Day 100 – Genetic research and echo number 15

IMG_0090Today we went to Leeds for your follow-up appointment.  We went over on the train because it is so much easier than parking.  I’d end up getting myself lost or not being able to find a space and I could do without the anxiety, especially on a day like today.  Not only that, but the car park Daddy parked us in last time didn’t have a lift, so it was lucky he was with me as we had a flight of stairs to carry you up in your pram!  Thankfully the lift at Doncaster Rail Station was working today, and a kind man helped me get the pram onto the train.  We arrived around 1pm, and your appointment wasn’t until 2pm so I decided to go to a restaurant opposite the hospital for a spot of lunch.  I had hoped that it would make today feel more like a normal mother-daughter day out, but with the hospital looming in the distance behind us that didn’t quite work out as planned.  Still, I had a nice light lunch and a cheeky little glass of Prosecco.  I couldn’t help but worry that the Prosecco could be a bad omen; celebrating too early?

I was so pre-occupied when we left that I walked out into the road at the crossing, and an angry man in a van beeped his horn at us.  Ok, so I should have been paying more attention, but he saw us so there was no harm done, eh?! Surprisingly I managed to get you to the hospital in one piece and we headed up to the Cardiology Outpatients department.  We know the drill now, so I told them your name and went and sat in the children’s waiting room.  We weren’t waiting long before they came to weigh and measure you; today you weigh 6.07kg (around 13lbs I think) and you are 63cm long.  After that I took you down the corridor for an ECG.  You are pretty used to those now, but this time it was a bit more challenging for the nurse as you were wriggling all over the place.  In the end it took 3 of us to get the result, with me holding you on one side, one nurse on the other, and another pressing the buttons on the machine!  That was all clear as usual; there has never been any concern over your heart function.

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A little while longer in the waiting room and your Cardiology Consultant called us into her room.  There was a girl in there on work experience.  I love it when there are students in the room as I get to learn all sorts when the medical staff explain things that they might not have done if the student wasn’t there.  You were pretty good for your echo, although you did keep trying to take over by grabbing the probe, and you kept sticking your foot up the Doctor’s sleeve and jogging her! That’s not very helpful Peanut – we need her to get those measurements spot on!

We weren’t seen by your usual cardiologist last time, so I was anxious to see what your doctor thought.  She apologised for not being there last time, but I explained that I understood because I knew that one of her colleagues had died in a tragic accident that day and it most certainly couldn’t be helped.  She did say that she had seen the results from your last appointment and noted that there had been further improvement which she said was great.  At the last echo, we were told that your right coronary artery (RCA) had normalised to around 2mm (normal for a baby is approximately 1-2mm), and that your left coronary artery (LCA) was dilated to around 3mm.  It had been around 4mm at the echo prior to that so that was great news.  Today your doctor got slightly different measurements (or she was just more specific with the numbers), so your RCA measured 2.6mm and your LCA measured 3.3mm.  I wasn’t concerned about the slight difference in numbers, as we are dealing in fractions of a mm so it is negligible, plus could be attributed to slightly different positioning for measurement.

Once the echo was complete, the Doctor asked how long it has been since you became poorly.  I told her you became ill on the 31st May 2015 and was diagnosed with atypical Kawasaki Disease on the 12th June 2015.  She seemed pleased with the progress you have made in such a short space of time, and is happy to give it a bit longer before your next follow-up.  The next appointment will be in 2 months; thus far they have seen you once a month.  We had a lengthy conversation about risks and concerns, one of which was my confusion over whether or not you had an aneurysm or just (just, ha!) dilation.  In the reports I have received some refer to an aneurysm and others don’t.  We were told some time ago not to be too concerned with the name, as the risks are the same no matter what you call it.  Today the doctor explained that you didn’t have a typical fusiform aneurysm (what they describe as a ‘string of pearls’), but that your arteries were dilated all the way along (as far as they can see on an echo anyway).  She showed me your LCA on the screen and highlighted where the artery becomes normal so I could see the dilated section really clearly.  Apparently that is how your arteries were when the Kawasaki’s took its hold, except they were significantly dilated.  It’s like your LCA was one big aneurysm.  Whilst that was a reason for huge concern earlier on your journey, the fact that they have improved significantly is fantastic.  And as they are regressing, they are doing so in quite a uniform way which is further reducing the risks of anything nasty happening.  I asked the doctor outright if she felt there was any immediate cause for concern, and she said she was no longer concerned and that your arteries were healing well.  Today was the 100th Day of your journey, and this was your 15th echo.  It’s strange how this has become so normal to us now.  Are you really a little miracle, sweetheart?  Your recovery is beyond anything any of us were led to expect.

