Bone Marrow Transplant No 3.

Well Since last post we have had a crazy couple of months.
The port insertion went in and has been a true blessing for delivery of intragam each month. Harrison has been fairly well through all of this .

We moved! to a cute house on an acre. and then had amazing storms and lived with no power for 5 days.
During this stormy period I received a call advising that Harrison's enzyme count was dropping.

Sadly I knew before the words were said that Harrison would need another bone marrow transplant, as technically this is his first graft failing. So rather than wait for it to fail completely and him become very unwell, and those dastardly toxic metabolites to creep up in number and do damage again, we agreed to transplant Harrison in the beginning of our Australian summer.

What seemed like the slowest couple of weeks passed with no more news, yet everyone around us as anxious as we were to meet the new team of doctors who would do this process. Then the call came and within a week we met our new Doctor, Melisssa Gabriel. Along with the original clinical nurse consultant we had Kay and a new ( well we had met her before but only briefly ) Immunologist, Melanie Wong.

We talked about the dangerousness of giving Harrison chemo, and the two types he will be given. Thankfully I don't recall the names or I would be playing Dr Google probably to our detriment. But we also talked about Ella being Harrison's donor again. Ella was asked as soon as I had the call to consider her options for being a donor, and said yes. She knows that despite her great cells it is Harrison's body that is not working well, and that should anything go badly it would. never be her fault.

Ella then went down a week later for independant review, and passed this with flying colours. We filled out a rheem of paper work, and I had to laugh and nearly cried at the same time, when the BMT doctor told Ella it was very very rare of her to tick "have you been a bone marrow donor before?" questions box with a  yes.

We then got a call to give us a date ( after another couple of week wait ) . Last week of September. It is coming along much faster than we would all like.
The reality of this means we have to pack up and store the house contents and give up our rental home. I cannot afford the costs of renting, kids and school, extra hospital costs of food, etc and the extra treats that the kids will need to keep them sane . Its going to be very hard. But a house can always be found again. It puts me under a lot less financial pressure to manage.

Chemo will put Harrison under immense strain. He is having reduced cycle chemo but for him it would be like a fully intensive round. Ada Scid kids are particularly sensitive to chemo it seems. He will end up with mucositis ( infections and sores in the mucous lining of mouth throat etc.) will probably have feeds done via nasal gastric tube, will have red blood cell and platelet transfusions to keep him going until the new bone marrow cells take hold.

As with last time Ella will have her harvest same day as Harrison has his transplant. As with last time my mum steps into my role to care for Marlie, Sterling and Ella while I go into hospital with Harrison - an expected 3 month stay. I will now try to update more and add photos as we go about this process. Second transplants for this are very uncommon. We hope to help at least show that its a process that works ok.

Updates on eyesight and immune function

Well since August, we had a few bloods taken and Harrison's immune function continues to dwindle. Doctors had him on IVIG temporarily to work out if his immune function would improve. It did but only while receiving IVIG. As soon as it left his system his numbers drop, so IVIG for the long term it is. We went through discussion of having it via a PORT, by monthly canula insertion or by subcutaneous I fusion( into belly fat)

As Harrison is extremely hard to canulate we tossed about a PORT vs sub cut
The port insertion won due to his extreme hatred of needles. Doing subcut is two - three times a week and I would learn the method to do this. So for now until he learns to cope better with needles he underwent surgery to place a portacath in his chest wall that doctors access monthly to run his IVIG.

So far this is working very nicely. As each month passes he gets better at this - it was inserted in October.

We are now counting down days to 13/3/15. We go for a day visit down to kids hospital to see his bone marrow team, immunology team and have a repeat eye ultrasound. Sadly we discovered right on Christmas time that the tumour he has in the left eye has started to grow in his right eye also. It was a for way back at the middle of feb and then July last rate, but it is now visible to the opthamologist when he checked his eye in December.

