These last few months have been some of the toughest I have ever had to face, and I have experienced a whole plethora of emotions from sadness to anger, and every feeling in between. I have even experienced grief, which was a particularly hard thing for me to deal with as it has been well over 20 years since I lost someone dear to me. And I know that Freya is still here, very much alive, but I grieved in anticipation of the possibility of loss, and I grieve still for the loss of her healthy heart.
Kawasaki Disease doesn’t rule my life quite as much as it has done over the last few months. I still dedicate a lot of time and effort to making sure Freya is receiving the best possible care, but mainly to satisfy myself that I understand the care she is getting because that’s the way I am. I’ve never been one to accept something just because someone tells me that’s how it is. I like to be fully informed, and I like to question the ins and outs until I am comfortable that information and decisions are accurate because I know that people don’t always get it right. Not even me, haha! No, Kawasaki Disease has started to take a back seat to normality as we get on with being a family again.
One of the things that has niggled me the most is the notion that the hospitals took too long to diagnose Freya’s condition. I have at times been angry and judgemental about the fact that they were unable to reach a diagnosis within the ‘safe’ window, and that it was too late by the time they worked it out. After our stay in Leeds, where I had the worst day of my life (reference Freya’s Story (11)), I returned to Sheffield Children’s Hospital angry and bitter. I felt that Freya’s doctors had failed to prepare me for what was about to unfold, and accused them of sending me to Leeds like a lamb to the slaughter. I had not imagined that my worst fears were about to become a reality; up to that point I believed that my negative thinking was going to prove unfounded. Both the Rheumatology Consultant and the Immunologist came to see me when they heard that I had questions, and I asked them pointedly “Thirteen days?! Why did it take you so long?” I remember thinking that I could read the hurt in their eyes, and could see that they were genuinely upset that I had questioned them. I do believe that they worked incredibly hard to do the best they could for Freya, and that they were almost as saddened by the outcome as I was when the echocardiogram confirmed that she had indeed been fighting KD.
It is easy when you are in a particularly black moment to want to hit out at those around you, and I very quickly forgot what I had seen from them in the days that Freya was in their care. Looking back now, it was really no time at all. Sheffield reached a diagnosis within 5 days of receiving Freya onto the ward, and during those 5 days I could see that they worked tirelessly to try to fathom out what was making my baby so sick. And they were brilliant at keeping me informed. I had not expected to see the Consultants very often during our stay, but there wasn’t a day that passed (well, weekends maybe) that we didn’t see one or more of them, sometimes more than once. The Immunology Consultant was one of the most warm women I have had the pleasure to meet, and I always felt that Freya had touched her heart in some way. She would come and sit next to me on the fold away bed, sometimes holding my hand when she had to discuss something difficult, like the bone marrow procedure. The Rheumatology Consultant and the SHO that we often saw would come into our cubicle and talk us through their thought processes and plans, and I always felt that it was a collaborative process.
Amidst all the researching and fighting of the past 4 months, I have always felt that there was still a reason to be thankful. I have to remember that I took Freya to A&E at the very first sign of a temperature; she had no other symptoms of KD and would continue to hide those from the doctors for a number of days. It seemed like a long time in comparison to other KD stories, but most of those didn’t take their child to the hospital on day 1. There was so much luck involved in Freya’s story – had she been a week older, I would have begun to treat her fever with paracetamol at home instead of taking her to the hospital. Had she developed a fever one day later, on the Monday, I would have taken her to my GP and probably would have been sent home with “just a virus”. Had the Immunologist not asked for an opinion from Rheumatology, Freya would never have had that second echo. Had Freya not developed that angry rash on day 11, the Rheumatologist would never have been called. It’s like the doctor said, in the end “Freya showed [them] the way.”
