Today was day 3 of Chemotherapy for Chiara. She has tolerated it very well and tomorrow she will not receive any Campath. Today was her last day of that. Tomorrow and Tuesday she will receive her final Chemo drugs to kill off her bone marrow. Today Chiara's blood counts started to drop. So much so that tomorrow she will probably require a platelet transfusion. She is still feeding well and behaving in her usual mischievous manner. Chiara remains in source isolation due to her still being Norovirus positive but tonight she managed to open her door and run down the ward corridor in her pyjamas. She was the fugitive on the run.
Richard commenced his GCSF today to boost his blood counts. He will have another 3 days of this which consists of 2 injections each day.
Chiara has developed a slight rash, doesn't seem to bother her and is most probably related to the Campath.
Tomorrow will probably be her last day for visitors and by Thursday she will be in strict isolation.
Strict isolation consists of absolutely everything being wiped down before it enters her room. Basically anything she doesnt have in with her before transplant doesnt get to go in after.
Sunday, 29 April 2012
Thursday, 26 April 2012
26th April 2012
On the 24th April 2012, Chiara and I made the journey to hospital to start BMT workup. What can I say, to find myself packing cases for both of us to repeat the process of what we did last August heartbreaking but was a job that had to be done. Yesterday Chiara was fasted and had an MRV carried out to enable the surgeons to have a look at venous access for central line insertion. This will be Chiara's fourth central line therefore access is now becoming a little more difficult to obtain.
Today Chiara was fasted yet again and was taken to theatre at around 4.30pm. She was starving and grumpy. She returned at around 6.30pm with a new central line insitu.
Tomorrow Chiara will commence her first day of Chemotherapy. Her regime and workup is different this time and slightly different drugs will be used to ensure her own marrow is completely wiped out this time round. She will commence Campath tomorrow. Fingers crossed no side effects will floor my wee girl. She is in such a good place right now and looks fab but if making her ill to make her better and reap the benefits is what is needed then that is what we must do.
Richard will commence his GCSF on Sunday to boost his Neutrafils and he will have injections until Wednesday. On Thursday he will be admitted to hospital and he will be placed on the Apharesis machine where harvest will take place of his cells. This bag of cells will hopefully give Chiara yet another chance at a normal life.
It is hard to believe but on Sunday it will be one year since we were given the devastating news that Chiara had a life limiting Storage Disorder. A day I will never forget, the day the Royal Wedding took place and the day when MPS1H otherwise known as Hurlers changed a whole family's life forever. In all fairness though if I look at Chiara's quality of life now as to this time last year I am thankful for the diagnosis early that enabled her to obtain early intervention that led to her deterioration being halted.
Today Chiara was fasted yet again and was taken to theatre at around 4.30pm. She was starving and grumpy. She returned at around 6.30pm with a new central line insitu.
Tomorrow Chiara will commence her first day of Chemotherapy. Her regime and workup is different this time and slightly different drugs will be used to ensure her own marrow is completely wiped out this time round. She will commence Campath tomorrow. Fingers crossed no side effects will floor my wee girl. She is in such a good place right now and looks fab but if making her ill to make her better and reap the benefits is what is needed then that is what we must do.
Richard will commence his GCSF on Sunday to boost his Neutrafils and he will have injections until Wednesday. On Thursday he will be admitted to hospital and he will be placed on the Apharesis machine where harvest will take place of his cells. This bag of cells will hopefully give Chiara yet another chance at a normal life.
It is hard to believe but on Sunday it will be one year since we were given the devastating news that Chiara had a life limiting Storage Disorder. A day I will never forget, the day the Royal Wedding took place and the day when MPS1H otherwise known as Hurlers changed a whole family's life forever. In all fairness though if I look at Chiara's quality of life now as to this time last year I am thankful for the diagnosis early that enabled her to obtain early intervention that led to her deterioration being halted.
Friday, 13 April 2012
13th April 2012
Friday the 13th and today it lived up to its name..............it has been a day of tiredness, stress, arguing, tears and worry to say the least.
