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.
