Well its just after midnight and I have been awoken by one of the sleep study people to tell me that it looks as though Chiara is breathing out her mouth when her dummy is out so therefore she is not getting the best from her BI PAP machine. She tries to change Chiara's mask to a full face mask. Chiara does not tolerate this at all. Chiara fully awakes and we resort back to her normal mask. She fails to go back to sleep and I end up feeding her milk. She eventually goes back over to sleep at around 3am.
Result of the sleep study shows improvement so she will stay on pressures of 12 on BI PAP and no need for NPA and also has been decided no need for Tracheotomy.
All good news.
We get Chiara's ERT on the Ward today which goes well.
Chiara's Consultant comes to tell me that her GAGS in her urine have reduced from 140 to 68 which is good news.
We have in depth meeting with Transplant Team next Thursday to discuss everything involved over next couple of weeks.
I took her down to the Dentist in the hospital as this is another check needed before transplant.
Chiara now has 3 teeth cut through and the Dentist did her first brush.
All in all today has been a good day.
Thursday, 28 July 2011
27th July 2011
Well we headed back to Hospital today after a bad start to our day. While out walking Chiara in her pram this morning a bolt snapped and I had to end up carrying her home while pushing the pram.
Anyway, we went to Cardiology at 1.30pm and the result of her most recent Echo was missing so we had to go back round to Cardiology to have another Echo carried out. This time it was more in depth. We headed back round to see the Consultant and sat anxious waiting to find out whether or not she required medication and that would result in delaying transplant. He went over both results in front of us. Her heart appeared normal on the recent echo and she had a 30% fractional shortening which is normal so transplant goes ahead as planned as he says her heart is functional normally.
We are admitted to Ward for sleep study tonight. Her pressure on her BI PAP machine has been increased to 12.
I settle Chiara down for the night and keep my fingers crossed.
Anyway, we went to Cardiology at 1.30pm and the result of her most recent Echo was missing so we had to go back round to Cardiology to have another Echo carried out. This time it was more in depth. We headed back round to see the Consultant and sat anxious waiting to find out whether or not she required medication and that would result in delaying transplant. He went over both results in front of us. Her heart appeared normal on the recent echo and she had a 30% fractional shortening which is normal so transplant goes ahead as planned as he says her heart is functional normally.
We are admitted to Ward for sleep study tonight. Her pressure on her BI PAP machine has been increased to 12.
I settle Chiara down for the night and keep my fingers crossed.
Tuesday, 26 July 2011
26th July 2011
Chiara had her hearing assessment today at the Sensory Centre. I was looking forward to the results of this as I am pretty confident her hearing has improved.
Chiara had to turn every time she heard a noise and an animal would appear. She responded brilliantly to this and it appears she is hearing high pitched noises very well but still struggles with the the lower frequency. She was fitted with new wee cute pink hearing aids and will be see again after BMT for further testing.
HAPPY HAPPY HAPPY
HAPPY HAPPY HAPPY
Friday, 22 July 2011
22nd July 2011
Today I feel extremely tired. I have been up every half an hour to Chiara as her BI PAP machine keeps alarming as she has a leakage of pressure. Chiara remains unfazed by this though. She remains well without her NPA and her secretions have improved greatly. It is great not to be suctioning her all the time.
After lunch today I received a phone call from one of the Doctors at the Hospital. It appears Chiara's latest Echo has shown a 22% fractional shortening and she has made Chiara an appointment to see a Cardiologist on Wednesday. I explained that Chiara's first Echo was normal and what did this shortening mean but she did not know herself. This only thing she could tell me was that if she required medication for this then it would delay BMT. She said she couldn't risk putting Chiara through BMT if her heart wasn't functioning properly.
I just worried about this the rest of the day. I had built myself up for transplant on 25th and now it seems there is a chance it could be cancelled. I understand totally that they cant risk transplant if her heart is at risk.
Finding today hard, worried regarding her results and finding juggling home life stressful.
After lunch today I received a phone call from one of the Doctors at the Hospital. It appears Chiara's latest Echo has shown a 22% fractional shortening and she has made Chiara an appointment to see a Cardiologist on Wednesday. I explained that Chiara's first Echo was normal and what did this shortening mean but she did not know herself. This only thing she could tell me was that if she required medication for this then it would delay BMT. She said she couldn't risk putting Chiara through BMT if her heart wasn't functioning properly.
I just worried about this the rest of the day. I had built myself up for transplant on 25th and now it seems there is a chance it could be cancelled. I understand totally that they cant risk transplant if her heart is at risk.
Finding today hard, worried regarding her results and finding juggling home life stressful.
Thursday, 21 July 2011
21st July 2011
Today feels slightly strange as usually on a Thursday we are in Hospital for ERT.
