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keen to go ahead with spinal surgery late in the year or early next year if her 2nd BMT is successful and she has been withdrawn off all immunosuppressant medication.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.

130 days post transplant - what a year

Sorry for the delayed update on Chiara's progress but life has been pretty hectic.  After Chiara's last admission she was discharged home on the 22nd December 2011.  We were so excited to be getting her home for Christmas.  She still wasn't 100 percent herself but was certainly a lot better than what she had been.  Her story was to be published in the Glasgow Herald on Christmas Eve, Richard and I as her parents were so proud.

We were to return on Christmas eve to have her observations checked and her bloods checked.  As we sat anxiously waiting we just couldn't wait until we returned back home to prepare for Santa.  Our anxiety turned to fear as Chiara's FBC (full blood count) returned as showing she had platelets of only 2 and her Neutrafils had dropped to below 1.  Her Haemaglobin was also low and she was to receive a platelet transfusion and blood transfusion and also an infusion of GCSF to try and boost her Neutrafils.  My stomach heaved as I suddenly thought all our plans and hopes for our lovely family Christmas were just about to be shattered.  She received her GCSF and Platelets but her blood was going to take around 12 hours to be ready for transfusion so left later on Christmas eve afternoon and returned home. We would return on Boxing Day to receive her blood transfusion.

Christmas in the Petersen/Fox household on Christmas morning was magical and exciting as all 3 children scrambled round the living room opening presents.  It was a dream come true to be spending  Christmas morning together as a family in the comfort of our own home when only just over a week ago Chiara was connected to a ventilator. 

Boxing Day came and Chiara and I travelled through to hospital for 9am.  She had her routine FBC taken and again we waited on the result.  We already knew that she was to receive a blood transfusion but added to that she would again require another platelet transfusion as her platelets were only 2.  She would also require more GCSF as her Neutrafils today were 0.  This was just my worst nightmare, what was happening no one knew.  We would return on the 27th December 2010 for routine checks.

The 27th came and we attended for routine checks.  Her FBC came back as showing no Neutrafils and Platelets of 1.  Now we were faced with the news that we had always dreaded hearing, her new bone marrow graft was failing.  Chiara was admitted to hospital on 27th December and on 28th December was taken to theatre where she underwent bone marrow biopsy.  The knots and sickness I felt in the pit of my stomach I cant even begin to explain.  These feelings grew worse as I entered the Consultant's room to be told that there were very little cells available from her new marrow and was looking like she was going to be needed a 2nd transplant.  I went to pieces and sobbed like I've never sobbed for months.  All our hard work and effort and everything Chiara had gone through had been for nothing.  We were told there was always a chance that it was just a virus that had caused this and we would receive results on Friday.

On Thursday Chiara deteriorated and her breathing became hard work for her.  She was extremely irritable and agitated.  Not the girl we all knew.  It was heart breaking to watch.  Early hours of Friday morning Chiara was admitted into Intensive Care.  She underwent a CT scan for fear that she had developed a bleed on her brain due to the low platelets.  My stomach again heaved, how on earth could all this be happening.  She returned from the CT scan and we were told that after the CT scan they had aspirated her stomach and drew back about 20ml of blood, I wanted to turn and run away, my immediate thought was she had internal bleeding due to the low platelets.  However, her CT scan showed no abnormalities and the doctor didn't seem too concerned about the blood from the stomach.  It was possible that her irritability was caused from her Morphine withdrawal or simply due to her virus.  She had come back positive for Rhinovirus a 2nd time.  She was commenced yet again on Morphine and antibiotics.  Later Friday afternoon we had a meeting with her Consultants.  After discussions with other medical staff and transplant centres the general consensus was that it was possible that Chiara's marrow had been suppressed due to a virus or a drug.  It was also noted that she had developed antibodies in her blood and it could be possible that these were destroying her platelets, Neutrafils etc before they even reached her bone marrow.  This news albeit not great was better than the thought that she was losing her graft. 

Today (Saturday) Chiara was commenced on her new infusion to try and destroy the antibodies.  She seemed more content today than she has in days.  She remains in ITU on CPAP.  She will receive Platelet transfusions on a daily basis and blood transfusions as and when she requires it.  Her tiny body is covered in bruises and a blood pricked rash due to the low platelets. This is not how I imagined I would be spending the last night of 2011 or rather hoped I would not spending.  I head into 2012 with my 2 boys and Richard at home, myself in a hospital room and my daughter in Intensive Care.  What do I have to celebrate about 2011, absolutely nothing, my only hope is that 2012 is a better year for all of us. 

Happy New Year to all my friends, family and everyone who has followed Chiara's story.

A not so nice lead up to xmas (15.12.11)

Well today is Chiara's brother Joshua's 13th birthday.  Sadly yet again Chiara misses another family birthday as today she lies connected to a ventilator.

Last Thursday and Friday Chiara's blood counts had dropped to under one and we had the horrible feeling she was losing her graft as she appeared well and no signs of illness to account for these drops.  Later on the Friday afternoon she became grumpy and just generally not herself.  After a long battle with myself I decided later on that night to take her through to hospital.  She presented there well, no temperature and breathing normally.  On checking her oxygen saturation levels it appeared she was only 88%.  She was started on antibiotics and 1litre Oxygen.  She had a fairly unsettled night and by the morning she seemed to be getting a little worse.  On Saturday morning she had an xray.  We were informed later that this was awful and her lung had collapsed and she could possibly have Pneumocystic Pneumonia.  Once again I had that terrible feeling in my stomach and literally felt sick to my stomach.  What was to follow the next few days we did not know or rather not prepared for.

