23rd July 2012 Day T + 81

A wee quick update on our Princess Chiara. Since my last entry which found us in Hospital again being treated for the awful virus HHV6 Chiara was discharged 2 days later having had 3 blood results returning negative.  Within 2 days we went from being up high to down low to back up high again. 

Since returning home Chiara has thrived and progressed beyond words.  She now sleeps extremely well in a bed, still has her episodes of getting up but on comparison with previous times she is doing well.  Her speech is a little behind, she tries hard and seems to have her own wee language but considering this time last year she wore hearing aids and faced the future of being deaf and struggling with speaking she is doing fantastic.  She does however think my name is "Bob" and calls me this all the time which is cute and funny.  Her hair is regrowing and as yet I am unsure of what  colour she is going to be sporting this time around.  She still has an NG tube in for medicines only as presently she is on 37 syringes of various medicines per day. 

On 20th July 2012 Chiara celebrated her 2nd birthday surrounded by those who share her life.  Her brothers and Nana and Papa unfortunately could not make her birthday as they were enjoying a well earned break in Portugal. 

Chiara had her examination carried out last week by her Hurlers Specialist who gave us the great news that at the moment Chiara's only other problem appeared to be her spine which will be operated on later in the year or start of next year.  She remains off overnight BIPAP, hearing aid free and walks unaided.

Today we were told that Chiara's hospital appointments would be once weekly instead of twice weekly which is a big step forward. 

We have a family holiday coming up soon which I cant wait to go and make us feel like a "normal family". 

This hopefully will be my last entry to Chiara's blog until her next operation.  From now I will be concentrating on getting my family back on track after a long 2years. 

3 months from now Chiara will be able to attend soft play areas, mix with other kids, join kids clubs and I will say hello to employment again.

Last week we joined Ben Fraser at his home to mark his one year diagnosis of Leukaemia.  This was a fantastic day enjoyed by everyone.  There was a bouncy castle to which I took great pleasure in watching Chiara play with the other kids.  A first for Chiara as she is still unable to mix with kids.  All the families that were there all were in same position as Chiara with low counts etc.  I think I speak for everyone who was there that day when I say it was brilliant and thanks very much to Ben's family for a wonderful day. 

Sadly 3 kids from Chiara's ward received their wings and I myself found this a very trying and upsetting period of time.  It made me realise how naive I had been in thinking everyone who entered Schiehallion recovered and sooner or later went home.  For a long while everyone that I met in the ward became like family to me and when they hurt I hurt for them.  Its something I don't think anyone will ever realise or relate to unless they have been in that position or been one of those parents/carers.  I shared my feelings with them like I had known them forever, told them more than I would my own best friend.  Friendships that will not end, you all know who you are . x

For everyone who supported Chiara and followed her story thank you so much but for now its on a break !

18th June 2012

Thought it was about time I updated my neglected blog.  Chiara is now 6 and a half weeks post transplant.  So much has happened in the last couple of weeks I feel terrible for not keeping her blog updated.  Anyway, since my last post we were all set to return home on the Friday of Chiara's 4 week post BMT period.  On the morning we were due to leave Chiara's rash that was suspected to be allergy related had spread and was very florid and fiery looking.  I knew instantly we would be going nowhere in a hurry.  As expected when she was examined they confirmed my disappointment that we would not be heading home as it was looking like her rash was developing into GVHD (this is when the new cells recognise her own cells as being foreign and fight against each other) the good news is it is treatable with steroids as she developed this last year during her first BMT.  After a couple of days Chiara's skin improved remarkably and a week later than planned we packed up our room that had become home and returned back to our real home.  It was emotional leaving the ward as I had become friendly with some many parents and to some degree I had more of a life in the hospital than out of it as I thrived on the adult company I got from the other parents. Chiara ran about the house going from room to room examining everything.  That night she didn't settle very well and woke screaming almost in terror.  We put this down to the fact that the hospital had become like her home and now her own home environment seemed strange to her.   It took a while to settle her but eventually she did calm down.  The following day Chiara went out playing in the garden and had a ball.  On Sunday less than 48 hours after returning home I went upstairs to find Chiara screaming and riggoring in her cot.  She had spiked a temp and looked awful. We quickly drove her back through to hospital. Instantly I suspected a central line infection as I had seen this all too many a time with her.  She settled with Paracetamol but within 15 minutes of her line being flushed and in use she spiked another temperature and I could feel her riggoring in my arms, she started to turn slightly blue in her feet and she was needing fluids into her fast.  Her line use was stopped and her port was tried to be accessed but unfortunately Chiara's port a cath is not the easiest to insert the gripper needle into.  A cannula had to be inserted so that we could manage fluids into her quickly.  She was given Oxygen and fluids and after a while started to improve.  Cultures were taken from her line to see if this was the source of infection.  The following day Chiara was fasted from 5.30am and after a long day she was taken to theatre just after midnight to have the line removed.  We remained in hospital for a week to have antibiotic cover for the infection.  Today we brought our little girl home to try and focus on the future away from hospitals.  Chiara is now able to produce white cell enzyme on her own and no longer requires enzyme therapy and her blood counts have all been good with her incrementing her platelets on her own also.  She still has issues with her skin which after a biopsy has shown it is not conclusive of GVHD but suggestive of drug reaction.  To which drug we are unaware as she is on so many. 