Anyway, at around 2pm today I received a call from the Transplant Coordinator. Chiara's dates have now been discussed. Harvest is going to take place around 11th August, with BMT taking place on 25th August. Therefore she will receive Chemotherapy to destroy her own marrow 10 days before this date.
My stomach is in knots. It all seems real now. Even although I knew this is what we were preparing for I am beginning to think nothing can prepare you for it.
Its all very scary. She has been through so much in her short life, that it seems unfair to put her through more. I can only hope that one day she will be glad we did it. She has already went through more in one year than most people go through in a whole life time. Still she smiles through it all and looks as if she hasn't a care in the world. It takes a special sort of girl to be like that.
Anyway, at around 2pm today I received a call from the Transplant Coordinator. Chiara's dates have now been discussed. Harvest is going to take place around 11th August, with BMT taking place on 25th August. Therefore she will receive Chemotherapy to destroy her own marrow 10 days before this date.
My stomach is in knots. It all seems real now. Even although I knew this is what we were preparing for I am beginning to think nothing can prepare you for it.
Wednesday, 20 July 2011
20th July 2011
One year ago today I gave birth to my beautiful Princess. Unfortunately she is wakening up in hospital but we will go home later today after her ERT.
I am up and dressed and ready to leave with her off the Ward at 8.30am.
All the nurses come and wish her happy birthday and she gets gifts from the ward.
We headed down to another Ward where the nurses had decorated her cot for her. Unfortunately Chiara's prescription wasn't going to be up until about 10am.
Joshua, Lindsay and Richard arrived and she was delighted to see them. Also on this special day Chiara had cut her first tooth.
ERT went well today, dressings were changed and so were lumen ends.
We spoke with one of the Respiratory Doctors who wants Chiara to come in again next week for sleep study to have her pressures increased again on her BI PAP. Chiara was now completely NPA free.
We left hospital late afternoon and headed to my mum and dad for a birthday dinner for Chiara.
I am up and dressed and ready to leave with her off the Ward at 8.30am.
All the nurses come and wish her happy birthday and she gets gifts from the ward.
We headed down to another Ward where the nurses had decorated her cot for her. Unfortunately Chiara's prescription wasn't going to be up until about 10am.
Joshua, Lindsay and Richard arrived and she was delighted to see them. Also on this special day Chiara had cut her first tooth.
ERT went well today, dressings were changed and so were lumen ends.
We spoke with one of the Respiratory Doctors who wants Chiara to come in again next week for sleep study to have her pressures increased again on her BI PAP. Chiara was now completely NPA free.
Tuesday, 19 July 2011
19th July 2011
Its 6am and I must have dozed off to sleep as I am awoken by Chiara moving around. The machine has not alarmed so it must be a sign that she has maintained saturation levels. Therefore there is a chance she wont need the NPA in.
I sit nervously waiting on the Doctors to come and tell me the results. Eventually her Consultant arrives and tells me that it looks as though there was no difference having the NPA in as having it out therefore initial impression is that she no longer requires the NPA. She will only rely on the BI PAP machine for overnight sleeping. Her figures from the study shows room for improvement therefore the Respiratory Technicians will adjust the pressure on her machine for tonight to see how she copes with pressures of 10.
I sit and remember and that one year ago today I was standing cleaning my floor when my waters broke and that was the start of my long journey to where we are today. Its been a hard year but worth it, for all her troubles she had brought she is definitely our special little princess there is no denying that.
Chiara has to go down for another Echo cardiogram today. This apparently is all preparation for BMT. Chiara had more bloods taken today and I tried to get a urine sample from her today as well.
We headed out for a late lunch with Uncle Bobby up the West End which was lovely and then back to settle Chiara down for the night without her NPA in.
Chiara has been given a new mask as well tonight so fingers crossed she tolerates the new mask and the higher pressures.
Again I spend the night looking at the figures on the sleep study machine but happily everything looks within range.
I sit nervously waiting on the Doctors to come and tell me the results. Eventually her Consultant arrives and tells me that it looks as though there was no difference having the NPA in as having it out therefore initial impression is that she no longer requires the NPA. She will only rely on the BI PAP machine for overnight sleeping. Her figures from the study shows room for improvement therefore the Respiratory Technicians will adjust the pressure on her machine for tonight to see how she copes with pressures of 10.
I sit and remember and that one year ago today I was standing cleaning my floor when my waters broke and that was the start of my long journey to where we are today. Its been a hard year but worth it, for all her troubles she had brought she is definitely our special little princess there is no denying that.
Chiara has to go down for another Echo cardiogram today. This apparently is all preparation for BMT. Chiara had more bloods taken today and I tried to get a urine sample from her today as well.
We headed out for a late lunch with Uncle Bobby up the West End which was lovely and then back to settle Chiara down for the night without her NPA in.
Chiara has been given a new mask as well tonight so fingers crossed she tolerates the new mask and the higher pressures.
