12th October 2011 (T + 48)

Well this morning, Chiara awoke happy and smiley, as she always is.  She had a great night sleeping as she went down last night at 6.30pm and that was her until 7.30am this morning.  Richard arrived bright and early this morning so we all managed to go for breakfast together.  Over the last couple of days I have come to realise actually how amazingly good Chiara has done through this whole BMT.  I have had so many of the medical staff tell me how amazing it is that she has not required an NG tube for feeding or for medication through this whole process.  We were also extremely lucky that Chiara did not develop any problems with her mouth in that we had been prepared for her developing ulcers etc but she never did. 

At 12noon today Chiara received her last dose of IV antibiotic and had a repeat blood test carried out.  Today was a turning point in all of our lives as Chiara handed over her BIPAP machine as she no longer required it. Not having to be ventilated at night time will have a huge impact in our whole family's life.  We were given 2 bags of all Chiara's medication that she will be on for the foreseeable future but this was a small price to pay for having a healthier girl.

At 2.30pm Chiara, Richard and myself made the exciting journey of leaving the hospital and heading home with our girl for her to start her new life.  We will have a couple day visits back and forth but at this moment do not intend to make any more long stay. 

Tonight I sit writing this from the comfort of my own home again.  Tonight Chiara for this first time since 20th July 2010 (day she was born) is sleeping in her own room.  Her brothers leave tomorrow to go on holiday with their Nana and Papa so sadly she wont see them this week but it feels so good to be all under one roof again.  Today is the first day of the start of a new life for us all. 

We have all come such a long way since that horrible day in April but have all come out the other end having coped with everything that was thrown at us.  It is a well known saying that what doesnt break you will make you stronger and in this case it is so true.  I have went from being someone who was at breaking point with no way of seeing any light at the end of the tunnel to being a stronger person than I ever thought I could be.  Along our journey I have met some wonderful people, and without some of these people, my life in the hospital would not have been as easy to cope with.

We know Chiara has to endure spinal surgery next year but for now I am going to enjoy our time together as a family and try and forget the first months of her life that we were robbed of.

Thank you to everyone who supported us over these last few months.  We could not have done it without the help you have all given.

10th October 2011 (T + 46)

Well the weekend turned out to be a very eventful weekend.  Chiara had a comfortable night through Saturday night, no temperature.  On Sunday we waited for her to be taken to theatre which was around 11.00am.  Before she went she had to have more blood tests taken so this meant more needle pricks for Chiara which was very uncomfortable for her and very distressing to watch.  However the required blood was obtained so that was a result. 

She went into theatre just after 11am and Richard and I went for a coffee, we were called to collect her again from recovery around 12.15.  She looked great, I had been asked to give theatre down her home BIPAP in case they needed to use it but gladly it was still in tact and never been used.  Richard carried her back to the ward attached to the Sats monitor and drip.  She was a bit sleepy but still looked great considering she had just been to theatre.  She quickly drank a bottle of milk and settled down to sleep.

Shortly after her brothers, Nana and Papa arrived.  She awoke when she heard them and was happy to see them all again.  We all had a lovely afternoon and the boys stayed later and we had a cheeky wee chinese and watched the X Factor.

Chiara settled well overnight, her saturation levels remained above 95%, her heart rate within normal limits, her temperature normal and respiratory rate normal.  She continues on IV antibiotics for the next couple of days to cover any remaining infection.

Chiara awoke today (Monday) well and happy.  Her Graft V Host rash which was my main concern for not returning home seems to have faded into the background with all the drama around the central line. The rash most certainly is still visible but not as livid as before.  She remains on oral Steroids to try and treat this.  This morning I was told Chiara would remain on IV antibiotics for 2 days because of the Sepsis.  It had been decided that because Chiara really only requires her central line for blood tests and fortnightly IGG then she would have a couple of weeks break from having a central line in.  To make this possible she is receiving her IGG today which should be next week (this is her Immunoglobulin) This means we start the discharge process over again shortly but an exact day when we find out we are keeping to ourselves so we dont jinx it.LOL

Blood Counts today are:

WBC  10.1
Hbg 82
Platelets 54
Neutrafils 7.5

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. 

3rd October 2011 (T + 39)

Well it has been a few days since I have updated Chiara's blog but nothing major to document.

Chiara remains fabulous, so much so she looks a bit of a fraud. 

On Thursday Chiara received what was hopefully her last infusion of ERT.  Her urine has been sent away to check her GAG level and her bloods were taken to check her enzyme level and depending on the results of these that will determine whether she receives another dose of ERT. Hopefully no more ERT for her and we can put that part of her life and illness behind us. 

Her Graft V Host rash seems to have cleared well after her increased dose of Steroids so fingers crossed Graft V Host stays away.

On Friday we had a lovely visit from Papa and went for lunch and a play in the park which was great and we all had a super time. 

On Saturday it was my birthday, not the nicest of places to spend your birthday but that's life, I spent last years birthday in Stirling Royal so at least this year it was a different hospital.  Saturday was a strange day it was mixed emotions and mood.  A few things happened which I wont go into but I think I was beginning to feel like a caged animal.  Anyway later on in the day Richard and the boys arrived and we had a wee birthday night in Chiara's room.

On Sunday Chiara and I went into town and met Nana, this was the first time in 6 weeks that my mum had seen Chiara so to be honest I wasn't sure how the day would go.  At first Chiara seemed really strange and pulled some funny faces at my mum but within a wee short while she was laughing and at ease again with her Nana.  

Today (Monday) Chiara has had all her IV medicines changed onto oral, therefore this is the start of the discharge process for us YE HA,  within a few days we could be heading home.  Chiara needs to remain in for a few days after changing onto her orals so her blood can be monitored to ensure her Cyclosporin levels are remaining at the correct level.  We will return after discharge at least twice a week to have blood checks etc.  She remains on medicine for her blood pressure but this looks to have returned to normal or at least stabilised which is good.  Her steroids have been reduced by 20% every 48 hours so today we are on a really small dose.  The down side of having her IVs changed to orals is that it is extra oral medicine syringes Chiara has to take.

Currently Chiara receives orally 7 syringes of medicines at 8.30, 2 syringes at 12noon, 2 syringes at 2pm, 1syringe at 4pm and then another 6 at 7pm.  On top of this she will now receive Cyclosporin at 10am and 10pm and another anti-fungal and steroid.  She manages all these so well and I admire her for being able to take these orally without any feeding tube.  I myself, sometimes struggle to take even 2 Paracetamol.

To make things clear for everyone, if Chiara behaves, manages to take her medicines all orally and keep good blood levels, rash doesnt reappear and she doesnt spike any temperatures then we could be home to watch X-Factor in our own house at the weekend.  All cleaners and anyone willing to empty the overflowing laudry basket that is no doubt waiting for me at home are all welcome to go and make a start on the house before I come home.  Richard mentioned getting industrial cleaners in so I am now wondering what sort of a mess am I coming home to..........................................how bad can 3 boys/men mess up a house!!!!!!!!!!!!!!!

Yesterday her results are as follows:

White Cell  7.8
Hb  93
Platelets  44
Neutrafils  5.5