We have a long way to go but feel we have past the biggest hurdle.  In December 2011 when Chiara lost her graft I never thought I could have the strength  to go through this again with her but I cant believe she done so well through a 2nd BMT.  I am very proud of her and what she has come through and also very proud of my 2 boys and how they have coped through a difficult time. 

I have said before in my blog posts of everyone's kindness and again I have been overwhelmed by a charity event that takes place every year in memory of my cousins who sadly passed away with Muscular Dystrophy when my dad was presented with £500 in donation to Hurlers.  I am very grateful for this and give thanks for considering Chiara.

I wrote this update to the blog last night which was 18th June but today we returned to Yorkhill as Chiara's skin flared up and she was very uncomfortable.  Before we left to travel we received the phone call we had always dreaded, Chiara's most recent blood for HHV6 had came back positive.  HHV6 was the cause of Chiara losing her first graft.  All too soon our bubble had been burst.  We had not even managed 24 hours at home.  Tomorrow I will travel back to the hospital for Chiara to begin daily Foscarnet treatment in the very big hope that we can save her marrow.  Fingers crossed everyone please.  Surely my wee Princess deserves a break.

30th May 2012 Day 27

Time for a wee update.  So far Chiara's BMT has went fantastic.  Last Tuesday we were allowed to take Chiara out of the hospital and over to the park since the weather was just beautiful.  This had come so much quicker than we had expected as she was less than 21 days post BMT.  We continued every day to go out walking and have day trips to the park and enjoy freedom from the hospital room.  We were told to prepare for heading home on Sunday which would make Chiara day 24 post BMT which was amazingly fast recovery.  Unfortunately by Friday Chiara was covered in rash, she had been getting a few spots here and there for a few days but nothing really specific.  She was receiving IV Piriton for an itch coming from an undissolved stitch in her neck from her central line therefore the Piriton should be enough to help with treatment if the spots were an allergic reaction. 

On Saturday my brother came and spent the day with Chiara which was great as it allowed me to have a girls day out shopping with my sister in law and niece.  Before we left the hospital my brother decided he would take Chiara out for a walk.  As we all left the hospital we had a small incident involving a garment of my underwear being attached to a wheel of Chiara's pram, a story best kept within the hospital I think, as it certainly made the amusement for the day in the Ward.

On Sunday Chiara's rash appeared to be spreading and she was becoming a lot more itchy and uncomfortable. She had received more Piriton but had vomited this the minute it was injected into her line.  Her anti-histamine was therefore changed to Cetirizine.  Unfortunately that was not enough to subside the itch and Chiara received another 3 doses of Piriton over night.  By morning Chiara's whole body was covered in an urticarial rash with puffiness of her face and eyes, very typical of an allergic reaction.  Chiara was fasted and prepared for theatre to have skin biopsies taken.  She was evaluated by a Dermatologist who felt this was more a reaction to a drug or a viral infection.  Her biopsy was therefore cancelled as was theatre.  She was prescribed Elecon cream to apply and continue with Piriton.  Chiara's next dose of Piriton was not due until later on that evening and she was starting to show signs of improvement.  Her Piriton dose was given at around 6.30pm and she vomited immediately and became flushed again.  It was starting to look as if her reaction was to Piriton which is the most unusual medicine to be allergic to.  She has been on Cetirizine and creams since Tuesday morning and her rash is showing great improvement.  Unfortunately as you will have realised this kept us in hospital and we never made it home on Sunday.