Last week, at Freya’s follow-up appointment in Sheffield, I thanked the doctor for everything they did for her. I said that whilst I would probably always wonder if there was anything they could have done to diagnose her sooner and whether her heart would have suffered if they had, I had nothing but gratitude for them for the way they aggressively treated the condition once they reached a conclusion. They were as relentless as the disease itself, and had entered a battle that they were determined to win. And they did win. Because even though Freya has suffered complications with an unknown longevity, they stopped Kawasaki Disease in its tracks when it was intent on causing further damage. I know that they did more than was necessary, carrying out daily echocardiograms even though the likelihood of a day-to-day change was small. They often said that they thought some of what they were doing was “overkill”, but I know they did that because they wanted a positive outcome for Freya.
It has taken me a long time to stop trying to find a door to place my blame at. That blame landed well and truly at my own front door in the beginning, and there is still part of me that wishes I could have made her strong enough to have avoided this disease. I often feel like I failed her in that respect. I don’t feel the need to blame so much anymore, and whilst it has been a slow process I do believe I have found my peace with this situation sooner than most. Don’t get me wrong, I still believe there is more to be done to make the diagnosis of Kawasaki Disease more successful. I still think it is a travesty that this disease can have such a huge impact on a child, and yet parents and medical professionals alike are unaware of its existence. I still think that it is appalling that mums like me have to spend so much time and energy reading documents that may as well be written in Swahili, and trying to separate the wheat from the chaff of internet searches. Let’s face it, mums have plenty of other things to be getting on with. But, I do feel that I have a stronger appreciation of the medical profession and as a result I understand why our children are so difficult to diagnose. So what brought about this catharsis? It was in fact a letter that was delivered to me via Ed Miliband MP, whom I wrote to airing my frustrations as a parent of a child with this rare disease.
When I received the letter yesterday, I was anxious to pour over its contents. On first reading, however, I was angered and frustrated. He had written to the Secretary of State for Health (Jeremy Hunt) asking for his response to my concerns, and enclosed a copy of the response he received. It appeared to be, on the face of it, a justification of my perceived failings of the NHS, “…challenge for GPs..”, “…6000 rare diseases..”, “…not possible to be an expert in all of them..” In all honesty, when I read the letter yesterday I was all fired up to write a pretty curt reply. And then I remembered something that an old manager once told me; if you are angry, don’t respond, sleep on it and respond later. So I read the letter again a few hours later and it was like some of the clouds that occupy my mind began to disperse.
What the Rt Hon Jeremy Hunt MP was actually saying was that we have to appreciate that with over 6000 rare diseases to consider, and particularly with Kawasaki Disease affecting only around 8 in 100,000 children each year, it is unreasonable to expect every GP to immediately diagnose a child presenting symptoms. You think that doctors are trained to handle every medical eventuality, but it is like anything in life – no-one knows everything about everything. Why, even in my field I would say that customer service advisors cannot be expected to answer every customer query that reaches their ear. All we can do is equip them with the tools to provide the best possible solution. Sure, there will be common questions that arise that even the worst advisor could answer with their eyes closed, but for every 100 simple questions that come along, there will always be one, a little more obscure, that needs closer attention, perhaps even a second opinion. Why couldn’t I apply the same common sense to the medical profession? I guess because they are dealing in life and death, so you expect more from them to know everything. But how many times have you been to the doctor when they’ve had to use Google, or refer to a medical handbook? Doctors are incredibly clever people; they are not geniuses with greater memory capacity than you or I.
The MP went on to provide information about the steps that have been taken to support medical practitioners in the diagnosis of rare diseases. One of the resources he drew my attention to is a number of videos that have been produced by Health Education England, “in collaboration with the Department of Health, rare disease charities, patient groups and clinicians”. One of the videos is called “Rare Disease: The GP’s Role” and has helped to give me some grounding in this whole thing (you can view it at https://www.genomicseducation.hee.nhs.uk/resources/videos). The NHS appreciate the challenges surrounding the diagnosis and treatment of rare diseases, like Kawasaki’s. Yes, more needs to be done to inject funds into research projects, but who am I to say that Kawasaki Disease needs more attention than the other 5999 rare diseases in the mix? I know I run the risk of sparking political debate about government spending and the like, but I don’t claim to understand that in any detail, and I am sure that the solution isn’t quite as easy as we all might think. And if being a Doctor was easy, we could all be one, couldn’t we? I already feel like I’ve qualified with a degree in KD!
