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..

2 years post Bone Marrow Transplant & update on eyesight

Well it is almost 2 years since Harrison had his first transplant done and things have developed Slowly but hey, they are still developing.

The latest report from blood work done says this :
" I am delighted to say that Harrison has normal IgG, M & A but the IgE is still elevated. His Ig subclasses are normal and he has normal blood counts. His lymphocyte count is 1.3 and has a low B cell count and low NK cells. He has a good level of antibody response to his killed vaccines that were given (the 2, 4 and 6 month old immunisations) .
Harrison does not have complete immune recovery - which is normal in a patient who did not receive(pre BMT) conditioning. "

Basically Harrison was well enough that we tested the waters with him having the first three rounds of immunisations that all other kids have - these were successful and he has shown that he now has antibodies that have grown from this. We yesterday gave him the Chickenpox vaccine - which in itself would normally be ok, but because Harrison's still has very low NK white blood cells, he may not respond to the vaccine - or worse still could get chicken pox. If this occurs he will go to hospital for a course of antibiotics and anti chickenpox antibody blood transfusion.

It is a risky move, but in light of the nasty strains of chickenpox that can be out there it is better to try to give him some immunity by vaccinating him, than by not. So we wait and see how this vaccine goes. Hopefully - and I am crossing all of my fingers, he has a good outcome and we don't see him get chicken pox. Time will tell and I will keep the blog updated re this.

Other than than he is well, talkative and very cheeky. His eyesight is deteriorating - well at least I feel it is. And we have reviews coming up with neurology and the eye doctors. We are actively working with Vision Australia now to help Harrison to learn braille - we even have a braille writer to play with :)

He seems to love the bright coloured tatrapoint brailler. I have a correspondence course to learn braille too.. But I will be buggered if I can learn it by touch - lucky I have eyesight and can see what each of the dots mean, so it will be easy for me to learn and then teach Harrison braille. We will start with a cane soon to get him used to the idea of that also.

It is heart breaking to watch him fall over things. He dropped his dummy the other day - right in front of himself , and when he bent to pick it up - he could not find it . I had to point it out to him. He seems so normal though in every other way - ad so despite the eyesight and Retinitis pigmentosa we are very lucky.

Next blog updates on chicken pox and lots of photos - as FINALLY i can upload them again :)

Quick update

I know its May and still no recent photos. Computer issues hamper this badly but I have worked it out. So... While waiting for those to all upload, I thought I would write a quick update. Since the Central line came out in February - Harrison has had his second birthday. He has had a visited by Vision Australia to assess and start working with him for his growing blindness. And he has had two of the first 3 required immunisations. I am hopeful that he is well enough to have number 3 tomorrow ( Which will be the 6 month old immunisation).

Harrison is going along extremely well. Although I have not got a clue how any of his blood counts are going, and will find his out next month - or 4 weeks after the immunisation is done , at which point the hospital will test everything fully for the first time since January. The neurologist will review him then too , as there has been a change in his epilepsy med regime.

Also on the 11th June Harrison will have another MRI. Done under a general anaesthetic, this will check things like brain size, any potential problems caused by the epilepsy, and by the ADA SCID. Fluid amount around the brain, and the little bony skull growth that was growing weirdly at the last scan. Its mostly just to check on things and be certain all is 100% OK. Hopefully this will be positive, and also to be sure the Dr's will do a lumbar punch while Harrison is under to check on (and hopefully not grow) any bacteria or infection in the spinal fluid. All path of the course, but poor tike... Another GA. Grrr. Still its for a good reason and its about 12 months since the last scan so this is really good going.

That is it.

So.. watch this space, pictures a plenty a coming. He is quite the little man. He even speaks in sentences, and sings Twinkle Twinkle Little Star and Happy Birthday. Impressive for a kid who had such a rough start in life ! Just goes to show how super smart he is :)

Take care to all who still read, hope all is well.

Home

Harrison came home Monday just gone - he still has his wobbly boots on and his eyes are very shaky at the moment, but he is eating walking around and talking up a storm as per usual.

