeks on from some of the worst days of my life, and I really do feel calm and happy in our new ‘normal’.

I think I’ve already mentioned that during those dark days after we spent a week in the cardiac unit, I was referred to the hospital psychologist.  Let me tell you about that – it was quite amusing really when I look back.  I remember one night in the Children’s Hospital when I was feeling particularly melancholy.  I had either called the on-duty doctor to come and see Freya, or they’d come for a routine check or to take some blood or something.  Anyway, I was talking as the doctor was examining Freya and I must have started rambling a bit about how I felt during my pregnancy.  On the Monday following our stay in Leeds, both the Immunology Consultant and the Rheumatology Consultant came to see me.  The consultant over at Leeds had warned them that I might want to discuss why the diagnosis came so late.  I felt so angry and let down now that I knew that a late diagnosis might well have sealed my daughter’s fate.  They explained how difficult it was to diagnose Freya because she didn’t present the typical symptoms of Kawasaki Disease, and that some of the tests they needed to carry out to rule out other illnesses just couldn’t be done any sooner because Freya was too frail.  Then they said that they understood how terribly difficult this was for me, and that they had spoken to the hospital psychologist to see if she would meet with me.  It was then that I found out there was a note in Freya’s medical notes that I had told a doctor that I had predicted my daughter’s death when I was pregnant!!  Now, I know that I was an absolute mess at times and I rambled and cried to the nurses and doctors a lot, but I’m pretty sure I didn’t say anything as nuts as that!  What I think I said was that when I was pregnant I was convinced something bad was going to happen to my baby, and I was so pleased when she was born healthy and perfect; that it was so cruel that now she was so ill after I had been lulled into a false sense of security.  I guess in the middle of the night the doctor needed to paraphrase, so I became known as some kind of lunatic who believed she could predict the future!! You’ve got to laugh.  Anyway, I accepted the offer of a chat with the psychologist and (after a bit of a wasted trip to see her, a double booking and a few tears) she came to see me in our cubicle.  Seeing her was a turning point for me.  I was desperate for her to tell me how to deal with this, and she answered me simply.  She said you don’t deal with something like this, and there were no tools she could give me to help me come to terms with what we had been through or what was yet to come.

Tuesday 22nd June 2015, 17:00pm

“Mummy had a chat with a Psychologist today.  Her name was Rachel and she was really lovely.  This has been such a tough journey so far my sweetheart, and I’ve needed someone to talk to that isn’t also feeling sad that you are poorly.  Luckily she thinks I am normal, haha!  What I am going through is perfectly normal.

The fact is, no one can make this better.  I cannot control the outcome.  I can only hope that your little heart repairs itself or that it holds out long enough for something to be done.  The lady said that what I am feeling is called ‘anticipatory grief’ and is perfectly normal for someone who has been told their child has a potentially life-threatening illness.

I asked Daddy today if we could have you, Eliza and Finlay christened.  Not because I think we need to do it for any morbid reason, but because I don’t think it can hurt to order in a bit of extra protection for my beautiful children.  I have never been really sure if I believe in God or not…At Leeds General Infirmary I wrote on a prayer sheet “Dear God, prove them wrong”. The odds are stacked against you little one, so lets hope He hears our prayer and reaches out to heal that broken heart.

The doctors have been trying so hard to be positive, always focusing on the great blood results or your observations or just how great you look.  But every time someone says how beautiful you are or how well you are doing it feels like a knife jarring through my heart.  The fact that you look so perfect on the outside makes what is happening on the inside all the more cruel, and even harder to bear.  

Looking at you now, how beautiful and healthy you seem, it is hard to believe there is a possibility that this disease could kill you.  I will always be afraid.”