I am dreading this day. It will be a long day, bloods have to be taken & this ultrasound. Both tests mean I get to hold him down kicking and screaming for them. I also want to play ostrich and not think about this right eye.  I continue to scour the internet for any options to reverse the tumour in the left eye , and stop it growing more in the right. So far anything trial like I have found is not possible for Harrison, due to the continued immune deficiency and that the overall degeneration of his eyesight is too advanced to be considered for trials. That and he is only 7.
Oh well .. I keep looking , hoping , and praying that we come across a miracle.

This year Harrison's class have transitioned to his siblings primary school as a satellite class so educationally he is doing nicely and he has made some great little friends. There are always blessings in life.

Next post will be after the bloods and ultrasound to update on his progress

And while we are at it keep going strongly on your journey Katlyn... She has  had her bone marrow transplant and is waiting in T cells to engraft. We keep you in our thoughts  and pray you get grata news very soon!

Update - Eyes,Immune Function, Holiday & 2015 look ahead

Life as always seems to fly by so fast. Already it is a month since we did our life changing USA Disneyland holiday.

Two weeks ago Harrison has his portacath inserted (by surgery at Westmead Childrens hospital) into the right side of his chest so it can be easily accessed to delivery his intragam (immunolglobulins) every month. Harrison's immune function continues to be low, so this infusion gives him an immune system for a couple of weeks per month. It is not a perfect solution and not a fix , but the best of a number of choices, and will be a life long product Harrison will need. & as Harrison has very fine difficult to access veins we have opted for this port instead of canulas every month.

Prior to that we had a wonderful Disneyland adventure. Pictures will follow this post.
We visited California, Disneyland, Colorado and the Grand Canyon and Yellowstone National Park and Legoland. We saw extreme heat and drove through snow. Rode on many rides and celebrated Halloween Disney style
It was a holiday that delivered what it was intended to do, created a visual memory for Harrison and his siblings, so that when Harrison can no longer see, we all can talk to and with Harrison about this amazing holiday we had.

Of course, with such a holiday comes the realisation that this indeed is his lot in life, and while amazing, there were moments that were tinged with real sadness. Sadness that he wont be able to return to see this again in his lifetime, that even if we did go back to Disneyland, he wont be able to see it like he did this time, and that this really is a final farewell in the path to losing his vision.

The cataract has now completely overtaken the the left eye completely , and suspect the tumour has continued to grow. December 12 marks the next Eye doctor appointment and the day we find out about the surgical appointment we have in April 2015 for Harrison's eyes.

Harrison now only has 5 degrees vision in his right eye. He has a strange fixed focus on the left eye, and a glazed appearance always. Until recently noone really noticed his eyes and now everyone notices.

Next year there are plans for Harrison to go to the "big kids school" as part of his schools Satellite class program, which is exciting that he and his class mates will be able to have this fabulous opportunity.

Many changes are afoot for our whole family next year, and while Harrison has his big school adventure his siblings will all take on new paths also, high school for the oldest Marlie, a new support class for Sterling and Ella will go up into year 4.

I cross all fingers that 2015 is a better year for our family medically speaking and that we can plan a new adventure with lots of sensory input for Harrison to enjoy.. Maybe we go south to Tassie this time.!!
For now we are busy putting up Christmas lights and trying to make Christmas as memorable as possible -just in case this is Harrison's last Christmas to see things :) I feel an expensive light bill coming up but it will be worth it as he adores Christmas lights and decorations!

In case we forget to write Have a MERRY CHRISTMAS to ALL!!

