8 years post BMT -May 2020

Its May 2020 and we are living in unbelievable times, currently we are in isolation, 7 weeks lock down due to Covid 19.  Never would anyone have predicted these unprecedented times.  Last time we did isolation, it was following Chiara's bone marrow transplant in May 2012 which makes Chiara now 8 years post transplant.

A lot has happened since my post which is not uncommon in the life of a Hurler's child.  While Chiara has remained at Primary School currently in primary 5 she has watched her eldest brother, Joshua leave home and take up a new life in Dundee while her other brother Lindsay, left secondary school and went into further education at college.  How time has flown and whilst I sit and take in these lifetime changes my boys have achieved I long to slow time down.  The life of a Hurler's child is short lived and the coming years are not known to us, as they are to anyone however, for us we know that every year that passes is another blessing gained.  Lock down to us is the best way to keep Chiara safe and healthy however, I cant help feeling that every week that passes it's another week that we have lost gaining memories with our girl.  Weeks have passed since she has seen Grandparents, Uncles, Auntie's, cousins, close friends and sadly her brother.  The only way of contact is the gift of technology which allows her face to face.  Regrettably in, 2018 myself and Chiara's dad decided it was time to go our own separate ways, pressures of life and stresses we lived under made it impossible to continue.  However, Chiara has accepted this change and has continued to flourish and adjust.  Sadly because of this lock down Chiara is unable to spend valuable time with her dad due to risk of infection and sees him merely through a window or from a socially acceptable distance which is heartbreaking as every day having Chiara in our life is a blessing.

In June 2017 our community galaday was held and we were so happy that Chiara participated in this as Fairy, this was a day we thought we could never have seen.  To have her take part in such a big event with all her peers and see the enjoyment on her face there are no words.  It was a proud day for us all as a family and memories that will last forever.

In December 2017 Chiara became very poorly and was admitted to hospital.  There was no indication of the problem and Doctors were baffled by what could be causing her severe drowsiness and inability to carry out every day movement, she could not eat and was barely awake which lasted for 48 hours.  I can remember thinking that this had been the first time since her transplant that I was genuinely scared this was the end for my girl.  This was something out with my control, usually I would have confidence that diagnosis would be made and treatment commenced however, on this occasion I had no faith.  It broke my heart to watch my girl lie lifeless and have no idea what way this would go.  Then after 48 hours in true Chiara style she sat bolt upright in the bed and asked for a 5 item breakfast, yes she was back and within 24 hours she was discharged.  This is what happens in the life of Hurler's children every day things can change so fast.  We left hospital not knowing what had consumed her energy for those 48 hours however, only glad she recovered.

In April 2018 Chiara, myself and others took part in a major event, the Glasgow Kiltwalk to raise funds for a charity very close to our heart.  It was a small walk but it allowed Chiara to participate and feel the sense of achievement that so many others feel when taking part in events.  These are things that are all too often taken for granted but for Chiara this was a major life achievement.

Whilst Chiara continues to flourish I can see she has deteriorated in other ways sadly.  Her mobility at times is troublesome with pain and reduced movement.  She has lost her sight partially in one of her eyes and her hands have curled even more than before and she suffers pain most days.  Her hips in the morning are stiff and sore however, this girl never complains which makes me humble to think what a small child like her can endure and still remain positive. 

Chiara took up ice-skating for a while and loved it however, sadly her knees did not and this was short lived but I cant tell you how happy and proud I was to see my girl have that time on the ice mixing with other children.  She attended regular swimming lessons every week and loved them with nothing holding her back.

In October 2018 our very good friends Emma and Linda organised a fundraising event to raise money to buy Chiara a specially adapted trike to allow her freedom.  Although Chiara has a wheelchair to get around she is not keen to use this therefore a trike was felt to be the ideal solution.

The charity fundraiser was a sell out, everyone attended in fancy dress and the night was amazing and enjoyed by all.  The total amount raised exceeded £5000 and no matter how much I say thank you to every single person involved I would never be able to achieve the gratitude I felt and the overwhelming feeling of love for Chiara that so many people around us were willing to help.  We have always known how much Chiara has touched the lives of so many people however this to me was more than we could ever have known.

