I guess we all have one of those don’t we – not many of us know what lies ahead of us.  Some know what might be in their path, but nobody knows exactly how or when those things will happen. That’s why it is important to live life, not take things for granted, and do your very best to be the best you can be. And all the other clichés about living life to the full that might seem flippant, but are actually really true.

But how many people lose sleep worrying about what might lie ahead in our futures? People with anxiety, sure.  Anxiety makes you worry about almost everything.  I imagine the majority of people think about their future, but worrying about what might happen next year, in five years, in twenty years… I don’t think that is common.  Amongst the rare disease community uncertain futures are a given.  Many people living with rare diseases don’t have a diagnosis for their ailments and spend years searching for answers and fighting for treatment.  Some have a diagnosis, even treatment, but have no idea how their lives might take shape with the disease they are living with.  Kawasaki Disease is one of those.

Take Freya.  I think most people think that she is okay now.  She had this disease that made her really sick, but she got treatment and now she is at home living a ‘normal’ life and looks perfectly healthy, like any other kid her age.  And in the most part that would be true, except for the ‘normal’ part.  You see, if you weren’t already aware from my previous blog posts, Freya was one of the 6% of children with Kawasaki Disease who sustained damage to her heart despite treatment.  The disease causes inflammation of the small to medium arteries throughout the child’s body, and if treated quickly enough heart damage can be prevented.  In those cases where treatment is delayed, due to misdiagnosis (or a lack of diagnosis altogether) Kawasaki Disease is allowed to continue on its destructive path and cause damage that may never be reversed.  Once an aneurysm forms, the child’s heart will never be ‘normal’ again, even if the coronary arteries remodel and aneurysms regress to within otherwise normal parameters.  This leads to complications throughout the child’s life and into adulthood, which is why long-term care and life-long follow up are so vitally important.

The good news in Freya’s case is that her arteries have remodelled quite dramatically from when she was at her most sick.  There are no obvious aneurysms any more, and the arteries that had expanded to over 5 times the normal size for a baby of her age, have now regressed to what would be considered normal in a child that hadn’t been touched by heart disease.  The bad news is that the extent of the remodelling has her Cardiologist feeling a bit anxious.  They are concerned with what might be causing the narrowing of her arteries, and whether that narrowing might continue to progress until Freya’s arteries are too narrow to allow the flow of blood.  When blood can’t get around your heart, that’s when a heart attack occurs.  Sure, Freya takes aspirin every day to try and help keep the blood flowing, but nothing can get through an occluded artery.

That said, do I think that she is at immediate or short-term risk of a heart attack? Hesitantly I say ‘No’.  I say it hesitantly because whilst I have this pretty good feeling that Freya might just be some kind of miracle success story, my experience with this disease is not to let your guard down.  Complacency has no place where this disease is concerned.  Let’s say that I hope that she is not at immediate risk.  We will find out more in the Autumn when Freya will undergo the cardiac catheter angiogram that she was meant to be having last year.  It has been delayed to allow her more time to grow, so that she might be stronger to outweigh some of the risks associated with the procedure.  The rapid remodelling of her arteries has led me in the meantime to insist that Freya receives regular follow up between now and then so that we don’t miss something while we wait.  An echocardiogram won’t show what is happening with the arterial walls, but you can see the diameter of the arteries (up to a point – an echo can only see about 3mm into the artery) so you can get a view of whether they are continuing to narrow.

So what are my fears for Freya’s future?  

Of course I am anxious about the angiogram, but I’ll worry about that more when the time comes around.  I am confident in the ability of the intervention cardiologist, and am happy that it is a relatively low risk procedure.  I’m not too excited about her going under a general anaesthetic – I’ve seen that once before and it felt like I was watching my baby die.  I would prefer never to see that again, but that’s the reality and I will always be there by her side when she goes to sleep, no matter how hard I find it.

I am anxious about what is going on in that heart.  I’d love to have a window to look through so we could check in on it every now and again.  We assume from outward appearances and general health that nothing too sinister is going on, but the fact is we just don’t know.  I’ve read many stories from other KD parents who have experienced dramatic turns of events with their children, resulting in the need for heart surgery.  Sadly, some children have lost their lives to this disease – mostly those that were not diagnosed in time, or at all, but often because the changes in their hearts happened quickly and in spite of regular follow up and treatment.  Nothing is promised.  Do I spend every day worrying that Freya will have a heart attack? No.  Does my heart leap into my throat when I wake in the night and can’t hear her breathing on the baby monitor? Every time.

I am worried about what the angiogram might find, and whether there is any likelihood of Freya needing surgery in the future – around 80% of children who suffer giant coronary aneurysms will require intervention later in life, whether it be a stent or bypass surgery, for example.

I am worried about whether Freya’s diet is sufficiently ‘heart-healthy’ to protect her heart from disease.

