31st August 2011 (T + 7 )

Well yesterday was T + 6 but nothing had really changed. Chiara remained very well, still energetic and eating well.  She received her GSCF which is a grow factor for the new cells.  She settled well last night and still remains on her BIPAP. 

Today she was as happy as ever, she was sitting in her cot waiting patiently for Richard and I to arrive and pick her up for cuddles.  The smile she gives us in the morning is enough to lighten up the day.

She is still eating very well.  Her weight has dropped slightly by a couple of ounce but I suppose given the amount of soiled nappies she produces its not surprising. 

She received her Immunoglobulin today and will receive her ERT tomorrow. 

She had some physiotherapy this morning and has lots of new exercises for both of us to practice.  We are trying to encourage her to pull herself up by herself from her knees rather than pulling up straight from a sitting position.  She is able to perform all the different positions on the exercise sheet she just requires a little practice to put then all together in correct sequence. With a lot of hard work and determination I am hopeful Chiara will be walking by the time we leave here in a couple of months.

All in all I am delighted with her progress.

29th August 2011 (T + 5)

Chiara still remains really good today.   She had bright red cheeks when I arrived first thing this morning and I think she is in a bit of pain with her teeth.  She ate very well again today.  Another of her medicines have stopped therefore we are now down to 6 syringes of medicine. 

Today I was holding her hands trying to encourage her to walk around the room. She managed very well and with any luck we could even leave this hospital in a couple of months with her walking.

She was a bit cranky throughout the afternoon but her naps today hadn't went accordingly to plan and I am hoping her mood was due to lack of sleep and sore teeth rather than anything else.

A blood test today revealed her Haemoglobin was only 6.9 therefore she was given a blood transfusion today.  This was transfused over about 3 hours.  Before the transfusion she certainly didn't have a look of having a low Haemoglobin as she was quite pink around the face and still had lots of energy.  However the transfusion took place and the blood result afterwards showed her to have a Haemoglobin of over 12.

She settled down very well tonight and she even managed a wee flying visit from Daddy today. As Chiara is still in total strict isolation visitors are only myself and Richard therefore the day can be quite long when Chiara only has myself for company along with her non stop DVD Toy Story.

I on the other hand am finding it difficult to sleep and today felt extremely tired and drained. Richard stayed a couple of hours tonight so that he could have dinner with me as eating alone every night in a huge kitchen with a wee microwave meal for one proves to be very lonely after a while.   He left and I returned to my lonely little bedroom only to find a lovely bunch of flowers in my bathroom left by Richard to brighten up my wee home for now.

2 weeks down hopefully only about another 8 to 10 to go.

28th August 2011 (T +4)

Today Chiara remained very well again.  No sign of any mouth sores as yet.  She does have quite a lot of loose nappies though which I think at times maybe giving her a bit stomach cramp.  Also into the bargain she is teething.............nightmare at the best of time but we have waited a whole year for teeth and they now pick their time to come through.

At the moment Chiara is still taking loads of medicines, she has antibiotics, 3 anti-reflux, medication  to reduce risk of any Herpes virus as well as Cyclosporin (which is the medicine which is given Intravenous to hopefully help any rejection of the BMT)  She has blood levels taken for this first thing in the morning before she receives the medication. 

She had no routine bloods taken today but I think tomorrow she may have bloods taken to see what her levels of Neutrafils, Platelets and Haemaglobin are sitting at. 

Chiara still remains on her BI PAP at night but the good news is when she is off it during the day and sleeping her Oxygen levels are staying high so fingers crossed maybe sometime soon another sleep study will be performed to see if she is ready to come off her BI PAP at night, this would be another massive achievement for her and it would be great just to be able to put her to sleep at night without a mask.

She was eating and drinking excellent today. 

Sadly her hair has went thin, in a couple of days I am sure it is going to just fall out and I dread the day that happens although I knew it was coming.  Even although the hospital prepares you for everything that lies ahead you are never really completely ready for it.  Having said that I do have her the cutest little bandannas and head scarves so she will always still look gorgeous.

