Over the past two years, I have used my writing and my desire to make a difference to raise awareness of this little known illness called Kawasaki Disease. It all started with a journal that I was given whilst my daughter was in the hospital, a journal whose pages I would fill with thoughts and feelings every night whilst I lay there on a camp bed by my daughter’s side, a journal that would come home from the hospital 50,000 words richer.
Since I began sharing Freya’s Story, I have received many compliments about my writing, or my dedication to the cause. I’ve sometimes been called an inspiration! And whilst I enjoy those compliments very much, the words and comments leave a bitter-sweet taste because they are compliments I would rather not have been in a position to receive. At times I feel like a fraud, particularly when people say that without my perseverance, Freya might not have been here today. Those comments make me feel like a fraud, because in the beginning, in that first week of Freya’s illness, I did nothing. Nothing.
Freya had just passed the 7-week milestone when she became sick. Up to that point we had spent some pretty uneventful days at home, where I lapped up the new arrival. It is no secret to those of you who have read my blog from the beginning, from before Freya was born, that I was not particularly happy when I discovered I was pregnant. With two children, aged 11 and 6, I already had the family I wanted. I was approaching 40, and had just obtained my biggest promotion at work. I couldn’t believe that I had gotten myself into such a mess, and in all honesty the pregnancy severely affected my mental health for some time. And then over time my feelings settled, and I began to look forward to the arrival of our little girl. It felt somehow that she was going to change things dramatically, that she was going to change me. Boy, did I get that right! I will never forget the moment that I held her for the first time – I wanted to hold her so tight that I was frightened that I would squash her. I remember asking the midwife over and over “Am I hurting her? Am I hurting her?” and they laughed and said of course I wasn’t! I had been there twice before, but this was different. Like somehow the journey I had been on through the pregnancy made her arrival so much more poignant. She was a gift; a gift that I had failed to acknowledge for much of the time that she was growing inside of me, but my love for her once she arrived was more than strong enough to make up for that. I was smitten.
As I’ve already mentioned, those early weeks went by pretty uneventfully. I had to remain in hospital with Freya for a few days after the birth as I had tested positive for Group B Strep during the pregnancy and had to receive intravenous antibiotics during labour to prevent the infection from being transferred to the baby. Getting those antibiotics in time was a huge source of anxiety for me. Throughout the pregnancy I had been plagued with thoughts that my baby would not arrive, and when I discovered that I was GBS+ and learned of the associated risks to my baby, I felt I had met her killer. But once she was here, and we had the all clear to go home, all of those worries disappeared. Having learned the hard way from rushing to be supermum with the other two kids, this time I spent most days laid on the sofa with Freya in my arms, developing a bond like I have never known. I watched movies as she slept on my chest, and I can remember only the sweet joy I felt from being in her company, just us two. The first real adventure we went on was a trip to Cannon Hall Farm, a local visitor attraction. That was on Saturday 30th May; Freya became sick the next day*
*The cause of Kawasaki Disease is still unknown, despite 50 years of research. There is no evidence that I am aware of, of any connection between Freya’s illness and our visit to the farm, and I wholly believe that the timing was purely coincidental. That said it took us a long time to return there, mainly because of the pain of the memories of it being our last day with our healthy baby girl.
Freya’s illness initially manifested itself with a loss of appetite. She awoke in the early hours of the Sunday morning, but she seemed to have a cold and was struggling to take her milk with her nose so snuffly. I tried for a while, but she just fell asleep with the exertion so I let her sleep until she next awoke. It was after the refusal of the next feed, that I became concerned; not because I thought she was ill, but because I was worried about her not feeding. That’s when I noticed her temperature. It was my intention to give her Paracetamol, but when I saw the bottle said ‘from two months’ the goody-two-shoes in me wouldn’t let me give it to her without seeking medical advice. So it wasn’t instinct that made me call the doctor – it was my conformity to rules! Nevertheless, the call was an important one to make and the out of hours doctor said that I should take her to A&E as it was unusual for a baby of her age to have a fever without an underlying reason. That’s when I remembered the GBS – my worst fears were becoming a reality and the word ‘meningitis’ seeped into my subconscious.