Two days following the fundraiser Chiara was admitted to Glasgow Children's Hospital for an arranged operation on her knees.  She would receive 8 plates bilaterally and these would remain in place until her knees had straightened.  Because of the turning in of both knees Chiara had come to rely on a K-Walker for mobility and stop the falls.  Another operation is a frightening time as Hurler's kids do not do really well under anaesthetic due to small airways.  However, Chiara embraced this and a few hours later we had her back on the ward.  She wanted home so desperately that she constantly asked staff when she could leave until she was told once you can walk 6 steps we can let you go.  Well, this I knew was the worst thing to say to Chiara as she would take this as a challenge.  Within minutes she was "get me up Mum" and there she tried, tears streaming down her face in pain and a body full of Morphine she attempted these steps until she completed.  To watch your own child endeavour so much pain and have so much determination is heartbreaking but it is something that I suppose we are getting used to with Chiara.  She returned home and continued to progress until she no longer required the K-Walker and was walking alone.

Chiara's hearing was always a problem and from 5 months following being diagnosed a deaf she was fitted with hearing aids.  However, as the years have flown by her hearing improved, she no longer required the aids and to date she has achieved passing her hearing tests hearing aid free.  A miracle some would say.

As time passes I reflect on what has been and what has still to come and I appreciate nothing is set in stone, nothing is promised in this life and as Covid 19 consumes and affects everyone of our lives you realise that we are all in the same situation living in fear of something.  To me and my family living with Hurlers and having endured 2 bone marrow transplants which leaves your body immuno-compromised is like living in a world with Covid 19 every single day of your life. Since Chiara's transplant all those years ago we wish people would accept its safer to social distance from her, every day we step out, go to school have visits from family and friends is a risk of infection to Chiara.  Therefore Covid 19 restrictions to us is normal life for Chiara as we live every day in fear of infection. 

Due to the restrictions in place all of Chiara's hospital appointments are on hold or are being carried out over the phone.  Regrettably her 8 plates that are due to be removed from her knees is unable to be carried out and she is awaiting carpal tunnel surgery however, at present we eagerly await lock down end in order clinical duties can be resumed.

#staysafe

Now age 6

What can I say it's been 2 years since I last posted so quite a lot required to bring the blog up to date. Sadly I was prompted to update Chiara's blog due to the increase in diagnosed children with MPS1H    Theses diagnosis are all over the world and like myself and our family the families of these affected children are desperately looking for answers, hope, miracles and with their heads full of questions they are turning to the Internet in hope of news to give confidence that their child will live a life the the full. By keeping Chiara's journey up to date I hope that families can find some answers they are looking for and take comfort from our girl's life so far and all obstacles she has overcome. She has inspired others by the courage she shows and fear/defeat does not seem to be a word she has in her vocabulary or emotions.


April 2016

As everyone already knows Chiara's hearing has always been an issue so it came as no great surprise to us when she started showing signs of hearing loss. along with this she had abnormal snoring and a sleep study carried out overnight to measure her saturation levels was reported as showing some slight "dips" in her sleep suggesting her oxygen intake was being compromised at times. We had already experienced this and sleep apnoea in MPS kids was not uncommon.  Chiara went under anaesthetic and had adenoids and tonsils removed again not uncommon in MPS kids.  Also her hearing loss felt to be attributed to "glue ear" therefore she had grommets inserted into both ears.  This made a huge difference with hearing tests being reported as good and snoring albeit never disappeared it was not as bad.

In July 2016 as a family we managed to go abroad on holiday.  2 weeks of normality, where no one knew Chiara's history and she could blend in with the other kids.  This was a fantastic opportunity for us to show that MPS had changed our lives there was no doubt about that but our girl was not defined by MPS and with family support and encouragement will make the most of her quality of life.  Chiara was 3 years post transplant and living her life to the full.

In March 2015 Chiara was admitted to Glasgow to have a bilateral hip reconstruction carried out.  This was something that as a family we knew would be required at some point but nothing ever really quite prepares you for what it involves.  Chiara's surgery lasted around 8 hours.  We had been told that it may not be possible to achieve bilateral reconstruction during one operation therefore once we past the 5 hours we knew that the surgeon had managed to achieve this. We were taken to see Chiara in intensive care post op. She was in a spica cast hip to ankle both sides with a solid bar between both legs for stability.  She looked fantastic although upset.  The following day Chiara was transferred to the ward, and was requesting to sit up, this proving a bit tricky,  As the days progressed Chiara on day 3 was out of bed and in a wheelchair, she had an epidural for pain relief but this was removed and she managed her pain on oral pain relief. Less than a week post op she was discharged home where her rehabilitation would begin.  Needless to say 6 weeks later when the cast was to be removed she had almost worn away the knees as she had been pulling herself across the floors to hold on to her independence not liking the wheelchair dependency.Once cast was removed we were discharged home and within 2 days Chiara's walking had returned.On last X-ray her hips were adapting to this surgery,  for how long we don't know but we will take each day as it comes.