Today back home a charity fashion show was organised by a good friend with proceeds going to MPS Society and Yorkhill.  It never fails to amaze me how everyone shows their support in such are lovely way when times are tough.  I would have loved to have attended as everyone knows how I love a "wee social event" but my time was needed more through here.  Thanks to all of my family and friends who were able to attend and give their support to a great cause.  To the organisers, I will always be grateful to you for thinking of Chiara when nominating the charity.

I hope tomorrow continues to be a good day.

27th August 2011 (T + 3)

Well after a quiet day yesterday with Chiara and only a small update on her blog my night ended up eventful but through no fault of Chiara. I arrived back at Ronald McDonald and gave mum and dad a skype call.  Mum and dad are just getting used to all this fancy technology so I need to give them some practice (plus as my dad pointed out he had splashed out £30 on a dongle so he is going to make sure he gets his money worth ) so anyway as I just get into the start of the conversation with dad I hear my son Joshua shouting in the background something about blood and saying he is nearly fainting, my dad shouting at him to shut up and my mum saying "im alright", next thing I know my dad is saying he is needing to cut the call short as he has to take mum to hospital as she has split her leg and there is blood everywhere.  O M G, next thing I know, here I am, while on Skype to them I'm calling my sister to go and get them and take them to hospital. Through all this I then realise that they all must have forgot they were on to me and I find myself skyping a bloody cushion.  Anyway it all ended up fine in the end. 

Today Chiara was brilliant.  She is still so happy and alert and content.  Her eating is still fantastic for someone who has just went through Chemo and transplant.  Her diet today was readybreak, soup and 2 slice of bread, yogurt, chicken casserole and crisps and snacks.  I am still really anxious everyday though that she is going to start going off her food due to the side effects of the Chemotherapy coming out of her system.  This can result in gut problems and sore mouths.  Thankfully she has none of those yet but I am sure one of these days she is going to be not so lucky and that day I do not look forward to.  Her hair today had a mind of its own, I am unsure if this is the normal before it falls out or if she is just like every other woman in the world who does just have "bad hair days". 

Her dad and brothers were through today.  Sadly the boys could not see her but I had a lovely afternoon with them even if it was just watching DVDs and a takeaway.  I didn't realise how much I missed them until I spend time with them and then they go. 

Chiara had a great bath as always, she spends so much time in the bath here that I am sure one day she will grow a tail....................

She settled well after taking her syringes of medicine like a wee trouper.

All in all I am really happy with the progress she is making and hope that any hitches that may crop up in the coming months are only minor.  Now we just need the cells to grow, grow,grow.

26th August 2011 (T+2)

Nothing much has happened over last 2 days.   Yesterday Chiara remained well and received her weekly ERT (this will continue until the hospital is happy that the BMT has engrafted).  She also received an infusion of Immunoglobulin. This will give her a small amount of immunity while she is so low.  Apart from that she ate well as usual. 

Today she remained well.  Nothing much happened today.

Tomorrow I look forward to seeing Richard and the boys.  Sadly the boys are not allowed into see Chiara but I will enjoy spending some time with them while Richard spends quality time with his wee girl.

24th August 2011 (Transplant Day)

Today I awoke and reminded myself that today was one of the most precious days of my life, not only was my boy turning 10 years old but my baby girl was receiving the long awaited transplant.

I sat and watched Lindsay open his birthday presents through Skype which was great, I have to admit it would have been better to see it in the flesh but circumstances made it such that seeing him over a computer screen was better than not at all.  How thankful I am today for modern technology.

I headed over to the Unit at 8.30am as usual.  I had phoned over a couple of times through the night but she had been settled and nothing to report.  I arrived at Chiara 's room which now had "STRICT ISOLATION" signs on the door.  After I entered through the first set of doors to her room I removed my outer garments and shoes.  From today Richard and I will be required to scrub from our hands up to our elbows thoroughly and wear an apron before entering through the next set of doors to Chiara.  No bags etc are allowed into her room either.  This is all to limit her risk of infection as she no longer has any Neutrafils or Lymphocytes (these are the cells that fight infection.).

She was bright and alert.  I washed and dressed her and gave her breakfast as part of her daily routine.