Last night Chiara was to have a sleep study carried out, she commenced this at around 8pm when she was connected to various different wires over her body. I was anxious just at the thought of how on earth she would keep any of these on, but to my surprise she did better than I expected and lasted till around midnight, from then on it just fell apart. She struggled to go back to sleep and was very restless, then at around 1.30am she had a very large projectile vomit and that put an end to the sleep study. She received a final bottle of milk at 5am as from then on she would be fasting in anticipation that if her line failed its challenge in the morning then she would be prepared for theatre for removal of the central line.
Initially the morning went well given that she was starved of breakfast or even any fluid by mouth. At around 11.30am the line challenge was commenced. Half an hour later I could see Chiara start to shake and knew instantly she was spiking a temp and Sepsis was the cause. Her temperature was taken and was 37.8. IV Paracetamol was commenced and the decision to stop using the line was made as it was clearly holding some sort of bug. Thank goodness she had fasted as there would be nothing to stop her going to theatre shortly for removal. Nana came for a visit today and unfortunately did not enjoy seeing Chiara in her poorly state. It is hard to watch your child fall ill but I can only imagine as a grandparent it must be just as hard and especially when grandparents don't experience the hard times as often as parents. I myself feel I can cope with more than I ever thought I could and suppose sometimes expect others to cope just as well. Anyway, Chiara's temperature continued to rise and soon she was sitting a very burning 39.8 with a heart rate of high 180s to 200. We we preparing for surgery. At 5.30pm we were told Chiara would be going up to theatre shortly but required a platelet transfusion before theatre. We delivered Chiara to theatre at 8pm. A long very stressful day it had been with a very tired, grumpy starving girl and I can only imagine sore also. I myself have been fortunate never to experience Sepsis but I believe it is a horrible experience.
We received Chiara back from theatre in under one hour, she had only required Gas to remove the line and no anaesthetic. She will continue on antibiotics for a couple of days to cover any remaining infection.
The good news is she will now recover and feel better given that the infected line has been removed, the bad news is the transplant once again will be postponed.
Nothing ever runs smoothly.
Last night Chiara was to have a sleep study carried out, she commenced this at around 8pm when she was connected to various different wires over her body. I was anxious just at the thought of how on earth she would keep any of these on, but to my surprise she did better than I expected and lasted till around midnight, from then on it just fell apart. She struggled to go back to sleep and was very restless, then at around 1.30am she had a very large projectile vomit and that put an end to the sleep study. She received a final bottle of milk at 5am as from then on she would be fasting in anticipation that if her line failed its challenge in the morning then she would be prepared for theatre for removal of the central line.
Initially the morning went well given that she was starved of breakfast or even any fluid by mouth. At around 11.30am the line challenge was commenced. Half an hour later I could see Chiara start to shake and knew instantly she was spiking a temp and Sepsis was the cause. Her temperature was taken and was 37.8. IV Paracetamol was commenced and the decision to stop using the line was made as it was clearly holding some sort of bug. Thank goodness she had fasted as there would be nothing to stop her going to theatre shortly for removal. Nana came for a visit today and unfortunately did not enjoy seeing Chiara in her poorly state. It is hard to watch your child fall ill but I can only imagine as a grandparent it must be just as hard and especially when grandparents don't experience the hard times as often as parents. I myself feel I can cope with more than I ever thought I could and suppose sometimes expect others to cope just as well. Anyway, Chiara's temperature continued to rise and soon she was sitting a very burning 39.8 with a heart rate of high 180s to 200. We we preparing for surgery. At 5.30pm we were told Chiara would be going up to theatre shortly but required a platelet transfusion before theatre. We delivered Chiara to theatre at 8pm. A long very stressful day it had been with a very tired, grumpy starving girl and I can only imagine sore also. I myself have been fortunate never to experience Sepsis but I believe it is a horrible experience.
We received Chiara back from theatre in under one hour, she had only required Gas to remove the line and no anaesthetic. She will continue on antibiotics for a couple of days to cover any remaining infection.
The good news is she will now recover and feel better given that the infected line has been removed, the bad news is the transplant once again will be postponed.
Nothing ever runs smoothly.
