So today was Freya’s first cardiology follow up of 2017. In case you don’t remember where we are at from the last visit, I’ll quickly bring you up to speed. We had a cardiology follow up in September 2016 where I questioned the Cardiologist’s desire to carry our a cardiac catheter angiogram. At that appointment I was told that I needed to trust the doctor or she would have no choice but to refer Freya to another hospital. However, shortly after the appointment I received a letter which allayed some of my fears, and so I made an appointment with the Cardiac Intervention team to discuss the procedure. At that appointment I was made to feel more at ease about the procedure itself, however the doctor said he was not happy to perform it on Freya given she was still so young, and asymptomatic. I left it with the two of them to argue the case, and eventually it was decided that whilst there was a degree of anxiety about the speed of the remodelling of Freya’s arteries, it was in Freya’s interests to wait a while longer, and she was placed on the waiting list to undergo the procedure in the Autumn of this year (2017). That was good news for me, as it seemed like a good compromise, although I then felt quite nervous about what could happen in the interim, and gave my consent to wait as long as Freya was reviewed regularly in between.
I believe the idea was for Freya to be seen in 6-months (from the last appointment), however I was keen to evaluate Freya’s current situation before we go to Mexico, so her appointment was made slightly sooner than planned. And that brings us to today.
Now, at Freya’s last appointment they were unable to obtain an ECG reading as Freya was hysterical and kept pulling the electrodes off (“stickies” as she calls them). Even when we managed to get her to lay still, she was just too distressed to get a proper reading. Since then she has developed an acute fear of stickers – all kinds! She wouldn’t even allow us to go near her with a sticker, not even to stick one on her clothes. So we’ve been working really hard trying to beat that phobia, and we had a breakthrough last week after a visit to Cannon Hall Farm. I’ve spent the last week chatting to Freya about the hospital visit, talking about the ‘stickies’, where they’ll put them and that she has to be brave and not wriggle or cry. On the way to the hospital this afternoon she told me and her Dad that she liked hospitals, and chatted away about the stickies and how brave she was going to be (I say ‘chatted’ but it was more like a mantra – “I like hospital, stickies on me, my legs, my tummy, me not cry, me blave.”) I was hopeful that today’s appointment would be more productive.
When we arrived we were sent straight for an ECG. Sitting in the corridor, one of the nurses that had looked after Freya before came and had a chat with us. She made a fuss of Freya, and after a while asked if she would like her to do her ECG for her. Freya said she did, so the ‘nurse’ (not sure what her actual job title is) went and asked the other staff if she could do this one as Freya is very nervous and she knew how to handle her. She decided to place the electrodes on her arms and legs only, leaving her torso free. Despite her best efforts, Freya still screamed at them to stop. I managed to calm her down (by telling her that Paw Patrol would be on the ECG screen any minute!) for just long enough to get a reading, although I gather that it wasn’t a full reading with it only being on her limbs. Freya then refused to be weighed or measured so we had to leave that part too.
We went into the Cardiologists room almost straight away, and she asked how Freya had been. We updated her on the chicken pox vaccinations, which are now complete. The episodes of single fever spikes in the early hours of the mornings (can be associated with angina) had ceased, and Freya was no longer having periods of suddenly lying still on the ground, so it must have just been a phase she was going through. The doctor tried to do an echocardiogram but Freya resisted (for the first time so far on this journey), and the Cardiologist wasn’t able to keep her still for long enough to get a look at her coronary arteries, let alone measure them. She kept fighting to get away, shouting “Stop it, stop it, please help me!” which was so upsetting to watch. She did however get a look at the heart chamber and could see that the heart was functioning normally – this was also confirmed by the ECG results, and to be fair, heart function has never been a concern in Freya’s case. As long as Freya’s blood can keep flowing nicely through the arteries everyone is happy, and that’s what the daily dose of aspirin is there for.
I must admit I felt more than a little uneasy about not being able to get a close look at Freya’s arteries. I mean, it’s the arteries that are the issue and I really wanted to be sure that there had been no further narrowing from her last appointment. The doctor didn’t seem to be too concerned however so we sat down for Q&A before setting off home.
So, what did we learn? I am going to bullet point it to make some sense of it all:
- The Cardiologist referred to the latest paper on the long-term treatment guidelines for Kawasaki Disease, and said that what she had read had given her some reassurance that the speed of remodelling in Freya’s case is not as unusual as she first thought. I think she may have been referring to the latest JACC review which I actually sent her after our last appointment (I can forward it to anyone who would like it – you can contact me through the awareness page www.facebook.com/freyasstory). Her initial anxiety was that with Freya’s aneurysms remodelling so fast it might be a sign of stenosis occurring. However having read the paper, she established that actually it is common for remodelling to occur within the first 2 years, particularly in cases where children are treated more aggressively than the standard treatment, as in Freya’s case.