I think about when Freya might decide to have children of her own.  Seems like a long way off to be worrying about it, but the truth is that she will most likely need to have a cardiologist involved in her ante-natal care.  There have been cases of heart attacks during labour where the mother had KD in childhood – there’s a whole medical paper dedicated to this subject (yes, I have a copy!).  I even worry about whether I will still be here when she enters that stage in her life and whether she will remember that she needs to consider her medical history.

And this one you might think of as really strange, but I worry about them telling me everything is ok, and that she can stop the medication and live a normal life, with KD firmly in her past.  You would think that should be something I would hope for, and be pleased about.  But, first of all, I will never forget the words of Professor Jane Burns, World Leading Kawasaki Disease specialist, that once a child’s heart has been damaged by KD it is never considered healed, or normal again.  So if  a doctor tells me everything is normal, and tries to send us on our way, then I will be putting up a pretty big fight!  Freya, and all the other children like her, need to be monitored for the rest of their lives.  Rady Children’s Hospital recommend obtaining a CT calcium score 10 years post-diagnosis to help identify and prevent further complications, for example.  It may just be a check-up once a year to make sure there have been no changes over time, but it is the peace of mind that we all need to ensure that there are no hidden issues lurking that might cause a serious problem later on.  Complacency is a killer.

“Oh Jo, you’re so dramatic!” I hear you say.  But you need to understand that I read stories, comments and messages every single day from someone who has been touched by Kawasaki Disease and suffered terrible consequences and devastating loss.  Every time I feel like I will never make a difference where KD is concerned, I remind myself of those stories, or I stumble across a new member of the Kawasaki community and the fire in my belly is reignited.  It is one such story that got me thinking again about the future, and reminded me that although I can hope for positive change in Freya’s condition, I must never allow my own complacency, or that of the medical profession, to create a false sense of security.  The story is that of Lisa Connelly. Her sister Amy has given me permission to share it in the hope that it might help to raise awareness of this disease and the devastating consequences when we get it wrong.

Lisa Connelly was diagnosed with Kawasaki Disease at the age of three.  By age five she had been given the all clear, following an angiogram that showed no persistent damage to her coronary arteries.  I can only imagine the elation of the family as they put KD firmly in the past, and watched Lisa to grow and live a normal, heathy life.  She was very active growing up, and was an avid runner.  She had a beautiful son, Levi, and was very much loved by her family and friends.  Described as “the kind of person you want to be like”, she had a heart of gold and was the first to come to your aid if you were in need.

Out of the blue, on the 25th November 2015, 37-year old Lisa had symptoms of a heart attack. The hospital performed an echocardiogram and Lisa underwent a stress test, both of which cleared her of any issues. Four days later, on November 29th 2015, Lisa passed away as a result of a massive heart attack.

The pathology report on Lisa’s heart showed that her coronary arteries had muscle cell tissue build up on the arterial walls, which caused restriction of blood flow to her heart, thus causing a heart attack.  The family had no idea that Lisa would ever have to deal with Kawasaki disease again, and even after the heart attack they did not consider it might be linked to an illness that she had when she was a child.

Since Lisa’s passing, her sister Amy has done everything she can to raise funds and awareness to prevent another tragedy like this.  Professor Jane Burns has said that conducting a CT calcium test earlier in Lisa’s life may have prevented further complications from occurring.  It was from a Facebook post sharing a medical paper which discusses the importance of a CT Calcium Score that I found Amy, and I am honoured that she has allowed me to share her family’s story in my blog.

Amy said:

“My sister was truly a beautiful light in this world. My sister had a great smile and loved to see others smile. She helped so many people while she was living and we know she continues to help even today.  Our hope is that somehow she helps in the research efforts of KD. So, my prayer is that your blog post reaches someone that takes the information and gets the proper treatment they need to live a long, healthy and hopefully happy life. We’re making beauty from ashes…My mom and dad’s grief has been almost unbearable this past year. And the hardest part for my mom is that the cause of the heart attack was from KD.” 

As well as raising awareness of the devastating consequences of this disease, Lisa’s family have made sure that her legacy lives on, and donated some of her heart tissue samples to Dr. Jane Burns and Dr. Anne Rowley, leading KD experts in the US. Lisa’s organs have also helped to save the lives of four people, and the family are in touch with the gentleman who received her liver.

I know how important it has been to me to make sure that Freya didn’t suffer this awful disease in vain.  We still have our daughter here with us, and thanks to the awareness that Amy is continuing to raise about the potential long-term and hidden consequences of Kawasaki Disease, we can hope that Freya will long outlive us.  I don’t know if I would have had the strength to fight had the unimaginable happened in the Summer of 2015.  As we were starting to get our lives back on track that year, a family was being torn apart by the loss of a truly beautiful person.  I join Amy in their hope that this story might reinforce the need to ensure that Kawasaki Disease survivors are given appropriate attention from the medical profession throughout their lives, and shines a bright light on the insidious nature of Kawasaki Disease.

Writing this, I felt my heart break all over again.  This is Lisa.