47 days on and I am less afraid, in fact I go most days without feeling any fear at all.  As I write this, I am reminded of another mum just like me who is at the start of her own terrible journey with her baby boy who was just a little older than Freya when he was diagnosed at 10 weeks old.  I wish I could make her pain go away.  I know too well all the emotions she will be going through right now, because we lived through them at this stage in our journey with Freya.  Sadness, fear, despair, hopelessness, anger, guilt, grief; until all those emotions had taken any reserves I had and I was left feeling nothing at all.  But speaking with the psychologist helped me to understand that I wouldn’t be able to package up any of those emotions and move on.  So instead I started to read anything I could get my hands on, and my research skills and tenacity would really come into their own over the coming weeks.  I couldn’t change where we were, but I certainly wasn’t going to accept our fate without a fight.

So we had a follow-up cardiology appointment in Leeds on Thursday, and those of you who follow Freya’s Story on Facebook will already know we had good news.  The right coronary artery has normalised, measuring 2mm now.  The left coronary artery was the worst affected to begin with, with the largest dilation measuring 5.2mm.  That has now reduced to around 3mm, which was fantastic news!  I asked the registrar if it was unusual to see such dramatic improvement so early on, given we were told it could take 1-2 years for that to happen, and he said that it is unusual, but not so unusual in very young babies.  It seems they are more resilient and able to repair themselves more quickly.  I am still confused about the ‘a’ word.  When the first echo showed dilation of the coronary arteries the doctors didn’t use the word ‘aneurism’.  A few days after that, and after a dose of IVIG, the echo showed that Freya’s arteries were further dilated and there was at least one aneurism.  When we were in Leeds, the doctors talked about the aneurisms and told me the risks associated with that.  Now they don’t seem to use the word, referring more to a general dilation of the arteries, known as ectasia.  I asked the registrar on Thursday to clarify this, and he said that it didn’t matter what we called it, the risks were the same and we shouldn’t get too hung up on a name.  He used the phrase “as far as I can see”, which I questioned.  So now I know that an echocardiogram can only show so much of the arteries; they can see about 3mm into each artery, but no further.  In order to see further into the artery a cardiac catheter is required, which he said would be ‘overkill’ in Freya’s case.  You can’t help but worry when you hear things like that, but I have to put my trust in the doctors that if they felt it was necessary to carry out further tests they would have done it.  For now, I’ll take the good news!  I also asked if the risk of stenosis (narrowing of the arteries) would be increased due to the speed of recovery of Freya’s arteries.  He replied that this is the time that the risk of stenosis is increased anyway, but the risk wasn’t any higher in Freya’s case.  I guess that it is the period in which the repair is taking place that the risk of stenosis is there – it is basically a narrowing of the arteries caused by a build up of scar tissue.  Freya will be seen again in a month, so we will wait until then to see how things are going.  The other bit of good news is that they are going to see if we can be transferred to the care of the Cardiologist at our local hospital, which will make things much easier logistically speaking.  We are still having the battle over her medication though, so the hospital gave us a prescription for her aspirin for the next month and we have left it in their hands to battle it out with our GP.

The thing with this illness is that there are so many ups and downs.  As a parent it is so exhausting.  Being positive one minute and feeling hopeless the next is emotionally draining and really takes its toll on you mentally.  Reading back through my journal to bring Freya’s Story to you has really brought that to light.  One day the entry would be full of sadness and despair, and the next day would be a ‘normal’ day, sometimes even bright and happy.  If you read post number 12, you will have felt the fear and sadness I did on the days after we returned from the cardiology hospital.  The weekend was pretty tough.  I was devastated and I felt more alone than ever.  And yet my journal entry the following day was a complete contrast;

Monday 22nd June 2015, 18:00pm

“What have I got to tell you about today then?  Not very much to be fair.  We had a quiet morning and you slept through until 6am, clever girl!  After a feed you went back off to sleep so I managed to sneak out to Starbucks to bring back some coffee and breakfast.  You were still asleep when I came back.  After I’d finished eating you cried a little, so I took you out of the cot and we snuggled up on my bed just like we did at home sometimes, and you slept in my arms for 2 hours!