August 2016.

Well this month saw our brave girl put on a school uniform and accepted into mainstream school with assistance from enhanced provision.  This was yet another day we never thought we would see. Instead we stood with tears in our eyes while she sat in her classroom, smiled and looked like she had
just been given yet another chance to push the boundaries. Tears were shed from many, the times we
watched her fight for life attached to drips, ventilation machines, oxygen masks and have blood
drawn daily seemed like a bad memory as we  stood overwhelmed with emotion  to see her sit and begin her education.  To date Chiara enjoys school to the max. She can be overpowering, boisterous, loud and cheeky but this seems to be a common theme for our girl since she entered the world.

In December 2015 we suffered a slight decline in Chiara's progress when a spinal X-ray revealed another slight curve at the top of existing surgery scar. She was fitted with a spinal brace and to date still has this.  Again in true Chiara style she has adapted and accepted this. No parent wants this for their child but for us it's in the minority of issues, if wearing this brace corrects her curve and no near future surgery is required then it's worth every second.


In April 2016 Chiara required yet another set of grommets to correct the "glue ear".  We also await results of MRI to see if she has any pressure on spinal cord.

Chiara speech has developed at such a speed, it was almost as soon as she found her voice we could not shut her up.  She continues to have incontinence issues now and again, behaviour proves challenging at times, she requires knee correction and continues to undergo testing and will soon be wearing glasses.  Day to day life is as near normal as it can be, life with MPS was never going to be easy, it's a learning opportunity for everyone.  Chiara doesn't know or think there is anything wrong with her, in her head I believe she thinks everyone wears a spinal brace, everyone takes medication daily and everyone is as "special" as her. I don't look forward to the the day I break the news to her she has Hurlers so in a weird sort of way although for all these years I have written this blog for other families maybe it will end up being Chiara's source of living with Hurlers and our saviour.

18th February 2014 (21 months post bmt)

Recently I have found myself being asked on many occasion, do I still write up Chiara's blog, simple answer is Yes but only if I have news to tell.  When I started this blog it was to raise awareness of Hurlers and give future Hurlers sufferers or parents of affected children an insight into living with a Hurlers child.  I never ever imagined so many people would be interested in reading Chiara's story and following her journey, when I logged on last night her blog has had over 23500 viewings which is absolutely overwhelming. This is worldwide and if reading Chiara's journey can give any affected families out there hope and happiness then that in itself shows me that documenting every last detail of her journey so far has been well worth it.

Now for Princess Chiara's update............................................................

Last entry of the blog post spinal surgery where unfortunately Chiara developed an infection in the wound and had to return to theatre for manual washout and then continue on antibiotics.  It was advised that the course of  IV antibiotics should run for around 3 months which would give the spinal surgery time to fuse, which would mean that if Chiara should develop another wound infection then any metal work that was insitu could be removed with fusion having taken place, if infection recurred before spine had fused then it would mean all metal work having to be replaced which neither her family or Spinal team wanted.  We agreed to the commitment of attending our local hospital 3 times per day for Vancomycin (antibiotic).  In order for a therapeutic dose to be administered and retained then her blood Vancomyicn levels had to be taken regularly and dose adjusted accordingly.  Chiara spent every night overnight in hospital in order for her to have one infusion at 10pm then another at 7am and another at 2pm. We came home in between 7am and 2pm and then again after her 2pm infusion until her 10pm infusion.  Amazingly we still managed to fit in a family holiday to a caravan which we arranged so that we would be in a caravan park close to a hospital that was able to give the infusions.  It did prove stressful at times with all the running back and forth and not ashamed to admit that it did take its toll on relationships within our family.  However, we got through it as no matter how hard it was we did not want to take the chance that Chiara could end up back in theatre for further spinal surgery.  During these months of infusions Chiara turned 3, she attended hospital for infusion and found her hospital room decorated with birthday banners etc, then post infusion returned home for a family get together to celebrate her turning 3.  It was a gorgeous sunny day, kids had their pool out and Chiara joined in all excitement with no care that she was travelling back and forth to hospital.

