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 !

Pictures, updates and life in general

Harrison above in the swing seat on Christmas morning. He just loves sitting and swinging.

Harrison in hospital at Jan with Line infection around the central line site.


















The photo of Harrison with "santa" A blow up santa at the front of our house over Christmas - he kept asking to "See Santa" in his little baby speak - as very cute !

I have attached some pictures and video of the boy at Christmas and hope you all enjoy watching my amazing little man come of age. There was a short hospital stay in Jan for H with a line infection around the central line site - a skin infection of sorts so there are pictures of him then too.

In less then 6 weeks Harrison will be two - a milestone I thought we would not achieve some weeks, and he has proven many people - including myself to be wrong. He is nothing short of fantastic. He is down to 3 monthly visits at Children's hospital and there is talk of starting immunisations - then testing his immune function after the first course to see how his body reacts. I have no idea how the bone marrow stuff all is going, but tis must be ok f the doctors are starting to discuss all this.

I am writing this from hospital with Ella in this time - has a lovely infection in her jaw - and many swollen glands etc, which needed to be surgically drained.. Its cool being on a general ward though, and not stuck in an Iso room. She is doing well and went home 1 week after being admitted with nothing more than a red swelling and some stitches. At least Harrison seemed to miss this infection tho!

Soo.. when I get all the blood test results I will update I promise, Harrison has shown a slight growth in his lymphocyte count. This count was at 0.8 and is now 1.1, and we are aiming for 4.0 So still a ways to go, but certainly getting there. His next big review on 20th March will review immunology stuff, eyesight and hearing - so a big day for us


This video is of the water baby himself. It was a lovely day and he was desperate to get into the water and have a splash so we let him do it - given he seemed so well at the time and there was no one around.
Another update to come shortly ....

Christmas, New Year and hospital - again!! :)

Well we had a lovey Christmas and New Year, and a quick trip to hospital for a line infection on the 6th to 8th Jan. So far all is good after a few IV doses of antibiotics, and some to give him for the next 7 days orally.
He is almost back to his old self - albeit a bit sleepy still.

He had some bloods done in hospital which showed his neutrophils had dropped slightly below normal ranges, and that his lymphocyte count ans white blood cell counts had dropped a little too. This seems to have normalised again which is positive and shows he was in fact fighting an infection. On a plus side he did not develop a temperature and his immunoglobulins are in normal ranges, despite the infection, so hopefully when we have the bone marrow transplant revew next week, we will see signs of improvement and hopefully get rid of the central line :) I will do my best to convince the Dr's so that Harrison can go swimming, and keep out of the toilet( where he loves to splash and play when I don't watch him carefully enough). Then of course we get the disinfecting of him and all the toilet surrounds, and hope and pray for no infection.

So that is it - very short and sweet. A huge thanks to Mum, Nikki & Bill for looking after the kids in my absence and for my day care mum Jodie who also stepped in and really got us out of a bind too.
next wednesday is BMT review and all the sorting of bloods will be done to test the progress of the 2 bone marrow transplants.

I will then update all the info re that and add a stack of photos and a vieo of Harrison down at Gosford Waterfront New Years Day.

Harrison is speaking very well now too - the latest is him pointing at the television and saying press play - whether or not the DVD is in to press play on, which is funy and cool that he is talking so well already.

Life in the fast lane

Well I know it has been a while since my last post. Harrison (I feel) is doing well, and things are swiftly moving in life generally.


On the 3rd September Harrison underwent a second bone marrow transplant - basically just the second lot of cells frozen away were used as a top up - to try to boost along his first BMT progress. This was cool as I got to see the whole thing - as I missed most of Harrison's first transplant trying to be in two places at once - as that day Ella had the bone marrow harvested and I tried to be with her and Harrison by running around back and forth - so this was a lot calmer. Harrison was quite a stinky boy after this BMT, as the preservative in the stuff STUNK to high heaven! But 3 days later he was stink free and looking good - and thus far has gone well with no signs of infections, transplant problems and other things that can occur when no chemo is done!

As yet I don't really know how well this is going, but... Harrison is now 20 months old and is now 12 weeks post second bone marrow transplant. We find out on 3rd Dec 08 the results of this progress - a month of waiting for blood test results will finally be over :)


Lately things have changed at the home front too. The kids and I have moved to my mum's house and Scott & I have made our separation final. Our home of 4 and a half years is being sold and then everything will be really complete. The kids have really settled in well at my mum's though, so this is encouraging.


I have no pictures to add with this, but promise some shortly.


Harrison saw the neurologist regarding his epilepsy a bit over a month ago, and it was decided that he has some epileptic discharges on his EEG, but the Dr's felt that we could take Harrison slowly off one of his epilepsy medications as they felt the two he takes were no longer necessary. Unfortunately in the process of reducing him down off the medication he started to have seizures, where he lost muscle tone and his head would drop quite severely. So... Back on the medication he went, and now it has been increased further to get these seizures under control. At least we found out about it and it is being controlled now - but the seizures are not good and can make him quite unstable on his feet - he looks a bit bruised and battered on his forehead due to falling on it a few times!! Ah - he is a boy though, after all!