Daddy came to see us at lunchtime.  I think he must miss having us at home, especially you.  I gave him the Father’s Day card you made him…Yes, you made it last week at the hospital in Leeds!  They painted your little feet yellow and stamped them onto the card.  He really loved it.  We will save it for you so you can see it when you are older.

 

The doctors came to see you today.  They are very pleased with how you are doing.  Mummy got a bit upset because I find it so hard to hear the doctors talk about how well you are doing when inside your little heart is broken…They want to keep you here this week to continue to observe you, and keep taking blood so they can make sure the inflammation from the evil Kawasaki’s stays away.  The plan is to wean you off the steroids and keep an eye on your blood results to make sure it doesn’t come back when you stop taking the medication…

..We talked about your illness and whether anything could have been done to diagnose it earlier.  The Immunology Consultant said that you have taught them many things about Kawasaki’s as yours was such an unusual case.  

We went to x-ray again this afternoon for another echo scan on your heart.  The sonographer said it was your left coronary artery that has the aneurism.  She also said it has not got any worse since the last scan she did, so that is good news.  Hopefully this means the Drs have switched the inflammation off.  

We have had a lovely day together.  I’ve not thought about the bad stuff once, I’ve just enjoyed your smiles and almost-laughter, and lots and lots of cuddles.  I do hope your story has a happy ending.”

Saturday 20th June 2015, 23:55pm

“Nothing much to say today sweetie pie.  Very uneventful day.  I’ll be honest, I woke up feeling pretty miserable and angry at the world, and so I resolved to spend the day in my pyjamas with the curtains closed.”

We had arrived back at the Children’s Hospital by ambulance late on the Friday so didn’t get to see any of Freya’s doctors.  I remember feeling quite abandoned.  I felt betrayed; like the doctors had all known what was coming but chose not to prepare me.  I now realise that wasn’t the case.  Freya’s doctors knew that her situation was serious, but not being cardiology specialists didn’t feel it was there place to discuss it with us.  That’s why the sent us to the specialists to ensure Freya received the proper care, and so we could all truly understand the extent of her condition.

The Thursday had been all the harder to deal with as my husband was away on business that day, and wouldn’t be returning until Saturday.  I felt it was unfair to contact him while he was away, knowing that he would feel helpless being so far away.  So I held onto it until my mother-in-law arrived for a visit and I couldn’t stop myself from falling apart, despite my best efforts to shield my family from my real concerns.  I arranged for my mother-in-law to sit with Freya at the hospital on the Saturday so my husband and I could go out for a meal to discuss what had happened in Leeds.

As was usual over the weekends, there was very little activity.  That created a solitude that was unhealthy for me given the recent events, and I began to come apart at the seams.

Sunday 21st June 2015, 17:07pm

“I hope you are here to teach me a lesson about faith, because if I lose you to this illness I know that I will never believe in anything ever again.  I’ve reached a point where I can’t even begin to trust that everything will work out okay, because I don’t think that I can face the disappointment.  I haven’t given up on you sweetie, but I’ve given up on hope.  Daring to dream feels like a bad [omen].  Perhaps this is a lesson to teach me what real drama is.  All the thing that have made me angry, sad, hurt in the past were nothing compared to this.  Nothing else could hurt like this does.

I took a beautiful photograph of you today, but I can’t shake the feeling that that photograph is going to haunt me forever.   I’ve been holding you in my arms for 2 hours now, and I don’t want to let you go.”

 

22:50pm

“I am really struggling tonight sweetie.  People tell me i need to be positive, but I feel so much pain I can barely breathe.  I want to believe you can fight this and that your heart will get better, but everything I read suggests that your chances are poor.  If this illness doesn’t get you now, there’s a chance the complications with your heart will get you later.  You are too tiny to fight this.

I wish I could fill this journal with encouraging words so you can see that I always believed you would get better.  Tonight I fear you will never get to read this.  I am consumed with thoughts about losing you, and torn up by fear that I will wake one morning and find you dead.  Every noise, every cry, every wriggle I am jumping thinking something bad is happening.