We are hopeful that we will be homeward bound within the next few days providing the rash settles.

Chiara's medicines have increased yet again and we were handed 13 morning medicines alone to give her orally, I decided then that it was unfair on her to manage these, given that she would need to take another 4 at lunch, 4 at dinner and around 10 at bedtime therefore I opted for an NG tube to be inserted for medicines only.  This will be removed once her dependency on medicines reduce. 

She is eating as healthy as ever with steak pie, potatoes and beans for dinner.  Her fluid intake remains fab with her consuming at least 1400ml per day. 

Her sleep study from last week revealed that she no longer requires her  BIPAP machine overnight which is yet another huge step forward.  Today Chiara had bloods taken for her White Cell Enzyme level to see if she is producing enough on her own to allow her weekly infusion of ERT to cease.  She also had a urine test to check and see if her GAGS have reduced also.  Her blood counts remain brilliant , with high Neutrafil counts and acceptable Haemoglobin and Platelets and to date has not received transfusion since transplant.

Last week my girl and I appeared in the Sun Newspaper as I had been nominated for a competition as an Ideal Mum.  I dont think of myself as anything more special than any other Parent that is in this ward. We all endure so much and watch our kids go through so much pain and treatment it is hard to single one person out.  There is not one person in here that hasnt cried as much as me or felt isolated as much as me, we are all in the same position but every day we all face the challenges that are set in front of us and our children.  Every one of the parents I have had the experience of befriending all deserve the title of ideal mum or ideal dad.  Watching your child fight for life every day is one of the hardest tasks a parent will probably every have to deal with. 

I hope that my next entry will be from home and I will have ALL my family around me, it is becoming lonely in this hospital room just me and my girl, I miss my boys and look forward heading home for good with my ever improving girl and her new engrafted marrow.

22nd May 2012 (Day 19)

Well tonight I write Chiara's update with a smile from ear to ear. 

I headed home on Sunday night to spend time with my boys.  I left comfortably in the knowledge that Chiara was recovering well. Her counts were continuing to rise and on Sunday they were 0.8 therefore for the first time in 3 weeks Joshua and Lindsay were allowed to visit their sister.  It was fantastic to have them altogether again and Chiara was so happy, screaming and scrambling over them. 

I arrived back to hospital on Monday to be told that Chiara's Cyclosporin (her anti-rejection) drug was going to be changed from IV to oral which meant that was another step closer to home.  How close, I didn't realise until Richard said that it would or could be very soon as long as Chiara remained in the healthy state she was and she had a good holding Platelet count and good Neutrafil count.  To go home soon would be fantastic, she was in 8 weeks for her first transplant and I came in with the view that's how long we would remain and anything sooner was a bonus but never imagined it could be as early as just a few weeks.  As hard as it is I am trying not to get my hopes up as something is usually lurking around the corner to burst my bubble despite my positive mental attitude.

The side effects of her anti-rejection drugs are starting to show, her Electrolyte blood tests are showing her to have slightly low Magnesium, Calcium and Potassium despite oral substitutes.  She has had several infusions to try and replace this but until she stops her Cyclosporin then I have a feeling this is going to cause a problem.  We always knew that the Cyclosporin would be toxic to her kidneys but its outweighed by stopping graft rejection.