At the hospital, they checked Freya over and could see that she was working pretty hard to breathe (you could see her little tummy moving up and down instead of her chest), and the fever was soaring. I remember telling them about the positive GBS result, and that she had been born within such a short time of the antibiotics being administered. We went up to the children’s observation unit with her where we held her practically naked body as she slept in our arms. I remember having to obtain a wee sample, which involves catching a wee in a kidney dish – no mean feat with a tiny baby, and we celebrated when we managed it! What happened next is a bit of a blur, but I think because of her symptoms, and my warnings about the GBS, plus the standard protocol to treat a feverish infant for meningitis as a precaution, they decided to admit her to a ward and give her intravenous antibiotics and antipyretics.
I know I have told you all that already in previous blogs, but I’m telling it here to illustrate the point that this wasn’t because of my instincts. We were here because of the instincts of the medical professionals that this tiny baby was just too precious to send home.
If you’ve read my blogs you will also know that after the medication had been administered I laid Freya down in the hospital cot and tried to get some rest in the chair in the corner of the room. You might recall that as I laid her down, I could smell that she had dirtied her nappy, and you’ll remember that I chose to ignore it, to allow my exhausted baby to sleep without disturbing her again. You’ll also know that I hadn’t sat back down in the chair for any more than a few minutes before I decided I could not let her sleep in a dirty nappy, and rose from my seat to change her. She would sleep better if she was clean. Was it instinct that got me out of that chair? No, I don’t think so. I think it was common decency and a conscience. Whatever it was, it was the single most important decision that was made, because as I changed her nappy I noticed that her body was covered in a rash. I hadn’t noticed that Freya was unresponsive when I changed her nappy. She was sleeping, but I was pretty adept at changing nappies without waking the baby – I was like a ninja! I can’t remember if she felt different. I called the nurse, the nurse called the doctor, and from that moment I was introduced to a whole spectrum of feelings that I will never forget and never want to endure again. I can’t remember much at all, except the way the atmosphere in that room changed when the doctor and also by then a consultant, uttered the words “shutting down” and started to wheel my baby out of the room.
Two years on I can still see myself standing in that corridor in utter shock. It was like something from a TV hospital drama. It wasn’t real. I called my husband to tell him he needed to come to the hospital. I think it was around 6am. I had this sinking feeling that my baby was dying right there in that room, and I remember how alone I felt. I walked slowly into the high dependency unit where they were working on my tiny little girl, pumping her with fluid boluses to resuscitate her. I stood at the foot of the cot, my hands gripped around the bars, and just watched in silence as the doctors calmly gave orders to the nurses to hand them this, and pass them that. It was how I imagine an out-of-body experience to feel. Even now, I have to wipe away the tears at that memory.
Clearly they managed to stabilise Freya, but she was incredibly sick. They continued to keep meningitis at the top of the list, particularly as she had gone into septic shock. A number of different antibiotics were being delivered to her tiny veins through cannula sites in every place they could get one. We would remain in the HDU for 3 days, where Freya was considered stable but no better. The temperatures continued to soar, she was tachycardic (elevated heart rate), and she was being fed through a nasal NG tube. On the Wednesday (day 4) she was considered stable enough to move back onto the ward. All I remember about those days was how quickly I settled into a routine of getting up and showering in our en suite cubicle, before Freya would wake. I would try and sleep when she did, but I was too distraught to harbour much sleep. I chatted with the nurses as they came and went, and it felt like we had been there for weeks. I ate when family brought me food, but I rarely left the room. Sometimes at night, my best friend would come with reinforcements and entice me out for a break and some food. It was during those meals that I aired my fears to my friend; what if she dies?