I have never felt fear or sadness like I am feeling at this moment.  I have no idea how I am supposed to handle this.  Every time you smile, my heart breaks a little more.  I could not bear to see a day where I wasn’t greeted with that smile.  You have been here for only 10 weeks and yet I can already see what an amazing little girl you are.  I need you to get through this and live a long and happy life because I know you are going to be special.

I am not only scared of losing you from my life, but I am petrified by the unknown, and by how suddenly I would lose you if the worst were to happen.  I am scared that I could lose you right in front of my eyes and there would be absolutely nothing I could do to protect you.  If only love were enough to keep you safe; the love I feel for you would protect you from anything.  I don’t think I could live a life without you in it.  You have made me whole.  Without you I am nothing, I am worth nothing. The person I was before you came into my life is not someone I want to be.  I can only exist in this world whilst you are a part of it. You are the best of me sweetpea.”

The next morning the doctors would notice a marked change in my manner.  In the weeks before our transfer to Leeds, I had been inquisitive and challenging; the doctors knew they needed to come armed with information and one said they had a lot of respect for me for the amount of research I had done, and how much interest I had shown in the full details of Freya’s condition.  That morning (Monday 22nd June, Day 23) was different.  I had nothing left to give.  No amount of questioning was going to change the fact that my daughter was forever changed.  It didn’t matter to me anymore.  The doctors commented on how well Freya looked and I nodded, whilst inside I was screaming! Who cared what she looked like on the outside?  On the inside she was broken.  My changed behaviour would earn me a referral to the hospital psychologist who I would see the following day.

Writing about it tonight brings those feelings flooding back as if it were happening now.  Back then I would never have believed that I would be sat writing a blog whilst my daughter slept upstairs.  I would never have believed that I could feel any different than I did in those dark days.  Some days are brighter than others.  Mostly we just get on with daily life, I mean you have to when you have children don’t you?  I still have to listen to tales of Minecraft and complain to my daughter about her untidy bedroom.  I have ironing to do and meals to cook, and find that I can take my eyes off Freya for long enough to do all of those things.

Tomorrow we are off to Leeds for Freya’s 3rd follow up echo.  The first following our stay up there showed a 20% reduction in the most badly affected sections of Freya’s coronary arteries.  Even the Cardiologist remarked aloud, “Wow!”, when she took the measurements.  It was more than we could have hoped for so soon after her diagnosis and I wept with relief.  The next scan showed no change, however that was to be expected and it could take 2 years for us to understand the long term effects on Freya’s heart.  Tomorrow we also hope to secure a more robust plan for Freya’s medication.  Our GP is not willing to prescribe aspirin for an infant as young as Freya despite the necessity for it, so we are currently battling to get a better solution than hours of travelling to pick up a prescription whenever we run out.  It is a hurdle we could really do without.

Not many people like to say their worst fears out loud.  I guess I’m different.  For some people voicing their fears can give those fears validation that is not welcome.  Some worry about being judged by their words.  Some are too concerned with what they should say and not what they feel.  We all have our own ways of handling our issues.  For me it can be destructive to keep my thoughts in my head; kept in there they play over and over like a stuck record and my old friend ‘Ant’ makes them her friend (for those of you who haven’t read my earlier bluemama posts, let me introduce you to Ant (Automatic Negative Thoughts)).

When I started blogging, I made a decision to be completely candid about whatever I was feeling, be it light or dark.  For me that is the only way I can really even try to help others who may suffer similar feelings or worries; to help those people who might not want to say it out loud realise that they are not alone.

I said in my last blog that the most cruel thing about Kawasaki Disease is the unknown.  We don’t know what causes KD.  There are a number of theories, from carpet cleaner to soy, and a strong opinion that it is a combination of environmental and genetic factors with a viral trigger thrown in for good measure.  Because no-one knows the cause, no-one can create a test and as a result many cases go undiagnosed or receive a late diagnosis like Freya.  Whilst it is rare for KD to reoccur, there are no guarantees that it won’t.  We are unsure how long Freya will continue to display the symptoms, what other health issues she may encounter throughout her life, or whether her little heart is strong enough to repair itself.