Today the sun was beating down therefore Chiara and I were allowed to go out of the hospital to the park, loved loved loved it.  We ran into a slight sad hurdle on our way out, Chiara initially was happy to be toddling through the ward having left her room until we made our exit out the Ward doors, she then started screaming and cuddled herself against the wall, she look scared, I didnt realise how much she had become dependent on her room and the familiar, safe surrounding it provided for her.  Sad to see I have to say but before long she had overcome this and was enjoying freedom.    To be walking through a park with Chiara 19 days post transplant looking as good as she does was just a joy.  When I returned my day was crowned with the news that Chiara's bone marrow Chimerism has been reported as 100% donor.  She sits today with counts of Neutrafils of 1.6, Platelets 281 and Haemaglobin of 91.  I am so happy as is her Daddy who has now proven his marrow was definitely the "good stuff".  Hurlers patients only need to have 25% donor to replace the White Cell Enzyme they miss so to have 100% is just a bonus.  Chiara was 100% with her first transplant but this time round we are determined not to let this one slip away.

To date Chiara still remains NG tube free which is amazing since she is on over 20 oral medications per day.  Her appetite is good and remains on a normal diet.  She still has a way to go and we still have worry of her developing infections but we will take each day as it comes.

 

17th May 2012 (T + 14)

Day 14 today and Chiara is brilliant.  She has no problems with her mouth or any after effects of Chemo.  However she has become grumpy and generally fed up being stuck in isolation. 

Chiara's counts today were, Neutrafils of 0.2, White Cells of 1.4, Haemoglobin of 88 and Platelets of 276.  Chiara has never managed to increment platelets to this level before so its amazing. 

The best news today is that her HHV6 came back today negative therefore her rash and temperature that she spiked at weekend had nothing to do with the HHV6 virus that we feared it could be.

Now we wait for this engraftment to take control and just keeping pushing these blood cells to an acceptable level with minimal side affects, one of them being the possibility of GVHD.  (Graft versus host disease).

14th May 2012 (T + 11)

Well until yesterday Chiara was keeping amazingly well.  She awoke yesterday morning just not her usual self.  She felt warm and was grumpy.  As the morning progressed Chiara's temperature slowly began to increase, so it was just a matter of time before she actually spiked.  Considering she was day 10 and had not fallen unwell before now was actually quite good going.  It would have been more unusual for her not to have had at least one temperature. 

She had developed some spots on her side and on her back.  These resembled the spots that she acquired back in November last year, one month before she developed Pneumonia which lead to the diagnosis of HHV6 virus which was the cause of her new BMT failing last December.  This obviously has made us very anxious as there is a possibility that the HHV6 could have been reactivated which is common post transplant because of the immunosuppressive nature of Chiara. 

This morning Chiara was screened for the HHV6 and had her Acyclovir changed from oral to IV and dose increased.  If the HHV6 test returns positive then Chiara will need this treatment changed but the adverse affects of the new medication would be suppression of the marrow therefore her engraftment would be longer and put her at further risk of infections.

Today she still had zero Neutrophils but her White Blood Count had increased to 0.4 which is still incredibly low but at least its on the increase.  Also she had an increment in her Platelets which is good as this hopefully means the new marrow is starting to en-graft. 

There is a possibility that the spots and temperature could be related to engraftment but we cant rule out the chance that it is the HHV6 and if it is then we just have to keep fingers crossed and think positive that this graft will keep as long as prompt intervention with correct treatment is started.

I returned home last night to enjoy a well longed for night with my boys.  Unfortunately while I was at home Chiara's temperature increased over 38 and she was commenced on IV antibiotics as standard protocol and Paracetamol.

I arrived at the hospital today and was faced with a poorly looking girl with an almost bald head and a bed full of her hair.  Upsetting as it was her Daddy shaved the remainder of the hair off.  I watched as every last curl fell to the floor.  I remember going through this feeling last year but she had less hair and albeit hard to watch it did not compare to today.  The only good thing was she could now eat and drink without having hair in her mouth, on her bottle and on anything she came into contact with.

She became brighter later on in the afternoon, no more temperatures and with a fresh hair free bed, pyjamas and bath she looked great again.  She has remained well the rest of the afternoon.  

Her results for the HHV6 are still outstanding so we have another anxious night of awaiting the result.

9th May 2012 T + 6

Just a quick update.  Chiara remains extremely well.  She has started taking better quantities of food and continues to drink normally taking in over 1 litre of fluid by herself.  She doesn't appear to have any broken down skin or Mucositis yet due to the after affects of Chemotherapy. 

