130 days post transplant - what a year

Sorry for the delayed update on Chiara's progress but life has been pretty hectic.  After Chiara's last admission she was discharged home on the 22nd December 2011.  We were so excited to be getting her home for Christmas.  She still wasn't 100 percent herself but was certainly a lot better than what she had been.  Her story was to be published in the Glasgow Herald on Christmas Eve, Richard and I as her parents were so proud.

We were to return on Christmas eve to have her observations checked and her bloods checked.  As we sat anxiously waiting we just couldn't wait until we returned back home to prepare for Santa.  Our anxiety turned to fear as Chiara's FBC (full blood count) returned as showing she had platelets of only 2 and her Neutrafils had dropped to below 1.  Her Haemaglobin was also low and she was to receive a platelet transfusion and blood transfusion and also an infusion of GCSF to try and boost her Neutrafils.  My stomach heaved as I suddenly thought all our plans and hopes for our lovely family Christmas were just about to be shattered.  She received her GCSF and Platelets but her blood was going to take around 12 hours to be ready for transfusion so left later on Christmas eve afternoon and returned home. We would return on Boxing Day to receive her blood transfusion.

Christmas in the Petersen/Fox household on Christmas morning was magical and exciting as all 3 children scrambled round the living room opening presents.  It was a dream come true to be spending  Christmas morning together as a family in the comfort of our own home when only just over a week ago Chiara was connected to a ventilator. 

Boxing Day came and Chiara and I travelled through to hospital for 9am.  She had her routine FBC taken and again we waited on the result.  We already knew that she was to receive a blood transfusion but added to that she would again require another platelet transfusion as her platelets were only 2.  She would also require more GCSF as her Neutrafils today were 0.  This was just my worst nightmare, what was happening no one knew.  We would return on the 27th December 2010 for routine checks.

The 27th came and we attended for routine checks.  Her FBC came back as showing no Neutrafils and Platelets of 1.  Now we were faced with the news that we had always dreaded hearing, her new bone marrow graft was failing.  Chiara was admitted to hospital on 27th December and on 28th December was taken to theatre where she underwent bone marrow biopsy.  The knots and sickness I felt in the pit of my stomach I cant even begin to explain.  These feelings grew worse as I entered the Consultant's room to be told that there were very little cells available from her new marrow and was looking like she was going to be needed a 2nd transplant.  I went to pieces and sobbed like I've never sobbed for months.  All our hard work and effort and everything Chiara had gone through had been for nothing.  We were told there was always a chance that it was just a virus that had caused this and we would receive results on Friday.

On Thursday Chiara deteriorated and her breathing became hard work for her.  She was extremely irritable and agitated.  Not the girl we all knew.  It was heart breaking to watch.  Early hours of Friday morning Chiara was admitted into Intensive Care.  She underwent a CT scan for fear that she had developed a bleed on her brain due to the low platelets.  My stomach again heaved, how on earth could all this be happening.  She returned from the CT scan and we were told that after the CT scan they had aspirated her stomach and drew back about 20ml of blood, I wanted to turn and run away, my immediate thought was she had internal bleeding due to the low platelets.  However, her CT scan showed no abnormalities and the doctor didn't seem too concerned about the blood from the stomach.  It was possible that her irritability was caused from her Morphine withdrawal or simply due to her virus.  She had come back positive for Rhinovirus a 2nd time.  She was commenced yet again on Morphine and antibiotics.  Later Friday afternoon we had a meeting with her Consultants.  After discussions with other medical staff and transplant centres the general consensus was that it was possible that Chiara's marrow had been suppressed due to a virus or a drug.  It was also noted that she had developed antibodies in her blood and it could be possible that these were destroying her platelets, Neutrafils etc before they even reached her bone marrow.  This news albeit not great was better than the thought that she was losing her graft. 

Today (Saturday) Chiara was commenced on her new infusion to try and destroy the antibodies.  She seemed more content today than she has in days.  She remains in ITU on CPAP.  She will receive Platelet transfusions on a daily basis and blood transfusions as and when she requires it.  Her tiny body is covered in bruises and a blood pricked rash due to the low platelets. This is not how I imagined I would be spending the last night of 2011 or rather hoped I would not spending.  I head into 2012 with my 2 boys and Richard at home, myself in a hospital room and my daughter in Intensive Care.  What do I have to celebrate about 2011, absolutely nothing, my only hope is that 2012 is a better year for all of us. 