The videos that I watched with interest discuss genomics, a discipline in medicine concerned with DNA mapping (in simple terms!) There are currently 11 genomic medicine centres in the UK who are part of the 100,000 Genomes Project whose role it is to participate in “sequencing 100000 DNA codes of patients, leading to better, earlier diagnosis and personalised care, for cancer, rare diseases and infectious diseases” (https://www.genomicsengland.co.uk). I was pleased to see Imperial College London on that list, and proud that my family has played our part in the study to understand the genetic determinants of Kawasaki Disease. I was pleased to hear, in the letter, that “The Rare Diseases Advisory Group…is considering a proposed way forward on an approach to earlier diagnosis, which will be developed with the medical Royal Colleges.” So, work is being undertaken behind the scenes to try to deal with this disease more effectively, and a Professor leading one of these research projects in London recently told me himself that they were about to publish a paper which proposes a diagnostic test for Kawasaki Disease.
As well as this, the MP went on to tell me about the NICE Guidelines that are in place for the treatment of children presenting persistent fevers (“Feverish illness in children: Assessment and initial management in children younger than 5 years”) which “provides advice in relation to the diagnosis of Kawasaki Disease, including details of the main symptoms…” This information can be found on the website https://www.nice.org.uk. I followed the flowcharts on the website and it was like watching Freya’s journey played out in a diagram! The doctors weren’t pissing in the wind (pardon the expression), guessing, or coming up with miraculous discoveries. They were following an NHS guideline for diagnosis and treatment, and by a process of elimination were finally able to reach a diagnosis for my daughter. Given the red herrings that Freya threw at them along the way, I am not surprised at all by the route they took, and I have a new appreciation for the process that they had to go through. Perhaps this is common sense, and you are all saying “Well, what did you expect?!” But you know, I really didn’t expect anything other than to take my child to the hospital, for them to look at her, tell me what was wrong, treat her and send her home. Well. 100% better. And not broken.
So what has changed for me? I no longer feel the need to blame anyone for what Freya went through, or for her current condition. I feel that my respect for the medical profession in general has been restored; they can’t know everything, especially about a disease that many have probably not seen before. I feel like I have reached a better understanding of the process that all the medical staff went through to diagnose my child. Don’t get me wrong, there were a few ‘blips’ along the way, and I have no doubt that there will have been something that could have been done differently somewhere, but I don’t think I believe that a different course of action would have changed anything for us anymore. Freya would still have Kawasaki Disease, she would still have received treatment and sadly because of the nature of the disease she was one of those children who was unfortunate enough to suffer heart involvement.
And what remains the same? My determination to raise awareness of Kawasaki Disease. My wish to see increased funding for research into the disease, whether from the NHS or public fundraising. My commitment to use whatever spare time I have to produce information about the disease where I think it will help other parents in my position. My passion to research and share what I can, and my appetite to challenge our Doctors to answer the unanswered questions that parents have about the disease and related issues.
I never thought I would use the word ‘lucky’ to describe our experience, but do you know what? We are lucky. Because so many other stories have not panned out the way ours did. Yes, Freya was critically ill, and yes she has a heart condition that will continue to worry us for an undeterminable amount of time. I will continue to suffer anxiety when Freya screams out in her sleep like she did at 6am this morning and my first thought was is this it? And I will always wish that Kawasaki’s could have passed Freya by. But right now, a mum is holding her child’s hand as they are put to sleep for heart surgery. A GP is sending a child home because all they have is a virus. A child is being pumped full of drugs that are not going to work, and will allow the disease to hide a little longer. Right now, somewhere in the world, a parent is grieving the loss of their child to Kawasaki Disease.