I guess it’s time to share with you the worst day of my life.  Thursday 18th June.  Day 19.

Thursday 18th June, 07:30am

“I could tell you that during your time in hospital I never left your side, but that wouldn’t be true.  Sometimes your Auntie would come to visit and take me somewhere for tea to make sure I was eating a proper meal…And last night I decided that the best thing for me was a good night’s sleep.

I left you on the HDU ward at about 10:30pm last night.  I changed your nappy, fed you and gave you your medicine and left you in the care of the nurses.  I trust them to do a fantastic job of looking after you, but it still didn’t feel right walking away.

…I had a full 8 hours sleep and do I feel better?  No, I feel even more tired than I did before, and to add to that I feel wholly wretched for leaving you…I arrived at the ward only to be sent away because the nurses were doing their handover.  So now I am sat in the parent room…writing in this journal, with no idea if you are awake or asleep, happy or sad, well or unwell.

Today is a big day – new drugs.  If everything goes according to plan we could be home by the weekend.  But this rollercoaster journey has taught me not to look too far ahead for the good stuff because we only end up disappointed.” 

That day Freya received an infusion of a drug called Infliximab.  We had to give our consent for it to be given to Freya as it is not a human product; it is made from the antibodies of mice.  At around 4pm I received a message from a friend, “How did Freya tolerate the new mouse IV?!?!” to which I replied “She’s very chilled.  One might say as quiet as a mouse!!!”  It took a number of hours to infuse, and Freya was closely monitored for a number of hours after for any signs of her body rejecting the drug.  Just like everything else on our journey, Freya took it in her stride, and it was considered a success.

In the afternoon, Freya’s Cardiology Consultant came personally to perform an echocardiogram.  The Rheumatology Consultant had kept his promise, and the Cardiologist was very informative, explaining everything that she could see on the screen.  She showed me the normal sections of Freya’s coronary arteries, and the bulges (the aneurisms), the largest of which was 5.2mm in diameter.  To put that in perspective, Freya’s heart would have been about the size of her fist or a large walnut.  The coronary arteries in a healthy baby’s heart are approximately 1mm in diameter.  Freya’s arteries were the size of a teenagers.  The consultant asked me if I understood the risks involved with Freya’s heart condition.  I said that I thought I did, that I understood that her blood needed to be kept thin so it would continue to flow freely through her arteries and not swirl and clot in the aneurism to prevent a heart attack.  I then said that I felt like Freya was a ticking time bomb.  I remember looking right into the Consultant’s eyes, waiting for her to give me a smile and tell me that I was over-reacting and that Freya’s situation wasn’t that serious.  She didn’t.  Instead she said “Let’s go somewhere private and talk shall we?” 

My heart was beating out of my chest as she led me to a room down the corridor from the HDU.  We went in and sat down; the consultant, our nurse and me.

Friday 19th June 2015, 07:38am

“Yesterday I left you with a note about thinking positively.  What irony there is in that as I write this morning.  On Tuesday we were brought to [the hospital] to see specialists in cardiology.  Their job is to fix hearts, but instead they broke mine when they told me they can’t fix yours. Can’t.

I didn’t update the journal yesterday even though it was an eventful afternoon and there was lots to tell you.  But I couldn’t face telling you.  It seems that in all of this ‘excitement’ and all the knowledge that I have picked up along this journey, I appear to have suppressed the bit about the risk of a coronary aneurism rupturing.  That’s a pretty big deal.  And it could happen to you.  And they’ve told me that there is nothing they can do about it now, and there will be nothing they can do about it if it happens.  So that’s that then.  Seems I was right about you being a ticking time bomb sweetie pie.  And right now I am really not sure how I am supposed to deal with that little nugget of information.

I have never been so scared of anything in my whole life, and I will never feel this scared again.  Losing you would be unimaginable.  My heart is already broken at the very thought of it.”