Thursday, 12 April 2012
12th April 2012
Easter weekend for us as a family was good, Chiara attended her brothers football festival and loved it even although the weather was poor. She was dressed for the weather in wellies, winter jacket and umbrella. It was great to see her running around and having fun. Unfortunately the end of the Easter weekend didn't turn out that good. After returning home on Monday night and been bathed Chiara spiked a temperature of 38.2 and vomited and became rigger. She lay shaking and looked awful. We drove through to hospital immediately where she presented then with a temperature of 39.2. Instantly I suspected her central line to be the cause of the problem as I had experienced it once too many times. Chiara had blood taken for culture and was examined. Her ear was found to be slightly inflamed and I thought there might be a small chance this good be the route of the temperature as she had been poking at her ear for a couple of days. She settled with IV Paracetamol and 2 fluid bolus and commenced on IV broad spectrum antibiotics. Unfortunately through the night Chiara lay shaking again and temperature again spiked. Her routine bloods were taken in the morning and slightly after that she spiked another temperature and was shaking and vomiting. I received the news that the cultures taken had started to grow bugs. She remained well the rest of the day and had fun with her Nana who visited. Unfortunately by 6pm after Chiara received her IV antibiotic she spiked another temperature and she vomited. It was almost certainly a line infection so decision was made to stop using the line and transfer any IVs onto her portacath. After a few hours Chiara's temperature still remained high and decision then was made to stop using her port and have a cannula inserted. 10pm and Chiara had the horrible experience of having a cannula inserted. All IVs would run through this until it was felt appropriate to challenge the port or line. Her temperature returned to normal and she remained well.
Today she is having her Port challenged and if its okay and she has no spike of temperature then she will receive her ERT today via this (which she should have got on Monday). Tomorrow her central line will be challenged and if ok and no temperature then there is a chance her conditioning chemo prior to transplant will go ahead next week. If she continues to spike then I presume Chiara will lose that line. While she is in hospital tonight she will have another sleep study carried out to give an idea as to her nighttime use of her BIPAP machine.
Fingers crossed x
Today she is having her Port challenged and if its okay and she has no spike of temperature then she will receive her ERT today via this (which she should have got on Monday). Tomorrow her central line will be challenged and if ok and no temperature then there is a chance her conditioning chemo prior to transplant will go ahead next week. If she continues to spike then I presume Chiara will lose that line. While she is in hospital tonight she will have another sleep study carried out to give an idea as to her nighttime use of her BIPAP machine.
Fingers crossed x
Wednesday, 4 April 2012
4TH APRIL 2012
Time for a wee update I thought.
Well since my last blog entry Chiara has been a wee star. She has been keeping fantastic. She has not required any platelet transfusion or blood products for over a month now and therefore we have only been visiting Yorkhill on a Monday to receive her weekly enzyme. For the first time in over a year I actually feel like we are behaving like a "normal" family and enjoying daily living. Chiara remains Norovirus positive although she remains symptom free. Her latest date for transplant is 26 April 2012. The dates were postponed a few times to see if she could shake off the norovirus out of her system before she commenced her conditioning Chemotherapy in preparation for transplant. I understand the reason for this as going into Chemo with a virus present therefore could make her gut even more toxic than it is already and would leave her at risk of severe gut infection whilst going through conditioning. On the other hand I felt time was running out as Hurlers kids require transplant before age of 2 to obtain the benefit. This is something that I am not entirely sure about now as Chiara has already received treatment from an early age and also has had 4 months benefit of transplant. At present she shows no signs of any neurological damage or developmental delay. She had her spinal assessment carried out and this went well. Chiara will be admitted on 12th April for a sleep study to assess whether she still requires BIPAP overnight. This had been recommenced after her severe Pneumonia in December to give her extra support over the winter months.
Chiara has achieved a better quality of life this last 6 months than anyone could have ever imagined when we were given her diagnosis last year. It is almost 1 year to the day when Chiara was placed on the ventilator and our long journey started.
Family members have been tested and unfortunately my Dad has been found to carry the Hurler Gene and also Muscular Atrophy gene which is another condition in our family. This has led to extensive further testing with other family members.
