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.