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.