I then talked to the Doctor about your future and expressed my concern that if you healed quickly they could discharge you too early.  There is a change.org petition underway to enable the NHS to undertake some long-term studies because of a fear that KD victims may be discharged prematurely causing later issues to go undetected.  The doctor said that the longest study that has been undertaken is in Japan, where the results from the study cohort showed that not one of the girls went on to suffer further damage or issues.  It is well publicised that this condition seems to be harder on boys’ hearts.  She said that once they are happy that your heart has repaired itself and there are no signs of stenosis (where the arteries continue to repair too far and close up) or pain, that you will move to annual cardiology follow-up.  At those appointments you will have an ECG and echocardiogram.  The only real way to establish the full extent of any damage to the coronaries is through an angiogram, but she explained that this is tricky in babies as the arteries can be ‘sticky’ and when the tube is inserted the arteries can spasm and stick to the tube.  CT scans can also be carried out, but they involve radiation which is always best to be avoided if possible.  She said as a result they are tending more to cardiac MRI as a means of exploration, so that may well be on the agenda going forward.  When you get to about 7-8 years old, they will start doing exercise testing too, so that can ascertain any affect on your fitness as a result of the disease.  And if by the age of 15 you have shown no signs of continuing complications, you will be discharged, on the proviso that we act immediately upon any signs of potential issues; angina or breathlessness for example.

I ought to be celebrating the results, but I just feel a bit numb really.  I think it is a combination of things.  All the puzzle pieces have started to fall into place as we get more and more informed about your illness, and Kawasaki Disease in general.  Now I understand the looks on the doctors faces when we were sent to the cardiac unit in Leeds.  Now I understand why your cardiologist felt she needed to take me aside and explain the risks associated with the extent of the damage to your heart.  And as much as we need to put the past behind us, I don’t yet feel able to put it all in a box.  I just can’t seem to comprehend what you went through, and how close we came to a very different outcome.  I can also see how very lucky we were that the disease took hold of you so aggressively in that 2nd week.  The clear echo on the first Monday in Sheffield resulted in the doctors ruling Kawasaki Disease out.  Had you not shown us that rash, the Immunology Consultant may never have asked for an opinion from Rheumatology.  Had the Rheumatology Consultant not decided to be cautious and request another echo, you would never have received the IVIG to stop the dilation in its tracks.  As it was you needed a second dose of IVIG and a dose of Infliximab, a drug that is not yet part of the UK protocol for use in treatment of KD.  In some ways, perverse as it may sound, we were lucky you were so incredibly sick.  Had you started to recover with the cocktail of antibiotics they threw at you in that first week, we might have been sent home and your heart would have sustained damage that we couldn’t see or prevent.  Words cannot express how grateful I am to the medical staff who moved so quickly to treat the condition as soon as they had the diagnosis, and the aggressive approach they took to stopping Kawasaki Disease in its tracks.

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So now it is time to start to get back on track to a ‘normal’ life.  We still have to clear up the confusion over whether to immunise or not, and the doctors now want you to have the chicken pox vaccine to reduce the risk of developing Reyes’ Syndrome.  We are still in the middle of the aspirin battle, and your Cardiologist is writing a “strongly worded letter” to your GP to make it clear just how dangerous it would be for you not to have your medication.  Once those things have been cleared up I think we really can start to look forward.  But this is going to be part of our lives for a long time, and I for one will never forget what this disease did to you.  And what it has done to me.  So it is time to start thinking about paying it forward.

This morning we posted the genetic research pack back to Imperial College London, so we will be part of their research study to try to understand the genetic factors that might influence why some children get Kawasaki Disease, but others don’t.  Or why some children suffer coronary complications when others manage to escape that ‘gift’.  Your Rheumatology Consultant wants to present your case to a medical conference, to raise awareness of this disease in the neonatal population, as well as pushing the case for using Infliximab as part of the standard treatment of the disease.  I will never forget one of the consultants saying how much they had learned about Kawasaki Disease from you; you’ve been sent here for a reason little one.

And the next step?  I’ve emailed the professor who is leading the research study to tell him that I am keen to support his efforts wherever I can.  I’ve said that I understand my fundraising contributions will be just a drop in the ocean of the many  thousands of pounds that this research needs, but he has graciously accepted any offer of help and is putting me in contact with a charitable organisation that can help.  He has also said that his international links have donors who will match whatever we raise.  Right now I need to focus on getting over some of the hurdles we are facing, and invest some time in doing some of the things together that can help us to heal from this experience.  But I have my thinking cap on, and will hopefully be able to share with you some ideas that I have to make sure that your story doesn’t only have a happy ending for you, but helps the doctors to find a happy ending for Kawasaki Disease.

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