17th February 2012

Quick update.

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

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

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

9th February 2012

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

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keen to go ahead with spinal surgery late in the year or early next year if her 2nd BMT is successful and she has been withdrawn off all immunosuppressant medication. 

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

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

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

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

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

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

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