Eyesight update

We have had news recently that the growth in Harrison's eye - known as vasoproliferative tumour- may be a cancer. Dr's took some time to further check and we have had reassuring news that indeed the tumour is not cancerous. It is an aggressive benign tumour so there is still much time to be spent checking and monitoring. Eventually the eye will have to be operated on in some form and at that time the surgery will send his right eye blind too. Naturally, we are keen to delay this as long as Harrison is not in pain.
So the disneyland trip is now officially on - we have been given ok from every doctor to allow this trip to go ahead.
We plan to have a month in the USA seeing the various sites of Disney and the countryside - mostly in a giant rv .
Harrison's grandmother has taken an early retirement to fund this trip but we have had a few amazing people on our side donating and organising donations to cover Harrison's share of our costs. So far that equates to nearly $1900 dollars and we are very thankful . Once the final figure is in we will be naming and thanking our sponsors and donators here online and in person .

Flight centre are may now trying to work magic to get us fantastic flight deals and help with booking everything for our trip- as we literally put on hold doing everything until we had news re the eye growths .
Next step passport applications finalised and off on a big plane trip.
I for one spend a Lot of time thanking the stars for making this growth to be a benign one. I think my little boy has had enough to contend with as it is :)

So two more doses of intragam to go and we say Bon voyage. It's sad and exciting at the same time to do this trip.
I wish we just had 2 more years so Harrison was that much older to really remember everything of the trip and to allow us more time for all the things we want to achieve.

Harrison left eye February 2014 

Small vasoproliferative tumour

Harrison left eye June 2014 

Larger more complex vasoproliferative tumour growth 

A quick shout out to Katlyn!
Katlyn is about to undergo a bone marrow transplant. She has made it this far and we know how hard that struggle has been!!!  When a donor match is made which we hope is very quickly, we pray for her that it's a perfect Match and it takes quickly and  complication free!!!

Our thoughts are with you on this next phase and we know that you will conquer this hurdle  as you have all others !

To track her journey here is the link ...Katlyn's blog

The vision is dying and quickly! Sometimes it feels like its one step forward and 7 steps backwards.

What a roller coaster the last 3 months has been. Harrison has had his usual round of blood tests last year to work out if his immune function is going ok. We were very surprised with the result. His B cells which have somehow reached 50% don't actually seem to be doing what they should - meaning despite injections and immunisations, Harrison has not immunity - to anything! So the doctors (luckily) ruled out another bone marrow transplant and have placed him back on a cycle of IVIG - aka intragam. Its the stuff they syphon out of plasma that is pure antibodies. It is what is now keeping him well and reasonably infection free.
But wait there's more. In the midst of this turmoil and upset , we discovered at a routine eye exam in February that his left eye has a growth in it. Initially the doctors thought it was CMV - cytomeglovirus - and that would have spelled disaster for us. Had it have been that, we were for certain, having another bone marrow transplant. Luckily and after a biopsy, retinal eye scan and MRI, it proved to be a hyper vascular growth in the back of the eye.

Known as "Coats like fundus" - it is a growth caused by the blood vessels growing at the back of the eye causing some kind of lumpy growth. Unfortunately it is very very rare - (and I note here with just a drop of horror and sarcasm combined- how 'surprising' is it that Harrison should get something super rare on top of his already rare immune deficiency and retinitis combination.!!!!! ) The even more worrying part to this rare growth is that it has well and truly taken hold of his left eye leaving the eye completely blind and has started growing in the right eye. Something the eye specialists tell me is highly unusual and they now have to check him 3 monthly with lots of scans and tests.