On the 29th March 2012 Chiara had further hearing tests carried out and thankfully we were given the news that she was hearing and if any hearing loss was evident it was only slight but unfortunately due to Chiara's unwillingness to cooperate complete hearing test was not carried out. She wears no hearing aids and appears to be hearing extremely well. She is trying hard to talk and communicate so much so she blethers all day but I find it hard to actually recognise the words which sometimes leads to her getting frustrated.
Life is never dull when Chiara is around, especially now she is walking and talking. I think it is safe to say I certainly have my hands full with her and her brothers. Whilst I do not look forward to enduring another 8 to 12 weeks in hospital I know this time around what lies ahead for her as I have experienced it before. My only hope is that she keeps as well 2nd time round as she did the first. I
hated her losing her hair the first time round but this time I think I will be even worse as it has come in so dark and curly it will break my heart to see it fall out.
Richard carried out a 10k run in Peebles which involved a kind of assault course and raised a considerable amount of money for Schiehallion Ward that Chiara is spending most of her time. This I feel is the least we can do to show our appreciation for all the help she is given.
Richard will commence his course of GCSF to boost his Neutrafils 1 week before Chiara's transplant date and then will be admitted to Beatson to donate the stem cells.
I will of course keep Chiara's blog up to date with her progress.
Well since my last blog entry Chiara has been a wee star. She has been keeping fantastic. She has not required any platelet transfusion or blood products for over a month now and therefore we have only been visiting Yorkhill on a Monday to receive her weekly enzyme. For the first time in over a year I actually feel like we are behaving like a "normal" family and enjoying daily living. Chiara remains Norovirus positive although she remains symptom free. Her latest date for transplant is 26 April 2012. The dates were postponed a few times to see if she could shake off the norovirus out of her system before she commenced her conditioning Chemotherapy in preparation for transplant. I understand the reason for this as going into Chemo with a virus present therefore could make her gut even more toxic than it is already and would leave her at risk of severe gut infection whilst going through conditioning. On the other hand I felt time was running out as Hurlers kids require transplant before age of 2 to obtain the benefit. This is something that I am not entirely sure about now as Chiara has already received treatment from an early age and also has had 4 months benefit of transplant. At present she shows no signs of any neurological damage or developmental delay. She had her spinal assessment carried out and this went well. Chiara will be admitted on 12th April for a sleep study to assess whether she still requires BIPAP overnight. This had been recommenced after her severe Pneumonia in December to give her extra support over the winter months.
Chiara has achieved a better quality of life this last 6 months than anyone could have ever imagined when we were given her diagnosis last year. It is almost 1 year to the day when Chiara was placed on the ventilator and our long journey started.
Family members have been tested and unfortunately my Dad has been found to carry the Hurler Gene and also Muscular Atrophy gene which is another condition in our family. This has led to extensive further testing with other family members.
On the 29th March 2012 Chiara had further hearing tests carried out and thankfully we were given the news that she was hearing and if any hearing loss was evident it was only slight but unfortunately due to Chiara's unwillingness to cooperate complete hearing test was not carried out. She wears no hearing aids and appears to be hearing extremely well. She is trying hard to talk and communicate so much so she blethers all day but I find it hard to actually recognise the words which sometimes leads to her getting frustrated.
Life is never dull when Chiara is around, especially now she is walking and talking. I think it is safe to say I certainly have my hands full with her and her brothers. Whilst I do not look forward to enduring another 8 to 12 weeks in hospital I know this time around what lies ahead for her as I have experienced it before. My only hope is that she keeps as well 2nd time round as she did the first. I
hated her losing her hair the first time round but this time I think I will be even worse as it has come in so dark and curly it will break my heart to see it fall out.
Richard carried out a 10k run in Peebles which involved a kind of assault course and raised a considerable amount of money for Schiehallion Ward that Chiara is spending most of her time. This I feel is the least we can do to show our appreciation for all the help she is given.
Richard will commence his course of GCSF to boost his Neutrafils 1 week before Chiara's transplant date and then will be admitted to Beatson to donate the stem cells.
I will of course keep Chiara's blog up to date with her progress.
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