Happy New Year to all my friends, family and everyone who has followed Chiara's story.

A not so nice lead up to xmas (15.12.11)

Well today is Chiara's brother Joshua's 13th birthday.  Sadly yet again Chiara misses another family birthday as today she lies connected to a ventilator.

Last Thursday and Friday Chiara's blood counts had dropped to under one and we had the horrible feeling she was losing her graft as she appeared well and no signs of illness to account for these drops.  Later on the Friday afternoon she became grumpy and just generally not herself.  After a long battle with myself I decided later on that night to take her through to hospital.  She presented there well, no temperature and breathing normally.  On checking her oxygen saturation levels it appeared she was only 88%.  She was started on antibiotics and 1litre Oxygen.  She had a fairly unsettled night and by the morning she seemed to be getting a little worse.  On Saturday morning she had an xray.  We were informed later that this was awful and her lung had collapsed and she could possibly have Pneumocystic Pneumonia.  Once again I had that terrible feeling in my stomach and literally felt sick to my stomach.  What was to follow the next few days we did not know or rather not prepared for.

Sunday she was still lively but her Oxygen requirement had to be increased and she was started on more antibiotics.  Sunday afternoon seemed to change drastically so quickly.  She started working harder to breathe and her Oxygen requirement was being increased more and more.  Within half an hour she had deteriorated so much that she was completely Oxygen dependent.  She was then transferred from the Ward to Intensive Care.  She was tried on BIPAP for a few hours but she still had enough energy to climb all over the cot and sit up.  Her saturation levels were dropping slowly and not managing to increase.  We were told she required a blood transfusion also and this would hopefully maybe help he Oxygen levels.  The blood would be available around 1 or 2 in the morning.  Unfortunately by 2am Chiara had become so distressed that her Oxygen levels just dropped and dropped and I stood and watched them go down to 40 until I could not watch any longer and I left the room.  It was then I knew intubation would be taking place very shortly.  I sat for around an hour waiting and reliving all the feelings I had went through on that horrible night back in April when she was first ventilated.  Here I was 2 weeks before Christmas waiting for my wee girl to be connected back up to ventilator.  She was placed on an Oscillator which is a special ventilator that is kinder to her lungs.

On Wednesday Chiara was changed from the Oscillator to conventional ventilator as her blood gases etc were all good and her xray was improving and showing her lung was re inflating.  She was found positive for Rhinovirus which basically is just a common cold.  Through all this she still remains feisty and has managed to disconnect herself from the vent twice and is keeping medical staff on their toes as she tries to climb out of cot so she is nearly always kept heavily sedated and paralysed.  It once again is horrible to watch especially at this time of year but she is making progress.  I am only thankful that my gut instinct was right to bring her through to hospital even although she never appeared unwell.

Today we are hoping she will improve even more and hopefully be excubated very soon.

1st December 2011 (100 days)

Sorry folks for not updating before now but I have just been enjoying having Chiara home and gave us all a bit of time to forget she actually had Hurlers.  Today marks Chiara's 100 days post transplant.  This is a huge milestone for Chiara.  Getting through 100 days with minimal problems and no graft rejection is a huge acheivement.  Tonight we marked this occasion with a bottle of Champers.......to follow on the bottle we had when we brought her home from transplant in October........if we are going to be marking
every special milestone in her life then we are gonnae be needing a lot of Champers lol.  

Anyway after returning home in October Chiara has kept extremely well.  It has been an emotional rollercoaster for all of us.  I  am finding the constant travelling back and forth 2 to 3 times a week to Yorkhill very stressful.  I miss working terrible although I love spending loads of time with Chiara albeit she is extremely hard work.  On the 8th November we were given the fantastic news that Chiara's hearing levels had went from 80 down to 30/40 which is level of conversation so currently Chiara has no need to wear hearing aids.  As a family we know she can hear everything we are saying and she responds to all noise we make.  She is making great progress with her teacher and currently she is not developmentally behind schedule.  She crawls around like a normal child, she can pull herself up at furniture and walk around or walk around with her walker. 

