That night (Tuesday 23rd June) I began researching in earnest. I had spent a fair amount of time and energy researching Kawasaki Disease and certain medical terms up to that point, but mainly to educate myself on the subject so I understood what the doctors were referring to. One of the doctors commended me on the interest I had shown and the research I had undertaken, adding that they never came into our room without all the information to hand as they knew that I would ask questions like “What were her last CRP levels?” CRP (C-reactive protein) is a marker of inflammation in the body that is measured from a blood sample. I had some understanding about CRP levels from a period of time when I had an illness and my CRP levels were elevated – normal is below 7, and mine had risen to over 140. When Freya was at her worst, the CRP levels in her blood had reached over 300. She wouldn’t be allowed to leave the hospital until her CRP was within normal levels, so that, along with other markers was to be the success criteria for her release. Freya’s blood would be taken every day, mostly from a heel prick, but sometimes from a vein.
This time my research was different. Not only did I need to understand the issues surrounding Freya’s heart, but I needed to find out what Freya’s chances were. So I took to the internet and searched for anything related to Kawasaki’s and acquired heart disease. I felt desperate to find a story that resembled Freya’s that had a happy ending. I can recall the feeling of despair building as I searched for positive stories with no luck. I did come across one story that seemed to resemble Freya’s pretty closely – same age, same delay in diagnosis, almost identical issues with the heart. Before I reached the end I realised that what I was reading was an autopsy report. I decided to stop looking. I found a couple of support groups on Facebook and joined, spending hours reading story after story from parents across the globe. I posted questions and a brief synopsis of our situation and although I couldn’t find a case exactly like ours, I got a lot of comfort from reading stories about babies who had been through what Freya had and had grown up to be happy and healthy.
During my internet search, I came across the Kawasaki Disease research centre at Seattle Children’s Hospital in the United States. I found a ‘contact us’ section and decided to send a message to ask if they had any stories like ours that they could share. You see, I had asked my consultant if they had any, but they were not aware of another baby as young as Freya that they could use as a comparison. In fairness, it’s difficult to provide any real prognosis of recovery with a disease that has only been around for 40 years or so as that there have been no long-term studies that can help to predict the future. To my surprise, I received an e-mail from the Dr who leads the Seattle KD research centre. This was his response:
“…I’m sorry about the delayed diagnosis and treatment. But it sounds like she’s getting good care now. Unfortunately most children under 6 months old develop narrowing or stenosis in their coronaries. It’s going to be scary for a while. I’m sure you are giving twice daily injections of Lovenox. But there is certainly hope. I follow 3 children with huge aneurysms under 6 months which resolved completely within 2 years. I have many others living pretty much normal lives with some exercise restrictions and on aspirin and blood thinners…So far, we have had no deaths and only one heart attack, who totally recovered and is now doing well.” Wednesday 24th June, 00:19
It was scant information, but it was positive news, and a direct response was more than I could have hoped for. I read it a couple of times. “I am sure you are giving twice daily injections of Lovenox”…no we weren’t. I replied to say that the use of Lovenox hadn’t been mentioned and explained the treatment Freya had received. He replied almost immediately;
“Standard of care in US and UK for large aneurysm includes anti-coagulation or a blood thinner in addition to aspirin. That means Lovenox …or low dose heparin if the aneurysm is very large near 4mm in a baby your daughter’s age. Sometimes warfarin is used but doesn’t work well in small babies…” Wednesday 24th June, 00:38
Freya’s aneurysm measured 5.2mm. My heart was racing. I felt the panic building; my daughter wasn’t receiving the treatment necessary to prevent a heart attack! The Dr asked a few more questions about where we were being cared for, etc, and then responded again to tell me that I needed to phone our cardiologist first thing in the morning. He said that I could use his name, that they could look him up. He told me that in the US they do not use the 8mm+ criteria for a giant aneurysm in small babies stating that it is relative to body size, and that “…5mm in a 2 month old needs anticoagulation asap.”
That night I wrote in my journal:
“…I do feel that this disease will take you from the world one day. Hopefully not until you are 90 years old, and the rest of us are long gone. Because of that I want to make sure we make the most of whatever life we have been given, and if we end up having 50 wonderful years together then what an amazing life we will have had…I don’t know why my eye is drawn to the stained glass window of the chapel just outside the window to our room, but God bless you tonight my little Peanut. I pray He keeps you safe.”
The following day (Thursday 25th June, Day 26) we would travel back to Leeds for Freya’s first cardiology follow up since we received the worst news. The echo showed no change from the previous scans which was good news, but I left with an uneasy feeling in my gut. I’d tried to ascertain what the likelihood was of an undesirable outcome. I remember asking if they could tell me how many young babies they had treated with Kawasaki Disease and how many of those had a favourable outcome. The Dr said that they didn’t really see babies as young as Freya with KD. So I asked if they could forget the KD and tell me how many babies they had treated with coronary aneurysms, and she replied that babies don’t get coronary aneurysms unless they have had KD. I wasn’t going to get any answers, because there weren’t any answers to give. Even the nurse that travelled with us in the ambulance said that it wasn’t a very reassuring visit. The journey back to Sheffield was a sombre one. I saw one of the on-duty Dr’s that evening and she said I was entitled to ask for a second opinion, so that was what I resolved to do.
…”I don’t want to cause a fuss or seem like I am being over-dramatic, but I think I need to trust my gut instincts on this one. I’m not losing you without a fight my darling.”
Later that night I received an unexpected e-mail from the Dr in America, stating his concern for the advice we were receiving. He had also asked the opinion of a KD specialist in Boston and she agreed. They mentioned something called a ‘z score’ which is apparently used to ascertain the correct treatment for coronary aneurysms in a small baby. The Dr had calculated Freya’s based on the information that I gave him. He said that Freya’s ‘z score’ was 16.9, and was well above the range at which US specialists would treat with Lovenox. He attached a paper from Great Ormond Street which details the UK advice for the treatment of Kawasaki Disease, and pointed out the section where our own advice states to treat a z score higher than 7 with warfarin; “Take the paper with you and good luck.” I felt sick. That niggling feeling in my gut that everyone tells me is me being negative, was right on the money. Later I would receive another e-mail. The Seattle Dr had queried the rheumatologist whose name was mentioned on the Great Ormond Street paper and asked her to recommend a Cardiology Consultant for Freya. 24 hours later he sent me the name of a Professor of Cardiology in the South West of England who had been involved in the writing of the GOSH paper. I searched online for his contact details, and immediately e-mailed him. I could feel the adrenalin rushing through my veins. I had information, expert medical opinions from people who have dealt with more cases than we have here in the UK, I had the official guidelines for treatment of Freya’s condition, and now I had a lead to someone who was within our reach who might just know more about this disease than anyone. All I had to do was sit back and hope for a reply.