Again I spend the night looking at the figures on the sleep study machine but happily everything looks within range.
Monday, 18 July 2011
18th July 2011
Well today Chiara and I head back into hospital which I am not looking forward to.
Before we go I head to the shops and treat her to a new pair of shoes. This would be her first ever pair of pre-walkers, what a big girl she is getting. Retail therapy always helps.
We head through later on in afternoon. Ward 5 we are going to today.
When we arrive its so nice to be greeted by all the familiar nurses who know Chiara well. Everyone comments on how well she looks and how big she had become. We quickly feel at ease again.
Sleep study commences around 10pm so I settled Chiara down at about 9pm. She was to sleep with her NPA in and on her BI PAP until around 1am and then I was to take the NPA out and put her back on BI PAP to see how she would cope. The sleep study machine would alarm if her saturation levels were going too low or her CO 2 too high. If the nurses felt there was any risk then I would insert a new NPA back in.
I settle down to sleep, fingers crossed that the study goes well.
At 1am Chiara awakes so I take out her NPA and put her back down to sleep only with her BI PAP machine. I lay awake for hours just watching the machine praying it doesn't alarm.
Before we go I head to the shops and treat her to a new pair of shoes. This would be her first ever pair of pre-walkers, what a big girl she is getting. Retail therapy always helps.
We head through later on in afternoon. Ward 5 we are going to today.
When we arrive its so nice to be greeted by all the familiar nurses who know Chiara well. Everyone comments on how well she looks and how big she had become. We quickly feel at ease again.
Sleep study commences around 10pm so I settled Chiara down at about 9pm. She was to sleep with her NPA in and on her BI PAP until around 1am and then I was to take the NPA out and put her back on BI PAP to see how she would cope. The sleep study machine would alarm if her saturation levels were going too low or her CO 2 too high. If the nurses felt there was any risk then I would insert a new NPA back in.
I settle down to sleep, fingers crossed that the study goes well.
At 1am Chiara awakes so I take out her NPA and put her back down to sleep only with her BI PAP machine. I lay awake for hours just watching the machine praying it doesn't alarm.
Saturday, 16 July 2011
16th July 2011
Early rise for us all today, so much to do for the Princess's big day.
Not surprisingly we awoke to rain.
Richard was working this morning so he dropped Chiara off at my mum's and myself and the boys set about decorating the Gazebo. All the decorations I had bought were pink, everything was to be pink, even the cup cakes.
I covered the table in the conservatory with cakes and it looked great.
Chiara arrived home at around 1pm and I quickly changed her into her gorgeous party dress and put on her "Birthday Princess" tiara. She was just gorgeous.
Shortly after 3pm the visitors started arriving and before long the place was packed and the party was going. The sun came out for a while and then it started to thunder.
Everyone had a great day I think!!!
We went inside later on and opened all Chiara's presents. She did extremely well and I was so happy that the day had went well.
I was exhausted now as was Chiara and everyone else.
Not surprisingly we awoke to rain.
Richard was working this morning so he dropped Chiara off at my mum's and myself and the boys set about decorating the Gazebo. All the decorations I had bought were pink, everything was to be pink, even the cup cakes.
I covered the table in the conservatory with cakes and it looked great.
Chiara arrived home at around 1pm and I quickly changed her into her gorgeous party dress and put on her "Birthday Princess" tiara. She was just gorgeous.
Shortly after 3pm the visitors started arriving and before long the place was packed and the party was going. The sun came out for a while and then it started to thunder.
Everyone had a great day I think!!!
We went inside later on and opened all Chiara's presents. She did extremely well and I was so happy that the day had went well.
I was exhausted now as was Chiara and everyone else.
Friday, 15 July 2011
15th July 2011
Well today is a bit manic. We are having a party tomorrow for Chiara's 1st birthday. Weather has been great this week but now looks like rain tomorrow. Typical, after everything she has went through she at least deserves some sun on her party day. My brother is coming down later to put up a huge gazebo so the party will still go ahead.
Community Physiotherapist came to visit Chiara today. Chiara performed delightfully for her, by rolling over, sitting up alone, standing against the furniture. It was great to see and the Physio was very impressed and said she would be letting Hospital Physio know how well Chiara has caught up. Eventually daily physio with her every day has paid off. Chiara is full of determination so I am doubtful that she will let anything beat her.
Rest of the day goes well, the usual cleaning the house in preparation for party. Kevan arrives down to put up Gazebo. My sister arrives later and we set about cake baking and organising for tomorrow.
Tomorrow is so special and I want it to be always remembered as only 3 months ago it was a day we never thought Chiara would live to celebrate. She is a little miracle and very brave.
Community Physiotherapist came to visit Chiara today. Chiara performed delightfully for her, by rolling over, sitting up alone, standing against the furniture. It was great to see and the Physio was very impressed and said she would be letting Hospital Physio know how well Chiara has caught up. Eventually daily physio with her every day has paid off. Chiara is full of determination so I am doubtful that she will let anything beat her.