Sunday she was still lively but her Oxygen requirement had to be increased and she was started on more antibiotics.  Sunday afternoon seemed to change drastically so quickly.  She started working harder to breathe and her Oxygen requirement was being increased more and more.  Within half an hour she had deteriorated so much that she was completely Oxygen dependent.  She was then transferred from the Ward to Intensive Care.  She was tried on BIPAP for a few hours but she still had enough energy to climb all over the cot and sit up.  Her saturation levels were dropping slowly and not managing to increase.  We were told she required a blood transfusion also and this would hopefully maybe help he Oxygen levels.  The blood would be available around 1 or 2 in the morning.  Unfortunately by 2am Chiara had become so distressed that her Oxygen levels just dropped and dropped and I stood and watched them go down to 40 until I could not watch any longer and I left the room.  It was then I knew intubation would be taking place very shortly.  I sat for around an hour waiting and reliving all the feelings I had went through on that horrible night back in April when she was first ventilated.  Here I was 2 weeks before Christmas waiting for my wee girl to be connected back up to ventilator.  She was placed on an Oscillator which is a special ventilator that is kinder to her lungs.

On Wednesday Chiara was changed from the Oscillator to conventional ventilator as her blood gases etc were all good and her xray was improving and showing her lung was re inflating.  She was found positive for Rhinovirus which basically is just a common cold.  Through all this she still remains feisty and has managed to disconnect herself from the vent twice and is keeping medical staff on their toes as she tries to climb out of cot so she is nearly always kept heavily sedated and paralysed.  It once again is horrible to watch especially at this time of year but she is making progress.  I am only thankful that my gut instinct was right to bring her through to hospital even although she never appeared unwell.

Today we are hoping she will improve even more and hopefully be excubated very soon.

1st December 2011 (100 days)

Sorry folks for not updating before now but I have just been enjoying having Chiara home and gave us all a bit of time to forget she actually had Hurlers.  Today marks Chiara's 100 days post transplant.  This is a huge milestone for Chiara.  Getting through 100 days with minimal problems and no graft rejection is a huge acheivement.  Tonight we marked this occasion with a bottle of Champers.......to follow on the bottle we had when we brought her home from transplant in October........if we are going to be marking
every special milestone in her life then we are gonnae be needing a lot of Champers lol.  

Anyway after returning home in October Chiara has kept extremely well.  It has been an emotional rollercoaster for all of us.  I  am finding the constant travelling back and forth 2 to 3 times a week to Yorkhill very stressful.  I miss working terrible although I love spending loads of time with Chiara albeit she is extremely hard work.  On the 8th November we were given the fantastic news that Chiara's hearing levels had went from 80 down to 30/40 which is level of conversation so currently Chiara has no need to wear hearing aids.  As a family we know she can hear everything we are saying and she responds to all noise we make.  She is making great progress with her teacher and currently she is not developmentally behind schedule.  She crawls around like a normal child, she can pull herself up at furniture and walk around or walk around with her walker. 

Chiara still has no need for night time ventilation but on a recent visit to hospital she has another sleep study carried out which showed that her results weren't as good as the last.  A lot of Chiara's problems are being put down to the fact that she is carrying so much extra weight.  She has gained 20% of her body weight in 2 months.  Unfortunately this has been down to the steroids so currently she is on the her last reduction and hopefully when these are completely finished she will lose some weight and hopefully her breathing etc will return to normal.  It is not a huge problem but you can tell she gets out of breath quicker than normal because of the extra weight.

Chiara has also developed a lot of excessive body hair over her shoulder and back, this is a side effect of her Cyclosporin which is the drug to stop rejection so its a small price to pay although not nice to look at on your wee baby.  We have been told this will disappear once the drug is finished and hopefully this will be in a few months.

A few weeks ago Chiara was admitted to hospital due to her contracting what was thought to be hand, foot and mouth which put her off her food and fluid.  I myself didn't cope with this admission too well and went into complete melt down and Richard had to come and stay with her in the hospital.  My feelings at that time were that my whole life now was Chiara, my whole day was Chiara, my boys no longer had a mother with them since Chiara was born, my thoughts were that the boys gained a sister and lost a mother. I know Chiara didn't ask to be born the way she was but sometimes the pressure and strain can just get so much that you forget who you actually are.  Most of my days are spent travelling to and from appointments and the rest of my family are left to bring themselves up.  I hate the thought that one day I will wake up and my boys will be all grown up and I will have missed out on all those years of their lives.  I know many of you reading this wont understand what it is like and possibly even think I am selfish for thinking feeling like this but having a child with disabilities no matter how small or how huge the pressure and strain it puts on a family is immense.  I am now in the process of getting shared care hopefully for Chiara with a closer hospital and she is having home visits to have her bloods checked so slowly we are returning to a normal family life and my hope is that next year I will be able to obtain some work. 

We have been through so many highs and lows this year that I now look forward to Christmas with my family and hope that next year is a better year for all of us.

Chiara has her spinal appointment in January when we will probably be told when she will receive her spinal correction.  Hopefully the last surgery for a while.

On Saturday I am attending a charity disco, the proceeds of which are being donated to Hurlers.  I am so grateful to everyone who has shown support to Chiara and the MPS society.

We were asked to be included in a television programme to be aired in December regarding Chiara's story but unfortunately in the last week we have been told that she is no longer going to be appearing.  I have just finished writing an article about Chiara's story which will appear in the next issue of the MPS magazine so hopefully this will bring hope and smiles to a lot of peoples faces.

Merry Christmas everyone x