She fell asleep shortly after I arrived. I headed off for coffee and Richard arrived as we had been told yesterday that there was a possibility of the transplant taking place at 11am.  When we arrived back at Chiara's room where we were told that it would be 2pm.  We gave her lunch and then sat anxiously watching Toy Story for about the 20th time with Chiara.  She fell asleep at around 1.30pm.  2pm came and went.  We then received a phone call to say there had been a slight hold up with the cells.  At 4.15pm the transplant team arrived with the bag of cells.  This big life changing item looked like a normal bag of blood.   Chiara sat in her buggy at the side of the machine while the stem cells were connected to her central line.  At 4.25pm we all sat and watched this tiny bag of red liquid being transfused into our little girl and suddenly give us the hope of that promise of a future with her that we once had before that fateful day on the 29th April 2011 when our dreams and promising future were shattered.  By 5.10pm the transplant was over.  She had no reaction which was fantastic, she had a slight strange smell around her which to me smelled a bit like burnt sweetcorn. This was from the drug they use to store the cells in.  Hopefully this scent will have gone by tomorrow. 

She ate dinner but looked exhausted, it had been a long day for her, for us too emotionally.  She had her bath, bottle and settled down to sleep.

The only thing we can do now is sit and wait and hope for these tiny immature cells to grow and multiply.  We have to pray that from now on she doesn't catch any infections as that can be fatal.  Hopefully in a couple of weeks we should know by her blood tests if it has taken and pray she doesn't develop "graft versus host" which is when the new cells start to attack her own body.  She gets lots of medicines to help the prevention of this so fingers crossed the medication does its job. Chiara's stem cell transplant was also a full match 6 out of 6 so really we could not ask for any better.

Tomorrow we have ERT and IVIG.  Tonight I am too tired to even start explaining what that is ...............

23rd August 2011 (Day 7 of Chemotherapy)

Well we have now reached the 7th and final day of Chemotherapy.  After an eventful day yesterday I did not have a good nights sleep. I phoned the Ward every 2 hours only to be told she was comfortable and all her readings were good.  My final call was at 7.30am this morning and unfortunately this time I was told she had suddenly become Tachycardic, high temperature of 40.5 and starting to go down same route as yesterday. They had commenced Oxygen and Doctors had been called.

I headed straight over to find her more responsive than what she was yesterday but her heart rate was over 200 (this should be 140 or less).  Her temp was still high at 39.  She was off the Oxygen and quickly settled back down, her ATG drug had been slowed down and 2 fluid bolus had been given. They had tried to give her Brufen to reduce the temperature but this had made her sick.  I hate you ATG.

At around 9am I gave her a bath as this helped reduce her temperature last night and in turn then lower her heart rate.  She seemed to bounce back yet again to her normal playful self. 

Temperature came down to 37 and gradually her heart rate reduced down to under 140.  She fell asleep in her buggy and all her readings looked back to normal.  Her ATG was commenced as this was now running several hours behind and this had to finish quickly as she still had another ATG of 17 hours to run today to keep on track for the Transplant tomorrow.

She managed to eat lunch well again today and was playful and slept well in afternoon.

I bathed her and weighed her to find she has gained another 4 ounces.  (Well done Chiara, not many people gain weight going through Chemo). She is now 20lb 11 which is fabulous for Chiara as she has done so well to have gained 7lb in under 4 months and manage to position herself on the 50th centile when in April she was completely off the chart as she was so underweight. I gave her only 7 syringes of medicine tonight and then her milk.  She settled well and hopefully will have a good night and be prepared for her life changing event tomorrow.

Today the schools returned after 8 weeks of summer break.  I missed my 2 gorgeous boys heading off in their new uniforms but as we do every year pictures were taken and sent to me.  I'm sad I missed them but I am sure they will understand.  I also missed out on the opportunity of seeing my lovely niece in her uniform on her first day of school, unfortunately this is a day I will never get back and I am sorry Paige I missed that. 

