I don’t remember much about the day we got a diagnosis for Freya’s condition (“D-Day”). Perhaps I was numb with all the information I had to take in? I remember my friend was visiting. I remember going for the echocardiogram. The consultant who carried out the echo was kind of eccentric, and Freya loved him. She cooed at him and laid there smiling while he did his work. It was him that told me he had been asked specifically to look for Kawasaki related issues with Freya’s heart. He mentioned Kawasaki Disease, and was profusely apologetic when he realised that was the first time I had heard that word mentioned. I immediately googled it on my i-phone, attempting to surreptitiously arm myself with information as he continued to scan Freya’s heart. I wasn’t able to read much, as the consultant was talking to me about what he was doing, so I resigned myself to waiting until I got back to our cubicle. He finished the scan and said that his report would be available in an hour or so.
My friend might remember what happened when I got back up to the ward better than I can, but I can only imagine that I began to read what I could about the illness. If I recall correctly, I don’t think we had much time to research before Freya was called for an eye test and my friend and I went together to Ophthalmology. The eye test was pretty uneventful – a 9-week old Freya was too distracted by the things around her to pay any attention to what the optician wanted her to look at. They put some drops in her eyes to look more closely for any conjunctival problems (a common symptom of Kawasaki’s), but saw nothing too concerning.
Shortly after we arrived back in my room, we were visited by the Rheumatology Consultant who was supporting Immunology with Freya’s case. When the rash on her legs presented itself on the Thursday afternoon, he had been called to come and take a look. He did a thorough examination of Freya and concluded that he still felt that we were dealing with an infection, but that it wouldn’t hurt for Freya to undergo another echo. He had no idea that his decision would end up being key to Freya’s recovery, as it was that echo on the Friday where they discovered that Freya’s coronary arteries were dilated. That final symptom was to confirm that Freya was a victim of Kawasaki Disease, a rare vasculitis that causes inflammation of the blood vessels. Freya’s liver, which should measure 1-2cm had increased to 5cm in size and was pushed down in her tiny body. Her spleen was also enlarged. KD is the leading cause of acquired coronary complications in children as a result of inflammation in the blood vessels within the heart. Diagnosed early enough (within 10 days) medication is administered which reduces the risk of coronary complications from around 25% to 5%. Freya was diagnosed on Day 13.
Soon after we received the diagnosis from the Rheumatologist, the Immunology Consultant came to see us. She said that it was crucial that Freya receive treatment for the condition. She had to obtain my consent to give Freya intravenous immunoglobulin (IVIG), and explained that it is a blood product made from the antibodies of many blood donations. I asked, hypothetically, what would happen if I didn’t give my consent. She replied that it was imperative that Freya receive this treatment, and that she wasn’t asking for permission. I would not have stood in their way. They moved quickly with a sense of urgency to get the IVIG ready for Freya as soon as possible. It would take a number of hours to infuse into her blood stream and they wanted to get it started. i now know that Freya’s condition was considered critical and they had to try to stop the inflammation quickly before it did any further damage to her heart.
Kawasaki Disease is very rare in itself, but it soon became apparent that it was even more rare for an infant as young as Freya to be diagnosed. The Children’s Hospital had to liaise with Great Ormond Street in London and other specialist hospitals to reach a diagnosis, and to decide on the appropriate treatment for such a young baby. I believe it was GOSH that advised that Freya should be prescribed steroids in addition to the IVIG. To think that 2 weeks before, I was worried about giving her paracetamol!
Everything that day happened so quickly I don’t think I really had time to think. I can’t remember if I became upset when we received the diagnosis, or whether I was relieved to finally know what we were dealing with. I do know that it was a tough day because it was that night that I shouted hysterically at the doctors who came to take a blood sample! Relief was soon replaced with fear and anxiety about Freya’s condition. I had never heard of Kawasaki Disease. There were many medical staff who hadn’t heard of it either! I do know that my journal entry that Sunday (Day 15) was optimistic.
Sunday 14th June 2015, 21:25pm
“You had another heart scan this morning. The coronary aneurism that was caused by this nasty disease is still there. It is no better, but it is no worse either. To us you seem like our beautiful Freya again. The rash has gone, there have been no temperature spikes in three days, and we just had the best news about the inflammation markers (CRP) having dropped to 49. We’re not out of the woods yet sweetheart, but it’s looking good. Now all you need is lots of cuddles to help mend that broken heart of yours.”
Freya had an echo on both the Saturday and Sunday and neither showed any change. The inflammatory markers (CRP and ESR) in her blood were coming down too. All good signs that she was on the road to recovery, weren’t they? I wouldn’t get much sleep that night and in the morning I would learn how naive I had been.