Freya’s CRP levels (the markers in her blood that show infection/inflammation) were dropping slightly day by day. When she went in her CRP was over 340 (normal is under 7). I took the downward trend as a good sign, but hadn’t considered that even as it was dropping it was worryingly high. To give you some context, Freya wasn’t released until her CRP dropped below 7, which would be 6 weeks after she was admitted. I don’t know what I thought was happening. I think I believed that she had meningitis, and that she was on the right medication to treat her for that. She needed a lumbar puncture to be sure, but was too sick to undergo the procedure until the Friday (day 6). They took blood samples every single day, but none of them grew any cultures. Around the middle of the week, a nurse came in and said “You know they’ve found it don’t you?! They’ve found rhinovirus!” I remember feeling so silly! I had taken my daughter to the hospital with a cold! I think I must have been in shock, because I actually believed they would say “Mrs McBride, Freya has a cold, you can take her home!” Even with the heart monitor constantly alarming as her heart rate soared, even as her temperature continued to spike, even when she was unable to feed on her own, with cannulae in her ankles, her wrists, her head! Even when she stared at me vacantly from that cot, and I yearned for cuddles that I couldn’t give for fear of dislodging the wires and tubes. What was going through my head? It was like I really believed they would just switch all that off and let me take her home. And yet, deep down I knew that something wasn’t right. I still had an overwhelming fear that she was going to die. But I’ve been told all my life that I am a neghead, a catastrophic thinker, so I batted that away as irrational and ignored my instincts, even though the doctor’s faces betrayed their fear. I remember remarking to the consultant that Freya had rhinovirus, hoping that he would confirm how stupid I had been to worry. But his response was “Yes she does, but rhinovirus doesn’t make a baby this sick.” I think that’s when I finally allowed myself to believe what my gut was telling me; that Freya was gravely ill.
The lumbar puncture would come back negative. Elevated white blood cells gave them reason to believe she may well have had meningitis, but that the medication had already begun to work, but the test was inconclusive. I remember how scared I was when they told me to hold her after the procedure. They said I had to keep her flat – I was petrified that I would make a move that could cause her a serious injury. Every test she endured during that week she had me by her side. All but the lumbar puncture. For that, the nurses asked me to stay away, and to be there for the cuddles afterward. I took myself far away from the treatment room, but I knew my baby’s cries when I heard them, as they bent her tiny body into the position necessary to carry out the test. That would be the first of the three lumbar punctures they carried out, but the other two were done under general anaesthetic in theatre so again I wasn’t present.
It was on day 7 that I told my husband how afraid I was that the doctors weren’t telling me something. I said I could see the concern in their faces, eyes that betrayed their calm words. It was then that I asked my husband to meet with the consultant. He had to do it, as I needed him to ask them a question that I couldn’t bear to hear the answer to. I needed to know if they thought there was a possibility that she could die. That is when she told my husband that on a scale of 1-10, with 11 being dead, Freya had been a 10. My instincts had been right, and I just didn’t have the faith to believe in them. The following day, Freya would be transferred to the specialist children’s hospital as the local hospital had exhausted its investigative scope. I do recall the consultant saying that if it were his child, and they had been sick for 8 days without diagnosis, he would be demanding answers. He said that the children’s hospital would be able to scan Freya’s brain, and do an echocardiogram on her heart. Still no alarm bells rang for me – belts and braces, I thought.
It is worth stating that in that first week, the only notable symptoms were a fever that would not abate with the usual antipyretics (Paracetamol/Ibuprofen), and a rash. Freya’s lips were very red, sore and cracked, but this considered a symptom of dehydration with her being tube fed. I had commented that her ankles and wrists looked swollen, but it was attributed to cannula damage, particularly as the cannulas were constantly having to be replaced due to failure. The staff did not give me any cause for concern, and I didn’t therefore consider that all of these were possible symptoms of some strange disease. Why would I? I mean that kind of stuff happens to other people doesn’t it!
It was in that first week that I should have asked Dr Google’s advice. The fact that I didn’t will haunt me forever. Do you know what the top result is if I put this question in my search engine…
Question: “My baby has a fever, a rash and cracked lips.”
Answer: Kawasaki Disease – Symptoms – NHS
I didn’t ask Dr Google though, and I have no idea why not. Trust? Complacency? Ignorance? Perhaps all three, and other reasons too. I simply had no reason, or point of reference, to lead me to believe that I would need to look it up myself! We were in the hospital, surrounded by doctors and nurses with many years training and experience. You don’t ask Dr Google, you sit back and watch and wait and let the professionals do their job. You are in the right place, and they will make your child better. That’s how it works. Perhaps I had the instinct, but didn’t know what to do with it? Not once did the thought enter my head that my child could have something seriously wrong with them that the doctors were missing. Not once. I’ve literally driven myself crazy for two years asking myself why I didn’t see it. Why I didn’t ask the right questions. I’ll never get that answer, and will have to live with the consequences of that.