We are nearly back to the 3 month mark and due to go down for the next anaesthetic and checkups again. On top of this Harrison has a cataract over the left eye that has finished off his vision anyway. Surgery and freezing are the only ways to stop the growth as without stopping it - well lets just say I want him to still have beautiful eyes to look at - and no surgery means literally losing his eyes!! That is not an option. It is incredibly frustrating, saddening and maddening that I as a parent cannot make this better for him. Its a bit of a kick in the teeth and we have been given now a deadline without a damn date!! Maybe 12 months. But we aren't really sure even if that long, say the doctors. as a family we are in shock, as I think like me , the rest of the family thought we might have lucked out and Harrison's vision would hang around for a few more years to come. The solution to our problem is no less complex. One way to stop the growth is to cryofreeze - however in freezing the growths it damages other good cells, and so will damage the little tiny bit of good vision he has left - currently 5 degrees of central vision only. It will damage the way he sees too - and so while he sees in a limited field he sees nearly as well as most of us do with the right eye. As an example of what vision he has left .. If you closed your left eye, and looked through a black drinking straw - that is the field he sees. His acuity - is about 3/12 ie what he can see clearly at 3 metres, we can see clearly at 12 metres. If the surgery happens his acuity could be significantly damaged and he would be able to barely see in front of him, let alone anything at distance. and if the growths are left he goes blind very quickly and it damages the eyeball itself. So here in lies our problem. Today is a pediatrician visit to discuss this all. and I am getting an approval for Harrison to travel to Disneyland. I am appealing to all and sundry for help to get us all there and finish off Harrison's visual bucket list. He adores Mickey Mouse - spends hour and hour watching youtube clips of old Mickey Mouse cartoons and when he saw the ad for Disneyland asked me to find in on the computer for him - the scoured the Disneyland web page and asked to go visit. How can I not grant that one wish? The plan is simple - hope to head off in September and travel in an RV for a month. An RV we can clean down and keep infection free - something we are unable to do travelling between and staying in various accommodations. My mum has blown me away this week - as she seems to do on occasion - by offering to retire early so that her superannuation can be used to fund this trip for us all. Its a gesture of unbelievable selflessness and one I could never ever repay. I hope that somewhere with a little ask from me someone can help us get there - even to fund Harrison's costs and we raise the money for the kids and my mum and myself to go take Harrison to see Disneyland. In the interim it is back to see doctors today, 2 July and 2 September with a few blood tests, intragam doses and eye exams in between . Here are a few photos of what the eyes look like in a retinal scan and the MRI image that clears shows the growth in his left eye .... and a few of him just being Harrison !!

Left eye through cataract seeing the growth



Growth again from a different angle


Right Eye showing the damage the retinitis pigmentosa has done - the black part is the dying and dead retina the part just left of centre that is still showing no blackness is the part Harrison sees from. The white spot above right of the clear bit with no rp damage is the start of the growth that can be seen in the left eye above and below

Left and right eye showing the retinal damage being done by this terrible disease !

Our Uluru Adventure from 2013

Due to Harrisons vision going we took a trip to Uluru. One car, One camper Trailer, 4 kids and 2 adults. We drove an amazing 7,500 klm in 3 weeks but Harrison got to see with his siblings every step of the way by his side, a memory that he will never forget.,

Here is a visual display of that trip. The plan for now is get him to Disneyland . We were plannig by Sept 2015 but the recent news on his reapidly deteriorating sight means it has to be this year as he simply wont see it next year

