Now for Princess Chiara's update............................................................
Last entry of the blog post spinal surgery where unfortunately Chiara developed an infection in the wound and had to return to theatre for manual washout and then continue on antibiotics. It was advised that the course of IV antibiotics should run for around 3 months which would give the spinal surgery time to fuse, which would mean that if Chiara should develop another wound infection then any metal work that was insitu could be removed with fusion having taken place, if infection recurred before spine had fused then it would mean all metal work having to be replaced which neither her family or Spinal team wanted. We agreed to the commitment of attending our local hospital 3 times per day for Vancomycin (antibiotic). In order for a therapeutic dose to be administered and retained then her blood Vancomyicn levels had to be taken regularly and dose adjusted accordingly. Chiara spent every night overnight in hospital in order for her to have one infusion at 10pm then another at 7am and another at 2pm. We came home in between 7am and 2pm and then again after her 2pm infusion until her 10pm infusion. Amazingly we still managed to fit in a family holiday to a caravan which we arranged so that we would be in a caravan park close to a hospital that was able to give the infusions. It did prove stressful at times with all the running back and forth and not ashamed to admit that it did take its toll on relationships within our family. However, we got through it as no matter how hard it was we did not want to take the chance that Chiara could end up back in theatre for further spinal surgery. During these months of infusions Chiara turned 3, she attended hospital for infusion and found her hospital room decorated with birthday banners etc, then post infusion returned home for a family get together to celebrate her turning 3. It was a gorgeous sunny day, kids had their pool out and Chiara joined in all excitement with no care that she was travelling back and forth to hospital.
One week later Chiara had blood taken as I wanted to keep a check on her counts, I was given the results and these showed that Chiara's counts had dropped so much so that she had become neutropenic (neutrafils under 1 which makes you more susceptible to infection) I panicked as Chiara had never been neutropenic since transplant. Immediately I tried researching in vain to find a possible explanation, I found what I was looking for, prolonged use of Vancomycin antibiotics suppresses the bone marrow. I contacted her Spinal Surgeon who then confirmed after his research that in fact it was highly possible her drop in counts was Vancomycin related and the antibiotics were stopped immediately. I then went through to Yorkhill couple days later to check counts again and thankfully they had returned to normal levels. Chiara then had to return for spinal x-rays to see how the fusion was progressing and if there was any sign of infection deep in the metal work. X-rays revealed no infection, spine had fused perfectly and in fact there was no further need for Chiara to commence with the Spinal brace and she would be followed up 6 months later.
In August we had the joy of watching Chiara attend her first day at mainstream nursery, this was a day neither myself or her dad ever thought we would see. To think that 2 and half years previously we had been given the news no parent ever wants to hear that our child had a life limiting illness, then watch her fight for life more than once connected to ventilation equipment, we had truly been blessed and had every one of prayers and wishes answered. On this day I also saw my youngest son attend his first day of High School and realised how my 2 boys who were once my babies were becoming young men. Chiara and myself found ourselves for the first time in 3 years at home on first day of school recommencing after summer holidays. An event we had missed out on for the last 3 years.
Chiara is fully toilet trained apart from the odd accident, she is big for her age especially for Hurlers as MPS affected individuals are usually smaller in height. Her speech could be better but as Chiara still uses her sign language to communicate she has no reason to actually speak, this is something we are working on.
She has had a couple of visits to the local hospital since start nursery but thankfully nothing too serious, common colds, sickness bugs etc.
On 6th December 2013 Chiara had her routine appointment at the Sensory Centre. 3 years ago on that day tests revealed Chiara had nerve damage and was deaf and fitted with hearing aids. On this visit today tests revealed Chiara had normal hearing and no hearing aids. This was almost impossible but true, I don't need to know how or why she can hear now ( I have my suspicions but only selected people know) all I am thankful for is she can hear.
December saw Chiara attend her first Christmas parties ever and meet Santa Claus for the first time, it was brilliant, a most enjoyable experience for us all. Christmas Eve was spent with the 3 kids baking cakes for Santa Claus and being hyper for the visit from the man in the red suit. Christmas came and went uneventfully illness wise.
In January of this year Chiara had been suffering from recurrent colds, runny nose etc, she was scheduled to have her portacath removed on 27th January as this was no longer required as she was not receiving any treatment now. Unfortunately Chiara developed a temperature 3 days prior to this and was admitted to the local hospital and commenced on IV antibiotics, she was transferred to Yorkhill the day before theatre was planned. While in hospital she tested positive for Adenovirus which is just really like a common cold. Her portacath was removed as planned and Chiara was discharged a couple of days later. While she had been I had asked regarding removal of Tonsils and Adenoids for Chiara as she frequently had such a lot of colds, this is now ongoing and she has appointments arranged to see if these need to be removed. Chimersm (which is donor blood) remains at 100 percent, something I still worry about to this day and think possibly will every day.
Chiara has had Orthopaedic appointments regarding her "Knock Knees" but as her Papa points out a lot of super models display these features so maybe she is going to become a model!!!!! !! Xrays reveal that there is no real concern for any surgery at the moment but she will continue to be monitored. Hips look good. Due to the Hurlers Chiara will always require Orthopaedic intervention due to her skeletal frame. We will deal with all little problems if and when they occur.
Over the coming months Chiara has routine Eye, Heart, ENT appointments as May will see her 2 years post Bone Marrow Transplant. Chiara is due to restart childhood immunisations next week which we are desperate for so she can enjoy an experience in the swimming pool.
As life is slowly developing for us now as a semi normal family we look to take Chiara abroad in the summer and enjoy family time with the boys. 3 years in and out of hospitals constantly eventually takes its toll on relationships between everyone in the family and hopefully a small glimpse of normality might be the answer we are all needing.