Chiara still has no need for night time ventilation but on a recent visit to hospital she has another sleep study carried out which showed that her results weren't as good as the last.  A lot of Chiara's problems are being put down to the fact that she is carrying so much extra weight.  She has gained 20% of her body weight in 2 months.  Unfortunately this has been down to the steroids so currently she is on the her last reduction and hopefully when these are completely finished she will lose some weight and hopefully her breathing etc will return to normal.  It is not a huge problem but you can tell she gets out of breath quicker than normal because of the extra weight.

Chiara has also developed a lot of excessive body hair over her shoulder and back, this is a side effect of her Cyclosporin which is the drug to stop rejection so its a small price to pay although not nice to look at on your wee baby.  We have been told this will disappear once the drug is finished and hopefully this will be in a few months.

A few weeks ago Chiara was admitted to hospital due to her contracting what was thought to be hand, foot and mouth which put her off her food and fluid.  I myself didn't cope with this admission too well and went into complete melt down and Richard had to come and stay with her in the hospital.  My feelings at that time were that my whole life now was Chiara, my whole day was Chiara, my boys no longer had a mother with them since Chiara was born, my thoughts were that the boys gained a sister and lost a mother. I know Chiara didn't ask to be born the way she was but sometimes the pressure and strain can just get so much that you forget who you actually are.  Most of my days are spent travelling to and from appointments and the rest of my family are left to bring themselves up.  I hate the thought that one day I will wake up and my boys will be all grown up and I will have missed out on all those years of their lives.  I know many of you reading this wont understand what it is like and possibly even think I am selfish for thinking feeling like this but having a child with disabilities no matter how small or how huge the pressure and strain it puts on a family is immense.  I am now in the process of getting shared care hopefully for Chiara with a closer hospital and she is having home visits to have her bloods checked so slowly we are returning to a normal family life and my hope is that next year I will be able to obtain some work. 

We have been through so many highs and lows this year that I now look forward to Christmas with my family and hope that next year is a better year for all of us.

Chiara has her spinal appointment in January when we will probably be told when she will receive her spinal correction.  Hopefully the last surgery for a while.

On Saturday I am attending a charity disco, the proceeds of which are being donated to Hurlers.  I am so grateful to everyone who has shown support to Chiara and the MPS society.

We were asked to be included in a television programme to be aired in December regarding Chiara's story but unfortunately in the last week we have been told that she is no longer going to be appearing.  I have just finished writing an article about Chiara's story which will appear in the next issue of the MPS magazine so hopefully this will bring hope and smiles to a lot of peoples faces.

Merry Christmas everyone x

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

18th September 2011 (T + 24)

Today is Day 25 of transplant and following a great success story until now Chiara took a slight turn in her recovery.  I am at the moment looking at this as only a slight complication post transplant and keep my fingers crossed things will improve and not head in a downward slope.

Following my entry on Wednesday, Chiara developed a redness around her neck which was being treated with cream but she was becoming slightly flushed around her face. She had spiked a temperature overnight through to Thursday so as per protocol she had several swabs taken for culture and was commenced on a broad spectrum antibiotic to cover any infection.  She was tired and out of sorts all day Thursday and her redness had progressed into a rash over her body.  At that point it was thought it could potentially be a drug allergy.  Her CRP markers had increased slightly.  Her Neutrafils were  1.9.  I settled her down on Thursday night and headed over the road and at that point I didn't feel overly concerned.

On Friday I arrived and she was extremely flushed and the rash had no improvement at all.  Her temperature had increased through the night and she certainly wasn't her normal self, she was slightly off her food and had vomited a couple of times with her medicines.  Later on in the day I was told that it looked as if she was developing Graft V Host Disease.  This is when the stem cells start fighting against her own body.  I was hoping and praying this wouldn't happen but despite all the prophylactic steroids and medication she is given to prevent this I suppose there was always the risk that this could happen.  I did read that if it happens within the first 100 days then its considered acute so I am hoping this is true. Chiara was prescribed 2 lots of steroid cream to be applied twice a day.  In addition to this she was given a platelet transfusion again as her platelets were low.