I will never be able to forget that day.  When we were all sat down, the doctor began to talk.  She reiterated what she had already said about the risks when we were out on the ward.  She said that one risk was that if the blood was able to swirl around within the bulge, it could cause a clot which would result in a heart attack.  That is why Freya would be taking aspirin for life.  She told me that there is a risk that if the aneurism begins to repair itself, it can become too narrow which would also cause a heart attack.  That is why Freya would have regular monitoring, ECG and echo scans to keep an eye on things.  Then she told me of the third risk.  She said, “There is a risk that the aneurism could rupture.  There is nothing we can do to prevent that from happening, and I’m sorry to tell you that if it does happen, there will be absolutely nothing that we can do.”  Those words will never leave me.  I immediately broke down.  I thought I’d cried before, but I literally sobbed so hard I could hardly breathe.  The doctor continued to talk, but I couldn’t tell you what she was saying because all I could hear were the words that will haunt me for the rest of my life.  Both the nurse and the doctor eventually fell silent and all that could be heard were my cries.  I think deep down I already knew.  I’d referred to her as a ticking time bomb on a number of occasions, and no-one had corrected me.  But I’m a neghead aren’t I?! I’m the one who always looks on the negative side and should think more positively.   They were supposed to tell me to get a grip, that I was being a drama queen.  They weren’t supposed to tell me that this disease could take my daughter away from us.

Eventually I composed myself and felt that I just needed to get back to Freya, so I left the doctor and headed back to the HDU with the nurse.  I can picture the scene as if I were watching a silent movie.  I turned the corner into the room intent on picking my baby girl up in my arms and never letting her go.  Except when I rounded the corner and saw her I couldn’t bear it, and I backed away and ran for the door.  The nurse followed me and I fell into her arms.  I didn’t know how I was ever going to embrace my little girl again, knowing that she could be taken from me.  I talked for a while longer with the nurse, and after some time returned to the ward.  The parents of the other 3 babies on the ward were with their children, so the nurses closed the curtains around Freya’s bed.

That evening I cried and cried, and I emotionally distanced myself from Freya.  I dreaded feeding time, using the fact that she was attached to the monitors as an excuse for not picking her up.  I felt like I had to stop myself from loving her any more than I already did.  We were sharing the HDU with 3 others; 2 babies and a 7-year old boy who had all undergone heart surgery.  I felt like we were frauds; that it was unnecessary for Freya to be in a high dependency unit and that she was taking the nurses time away from those more severe cases.  That morning I had cried with the nurses about the fact that I felt we were in the way, that we were using up valuable bed space for more serious cases than Freya’s.  The nurse said Freya wouldn’t be in the HDU unless it was considered necessary, and I sneered that they would if they didn’t have a bed for her anywhere else.  That night I cried bitter, angry tears.  I could not believe that earlier that day I had felt we were a burden, that Freya wasn’t as sick as the other babies on that ward.  I suddenly felt angry at the other babies.  They were being fixed, but they wouldn’t fix mine.

I could leave it there, but I don’t want to leave you with the fear that I had on that day.  A lot has happened since then, I have a much better understanding of Freya’s heart condition now, and there has been some improvement since that day.  Freya is currently what her consultant describes as “nice and stable”.  The build up to her follow-up appointments causes me huge anxiety that manifests itself in shouting at the kids or nagging at my husband.  It also awakens the fears that I try to suppress on a day to day basis.  I feel physically sick as I climb the stairs when I go to check on Freya whilst she is sleeping.  I am always afraid of what I might find.  People have asked me if I sleep ok.  I sleep perfectly well.  It’s waking that I am most afraid of, too scared to open my eyes sometimes.  The relief I feel when I hear her move or breathe is intense.