One week later Chiara had blood taken as I wanted to keep a check on her counts, I was given the results and these showed that Chiara's counts had dropped so much so that she had become neutropenic (neutrafils under 1 which makes you more susceptible to infection) I panicked as Chiara had never been neutropenic since transplant.  Immediately I tried researching in vain to find a possible explanation, I found what I was looking for, prolonged use of Vancomycin antibiotics suppresses the bone marrow.  I contacted her Spinal Surgeon who then confirmed after his research that in fact it was highly possible her drop in counts was Vancomycin related and the antibiotics were stopped immediately.  I then went through to Yorkhill couple days later to check counts again and thankfully they had returned to normal levels.  Chiara then had to return for spinal x-rays to see how the fusion was progressing and if there was any sign of infection deep in the metal work.  X-rays revealed no infection, spine had fused perfectly and in fact there was no further need for Chiara to commence with the Spinal brace and she would be followed up 6 months later.


In August we had the joy of watching Chiara attend her first day at mainstream nursery, this was a day neither myself or her dad ever thought we would see.  To think that 2 and half years previously we had been given the news no parent ever wants to hear that our child had a life limiting illness, then watch her fight for life more than once connected to ventilation equipment, we had truly been blessed and had every one of prayers and wishes answered.  On this day I also saw my youngest son attend his first day of High School and realised how my 2 boys who were once my babies were becoming young men.  Chiara and myself found ourselves for the first time in 3 years at home on first day of school recommencing after summer holidays.  An event we had missed out on for the last 3 years.

 

 

Chiara settled into nursery fantastically, and remains to this day loving every minute of it, she does frequently get into trouble for her mischievous behaviour but through all Chiaras problems and illness her behaviour is proving my most difficult condition to treat yet. !

Chiara is fully toilet trained apart from the odd accident, she is big for her age especially for Hurlers as MPS affected individuals are usually smaller in height.  Her speech could be better but as Chiara still uses her sign language to communicate she has no reason to actually speak, this is something we are working on. 

She has had a couple of visits to the local hospital since start nursery but thankfully nothing too serious, common colds, sickness bugs etc. 

On 6th December 2013 Chiara had her routine appointment at the Sensory Centre.  3 years ago on that day tests revealed Chiara had nerve damage and was deaf and fitted with hearing aids.  On this visit today tests revealed Chiara had normal hearing and no hearing aids. This was almost impossible but true, I don't need to know how or why she can hear now ( I have my suspicions but only selected people know) all I am thankful for is she can hear. 

December saw Chiara attend her first Christmas parties ever and meet Santa Claus for the first time, it was brilliant, a most enjoyable experience for us all.  Christmas Eve was spent with the 3 kids baking cakes for Santa Claus and being hyper for the visit from the man in the red suit.  Christmas came and went uneventfully illness wise. 

In January of this year Chiara had been suffering from recurrent colds, runny nose etc, she was scheduled to have her portacath removed on 27th January as this was no longer required as she was not receiving any treatment now.  Unfortunately Chiara developed a temperature 3 days prior to this and was admitted to the local hospital and commenced on IV antibiotics, she was transferred to Yorkhill the day before theatre was planned.  While in hospital she tested positive for Adenovirus which is just really like a common cold.  Her portacath was removed as planned and Chiara was discharged a couple of days later.  While she had been I had asked regarding removal of Tonsils and Adenoids for Chiara as she frequently had such a lot of colds, this is now ongoing and she has appointments arranged to see if these need to be removed.  Chimersm (which is donor blood) remains at 100 percent, something I still worry about to this day and think possibly will every day. 

Chiara has had Orthopaedic appointments regarding her "Knock Knees" but as her Papa points out a lot of super models display these features so maybe she is going to become a model!!!!! !! Xrays reveal that there is no real concern for any surgery at the moment but she will continue to be monitored.  Hips look good.  Due to the Hurlers Chiara will always require Orthopaedic intervention due to her skeletal frame.  We will deal with all little problems if and when they occur. 