She has a low Platelet count of 42 which is to be expected post transplant.  Unfortunately this low Platelet count comes with huge drawbacks, one of which is bad bruising therefore it is advisable to avoid falling at all cost.  Obviously no one thought to tell Chiara this, as yesterday she took a big nose dive into the bed and instantly the damage started to appear.  She  was examined by the Doctor and treated with an ice-pack.  Unfortunately this morning she woke with a black eye.  Still cute but looks even more wild with a bruise on her forehead and a black eye. 

Today her Platelets are only 24 so tomorrow she will receive a transfusion. 

We still have  zero Neutrafils or White Cells so every day her number obsessed mum is annoying nurses for her counts in the hope that one day I will see even a slight increase.

Chiara herself, is unaware of everything she is enduring, the biggest trauma for her through all this is being not allowed out of a door.

Yesterday one of Chiara's samples came back as showing she was Norovirus negative.  She has had this virus since February and ironically now when going through a BMT she loses the Noro eventually.  Good thing with this is that when she eventually comes out of strict isolation she will be able to have a bit more freedom outside of her room.

Whilst in strict sadly for Chiara she only has the company of myself and her dad when he is able to come through.  She misses her brothers terribly and is constantly looking at the camera and giving them a kiss when she sees their picture.  I showed her a picture of them on my phone and she looked behind the phone thinking they were there.  Hopefully soon she will see them.

7th May 2012 Day T + 4

Well so far so good.  Chiara's counts are now at 0 for Neutrafils and White Cell Count.  She required a blood transfusion on Friday so today she has a Haemaglobin of over 100.

Clinically she remains well, she has developed a redness almost sunburn like.  This is the result of the Treosulphan Chemotherapy that she received prior to Transplant.  This will be treated with an 50/50 emollient and should hopefully improve.

At the moment her mouth appears fine, no sign of mucoscitis which is extremely common post Chemo.  She continues to have many loose stools and therefore her little bottom is a bit on the red side but so far it has not become broken down.  Basically every time she required a nappy change I plaster on Orobase in Vaseline in the hope this will prevent any problems in that area.

I myself went home on Sunday to spend a night with my boys, which was lovely.  We didn't do much but to spend some quality time just watching TV and talking made all the difference to my otherwise busy days with Chiara.  As much as Chiara requires attention it is also important for me to try and make my boys feel equally as special as her.  Again, like last year, I feel my boys are growing up before my very eyes and I am not there to give them my time and my sadness will come when one day they are all grown up and I realise how much I missed out on.


Chiara still remains off her food but continues to take milk and juice orally and some pureed fruit.  I am hopeful her appetite will return in the near future.  She still is NG tube free and takes all numerous amounts of medicines orally.  Her Potassium has been a bit on the low side therefore she has received 2 Potassium infusions.

As yet she still has a full head of unmanageable hair.  She also continues to misbehave having found a vent in the room under a desk and is able to stick her arm out this into the corridor and is shouting at anyone who can hear her. 

We now wait wait and wait on these counts to rise.

3rd May 2012 Transplant Day

Well today was yet another day that we will never forget.  At 3.58pm Chiara received her Dads stem cells in the hope that this will stop her Hurlers from progressing and hopefully give her the best chance of a normal life. 

This morning Richard left myself and Chiara to go to the Beatson to have his stem cells removed.  He had been on GCSF for the last few days to boost his counts and I have to say last night he looked like he was feeling the side affects from the injections.  These injections were given to him to fool his marrow into thinking he had an illness so that his blood cells that fight infection could increase.  Although he felt slightly sore it was a small price to pay for what could be the benefits and what greater feeling to have than knowing a few days of discomfort gave your daughter back the gift of life. 

Richard returned just after lunch, slightly weak but generally well.  They had managed to obtain a large cell dose from him, more than they expected therefore Chiara would receive her quantity and the rest would be stored in case she required this in the future.


We waited and then watched a tiny bag of cells getting transfused into Chiara while she sat on her cot unaware of what a huge moment this was for myself and her dad, a moment that on the 24th August 2011 we witnessed and hoped we would never witness again but sadly today we went through it a 2nd time but hopefully the last time.  Richard is always bragging that he has the good stuff so I am praying that he is right and it was delivered to Princess Chiara today.

