8th October 2011 (T + 44)

Well on day 45 unfortunately this week has not ended the way I thought it would have at the start of the week.  Chiara has done so well all week, managed all her oral medications and we had been told we would be heading home on Friday if Chiara continued to keep well.  I was getting so excited, I packed up all our belongings on Thursday morning leaving only a selected couple of items that we would require for our journey home on Friday.  I couldn't wait to return home, this week I have missed my boys so much.  On Thursday morning to make our day even better we received the news that Chiara's enzyme level which was measured through blood test had returned showing she was sitting with normal levels and producing the missing enzyme she required on her own therefore there was now no need for ERT, our weekly routine that had been since May had now come to an end and her new bone marrow was carrying out his job nicely.  I was so happy, her transplant had went so well and it was good news all round.  Going by my experiences in my life good news to me usually always means that there is bad news round the corner.  I could of course always look at it another way, that, being that maybe we had had our run of bad luck and we now deserved a break.  My first instinct was correct.  About an hour or so after receiving the news of Chiara's enzyme levels Chiara had a vomit in her pram and spiked a temperature.  Oh no, this cannot be happening but oh yes it was.  Before long Chiara was sitting with a temperature in the high 38 region.  She was commenced on antibiotics and cultures taken from her line for infection.  Her heart rate increased until she was sitting at around 200.  Her respiratory rate increased and she looked unwell.  We struggled most of the day to reduce her temperature but nothing was working.  It was decided later in the afternoon to try and insert a cannula so that we could stop using her central line in case using the line was in fact keeping her temperature high.  Unfortunately we failed trying to insert a cannula after several attempts.  By early evening her temperature slowly came down and her heart rate in turn slowly came down to around 150.  She remained well overnight. 

Friday came and Chiara looked well.  I on the other hand was a wreck.  I couldnt speak to anyone but I was ready to burst into floods of tears.  I had built myself up so much to return home and spend time with my family I was so disappointed.  I looked at Chiara and I felt upset for her also, I wanted so much to take her home and let her play with her brothers as she has improved so much in hospital that she will be like a different girl going home.  All day Friday I cried, I couldn't eat as I was so upset.  I go to a group here on a Wednesday and we talk about "good days and bad days" and this was most definitely one of my bad days. Chiara's results had returned confirming my worst fear, she had a line infection which meant another 7 days IV antibiotics.

I went to bed early on Friday night and Chiara settled well.  Saturday would hopefully be a better day.

Saturday (today) well Chiara slept well awoke at around 8am after having a blood sample taken and sat in her cot drinking her milk happily while I showered.  By the time I came out of the bathroom Chiara was hot, grumpy and shaking in her cot.  Examination revealed she was spiking yet another temperature.  Before long she starting getting a higher and higher temperature.  Every time her central line was being used her temperature soared.  I sat with her in my arms and couldn't believe that 48 hours previously we were planning to be discharged home.  My upset regarding not returning home soon changed to worry and anxiousness regarding Chiara.  She had required more medical intervention in the last 48 hours than she had all the way through transplant.  This girl will do anything to avoid returning home.  It was decided that her line needed to be removed and would have been done almost immediately had I not given her the milk.  Since she had received the milk we would need to wait until lunch time before she could be taken to theatre.  The other problem we faced was that Chiara's platelet count was too low for her to go into theatre therefore she required another platelet transfusion.  Around 11.30am we were told that it would be better to take her to theatre tomorrow morning when they are more prepared especially as Hurler's children can prove to be quite difficult to intubate.  As we were waiting until tomorrow, Chiara's central line would not be getting used to hopefully stop anymore spreading of the infection.  Therefore Chiara had to have a cannula inserted into her foot so that she could receive fluids etc through this.  It was extremely difficult to cannulate her but this is now in her foot.  She settled down over the course of the day.  She had a visit from Uncle Kevan and Auntie Sylvia but sadly no visit from her brothers today.

Tomorrow we face Chiara going into theatre first thing to have her central line removed and hopefully she will improve after this is out of her system.  We are hoping to see the boys tomorrow if Chiara is well enough for them to visit.