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

27th September 2011 (T+ 33)

Well Chiara is still keeping extremely well.  She still has slightly high blood pressure but nothing to cause too much concern.  Yesterday Chiara and I went out for the afternoon on a lovely relaxing retail therapy session.  All for Chiara as she has grown so fast she has outgrown most of the clothes I had brought in to hospital and to be honest she was needing a winter wardrobe anyway. 

Yesterdays bloods were excellent with Neutrafils being 4.1 and having got there on their own with no help from GCSF in days.  Platelets are sitting at 39.

Today (Tuesday) examination of Chiara proved unremarkable with her rash practically away.  Her medication is now starting to be reduced as from today.  Yeh, this must mean as long as she behaves then we could be heading on our way out of here.  Chiara is now only 5 weeks post transplant and is doing so brilliant.  To think she came into hospital on home ventilation, problem airways and was slightly higher risk for transplant due to her airway problems and now shows no sign of airway problems, off the home ventilator and looks fabulous. 

Yesterday Chiara was introduced to the playroom and made herself at home by demolishing the train set and removing all the furniture from the doll's house............................I can see I'll have my work cut out with her and lessons in "playing nice"...

No more sitting around quietly playing for this wee girl, she is off, pulling herself up at anything she can reach and wanting to be on her feet all the time. 

Today Chiara received her Immunoglobulins which she will receive fortnightly for some time to come to give her some additional immunity.  She has 2 more weeks of ERT left and then she finishes with that. 

Today I was chatting regarding Chiara's condition and when she was diagnosed etc and I find it hard to believe how far we have all come in such a short space of time.

Yesterday blood results:

wbc 6.2
hb 106
platelets 39
Neutrafils 4.1

Today blood results:

wbc 7.7-
hb 105
platelets 43
Neutrafils 5.4

WoW - thats all I have to say, how good are these bloods

25th September 2011 (T + 31)

Well today sees the end our Chiara's 5th week in hospital and starting on her 6th and if you had asked me 6 weeks ago how I felt about embarking on a long haul stay and a terrifying experience of transplant I would have said "I am absolutely dreading it" but in actual fact the time has passed quite quickly, I am not denying that on days I had "lows" but equally on days I also had "highs".   I look at Chiara now and see that however scared I was of putting her through transplant it was the correct and of course the only decision myself and Richard could have made.  Her progression and development from transplant alone has been amazing.  I look at this little girl who can now sit unaided, sit up from lying down, pull herself up at furniture and now stand and have to remind myself that at the start of June 2011 Chiara was still unable to sit unaided and we faced the future of her having of delay meeting even the simplest of milestones.  On Friday I was told that my wee girl now no longer looks so much the baby she once was but is developing into a little girl, although this is hard to accept as no one wants their baby to grow up I at the same time have a smile knowing that she has reached a point that back in April 2011 I never thought she would reach.
Anyway, on Thursday I was told that Chiara's IV medication were going to start being changed onto oral.  This is extremely good news but as equally a nightmare as it is yet more syringes of oral medication Chiara has to endure.  Anyway I also was given the fantastic news I could take Chiara out,  not just around the hospital but actually outside in the fresh air.  I thought I was weeks away from going outside with her but on Thursday I was delighted as I now knew I was approaching a step closer to going home.  Chiara got wrapped up and out we went.

Unfortunately that one day soon turned into a wee nightmare as later on that night Chiara woke up vomiting and just not herself.  On the Friday morning she awoke grumpy, covered in a rash again and off her food.  She was started on Steroids again and changing her IV medications to oral was put on hold for the time being.  She was given maintenance fluids as she was a little bit flat looking.  She didn't eat so well for the rest of the day but she picked up after lunch which was good as her Papa arrived to see her and had not seen him for 4 weeks.  By later on that night the rash had faded but she was still off her food. 

On Saturday she awoke in better spirits, was drinking better and was attempting to eat small amounts of food.  Her brothers and Richard came today and we all went out for a walk to the park which was lovely as Saturday was a glorious day.  Chiara was definitely getting back to herself.  The rash was better again today.

Today (Sunday) Chiara is back to herself.  There is one wee small problem, she has developed high Blood Pressure but this can be a side effect from the Steroids and the Cyclosporin medication she takes.  She had a slightly positive fluid balance this morning so she was given a water tablet which resulted in the heaviest wet nappy ever.  This wasn't enough to reduce her Blood Pressure so she was commenced on a Blood Pressure tablet.  Apart from this new wee problem Chiara remains brilliant, happy, eating well again and sleeping well without her BI PAP.  She had visitors today, Uncle Gary and Auntie Linda who I think were pleasantly surprised by her.

