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.

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).