Perhaps if I’d read about this weird thing called Kawasaki Disease, I’d have associated the lips and the swollen extremities as symptoms and made them see? Perhaps when the consultant mentioned a heart scan, the penny would have dropped and I would have asked him, “Do you think she has Kawasaki Disease?” Perhaps if I had, they would have talked to me about it, and we would have agreed that she met the brief, and not for just an incomplete case as was finally diagnosed on day 13 of her illness. No, she had all the symptoms they needed for a complete diagnosis. My old boss once described me as being like “a terrier after the postman.” When I get a bee in my bonnet, I rarely let it drop. Had the KD seed been planted, I know I would have done so much research in those twilight hours when I dared not go to sleep that I would have made the connection. I know that when they did an echo on day 9 that showed no damage that I would have told them that didn’t mean she didn’t have it! 75% of children with KD don’t sustain any heart damage, and you can’t use a clear echo to rule the disease out! I know when the new rash appeared, that I would have recognised it from pictures I would have seen plastered all over the internet! There’s no mistaking it. I might even have known that young infants are more likely to suffer large and giant coronary aneurysms, that they are more likely to be resistant to standard treatment (IVIG) and that they are more likely to get a severe case of the disease that results in a dangerously low platelet count (thrombocytopenia), not the usually high platelet count that is characteristic of Kawasaki Disease. Had they decided to be open with me about their fears or suspicions, had they told me that KD was the prime suspect, I would have made them give her the treatment and not allowed them to rule it out. Which is what they did. They ruled it out on day 9, and she went through further tests including an MRI and a bone marrow aspiration in theatre, both of which failed to give them an alternative. I wonder how it felt to see her coronary aneurysms when they showed up on an echo on day 13….
Now that sounds like I am blaming the hospitals. Maybe I am? I don’t know. I do know that it wasn’t negligence on their part. They worked so hard to find the answers, and were thrown off the scent by this insidious disease. I know how important it was to them that Freya get well again. They know I was angry, in fact they told me how upset they were that I was angry with them because they felt that they did everything they could. I won’t lie. I will always feel angry that they were looking for it and still allowed themselves to be misled. Could they have known more about the disease? Enough to give them the conviction to go with a diagnosis regardless of that first echo? I know they had to make certain decisions, and that the treatment for KD could have proven catastrophic had it had turned out to be Leukaemia as they had also suspected. And no amount of blame will change where we are. What I am thankful to them for is how they treated the illness once the diagnosis was confirmed. Their aggressive treatment of the disease and their over-cautious approach to monitoring from that moment was fundamental to her recovery, and I truly believe that their actions from that point saved her life. I will always question the decisions made by both hospitals leading up to Freya’s diagnosis, perhaps because it would feel easier if I had someone to blame, but their actions afterward more than make up for any misjudgement.
Perhaps it’s one of the reasons why I can never forget those early days in the hospital? Perhaps it’s the main reason why I do what I do to raise awareness of the disease? I cannot bear the thought of another parent feeling the regret that I do over questions I should have asked…
Whatever the reason, I know that I will always wonder if I could have helped my daughter get an earlier diagnosis and more timely treatment. I will always wonder if I could have stopped her heart from breaking. I can’t change what happened to her, but perhaps I can swallow some of that guilt and grief if I can change it for someone else. So know the symptoms! Let our story show you how important it is to advocate for your child. Ask questions – direct ones! What do you think could be wrong with my child? Make them tell you what they are thinking! And when you get some idea, and you think your child is displaying symptoms that give you even the remotest possibility of this disease, then ask them this…
“Tell me why it ISN’T Kawasaki Disease!”
It could be the most important question you ever ask.
If you would like to follow Freya’s progress, you can like and follow her story on Facebook (www.facebook.com/freyastory), Twitter (@freya_story) and Instagram (@freyas_story).
For more information about Kawasaki Disease, please visit the NHS Choices website: www.nhs.uk/conditions/kawasaki-disease/pages/symptoms.aspx
If you know of anyone who is dealing with this disease and would like support, they can contact the UK Support Group through their website www.kssg.org.uk, or visit the KD Foundation (US) www.kdfoundation.org
To donate to Kawasaki Disease Research, please visit my fundraising page: www.virginmoneygiving.com/joannemcbride – all funds raised go directly to the COSMIC Kawasaki Disease Research Fund, registered charity number 1043697)
You can also visit the UK Foundation for Kawasaki Disease, Societi, to see the work they are doing to change the face of this disease: www.societi.org.uk