My husband and I thought long and hard about what we should or shouldn’t share about Freya’s condition.  We didn’t want our children to find out things we’ve decided not to tell them.  We didn’t want Freya herself to be told before we have told her what she needs to know.  We didn’t want to be judged by people who might see us doing things with Freya that they think we ought not to be.  And we didn’t want pity.  I don’t want people to look at Freya with sadness.  There is a strong chance she will beat this thing; she is after all our miracle.  She is a beautiful girl with a lovely temperament. She sleeps through the night, and wakes up smiling.  It is impossible to stay sad for long in her presence – a less content baby would have made this journey all the more difficult.  She has softened every blow with her bright blue eyes and cheeky smile.  My love for her is overwhelming and I will forever be in awe of her.  Sometimes when I’m holding her and she is smiling up at me, a sadness seeps into my consciousness as I contemplate a world without her in it.  But I bat it away because although it is true that the day her heart broke mine did too, she doesn’t need a mummy who is sad all the time.  She needs me strong, smiling and by her side.  Always.

http://www.facebook.com/freyasstory

The most cruel thing about this illness is the unknown.  The second most cruel thing about this illness is the false sense of security that it lulls you into when everything seems fine on the surface.  You can’t see what Freya has been through when you look at her.  What you see is a beautiful, happy little girl.  She is in all aspects (with the exception of the Kawasaki Disease and it’s ‘gifts’) the perfect baby.  She sleeps through the night from 7pm to 7-8am, and when she wakes it is not with a cry but with chatter and that beautiful smile.  With the exception of a couple of symptoms that remain – thick yellow finger nails, one of which came off yesterday and sore, dry lips that crack and bleed when she cries – she looks the picture of health, and other cliches.  Her last follow-up appointments were 3 weeks ago.  Since then we have been allowed to ‘forget’ about the journey we have been on.  Life has become the usual routine again; washing, ironing, picking up after Freya’s siblings.  The 6 weeks we spent in hospital seem like a distant memory, the only reminder being in the mornings when I prepare Freya’s daily medication.

Tomorrow is Freya’s next cardiac follow-up appointment.  And just like that, we are shunted from our 3 weeks of ‘normal’ and back into that dark place where we live with Kawasaki’s.  Just then my fingers hovered over the keyboard for minutes trying to think of what to write next.  I don’t have the words.   I’ve often referred to this journey as a rollercoaster, but the more I think about that the less appropriate it seems.  I love rollercoasters.  Love the breathless anticipation as the car makes it’s ascent up the tracks.  Love the exhilaration as you tip over the brow and race downwards, screaming wildly with joy.  I’d say the climb has some similarities, it’s just that the drop brings no joy.  It is a nosedive into a deep dark chasm.  Tonight I have a heavy heart, as again I am given a stark reminder that we are still on this ride, and we have no idea when we will ever get off.

Returning to the hospital where my worst fears became a reality always shakes me.  The memories of that week at Leeds General Infirmary are still so raw.  Up there I felt my most vulnerable, isolated and alone.

Tuesday 16th June 2015, 23:00pm

“I am sat by the side of your cot in your new temporary home.  We arrived at Leeds General Infirmary around 1pm and you were taken immediately into the ultrasound room for the new doctors to carry out another echo on your heart.  I can’t remember much about what was said as I seem to have hit a brick wall this afternoon after very little sleep last night.  What I do remember is that they confirmed what Sheffield had reported…I am too tired, and I am too exhausted with bad or worrying news.  You got very hot in the ambulance and all through the scan you just got redder and redder.  I could feel the panic rising inside of me like bile from the pit of my stomach.  They took your temperature: 36.4. Phew!  I cried with relief.  Last week I cried every time your temperature peaked, and now I’m crying when it doesn’t!

A new doctor came to see you today.  He is [the rheumatology consultant’s] equivalent…He wants to test your blood in the morning, and see the results of a further echo before he will make the decision about giving you another drug (Infliximab).  At the minute we are not sure whether you have beaten the Kawasaki’s, nor do we have a full report on the condition of your heart.

