It was three years ago last week that James had his Bone Marrow Transplant at Starship Hospital.
Today I joined 261 Bikers for the Annual Fundraiser Bike Run for Ronald McDonald House. We rode from North Harbour to RMH Grafton Mews where we spent months living in and off ward when James was transplanted. I spent some time talking to a few families that were there today, and it reminded me how important it is to share stories in support of what comes after.
We’re doing good!
Its not without its challenges still….schooling, adjusting to a cochlear implant, ongoing toileting issues, and then the serious issues that JAmes will need two new heart valves and a C1 and C2 vertebrae replacement/fusion are in our future.
But for today, we have a happy 8 year old boy. We are reminded every day, to make the most of now, and that we have the strength as a family to deal with what comes next.
Doing a BMT seemed so long ago now, although at the time it feel like the world had come to a standstill.
We will continue every day enjoying the simple things and sharing smiles.
Thats the way everyone should live….rare disease or not.
Last night the Campbell Live story of James cochlear implant turn on aired. A link the the story is below.
James reaction to Johns personal message at the end of the story was priceless, and really drove home the difference this implant will make in his life (and caused a few wet eyes in our house as well.)
Thanks to everyone for the humbling messages of support this week. Its the stuff that keeps us going.
Paul, Fiona, James and Maggie
Today was James cochlear switch on day.
The team at Audiology at Greenlane Hospital were fantastic today, and helped James feel at ease with his new hearing device.
After some testing of electrodes in three different frequencies with some tonal sounds they then turned the device on at a low volume and raised it to a point where James was responding to our speech.
Amazing to think that a child that has 85% hearing loss can hear with a bionic ear.
We were again humbled by the growing number of people on TEAM JAMES, and today have added some more friends.
James will have ongoing habilitation to learn to how to hear with his cochlear implant, weekly and then monthly for the next year, followed by the implant for the right ear.
James seemed at ease during the whole process today, and all he wanted to do was get back to school and show his friends his new cochlear and the cool school bag the team at MEDEL had given him.
Campbell Live were there filming the switch on as they had used a new facial monitor that had been donated by NEW WORLD that cost $75k when they did his surgery. It should air later in the week and I will post a link to that when its available.
We also filmed the whole switch on on our camera and I will post some video later tonight when I get the chance.
Today was a good day in our journey with Hunter Syndrome, as we took another step forward in helping James have a normal life living with this disease.
As usual, his sister Maggie was by his side in support and friendship.
Tomorrow is switch on day for James cochlear implant.
We were finally able to remove the dressing that was covering it and got to see the incision where his implant was inserted. Once the hair grows back over it you won’t be able to see the mark, but for now we can tell him it looks a bit like Harry Potters wizard scar on his forehead.
We plan to video the switch on and will post his reactions when we get some time over the next few days.
This week James had the surgery for his left ear cochlear implant.
He is having Medel implants, as they are easier to use should he need MRI scans in the future
The surgery took about 3 1/2 hours and went well. He had some bruising and swelling for a few days, and had some time off school.
He is still wearing his right hearing aide so he has some hearing, and this will need to come off once his left implant is switched on to help his brain adjust to the different way of hearing.
Switch on for his implant will happen mid- May, and we will video that and try to post a link of the turn on and his new hearing.
For the last few months James has been assessed as a candidate for cochlear implants. He has severe hearing loss because of his disease and currently uses hearing aides and a wifi microphone at school.
On Thursday this week James was approved for bilateral cochlear implants. It will mean surgery to do the implants. They won’t do them at the same time but will do one and let him adjust to it, and then do the second one.
We are just going to discuss with James main metabolic specialist this week if there are any pitfalls or future things to consider before proceeding.
Here is a video that explains about implants.
Its been a long time between posts on James blog, more than a year has past. That certainly not because we haven’t had anything to share, but because life has a new normal for us.
James is doing great, and now a big 7 year old who goes to school, enjoys Keas and bossing his sister around. Maggie is now 5 and at school at well.
James doctors have all been very positive about his post BMT results and his blood work has been encouraging.
His chymeric results last week were 96.2% which is the highest its been. This should mean that he has enzyme being produced.
The last month has been a flurry of hospital appointments that have included an MRI, a holter monitor study for his heart, blood tests, dental, ENT, and and ECG. Constant monitoring is part of the schedule and part of the new normal.
James has also been assessed for Cochlear implants, as his hearing due to the disease has always been a concern for his learning. We dont have an outcome or decision about the best way to proceed as yet, but we are getting close now.
We have moved from Christchurch and are now living in Auckland, which is where Starship Hospital is. Its been much easier getting to appointments when its just up the road.
We will try to write more regularly, as a record of our journey and to share information with others who are on the same path as us.
Tomorrow it will have been one year since we had the call from Starship to say “Could you come tomorrow??”
A lot has happened in our lives in the last 12 months, and in a few more weeks we will have a little celebration for the 1 year anniversary of James Bone Marrow Transplant, which was on 19th September.
We left our home for two months and lived in isolation in the hope that a procedure we were told was uncertain in outcome would save our sons life, or at least improve its quality.
A year on and James is doing just awesomely.
His chimeric result we got back a few days ago is back up to 86 from the last result of 78 which is awesome news.
Hes a happy, healthy and vibrant 6 year old boy who can read, write, attends a normal school, loves his little sister and is a joy!
Callum, Emma, Becs, Lochie, Nicola – You are all amazing for the support and reassurance you have given us on the start of this journey and that the work you all do is not only important, but changes peoples lives for the better. My thanks.
The last few weeks have been busy with James having visits at Christchurch hospital for annual checks on his eyes with the optician, bones with the osteopath and cardiology did a full day wearing a holter monitor (ECG heart monitor).
Its coming up to the time when we head back to Starship for the 1 year reveiw of James BMT results. His chymerisim is lower than expected at the moment, and the hematologists are doing some T cell analysis to see if they can find some more information on where the graft is at.
All in all, James is doing GREAT!! His eyes are good and his bones seem to be ok, and he will have some more x-rays over the coming months to check mainly his hips. Fingers crossed the ECG results are good, but that was only done yesterday so we wont expect anything for another week.
James has been doing well at school and his language and learning are improving in leaps and bounds. Heres some photos of James over the last few weeks….
It took Dentist 1&1/2hr to take out four teeth & do a couple of fillings for James under GA this morning. On waking James was quick to take up the lemonade ice block offer & down two yoghurts!! This evening was like any other at the Marquardt so let’s just say James has recovered well:)