Tonight I am so tired but not sure if I will sleep as tomorrow could be the first day of a whole new life for my wee Chiara, fingers crossed and praying she doesn't reject it.  She couldn't start her new life on a better day as tomorrow is also her brother Lindsay's birthday.  Sadly I will also miss this but I am sure he will agree if me not being there gives his sister her life back then it is probably the best birthday present he could ever have asked for. x

22nd August 2011 (Day 6 of Chemotherapy)

Well today was the day I was not looking forward to.  It started off good, with Richard heading off to hospital first this morning to let me catch up with the dreaded washing.  When I appeared at the hospital Chiara had refused breakfast from her Dad.  We washed and dressed her and she went down for a wee nap.  The nurse said she had had a settled night last night and had awoke only at 6am for a bottle of milk. 

Today was the day of the ATG commencing.  She was to be given a one hour trial to check for any reactions.  This was started at 11.00am.  She was attached to the monitor and her heart rate, sats and respiratory rate were closely monitored.  Her heart rate went from 135 up to 180 but she was moving about a lot so it was probably more due to activity rather than Tachycardia.

The trial went well so at 12 noon her ATG was started, this would run until 5am tomorrow morning. She settled for a sleep and Richard and I left for lunch.  We returned at around 1.40pm to find Chiara starting to wake.  She required a nappy change so Richard lifted her onto the bed, as he took her out of her buggy I noticed her colour was not as good as usual.  Within minutes of being on the cot she vomited everywhere, her colour drained, she became limp and tachycardic.  Doctors were phoned and arrived within minutes.  We gave her a quick bath but she became extremely shivery, and spiked a temperature.  Her Oxygen levels dropped and she was commenced on Oxygen.  She also was started on another IV antibiotic incase of infection and saline bolus given.  She remained tachycardic for a few hours after this episode but after a hour her Oxygen was stopped and she was back to her lively self. 

At around 7pm after I had bathed her her ATG was commenced again at a lower rate titrating up slowly until it was at its full rate at 9.30pm.  She settled down to sleep at 7.30pm and all her observations ie heart rate, respiratory rate and Oxygen levels were normal. 

Frightening day today as today for that short while she looked extremely unwell.  All I could think was this was the start and she was going to look like that for the next few weeks.  Hopefully she will remain settled overnight and will cope with her final dose of ATG tomorrow and transplant will go ahead as scheduled on Wednesday.  The pictures in the bath below are after her period of being unwell so you can see she bounced back quickly.

21st August 2011 (Day 5 of Chemotherapy)

Well today was Chiara's 5th and final day of the first batch of Chemotherapy I(Fludarabine and Busulfan).  Monday and Tuesday she will be given ATG which is the Chemotherapy we are not looking forward to as it can cause loads of side effects and we have been told this is the one that will most probably make her fall ill.

Anyway today I arrived at 8.30am to find Chiara still in her cot and only just awake.  She had been up at 5am for a bottle of milk but had then went back to sleep.

Unfortunately her lying longer in bed meant I had to endure the 9 syringes of medication.  She took the first couple as good as gold but as soon as I tried to give her the Cipro antibiotic she gagged and puked all over floor.  I seriously think now that this has to be either changed to IV or something else. 

No 10am Chemotherapy this morning YE HA.  Examination with Doctor was good.  . 

She ate well at lunchtime , beef casserole today.

She played normally and was lively today. 

Her wee bottom has broke down though, it has been like this for some days now.  Not as red as I suppose it could be but then that might have something to do with the fact that everytime I get near her for a nappy change I slap on layers of Orabase. (To me it just looks like really thick brown sugar ).  I am now cleaning her with Hydromol, warm water and swabs.

She had what was probably her last bath in a proper big luxurious bath for a while.  Jammied up and ready for bed.  6.30pm and I was pestering the nurse for all her 9 or 10 syringes.  Tonight I was going to try the Cipro first and any other meds that were dissolved tablets.  7.15pm and I eventually got them, one down and another 8 or so to go.  Cipro went in, gagged and the whole dinner, etc came hurling back up and all over the floor.  Definitely no way I am giving her that again, she is coping so well with her Chemotherapy and eating so well all day and then one medication just makes her throw the days meals back up, just not worth it.   I gave her the rest of the syringes no problem followed by a big
bottle of milk. 

Now came the part of trying to get her to sleep, not having any of it as after a huge vomit she is now hungry.   Anyway before long she fell over to sleep.  She was being given her Steroid IV tonight which she requires to be commenced on before her ATG tomorrow. 