- Freya was treated with the standard IVIG and high-dose aspirin at the time of diagnosis, with the addition of intravenous steroids. Resistant to two doses of IVIG she was then given another drug called Infliximab. This is not widely given in the UK, although there is a strong case for it’s use, particularly in severe or high-risk cases. As a very young baby, Freya was in the high-risk category for heart damage, and also presented with a severe case of the disease. The hospital had to apply to NHS England for permission to administer Infliximab to Freya – I still have a copy of the letter granting that permission. Freya’s Cardiologist believes that the Infliximab was instrumental in Freya’s cardiac recovery from the disease and I have to say that I agree. I think Infliximab saved Freya’s life.
- I asked about our impending long-haul flight, and we were reassured that there should be no concern. We should make sure we don’t forget Freya’s aspirin, and should keep her hydrated on the flight, but that was the only caution. Oh, that and that we had to remember to send the doctor a postcard 😉
- I also asked about cholesterol testing, as there has been some information circulated about undergoing the test one year post-diagnosis. We were advised that it may be standard in cases fitting the ‘normal’ age bracket for KD, but that Freya is too young for a low-cholesterol diet and would not be treated with statins for high cholesterol as her brain is still developing. I guess that’s where we have to trust the individualisation of our children’s cases compared to the standard protocols.
- I have also read that aspirin is best taken at night to reduce the risk of cardiac events during the nighttime hours. Freya has always had hers in the morning, but with her soon going to be with a childminder, I wanted to switch it so I can give it to her before bed. This is not a problem, and the anti-platelet effects of the drug will remain long enough for the longer delay between doses when I make the switch.
- The last thing I asked was in relation to specific care plans/patient specific protocols required for the childminder, for example. The doctor said that as long as the childminder knew what to look out for – becoming pale, sweating and quiet – there were no other ‘special’ arrangements to be made. I expressed my concern that in the event of a potential cardiac issue, were an ambulance called it would take Freya to our local hospital which is not a PCI centre. The doctor is going to provide me with a letter outlining the care Freya should receive in such an event, and advised that in coronary cases, Freya would be better transported to a local hospital for immediate care than risk the journey to Leeds. Once stabilised, she could be transferred by the EMBRACE medical transport service to the cardiac unit if necessary.
We agreed that Freya will be seen again in 6-months, and that although they feel less anxious about the progress so far, they will still carry out the angiogram later this year. I agree that it is prudent to take a closer look to be sure we know the full picture. I also explained that whilst in the past I have questioned the steps they wanted to take, it was never from a place of distrust but only from making informed choices about what was best for Freya. Even though I was the one who wanted to delay the procedure, I have still had to live with the fear that I may have made the wrong choice, and something terrible could happen without warning. Overall it was a very positive visit today.
But (yep, there’s always a but with me isn’t there!), I can’t help but still feel a little uneasy that what was decided today was not based on what we could actually see (with the exception of the heart function being ok). For all I know, Freya’s arteries could have remodelled even more than the last appointment and now be heading dangerously towards narrowing. I guess that is a possibility, but we have made decisions today based on probability. The doctor thinks it is improbable that Freya is in any real or immediate danger. Hopefully the aggressive treatment Freya received in the acute stages of the disease have set her up for a bright future. There are still many unknowns, some of which we won’t get answers to until much later in Freya’s life. For now, perhaps it is time to have a little faith…
For some reason as we reached the exit level of the hospital, we turned the wrong way out of the lifts. It wasn’t a big problem as you can get out either way. This way, though, took us past the Brotherton Wing – the old part of the hospital which houses the parent accommodation where I had to stay, alone and far away from Freya when she was being taken care of in the cardiac high dependency unit. As I walked past the entrance to the old wing, I couldn’t help but feel a deep sense of sadness at the memory. And then I remember the chapel that I used to pass on that long, lonely walk to my room every night when I couldn’t keep my eyes open any longer. I don’t consider myself a religious person, but not wanting to leave anything to chance, I had entered the chapel the day after being told there was a chance we could lose our daughter. It as the day Freya was taken back to the Children’s Hospital, and as a parting gesture I had taken a slip of paper from the table inside the doorway of the chapel and wrote on it, “Dear God, prove them wrong.” I am beginning to think He might just have been listening.