All we can do now is wait, tomorrow I am expecting her counts to be at 0.  After that we wait and wait and hope that the cells will take and while we wait we pray she doesn't catch any infections as she has no immunity at all at this moment.

All in all another emotional day but a worthy one.

1st May 2012

Today Chiara received her final dose of Fludarabine Chemotherapy. 

She again 2nd time round has coped with the Chemo remarkably well.  She has experienced a slight sickness yesterday and today so much so that it has put her off her food.  By this I mean instead of sitting down to Chicken and Potatoes for lunch and Mince and Potatoes for dinner she is only managing a bowl of soup and bread followed by a yogurt, with snacks in between and about 4 bottles of milk in a day so really at the moment I am not too worried!!!!!!!

Her and I both are finding it very hard to be kept in isolation.  After escaping out her room the other night she has now worked out if she opens the door and pops her head out to check if anyone is there gives her a better chance of escape.  Last night I closed all blinds and tried to settle her but she stood in her cot and shouted "Hello" to anyone that was listening.  Frustrating as it is , it is also very funny

Her curly hair is day by day getting more out of control so I guess by the end of next week she will be starting to lose it.  I am making the most of bows and clasps in it the now but its a fight between me and Chiara to see who can keep it in her hair the longest.

Tomorrow Chiara will receive her Enzyme Replacement Therapy. 

Richard has been receiving his daily GCSF and today had a blood sample taken which will report whether he needs another dose tomorrow.  On Thursday he will be admitted to hospital in the morning and the donation will start. Chiara is due her transplant of the cells in the afternoon so as yet we are unsure if Richard will make it back in time from donating to actually witness Chiara receiving his cells.

Today Chiara's blood counts have started to decrease and she required a platelet transfusion so I presume by tomorrow they will be even  lower.  It is when the counts go so low and to 0 that Chiara is at increased risk of infection hence the need for strict isolation.

29th April 2012

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.

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.

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.

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

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. 

17th February 2012

Quick update.

Chiara attended Day Care on Monday for her weekly dose of ERT and was found to have lowish oxygen levels.  This was thought to be probably due to a build up of mucus as her chest sounded clear and her x-ray was okay.  We were admitted to the ward and have remained here since.  She has had numerous suctions and has been found to have extremely thick sticky secretions but after a good number of suctions her oxygen levels have increased and her need for oxygen stopped.

On Thursday Chiara went to theatre for her scheduled central line insertion procedure.  She was also going to be having a cyst removed from her gum which was stopping her tooth from coming through.  This is huge and black.  Unfortunately this could not be carried out in theatre at the same time as she was also having a bone marrow aspirate taken too.  She remained well after theatre but was taken into ITU for observation.  She was extremely active and when I arrived this morning she was hyper.  She was transferred back down to the ward at around 10am.  I left Chiara for only 2 minutes while I went to obtain a "wiggly bag" for her new line and when I returned I was faced with a cheeky little monkey sitting laughing with her new line lying at her side . O M G............................................... wild child had eventually done it.

We now sit fasting awaiting another theatre slot for yet another go at a central line. 

9th February 2012

Just a wee update on what the Princess has been up to since my last entry almost one month ago.

Chiara at present remains remarkably well, almost too well to be requiring a 2nd transplant but the blood results and marrow results tell us otherwise.  She is behaving now what I can only describe as a normal healthy 18 month old girl, mischievous, wild and happy.  She has a wonderful appetite, her weight is steady and she is toddling about extremely well with no debilitating effects from her kyphosis (lump in her spine).   She was reviewed by Spinal Consultant in January who had no concerns at present but is keen to go ahead with spinal surgery late in the year or early next year if her 2nd BMT is successful and she has been withdrawn off all immunosuppressant medication. 