On Saturday I had been telephoned at 2am in the morning as Chiara had become unwell with high temperature, increased heart rate and "out of sorts".  The hospital had said they were going to put her back on her BIPAP machine.  I quickly dressed and ran over to hospital anxious to see what I was going to be confronted by.  When I arrived Chiara lay struggling around her cot, anxious, agitated, extremely flushed and very uncomfortable looking.  I quickly placed her on her BIPAP machine and she settled down.  I felt sick at the thought of her requiring her BIPAP again after getting her off it for only a few nights.  It was pointed out to me that it wasn't through respiratory compromise that she was given her BIPAP mask it was more just to give her a rest as she appeared to be working harder at breathing which was probably a complication from the high temperature which in turn had given her the increased heart rate.  She slept for a few hours comfortably as I sat by her bed, her temperature was struggling to decrease and I had discussed with the staff at the possibility of her being allergic to one of her antibiotics.  Later on in the morning one of her antibiotics was stopped to allow them to see if her symptoms improved.  She appeared to get better as the day progressed and her temperature dropped and stabilised.  Her colour became less flushed and I thought she was on the road to recovery but by early evening after her bath she became red and inflamed therefore it was not a side effect from the antibiotic.  Now I had to  digest the news that it was most probably Graft v Host or possibly an engraftment side effect that sometimes happens in the early stages, either one had the same treatment.  She recevied a blood transfusion today also and was given her GCSF again tonight as her Neutrafils were only 1.1.

The boys and Richard had arrived on Saturday but as Chiara was not 100 percent the boys didnt get in to see her.   Neutrafils today are over 3 but she did receive the GCSF last night.  She was reviewed and it was decided that she would be commenced on an additional dose of Steroid to try and improved the rash and the redness.  Chiara already receives a prophylactic dose of Steroid in the morning she now she will receive an additional dose at night for approximately the next 48 hours.  We settled down on Saturday night and I prayed the phone didnt ring with more bad news.

The news today (Sunday) is her temperature remained normal overnight last night.  She ate well today, played well.  The rash most of the day seemed to be improving along with the redness of her face although yet again after her bath and after I applied her cream her face became extremely flushed and inflammed.  I settled her down tonight (for the first time all week since giving up her BIPAP mask)on her side cuddling into her blanket.  Fingers crossed that she keeps well with no temperature.  If she remains well then tomorrow we should be on a move to a  different ward.  This would be great as it is another step closer to getting home but I am not getting my hopes up as the complications in the last few days has been enough to dampen my spirits. 

14th September 2011 (T + 20)

Today is Day 21 of Transplant.  3 weeks on and Chiara is still keeping excellent heath.  Eating and drinking are still normal.  We had stopped her Thick and Easy (a thickner) in her milk yesterday to see if she could tolerate the milk on its own but after a tiny vomit this morning and a rather large one at lunch we have decided to go back to adding the Thick and Easy. 

Monday night Chiara was tried of her BI PAP machine to see if she could sleep all night without requiring the added pressure.  Trying to put her to sleep proved to be quite a problem as she is so use to going to sleep with her mask on but after a lot of crawling around the cot she eventually fell asleep.  I myself spent a lot of the night phoning over to the unit to check how she was getting on and asking what the figures were on the sleep study machine.  Every time I phoned the figures were great but I didn't want to get too excited as it was really only spot checks and the real conclusive result would come from the machine itself once the pattern of the whole nights sleep was uploaded and checked by the Doctors.  Anyway after a very anxious night the news was Chiara now no longer requires her BI PAP machine.  After long term ventilation at night Chiara is now mask free going to sleep.  So happy, happy,happy.  She will remain in ITU for a couple more nights to make sure she is coping well without the BIPAP then she will be moved into a ward.

As Chiara's Neutrafils were high on Sunday it was decided to stop her GCSF to establish a more true reading.  The result of not getting the GCSF is her Neutrafils will drop slightly but hopefully will increase again without the GCSF.  Today they are sitting at 1.2 which is a lot lower than what they were but still good enough not to require GCSF.  This will be her 4th day without so fingers crossed the Neutrafils keep multiplying on their own.  Her platelets were low today so she required a transfusion of platelets. 