Over the coming months Chiara has routine Eye, Heart, ENT appointments as May will see her 2 years post Bone Marrow Transplant.  Chiara is due to restart childhood immunisations next week which we are desperate for so she can enjoy an experience in the swimming pool.  

As life is slowly developing for us now as a semi normal family we look to take Chiara abroad in the summer and enjoy family time with the boys.  3 years in and out of hospitals constantly eventually takes its toll on relationships between everyone in the family and hopefully a small glimpse of normality might be the answer we are all needing.

13TH June 2013 - post spinal fusion surgery

On 23rd May 2013 we dressed Chiara in gown and gave her a pre med and placed her on bed at 8.30am and wheeled her to theatre where she was put to sleep smiling at both myself and her dad, that was the last we saw of her until 7.45pm that evening, to say it was the longest hardest day of my life is an understatement......

We were fully aware of the lengthy surgery but nothing prepares you for what thoughts you have going round in your head and churning feeling in your stomach. Chiara was given her pre med and she laughed and giggled so hard she was so cute, it was almost cruel what we were about to put her through.

Spinal fusion took place, involving removing discs and vertebrae.  Surgeon went in through her chest and turned her over and then operated on spine, inserting screws, pins, rods and a hook.  We had been told if after he had went in through the chest it looked as if she wasn't coping then they would stop and perform 2nd stage at a later date.  Don't know what was worse waiting for a phone call half way through the day to say that they had no choice but to halt operation or the thought that she was stable enough to have front and back incised on same procedure.  Spinal cord electronic tests were carried out throughout surgery to ensure the cord was not in any danger.  Thankfully these were all normal.  Before operation began a Respiratory Consultant carried out a Bronchoscopy ( a small camera down to examine airways) given Chiara's previous respiratory problems and lengthy anaesthetic everything had to look good enough to carry on.  Thankfully her airways appear to have improved greatly with the time that has lapsed. She was admitted to ITU post operation which we knew was always to be the case.  She was ventilated and had chest drain insitu, along with arterial lines.  Her face was extremely swollen due to operation and ventilator but she looked well.

Doctors had hoped and planned to excubate Chiara  from ventilator the following morning, to enable them to carry this out her sedation had to be reduced.  If Chiara's past history of reducing sedation is anything to go by then I didn't think this was going to be as easy as what was initially thought.  I was right, I arrived Friday to receive the news that Chiara would spend another24 hours ventilated.  It was felt due to the fact she had chest drain, catheter and large wounds and she was posing to be slightly feisty that it was decided to enable to aid with the healing process and give her body a chance to rest she would remain intubated.  Disappointing yes but definitely a sensible decision.

Saturday I arrived to Chiara thrashing around trying to excubate herself.  Plan was for excubation  at midday  but by 11AM curtains were pulled and excubation was carried out.  She looked great, she looked in pain but well.  She was then placed on Bipap ventilation to help with her breathing, she tolerated this for one night and then decided this was no longer required.  By Monday she was off Oxygen , chest drain removed and catheter removed.  This then gave Chiara freedom to try pull herself out of bed.  Her pain threshold seemed to be higher than high.  She was transferred to a Respiratory ward on Tuesday incase she required help with breathing.  Tuesday night she was up on her feet, shaky and sore but up all the same.  Her dressing had some leakage on it and she had developed a bit of a temperature . Swabs were taken following day and confirmed she had a wound infection and required IV antibiotic therapy.  On a plus side we were allowed to take Chiara outside of hospital and went for loads of walks and picnics.  By end of the week Chiara was walking around great and complained of hardly any pain except for the cannula that was in her foot.  It was ironic, she had just had major spinal surgery carried out yet the cannula in her foot that was used for her IV antibiotics was what was causing her the pain.  It was decided she would go to theatre and have a temporary line inserted.  This was carried out and IV antibiotics were at midday therefore if Chiara's infection blood markers continued to improve we would be allowed home although returning every day for IV.  Obviously we jumped at chance of taking her home regardless of the daily journey.  Sadly on Monday our joy was diminished yet again when I showered Chiara and underneath her dressing revealed a very full blistering wound.  Swabs were taken and we were to return to hospital and admitted and Tuesday.  This was what we had feared. Chiara was taken to theatre on Wednesday with a manual wash out of wound performed and also the possibility of rod exchange to be carried out.   Until she was opened up we would not fully know how deep the infection had went.  It could be surface which was initial thoughts but it could also have gotten into all the metal work in her spine.  Thoughts raced through our mind, Chiara just has no luck but has lots of spirit and determination which has gotten her this far.For the first time ever when I took Chiara into the anaesthetic room she screamed "mummy no" and was sobbing uncontrollably, she knew, she remembered, something I had never experienced with her.  It broke my heart to leave her but no option was available.  Post surgery we were given the news that at the moment no rod exchange was required as the infection was not as deep as the metal work.  She has been stitched with different stitches this time and has a different dressing on which will remain on until Monday.  She also has a femoral arterial line in for bloods etc therefore we will not be returning home until next week.  She remains on IV antibiotics.  Despite this she is up on her feet with minimal pain relief.  We keep our fingers crossed that this dressing holds and the wound clears of infection.