With regards to her BMT, this has been brought forward to the 1st March 2012.  Due to the failure of Chiara's first graft her blood counts have never fully recovered and I am presuming this is because she has a bone marrow at present that doesn't have a good amount of cells to produce sufficient blood products.  She has been requiring platelet transfusions every 2 to 4 days and blood transfusion almost weekly.   Due to the amount of transfusions she is requiring a decision was made to admit Chiara on 23rd February to have another central line inserted and commence Chemotherapy the following day.  On this occasion her Chemotherapy protocol will be slightly different and slightly less intense.  It will consist of a couple of different drugs but will still be administered over the 5 days.  She coped extremely well with her last Chemotherapy so I keep my fingers crossed for the same results this time round.  Richard has been and had all his tests carried out for the donor process and we await the news that these are okay.  He will have to endure injections for around 4 days to boost his  blood counts and then will be attached to a machine to have his stem cells removed and these will then be given to Chiara.  The advantage of this is that if in the unfortunate event of this 2nd BMT failing Chiara can be topped up with a 2nd bag of cells from Richard.  This was one of the disadvantages of her 1st BMT being a cord, there is no way of topping up.

Chiara has a mop of dark curly hair at present and it seems so sad that yet again she will lose it.  On the plus side she will also lose all the hair that is covering her shoulders and back due to the side effects of her Cyclosoprin drug. 

Having to go through all the transplant process again is very daunting and anxious but I have spoken to a couple of people who have went through it twice and the general feeling is that it is a bit easier 2nd time round.  One of the other Hurlers families we have contact with sadly lost their girl a couple of weeks ago post transplant and I have found this incredibly hard to come to terms with.  The little girl was same age as Chiara and both went through transplant at the same time.  There is not a day goes by that I don't think about Olivia's family. 

Stress seems to never leave our family and as much as wee stay positive through it all we are certainly pushed to our limit.  Richard, I am sure is nervous about his part in all this transplant and he has had a stressful time with troubles with Chiara and also the passing of his dad on the 31st January 2012. 

I look forward to June when hopefully Chiara will have a fully engrafted new bone marrow and is keeping healthy and home from hospital so that we can watch my gorgeous niece in the Grangemouth Galaday as the flower girl. 

At the moment we travel 3 times a week to hospital and the last couple of weekends have unfortunately been admitted. She is going through a few pre BMT tests to ensure everything is okay for transplant.  Echo has been normal and yesterday she went through her kidney test, the results of which we await.  Through all this Chiara remains her usual self and lets nothing phase her. She keeps nurses on their toes and keeps everyone amused with her antics.  Sometimes I could scream at her for her behaviour but it is shortlived.  Unfortunately for me Chiara is still not a fan of sleeping but I am optimistic this will come in time.

I will keep her blog up todate when she starts BMT workup.  We express our biggest thanks to family and friends who have supported us through this last year and more, there is no way we could have made it this far without you all.  One day I hope to make it up to you all.

11th January 2012

Since my last entry Chiara was commenced on Foscarnet for her HHV6 virus.  Her blood counts have all increased and today was the first time in over 2 weeks that Chiara never received her daily Platelet transfusion.  She, in herself is back to normal and jumping all over the place.  We did however have to wait for her Chimerism result which tells us who the counts belong to, if its Chiara's cells growing back or if we had managed to recover the donor cells.  Later this morning I received the news I didn't want to hear, unfortunately there were no donor cells to be found.  We now face a 2nd transplant.  I am however, calm and coping with it a lot better than what I would have done this time last week.  We have a plan for where we go from here and in my eyes at least we can have a plan because believe me being in here there are so many people worse off than us that deep in my heart I just know they don't or cant ever have a plan.  I look at Chiara today and I am thankful that she got through the last 2 weeks of what could have been potentially fatal.  What has happened to her new marrow is annoying, unfortunate and extremely unlucky but it has happened so we just need to move forward. 

Chiara will remain on the Foscarnet for around up to 2 weeks, she will be given a period of around 2 months to regain her strength and around that time she will undergo her 2nd  BMT from whom her donor will be her daddy.  He will have the chance to do the most wonderful thing and that is give his wee girl another chance at a normal quality of life.  Although Richard carries the disease he has enough white cell enzyme in his blood to give Chiara what she requires.  When parents are carriers of Hurlers they both carry lower amounts of white cell enzyme that non Hurlers carriers have.  He carries more than myself and is a full match for Chiara.  I know he will take great pride in giving his daughter the gift of life.  We are hopeful that she will not endure as intense Chemotherapy as previously albeit still hard to go through. 