I enrolled Sterling in soccer today and am looking forward to going to days watching him play. Harrison's discharge summary said his lymphocyte count was 2.2. Now I hope this is truly the case as I can take Harrison (on not too cold and rainy days) to soccer to watch Sterling play and this will make it that much more fun and family oriented.

I decided to start looking at houses to buy - little ones that I can do improvements to, so that I can have a secure and steady home for Harrison with his eyesight and for Marlie, Sterling and Ella to have a family home, as time goes by.

The good news is that Harrison is home, seems stable and relatively unscathed and that life is moving along nicely right now. It is less than 2 weeks til Harrison turns two, and I am trying to get together a slide show of pictures from day dot to age 2, I see an amazing change and I hope you will too.. to be continued,

Update

Harrison has been moved to a standard Iso room, and has dropped off one antibiotic, so this is good, but the bad news is that he started vomiting tonight, as we have been force feeding him liquids in an effort to get his gut going again.

If this force feedng doesn't work, we will have to put in - and for the very first time- a nasal gastric tube to get some fluids and nutrition into him. I sincerely hope this is not an option we have to take up.

He has diarrhoea still, which is not good, and he is still very sleepy but is having more periods of wakefulness so I am hopeful he will recover soon. Mum has switched places with me tonight to give me a break and I really needed some time with the kids as I knew they were getting very anxious.

I had a lovely visit from my work friends yesterday - thanks for the cupcakes - they were a major part of my dinner and coping with blood tests being done!! :) And thanks to Donna, Jodie, Joanne and Nicola for keeping me sane with calls and sms messages checking how things are going.. also Nicola and Dave added Marlie , Sterling and Ella to their 3 last night and had them stay over while I was with Harrison and Mum worked. The kids really enjoyed the time away and were so excited telling me about staying there, again my thanks to Nicola and Dave for this.

Scott has called up each day to ask after Harrison and check on his progress.

Harrison is having a lot of difficulty having his blood being taken, as no central line means they have to take the bloods in the normal way, thru a vein. I remember as a baby how much trouble we had taking his blood this way, and a few months ago the line stuffed up and we had the same trouble taking bloods "peripherally" ( Via a vein). In the last 2 days he has had 2 tests taken to check his levels as things are very all over the place at the moment and some of his levels are very low. It has taken 8 attempts to get enough blood to be able to test. That is 8 separate goes at various veins. If you are horrified at this - imagine being there each and every time to hold him down screaming to have them done. In the end last night I very nearly cried as they finally hit a vein that gave just enough to test with. One doctor suggested he was a candidate for having a new central line or like put back in to enable bloods to be done.

God forbid this should happen, and I have to admit this kind of rocked me. I left the room post testing last night at nearly 8.30pm, had a coffee, cried my eyes out for a few minutes and went back with a more positive "can do" attitude to all this. I know it has to be done, and I know the only way to truly monitor him is to have regular blood testing done, but it makes me sick when they come in , again, to try again for more. The poor darling is sick enough, let alone this.

I have to hope that his veins are so poor due to the gastric bug, and that he is dehydrated, as opposed to him just having shitty veins. I have no idea how we will get the bigger tests done if this is the case and he doesn't have some sort of line or port put back in. But lines mean infections and we start the vicious cycle again. I tell you I am about in tears writing this, but I need to write it down and wipe my slate clean.

Also the ongoing virus of this gastric bug has meant his weight has dropped 800 grams in less than a week. He started to gain weight back yesterday and was 14.64 kg. Today he weighed in at a paltry 14.04 kg. At least he tried eating apples, so hopefully the pectin in it will help his but settle. Mum said he vomited tonight though after trying him on some neocate formula. This stuff is very elemental, so he should have tolerated it. Oh well, looks like we'll be in hospital a while yet - until at least he picks up and starts eating and drinking.

This one's not a very positive blog, but it is the truth and sometimes its good to read the bad - gives a better overall picture of what is going on.