20th August 2011 (Day 4 of Chemotherapy)

Today we are on the 4th day of Chemotherapy. Every day I awake I am always nervous about what is going to be waiting behind the unit door.... this morning I arrived to a very happy Chiara being fed a yogurt in her cot. I washed and dressed her and she went down for a wee nap at 9.30am.  Her Chemotherapy was administered at 10am.  She didn't sleep long this morning.
She ate all her lunch today, huge bowl of mince and potatoes.  She had a lovely visit from Nana.  She had another nap around 12.30pm.  Mum and I popped out for a bite to eat and returned an hour later to find Chiara awake yet again.  That was 2 rubbish sleeps today therefore I just knew she was going to end up crabbit.

The Doctors came to see her and found her to be extremely well, hopefully this will continue.

We had a small incident of her flush connection coming apart from her central line lumen therefore there was a small puddle of blood and water emerging on the floor.  Oops think Chiara had maybe managed to loosen this off a little.

By late afternoon she was crying, grumpy and not settling, the usual for her if she is tired.  She had dinner and then we waved Nana off and will hopefully see her in a couple of weeks when her blood counts increase again.

She had her bath and got dressed in THE most cutest Peppa Pig jammies.  We then settled down to receive her 9 syringes of medicines and was doing well taking them until she gagged on one and threw up everywhere............... NIGHTMARE, the new cutest jammies were not even on half an hour.  Another change and rest of medication was given and milk.  She fell asleep in my arms tonight and I just put her in her cot and attached her to her ventilator.  She looked so settled.  She still was to receive one more syringe of Aciclivor as this was not prescribed until 8pm and could not be given earlier as there had not been 4 hours between her last dose.   

19th August 2011 (Day 3 of Chemotherapy)

Well today we are in our 3rd day of Chemo.  I arrived at hospital to find Chiara awake and happy.  She had had a good night and slept reasonably well.  There did appear to be a slight problem with her central line as it was proving to be difficult to draw back any blood, this had happened yesterday but looked only like it could potentially be a positional problem (only when she was asleep and lying down was it refusing to give out any blood).  It had been suggested that a flush was kept on Chiara's central line to ensure patency (this means a line would be permanently attached to one of her lumens on her central line with a constant supply of saline being flushed through.)

She ate breakfast fine as usual, was dressed and washed and put down for her morning nap.

At around 10.40am her Chemotherapy was administered.  All her Chemos would run slightly late as the problems through the night had caused all the timings of her drugs to be slightly offset.

She was great today, Doctors appeared happy with her appearance, no cause for concern.  She seems so far to be tolerating the Chemotherapy.

She ate very well at lunchtime.

We had a visit from Papa today which was great and I am sure he enjoyed seeing how well she looked.

She had another Chemotherapy administered at 2pm, then again at 4.30pm.

We also had a visit from Richard and Joshua today whom Chiara was very pleased to see. This will probably be the last visit from Joshua until after Chiara's blood counts start to rise to avoid her catching any infection.

All in all today has been a good day, she is coping extremely well.  Chiara has lovely nurses who make it easier for her to cope being in hospital all day.

Chiara was still hyper tonight when I was trying to put her off to sleep so I left and had a bite to eat and returned at 9.30pm to find her sound asleep.

18th August 2011 (Day 2 of Chemo)

Well today I arrived at the hospital at 8.30am.  Chiara was awake and sitting in her pram.  She had been up quite a lot through the night, but not in an upset way, it was just that she was wide awake and ready to party the nurse said.  I gave her the daily 10 syringes of medicines and then changed her and gave her breakfast. She fell asleep for an hour and settled well.

At 10am her Chemotherapy was administered again and also she received her weekly ERT today.  It felt that she was hooked up to a machine most of the day and she was quite restricted in what she was able to do.

From my point of view today went well, she had a chest x-ray which was clear, bloods are still okay, she ate extremely well and weight increased.  She was settled all day had 3 naps and was playful and her happy self.  She received more Chemotherapy again at 4pm, and will get another dose at 10pm and then again at 4am.

She settled down to sleep tonight at around 7.30pm after yet another 10 syringes of medicines.