I do not look forward to the weeks and months we have ahead but have great faith in that this will give Chiara the chance she deserves.

Two things spring to mind..................someone once gave me a card for my purse called "Footprints in the Sand" and its not until now I fully appreciate the meaning.  Wise words once spoken to me "God will only give you what you can handle".  If this is true then I am telling him now...................enough is enough no more can I handle.

But then again what doesn't kill you only makes you stronger.........

One night I dreamed I was walking along the beach with the Lord. Many scenes from my life flashed across the sky.

In each scene I noticed footprints in the sand. Sometimes there were two sets of footprints, other times there was one only.

This bothered me because I noticed that during the low periods of my life, when I was suffering from anguish, sorrow or defeat, I could see only one set of footprints, so I said to the Lord,

“You promised me Lord,
that if I followed you, you would walk with me always. But I have noticed that during the most trying periods of my life there has only been one set of footprints in the sand. Why, when I needed you most, have you not been there for me?”

The Lord replied, “The years when you have seen only one set of footprints, my child, is when I carried you.”

---

6th January 2012

Well I am not really sure where to start tonight or how to explain it but I am going to try and present it as best as i can.  Last night was a very difficult night for me to write Chiara's blog as I felt it was probably the first negative piece of news I had ever had to write regarding her diagnosis, treatment and transplant.  Up until last night Chiara had defied all odds against her and surprised everyone who came across her so to be writing what I did last night filled me with sadness.....however in true Chiara style she turned things around. 

Today we were awaiting Chiara's old cells being transfused into her when I was told it was being put on hold for a few hours.  Chiara had been tested for a virus called HHV6 (which is of the herpes family) in most people this can be dormant or active but cause no symptoms but in immunosuppressed or transplant patients it can be fatal.  Yesterday we were told she had one positive for this and one negative so therefore it wasn't really sufficient evidence to conclude that was the cause of her bone marrow failing.  This morning I was told that old blood samples of Chiara's had been looked at again for this virus and there was another positive.  It had been pointed out that these results could actually be from the donor and not from Chiara but it would be difficult to confirm.  Therefore today a sample was taken from Chiara's mouth.  Later on today we were told that the sample confirmed it was Chiara that carried the virus therefore she could be given treatment to halt the virus and hopefully let her blood counts recover and maybe a chance that the donor cells would regrow.  This to me is nothing short of a miracle.  My gut instinct told me days ago that there was more to Chiara's illness and I had to ensure every avenue had been explored before the cells were transfused.  Well today I got my answer.  Not only this but Chiara woke this morning bright eyed, bouncing with fun and back to her usual feisty self, a side I have not seen for a long time. 

I cant explain how I feel tonight, its a mixed bag yet again of emotions and also anxiety and hope of the next step in the treatment.  I certainly didn't think I would be writing a blog entry with this context that's for sure. 

Princess Chiara fights on ...........

Keep your prayers, thoughts and healing going everyone as it definately works.

5th January 2012

Well  since my last blog post it has been a somewhat mixed bag of emotions over the last 5 days.  After finding out Chiara could possibly have an antibody causing her problem, we then discovered that treatment for that hadnt worked.  I had prayed and willed those counts to come back up but they never did.  She was screened for several viruses as she still appeared unwell.  Today she came back HHV6 positive and I thought this was the answer we had been searching for.  Later on in the afternoon I was given the news that Chiara's marrow was now only about 10 to 15 % donor and the rest was her old marrow coming back.  At it stands the now Chiara has zero neutraphils so therefore she is at higher risk of catching an infection.  The decision is now out of our hands and tomorrow Chiara will receive her old marrow back again.  The plan is that by topping her up with her own cells this will give her an immune system to fight off the virus.  She will commence ERT again and hopefully when she is well enough in around 2 months we will go through our 2nd and final chance at a transplant.  Although the news was devastating I am handling it better than I thought I would. 

Chiara in herself seems to be getting better each day unaware of what lies ahead of her.  Keep positive is all I keep hearing but I have to admit I find it very hard when its 1 step forward 15 steps back in Chiara's new path of life.

We all now have to juggle the stressors of life at a hospital, life living apart and of course life trying to raise a child with Hurlers.