Also the fact that I feel guilty for starting back at work and asking to make it 4 days per week as of this week , but then totally being unable to even go to work. I really thought that Harrison was past all this and so would never have committed to work knowing otherwise. I guess that is the way the cookie crumbles and another week off work- luckily with a very understanding boss. Again thanks.. :)

I will update on him again as things go - hopefully with better news than this one.

Back in hospital again ...

Its now 12 Feb 09 and Harrison has been here for 4 days with a central line infection and a really hasty gastro bug. He had his line removed on 11th feb- never to be replaced!!!

What a milestone - here is a picture commemorating the day of removal.. He looks very puffy in his face and he is very very sick - in fact he has been under high observation due to spiking temperatures and having had a febrile convulsion. This was probably the scariest thing I have ever seen - he has a huge spike in temp and went from 35 degrees to 40.4 degrees, his eyes rolled back in his head, he convulsed and stopped breathing. At first I knew he was seizing so was ok to start with , but he stopped breathing and by the 30 second mark i was really panicking - and then suddenly he stopped moving and took a massive breath, and settled down. I will never forget it though, and I realise now why they are so damned dangerous.

After that they transferred him to a high observation unit on the Children's ward at Gosford as they had to 24 hours a day monitor him to ensure that he didn't do it again. Mum has run herself stupid looking after Marlie, Sterling and Ella while I am here, and all the while managing to still work and keep the house immaculate! I still have a few lessons to learn from her - but without her and Jodie - my day care mum I would have been struggling.

The house Scott and I owned and lived in together has been sold and settles on 23rd March- which makes everything finalised with the financial aspect of our relationship and the child arrangements are yet to work out. He doesn't see the kids overly much and this is disappointing but at the same time it must be difficult to not come home and see the kids every day. I guess this is the way life goes. Some days are better than others and you can either go with it and just keep going or let it get the better of you - and I choose to just go with it - why sweat the small stuff ..

Harrison today has been moved from the high obs unit to a single Isolation room until he returns to being well - high central line was removed - with a little diffculty and a few extra nicks on his skin to get it out without tearing his skin. But it is goneand he will be so much better for it . At least it is one less source of infection for him . This infection is called psuedomonas. A respitory infection but was in his lines, and hwen they hooked up the IV that is why he had the spike in temp and the convulsions. They call it a septic shower - as the bug goes directly into the bool stream and vein near the heart so the reaction to the bug is almost instant - and very scary . HE does seem better today havng had the line out - and his gastric bug - Adenovirus is also responsible for chest infections too.. So they are being doubly cautious with him to ensure that these two bugs don't hit his chest area. So far, so good!

I have a few pics of him line free, and in hospital. We are watching him closely and it was a near thing him going into intensive care - simply that they had the available staff to put him on kids ward and not have to go to intensive care, the only other time I have seen him this sick though, is when he was a tiny baby and ultra sick.

I admit now even to myself that I held my breath this time hoping he wouldn't go backward, and his blood counts are all over the place and his platelets are very low and his blood protein too - so I am stll crossing everything that he improves quickly as I hate seeing his little body so ravaged with pain and him constantly sleeping as he's too sick to do anything else.

For now we wait - and a move to a standard isolation room is hopefully a step in the right direction.

Harrison in emergency this was post convulsion when we were moved to the resus bay in case he fitted again.

Harrison post surgery - LOOK NO LINE!!!!!








Today Harrison has been the most awake he has been for 4 days - he has not eaten a thing for the same amount of time - but he had his first bath with no line in today - a picture speaks a thousand words....


Also I got an email from my friend Narelle in Singapore and her daughter Ella who has SCID too, had her line taken out the other day also .. Ella and Harrison seem to do things in pairs - so a huge congratuations to Ella on another milestone, and it looks like her BMT was very successful as she has nearly completely normal blood counts for everything . I hope Ella's good counts begin to rub off on Harrison - but only tme will tell...

I will update in 48 hours how Harrison is going - as I find this quite therapeutic and stress relieving to write - it means I can allay my concerns here and stay positive in teh room with him.


Ella in hospital with her bad infection and second picture, you can see her cheek and jaw padded post surgical drainage. She is pictured with my nephew Seth - what a chunky 7 months old baby he is !