Her cells also are still sitting at 100% donor.  So all in all everything is still good.

To top it all off I am charging on with my online Christmas shopping in here!!!!!

21st September 2011 (T + 27)

Sorry about the delay everyone in the updates, basically not really very much been happening on a daily basis so really I like to gather up about 3 days worth of information.  So here we go...............

On Monday I arrived at ITU to find Chiara extremely well, no temperatures overnight which is great although she did have a small vomit at around midnight for no apparent reason.  Chiara still remains off her BI PAP and continues to improve extremely well off this considering all she has went through.  We moved out of ITU on Monday and now on a Ward.  I actually felt quite emotional leaving ITU (you are all thinking now I am mad) but everyone had been lovely, caring and took an interest in Chiara's condition and made me feel not so lonely as I could have done.  Anyway after 4 and a half weeks Chiara and I now embarked on the new chapter of our journey or rather her journey which every way you care to look at it. 

Our first night on the ward proved very tiring for myself as because Chiara is still on quite a lot of medications she still requires a fair amount of medical intervention all night and on top of this Monday night Chiara was getting another sleep study carried out to ensure she was coping ok of the BIPAP.  Anyway, by midnight Chiara decided to get up and vomit yet again at the same time as the previous night.  She settled down to sleep the rest of the night but in our room she was the only one that did.  I couldn't settle at all and before I knew it I was up getting dressed to face Tuesday.

On Tuesday Chiara's redness and rash had practically disappeared, which is great as it shows it is responsive to steroids.  She remains temperature free, again great news as it shows probably infection free.  She remains on some creams, increased dose of Steroid and she received a dose of anti-sickness IV at 10pm to hopefully stop the midnight vomiting.  Her appetite is still very good and still has had no signs of requiring an NG tube.  Tuesday night remained another sleepless night for myself but Chiara slept well.  I have a small problem of her now realising I am sleeping at side of her, so she now sits up and laughs at me through the cot bars, cute I know but at the small hours of the morning........ not so much. 

Today she is fantastic, happy, less red but has now become attached to my hip.  Everytime I am out of her sight she is crying her eyes out, this could be fun when we get home.......
Her Neutrafils today are over 3, her Platelets are in the hundreds but yesterday she received yet another platelet transfusion.  She has been receiving doses of GCSF the last couple of nights but tonight as her count was so good she didn't receive any.  We still remain in protective isolation so no visitors yet except for Parents, Grandparents and siblings.  Not as bad as you all may think as we have windows which look onto the corridor so she can see all the other kids running around which I think she enjoys as she misses her brothers.  We also have a window looking into car park so its great I can sit and people watch. 

No idea of when we will be heading home but first thing first we need to get out of isolation and that will be a huge step forward. 

I have one request from everyone who reads Chiara's page,  on the news tonight was the story of a little boy who potentially may need to receive a BMT.  Before Chiara was diagnosed with Hurlers the only thing I ever did was give blood and after now being on the receiving end of being a mother whose child required blood transfusions regularly throughout transplant I would ask if possible you could donate when and if you can.  Its a small, painless half hour or so out of your day but without people donating blood my little girl may not have continued to improve the way she has.  Also an even bigger request,  without donors on the Bone Marrow Register Chiara and other children like Chiara would not be given the chance of a new life through this life saving procedure.  Bone Marrow Donors are extremely valuable and my plea is if you have time to enter yourself on the Bone Marrow Register then please please donate.  One person is just not enough, there are so many children that require BMT and for some children matches are very far and few between.  I realise only now that I was ignorant to how important blood, bone marrow donation and other donations actually were.  I probably, no in fact I know I went about with an attitude that because it wasn't happening to me then I didn't really have to pay much attention to important pleas for donations.  After living and sharing my girl's story I will now pay more attention and will donate what I can if it can help save a life and hopefully will have continued support with this.  It is all too easy to turn a blind eye....................................

Anyone interested in registering as a donor you can go online.  www.anthonynolan.org/register
You can also go to East Kilbride Holiday Inn on Sunday 9th October between 10am and 4pm to join the bone marrow list.  The down side is you have to be over 16 and under 40 but if you dont fall into this age group then feel free to pass it on to someone who might be eligable.

In Chiara's situation her brothers were not a match, I was not a match but her dad was a match but unfortunately Richard obviously carries the Hurlers Disease.  Chiara received a Stem Cell transplant and if it weren't for donation from strangers then Chiara would not have been given a 2nd chance at life and you wouldn't be reading this blog..

Big Thanks