Update - long long time no post

Well It has been a long time between posts - primarily as I had forgotten the login name!! Oh my :) Harrison is now 6 and a half and a right cheeky monster. He has grown to be a funny crazy little man and has started his first year of school this year. Due to his complications along the way Harrison has a few ongoing issues such as his immune function is still sub par - his bone marrow transplants are now as complete as they will ever get , but his cell engraftment of all three kinds of white blood cells is incomplete . T cell are great at 99% B cells ( them that make the antibodies and help produce immunities) are at around 30% and his NK cells dont exist.. So he is still susceptible to a great range of things - and despite many attempts to vaccinate the old man for chickenpox, measles mumps and rubella he has not produced antibodies. Recently as a result he came into contact with chickenpox and we had to get him an antibody transfusion to ensure that he stayed pox free!!! Luckily that worked fine .. scary stuff even still:) Harrison is quite developmentally delayed. He has a moderate intellectual impairment, an autistic spectrum disorder diagnosis, epilepsy and the eyesight is degenerating now very very rapidly. He has now only 5 degrees ( of vision left in his right eye only. Effectively its like looking through a drinking straw. His left eye is blind. He now hits the legally blind status and soon enough that right eye will follow along with the left. Ad so because of all of this he attends a special needs school. They are fabulous and have really made him very indepedant - he uses his long cane daily now and most days impresses people with how well he runs and rides a bike with his lack of vision!! We are about to embark on a series of see Australia adventures.. Adventure 1 - Uluru(Ayers Rock) The Olgas and Kings Canyon and then to Victoria to see the 12 apostles. Time for him to see what he can while he can- we are crafting books that have pictures in them and some touchy feely bits that will help him remember the trip - and hopefully we can make a few of these give them to guide Dogs and Vision Australia so other peope with a visual impairment can "see" australia too.. We are about to go to the hospital for our last ever bone marrow transplant checkup in a month. I am so excited but so almost afraid for the next step. It seems like its been such a long tired road to get here. For now though Harrison and his siblings are amazingly happy and have just moved on with life like nothing ever happened - and for that we can only be very thankful.. We have been truly blessed by amazing family, friends and the huge amount of support the kids have received through all of this - their world turned upside down for a long time and its a happy place to be now looking back:) Updates will follow for the last lot of results and how he goes now with his varying diagnosis and vision.

More photos

Harrison is now 3 - March 6th marked this special day.

Harrison and Marlie, sterling & Ella at Gold Coast on Holidays this year. They all loved this and spent an hour climbing on and around it :)

Since the last blog Harrison has been immune wise stable but suffered from a number of bouts of pneumonia and ear infections.

He is now back on his bactrim daily dose to help prevent further bouts, has had Chicken Pox immune booster - as he came into contact with Chicken Pox and is unimmunised for this, and if all things go well with blood tests done this week we should be able to undertake further immunisations.

So.... Here is a visual update for you all. We are yet to see his eye doctor, but his neurologist is happy and wants to only see him yearly now - a definite step in the right direction. Also Harrison will be reviewed by sleep study to try to determine why he sleeps so little and has such disturbed sleep in the nights. (hopefully I will then get more than 5 hours a night :) )

A picture with Harrison and Seth in prams, Marlie Sterling Ella and Myself at the entry to the 2010 Sydney Royal Easter Show.

Harrison had a ball at the Show - he laughed so hard I thought he woudl burst on the roller coaster, and he especially loved the "PUPPIES" - those of the mooing variety - otherwise known as Cows- to the rest of the world.. Lucky he's going blind or I would have been really concerned!! ha ha ..

Harrison and me on roller coaster at the Easter Show

A birthday cake I made for Harrison's friend Indiana's party . Very proud of my creation : )

Updates with Photo's of the BIG boy!!!

I know it has been a long time between posts and much has happened.

The pictures above are of the little man -

Harrison is off all his prophylactic meds ( ie those meds to stop hm gettign sick) and has had a course of immunisations which have worked well. He had pneumonia a few weeks ago again, and for the first time ever, he had a normal reaction to an infection. His white blood cell count increased and his neutrophils increased too.. Which in lamens terms means his body is learning how to fight off infection. It was rather thrilling to see him have a normal response - even though he had pneumonia!!

He has a cane now to assist with his eyesight and has been given the clearance from the Dr's to go out into the big wide world - So... Off to preschool for Harrison next year :) And given all the hospital and setbacks he is very much like the average 2 and a half year old boy. He is well spoken, enjoys life and is a general handful.

There are still a few ongoing issues of epilepsy - which we are working hard to control & his deteriorating eyesight, but all in all, he is going great. Even the news in terms of the vision loss has been positive and it is not degenerating as quickly as before. He may well have vision until he is around 10 years old. Which is heaps better than the doctors expected.

And soo a few pictures to keep you amused. Mostly they are of him with the cane - which when you take a picture of it lights up - as it has light reflective coating on it! Cool huh..