Today Chiara also attended Audiology as her hearing seems to have improved and there is some questioning round if her hearing aids maybe slightly loud for her as she blinks all the time anyone talks or she hears a noise once she has her aids inserted.  For the first time in 3 weeks Chiara was allowed beyond the double doors to head over to the Audiology Department.  Unfortunately the hearing test failed as Chiara was uncooperative at carrying out the test. 

Our other news today which is just fantastic and I have kept to the end is that Chiara's blood test taken to see how much of her cells are donor and how much are her own has shown that Chiara is 100% donor.  How wonderful and happy do I feel today.  I have been on a high all day.  In the last few days everything that has happened has been positive so much so that  a small part of me hopes that nothing negative comes along to slap us in the face.

Fingers crossed everything continues to be positive and gives Chiara the break she deserves after all the bad luck and ill health she has had.

Through all this she still remains a happy, dancing, delightful beautiful girl who can put a smile on anyone's face that happens to pop into see her.

11th September 2011 (t +18)

Well today  Chiara is brilliant.  She still remains in good mood, eating extremely well.  She has a lot of thrush in her mouth but doesnt seem to bother her. 

Her brothers and Dad came through today and because her Neutrafils were over 1 the boys were allowed into the room beside her and it was so great to have us all together again.

Myself and Richard went out with the boys in the afternoon and spent time with them at the Transport Museum.  Before we left to go there Chiara's bloods came back showing her Neutrafil level was 6.8 so since this was such a huge jump from yesterday at 1.2 it was decided to repeat the test in case it was contaminated or something.  Because of this I couldn't wait to get back to hospital to find out her correct level.  I have become so obsessed with blood and numbers that  I'm mad.  The repeat test showed that her Neutrafil level was 7.9, her white cells had increased and also her Platelets had went up from 17 to 19.  I couldn't help but have the biggest smile.   These were the numbers we were waiting on.

Tonight Chiara will not get her GCSF (the drug that helps stimulate the cells to grow).  Hopefully she will still be able to maintain high levels of Neutrafils off this drug.

On weighing her tonight we discovered she has also put on more weight and is now up to 21lb 4. 

I settled her down to sleep , still on her BI PAP but hopefully this week she will get a sleep study carried out and maybe even a small chance of giving up the use of her BI PAP.

I left her and spent a lovely night with my boys and Richard having a nice meal.


t

9th September 2011 (T +16)

Well today is Transplant day plus 16.  I dont really have much to say today that is different except ......................................................................................Today's NEUTRAFILS have went up to 1.2,
 I  am so happy happy happy.  This means Chiara's brothers can visit her on Sunday. 

This more than makes up for all the other crappy stuff that has went on today such as my 2 loads of washing not spinning and dark colours dying Chiara's comfort blanket and the cash machine being out of order and I had no money and on and on and on but who cares....................Chiara has NEUTRAFILS and they have increased. 

Her platelets are still low at 19 so she will probably require a small transfusion over weekend but this we can handle.

8th September 2011 (T +13, 14 and 15)

Well today is Transplant Day +15.  Over the last 3 days  Chiara has still remained amazingly well.  No sore mouth, sickness or loss of appetite.  She is still eating and drinking as normal.  She is playing or rather throwing herself around and being boisterous but I would rather this than see her lying in hospital cot. 

Yesterday Chiara got her Immunoglobulin and coped well with this as usual with no effects.  She had a rather large vomit last night but unfortunately this was "mouthwash" induced and I wont be repeating that experience again.

Today she ate very well again all day. She received her weekly treatment of ERT which she will continue to receive until we are certain she has fully engrafted. 

The good good good news is today her blood level of Neutrafils were 0.7, so we are on the up.  Please please don't start coming down.............if they keep rising then before long we can have visitors again.  As much as Chiara loves me I am sure sometimes she looks and thinks how long have we been locked up together.  She looks longingly at the double doors as if to think "I wonder what is beyond there". ......one day Chiara you will find out.

Her skin is marking very easily at the moment but I am guessing this is because her platelets are still sitting too low.  She will probably require another platelet transfusion but hopefully after that she will start holding her platelet count.