Rest of the day goes well, the usual cleaning the house in preparation for party. Kevan arrives down to put up Gazebo. My sister arrives later and we set about cake baking and organising for tomorrow.
Thursday, 14 July 2011
14th July 2011
School holidays this week but we arrived at the hospital for Chiara's ERT earlier than normal which is so surprising.
Chiara had her pre meds, dressing changed and lumen ends cleaned and changed all by about 10.30am.
We spoke with Chiara's Consultant today who says it looks as though Chiara's transplant wont be taking place until August. She has to be admitted into hospital on Monday to have sleep studies carried out on Monday night and Tuesday night so therefore she will be waking up on her birthday in hospital. It has been discussed amongst the Doctors that Chiara's NPA proves high risk for trauma which should be avoided during transplant due to the risk of increased infection etc. The sleep studies will give the Doctors an idea if she still requires the NPA in or if her BI PAP overnight would be enough to cope with her airway problems. If she is unable to tolerate not having the NPA in then a Tracheotomy is being considered. This means the Doctors would have instant access in the event she needed emergency ventilation during transplant. All sounds very scary but I know they Doctors are just making sure that everything is in place in the event should she need it. Her Consultant told us that the Trachea would only be a temporary measure and would be taken out shortly after BMT.
Chiara also had to give a sample of urine so it can be checked to see if the GAGS ( the build up of the bad enzyme) has reduced any. She had bloods taken today for immunity etc.
Transplant Team spoke to us and told us decision has been made to have Chiara in ITU during BMT. Richard and I had long chat with them about all the procedures being put in place just in case Chiara should need anything during conditioning for BMT.
Chiara is due her immunisations around now but Doctors have decided to leave it as she will have to restart all her immunisations from the beginning anyway after BMT. I asked regarding the Chicken Pox injection as I know Chicken Pox can be fatal after BMT. This is not available in this country but an antibiody will be given should I feel Chiara is at risk after being in contact with someone with Chicken Pox.
Everything seems to be moving along now.
Chiara had her pre meds, dressing changed and lumen ends cleaned and changed all by about 10.30am.
We spoke with Chiara's Consultant today who says it looks as though Chiara's transplant wont be taking place until August. She has to be admitted into hospital on Monday to have sleep studies carried out on Monday night and Tuesday night so therefore she will be waking up on her birthday in hospital. It has been discussed amongst the Doctors that Chiara's NPA proves high risk for trauma which should be avoided during transplant due to the risk of increased infection etc. The sleep studies will give the Doctors an idea if she still requires the NPA in or if her BI PAP overnight would be enough to cope with her airway problems. If she is unable to tolerate not having the NPA in then a Tracheotomy is being considered. This means the Doctors would have instant access in the event she needed emergency ventilation during transplant. All sounds very scary but I know they Doctors are just making sure that everything is in place in the event should she need it. Her Consultant told us that the Trachea would only be a temporary measure and would be taken out shortly after BMT.
Chiara also had to give a sample of urine so it can be checked to see if the GAGS ( the build up of the bad enzyme) has reduced any. She had bloods taken today for immunity etc.
Transplant Team spoke to us and told us decision has been made to have Chiara in ITU during BMT. Richard and I had long chat with them about all the procedures being put in place just in case Chiara should need anything during conditioning for BMT.
Chiara is due her immunisations around now but Doctors have decided to leave it as she will have to restart all her immunisations from the beginning anyway after BMT. I asked regarding the Chicken Pox injection as I know Chicken Pox can be fatal after BMT. This is not available in this country but an antibiody will be given should I feel Chiara is at risk after being in contact with someone with Chicken Pox.
Thursday, 7 July 2011
7th July 2011
Another ERT day.
Infusion went well as always and she got her dressings changed and her lumen ends cleaned and changed.
The energy Chiara now has is amazing. She also sits up alone as her core stability has improved greatly. Her hearing appears improved also although reading articles, any damage to hearing cant be reversed. We will just wait and see.
We did not see the Transplant Team today so I am presuming that Chiara's transplant is not going to be the end of July as planned.
She still has her NPA in and this is now changed completely by me. I have eventually managed to gather enough courage to have the confidence to insert it by myself. She screams and wriggles as I insert it into her nose and its so upsetting to see her so distressed but after its in and taped she gets cuddles and forgets about it.
Infusion went well as always and she got her dressings changed and her lumen ends cleaned and changed.
The energy Chiara now has is amazing. She also sits up alone as her core stability has improved greatly. Her hearing appears improved also although reading articles, any damage to hearing cant be reversed. We will just wait and see.
We did not see the Transplant Team today so I am presuming that Chiara's transplant is not going to be the end of July as planned.
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