You are currently on the high dependency unit within the children’s cardiology wing of the hospital.  The staff and surroundings seem lovely, however I am not allowed to sleep with you on this ward, and have been given a key to a room in the parent’s residence.  I cried a lot when they told me I couldn’t stay.  I’ve been by your side all through this adventure sweetheart, I couldn’t leave you now in this strange new place.  I am sure they all thought me a very foolish and over-protective mother, and all of them tried to reassure me that you were in good hands and encourage me to go and get some sleep, but no matter what they say I will not leave your side tonight.

Daddy and I walked down to the parent’s room to drop my bags off.  The room they expect me to sleep in, away from you, is in a completely different wing of the hospital!  It is a 10 minute walk from where you are.  There is not a chance that I would spend tonight alone that far away.  I know you wouldn’t know I was gone, but I think I need you more than you need me at the minute. I love you dearly…These past few weeks when you were not yourself I have missed you more than you can imagine.  Now that you are back with me, and you know who I am…I don’t want to let you out of my sight. You are too precious to me.”

The nursing staff tried to encourage me to leave Freya and go to my room for some sleep.  They said that it was important that I look after myself to have the best chance of looking after her.  But I was stubborn and resolute; I would not be leaving her alone.  I fed Freya at around 1am and could feel myself dropping off as I held her in my arms.  After I laid her down to sleep I sat for some time considering what I should do, worried that if I stayed awake for the next feed I could cause her harm if I fell asleep.  I thought about it for 2 hours, and at 3:30am I slowly rose from my chair and with tears rolling down my cheeks I left her.  I am crying now, as I recall that memory.  I felt so much fear, sorrow and guilt; I still feel it now.  I guess it was around 4am when I finally settled down in my bed, although it was an anxious sleep mainly because of the situation, but partly because of the LGI ghost story that had gone viral on Facebook the week before!  I slept with the light on and one eye open!  When I awoke, I showered and rushed back to the ward where Freya was sleeping soundly.

“I guess you didn’t even miss me, but every minute I was away from you felt like an eternity.” (Wednesday 17th June 2015)

Freya had another echo that day (Wednesday 17th June, Day 18).  There had been no change from the previous day, but the doctor confirmed that Freya’s aneurisms were large and that the aortic valve continued to leak.  They said that otherwise her heart function was fine, but that she will need follow-up care for life, and would need to take blood thinners (aspirin) for many months to come, if not forever.

The Rheumatology Consultant came to tell us that he wasn’t happy with the inflammatory markers in Freya’s blood sample, and that it was time to tackle the Kawasaki’s more aggressively.  I had to give consent for Infliximab to be prescribed.  I believe it is unlicensed for use in children.  The hospital had to apply for funding for it as they did not have permission to use the drug in a baby as young as Freya.  The drug would switch off a protein in Freya’s blood called TNF alpha – one of the risks associated with that is increased potential of developing lymphoma, although the doctor assured us that this was more likely where there was prolonged use of the drug.  Freya would have only one dose to begin with.  It was no choice at all really, but we had to put our trust in the doctors and they were clear that we needed to switch off the inflammation to prevent any further damage to the coronary arteries.  I asked the consultant if I would have a chance to speak properly with the cardiology consultant as I felt that I understood the Kawasaki’s Disease pretty well, but was less confident in my understanding of the effects that it had on Freya’s heart.  He told me he would make sure I had some time with the consultant the next day.

And so we prepared ourselves for another drug.  Freya had been on various antibiotics (amoxicillin, meropenem, cefotaxime and gentamicin), antiviral medicine (acyclovir), paracetamol, ibuprofen, steroids, aspirin and a drug to protect her stomach from the drugs (lansoprasole).  She had received a blood transfusion, and two doses of immunoglobulin.  The next day she would receive yet another drug – the last they could really offer to help her situation.  I did not know that that day would also be one of the hardest days of my life.   Whether I am able to write about that tomorrow will very much depend on the outcome of our follow up appointment.  Keep your fingers crossed for her, will you?  And if you believe in God, please keep her in your prayers.