She no longer gets her anti-sickness infusion but still receives 6 oral medicines at night, slowly but surely these are reducing.

Over the next few days she will be assessed by respiratory who will make the decision if they feel she is well enough to have a sleep study carried out in view of her requirements of the BIPAP at night.

I know it is a huge hope but if Chiara could leave here having had a transplant with minimal complications and no longer needing her ventilator at night I would be absolutely on cloud 9. 

Having said this Chiara is so used to being put to sleep with her ventilator that she jumps around the cot with no intention of sleeping and then the minute I put her mask on she just rolls over onto her side and goes straight to sleep.  Without the mask she doesn't seem to associate night time with sleep, I think it will be a case of giving the ventilator back but keeping the headgear and mask so she can have that to put her to sleep at night.................or a more drastic action of a balaclava so she knows its bedtime. 

This weekend I look forward to spending time with the boys and maybe have them stay overnight with me.  I miss them so much and last night Lindsay was upset talking to me as he misses Chiara and me so much.  I cant wait for day when we all can become a family again or rather start living a normal family life.  From the day and hour Chiara has been born her whole first year of life has been spent mostly in hospital.  I know the boys understand why I am away and the reason why Chiara has to spend so much time in hospital but at the same time they are only children themselves and it is hard on everyone. 

Having one child with a health problem doesn't just affect that child it affects a whole family and more.

Anyway, happy September weekend everyone whatever you are doing and thanks to everyone who  has been following Chiara's progress and has been sending us messages through Facebook etc.  Although I dont reply to them all I read every one and realise how much my wee princess has touched so many people's lives. 

You will see by the pictures she is still as happy and certainly still as cheeky..

5th September 2011 (T +10,11 and 12)

Well today is T + 12 and Chiara is absolutely great.  She is still eating well, drinking well, taking all oral medicines no problem and is still her happy wild self. 

On Saturday she required a platelet transfusion as her platelets had dropped to 17 but she tolerated this very well and had no problems related to this. 

She continues to get her GCSF which hopefully should start to help stimulate her new cells to grow as her neutrafils are now currently sitting at 0 and she is at risk of getting infections so the quicker the neutrafils increase the happier I will feel.

She is continuing to try her best to walk and she is certainly not far off it now. 

What is left of her hair is still sadly falling out but to me she is still as cute as ever but it is sad after her bath not to be able to brush her hair like I always did. I am sure I will be more than making up for it in the years to come when I am sure her hair will grow back beautiful and healthy.

She still settles well at night and is still on her BIPAP. 

Fingers crossed the next few days will remain as good and as positive.  I cant really believe that I have been here for nearly 3 weeks already, in some ways it seems ages and in others it has flown by and yet so much has happened in a short space of time.

2nd September 2011 (T + 8 + 9)

There was no entry to Chiara's diary yesterday which was T+8 as there was nothing really happening which I suppose is good news.  She remains very well, still has frequent soiled nappies which is resulting in a bit of a red bottom but apart from that she is very very well.  She is still eating normally and drinking normally therefore to date we have had no need for an NG tube and hopefully this will continue.  Yesterday Chiara received her weekly ERT which will continue for the next few weeks until her new marrow has hopefully engrafted.  She settled well last night and still requires her BI PAP for overnight sleeping.  The only distressing thing more for myself rather than Chiara was yesterday her hair was noticeably falling out.  She became extremely thin at the sides and her wee cot sheets etc were full of hair.

Today I made the horrible decision to have what remained of her hair shaved off.  I came to the decision at lunch time when her hair was falling in her lunch, it was in her fingers and all over her clothes.  It was unfair to have her go through all that when really the only reason I was putting off shaving her head for was me.  After all I knew in my head that after the Chemotherapy had stopped then loosing her hair was ineviatable.  At 4.15pm we started shaving.  She tolerated the clippers very well and in the end it was not as bad as what I thought it would be.  Tonight she was bathed, fed and was looking extremely clean and hygienic again. 

She restarted her GCSF tonight which hopefully will stimulate her new cells to grow.  Her Neutrafils are 0.1 so now we just need to see them go up and we will all be happy happy happy.

She settled well tonight and still remains on BIPAP.

Tomorrow I look forward to my boys coming through for a wee visit.