Start of the next stage. 13.05.13

Well its been almost 10 months since my blog entry.  So much has happened and I apologise if I miss anything but I will let you all in on the main points.

My last entry saw us celebrating her  2nd birthday.  Since then Chiara has had quite a few admissions back into hospital due to viral infections and minor complications.  In March Chiara had her portacath removed as she had been admitted over 3 consecutive months and she had positive blood cultures from the port showing it was infected.  This was removed and as much as I'm delighted it also means that she has no immediate venous access therefore any IV medication or treatment required then Chiara has to endure cannula insertion which is not the most straightforward as Chiara's veins prove to be a problem.  In December of last year Chiara started the dose decrease of Cyclosporin (the medicine that stops the marrow rejection but also causes extra body hair growth and a decreased immune system) by end of January she had completely stopped this medication therefore meant her immunity would increase and she could mix with other children.  Unfortunately this came at a price.  Every time she she mixed at soft play etc she caught a virus and ended up back in hospital.  This is when she acquired yet another set back and developed a condition called AIHA which is Auto Immune Haemolytic Anaemia.  This means her body was failing to recognise her own immune system and had produced an antibody which was killing off her own Haemaglobin.  This ended up with her receiving a couple of blood transfusions and a weekly treatment of a drug called Retuximab to destroy the antibody.  At that time it felt like every time we made advances we ended up taking 1 step forward and 5 back.

Chiara's hearing is still great and she wears no hearing aids with her hearing appointments now 6 monthly.  Her extra body hair is slowly falling out and she looks more girl like rather than monkey! although she was kind of cute with it.

On 3rd May we celebrated Chiara's one year post transplant anniversary.  Her last check showed she was still 100% donor chimerism, Gags as low as 12 and normal levels of White Cell Enzyme.  Her heart echo was as normal as it was the previous time.

We now face a long 11 hour spinal surgery which Chiara will undergo next week in Edinburgh.  An operation I have mixed feelings about.  These being that Chiara's spinal Kyphosis proves no problem for at present, she walks, runs climbs and does everything normal therefore I think why put her through it, on the other hand she is young and to have it carried out just now means less stress for her when she is older.  Hopefully all going well she will make a speedy recovery as Chiara always does and is well enough to start nursery in August.   The protocol where we live is that the spinal surgery is carried out after transplant when the individual is well enough.

Chiara has grown into a gorgeous, curly haired cheeky wild child.  Would expect nothing less with her having 2 brothers to follow.  She now attends toddlers, soft play and parks.  Her speech is not the best but she signs very well and therefore has it in her head that there is no need to talk.  I am sure this will come with time.  Chiara is on a milk and gluten free diet and copes with this amazingly well and certainly  to look at her she is very healthy looking with a lot of reserves. We have enjoyed our first family holiday late last year and if Chiara is well enough will be going away in the summer.  I have normality being in employment again and slowly we are living a normal life.  On 31st December we sadly lost our beloved family cat after 16 and a  half years and it saddens me that Chiara will miss out on the memories the boys had with her.  Chiara certainly is not the angelic little girl I had dreamed of, instead she climbs, bites, and punches anyone that gets in her road.  She loves playing football with the boys and plays with her car and bike majority of time. I try my hardest to clothe her in dresses with ribbons and bows in her curly locks but the look is soon diminished when she is caught dress up in air throwing eggs at my windows.

After next week we will have another procedure ticked off for the time being.  We know that through Chiara's life she will have to endure probably hip and knee operations and anything else the life of a Hurlers kid is thrown but as family we will see her through it and ensure she has the best quality of life she can.

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.