We have recently had a review with Harrison's eye Doctor. Due to Harrison having Retinitis Pigmentosa he has 3-4 monthly reviews to ensure the eyes are still OK. Because Harrison has a turn now in his left eye we have to patch the right eye for 1.5 hours each day in an effort to strengthen the left. This works some days but mostly not as he really fights the patching process.. But in the long run it will help his eyes and that is the main thing - and so we persevere. Harrison's retina's both look quite patchy on the outside (peripheral) section - basically meaning that he is losing his peripheral vision and will slowly develop night blindness. Unfortunately for Harrison his retina degradation is already quite severe. Most people live a normal life til in their 50's when they start to be badly impacted by this disease. Harrison eye's, by comparison to a person who has a family history of retinitis (RP), looks like a person would in their 30's. So his degeneration is expected to be much quicker and the services of Vision Australia will be needed soon. His Dr anticipates him to need a laptop computer for use during primary school to assist him. He also says that because they have no information on the effects of immune deficiency, and bone marrow transplants on people with a retina condition, they have no idea if this will make things worse, or help Harrison's case. Time will basically tell.

In the 18 months since the Dr's diagnosed Harrison with RP, they have noticed a change in the optic nerve- gone from pink to rather white looking because the blood vessels have all thinned. For me I feel this means that he will probably not see adulthood with vision in tact. I have to say that it makes me feel quite sad that his life will not be as full as mine or his brother and sisters lives, and there are days- like today - that I could sit and just cry for what will never be. And I really wish I knew why. Noone can give us that answer though, and so I guess I will just have to settle for I'll never know.

I also wonder at how this will impact on Marlie, Sterling and Ella as they grow up- having to always watch out for their baby brother. Some days I look at them and see how grown up they have been forced to become. But without them life just would not be the same. And Harrison will always have lots of people to love and support him, no matter what happens in the future - and for that I am the most thankful.

And so life goes on, Christmas is approaching and Harrison will hopefully (no infections please !!) will have his first Christmas at home. And as we live next door to a man who has a thousand Christmas lights out already, and all of my kids love the lights, I too am going to take on the Christmas spirit and light up our house too!

Pictures coming next blog . and it won't take me 3 months to do this one I promise.

Bone Marrow Transplant Review

Well things are not as rosy as I was hoping! Harrison will need his second bone marrow transplant after all, but he does not have to have the conditioning before it - instead they will give him the cells we still have frozen as a kind of top up. This is great news as we are only adding to his progress this far, not restarting - good for him, good for us and good that he has no chemotherapy to add to his woes! :)

This can of course still have its own little complications but we'll get to that if anything bad happens. Harrison has stopped having IVIG for now so that his next lot of bone marrow cells (hopefully) start making B cells straight away. Harrison's B cells have not grown very well and by stopping the immunoglobulins being given monthly we will hopefully make the new bone marrow cells think these are lacking and therefore make new ones. We all know he is lacking in these cells and that he does not make his own immunity but his body seems to be thinking otherwise at this point. ( Harrison's T cell count is about 83% Ella's cells now - so we are making slow progress )

There are a few issues we therefore have to watch out for - making sure that we go back to Westmead Children's hospital weekly after the second lot of marrow, making sure that any signs of problems are seen to immediately - as there is a slight chance that these cells may react and cause Graft versus Host disease, a potentially lethal side affect of this bone marrow transplant process where the new marrow cells attack the body they are put into. Hopefully this won't happen. And Harrison and Ella are such a good match that he should be ok.

Then we have the worry about the cells themselves, given they have been frozen for longer than 12 months. They may not work as well, there could be other issues that come from this but again until they are delivered into his body we have no way of knowing what is to come.

Until the new marrow has had time to take effect Harrison will not have any IVIG temporarily, which puts him at risk a little of infection, but then he will restart a short course to give him the top up he needs monthly of immunity cells, and by the end of October we should have an idea of how things are progressing. If things are well then we may even be able to get Harrison about more, but I will hold by breath til January for this to truly happen, so if he goes well and he can start to go out and about more, earlier, then this will just be a bonus.

Otherwise he is doing well - bar a chest infection this week, and yet another course of antibiotics.. Oh well at least he doesn't get pneumonia!
I will update from kids hospital after the transplant with photo's for you all to see.

Harrison's Blood Counts thus far, eye sight review and neurology stuff

What a doosy this blog is - all very medical but I will try to keep it as simple as possible.

OK on the 31st July Harrison had bloods taken to assess the progress of his transplant, and to quote our BMT Doctor - slow and steady.. More like just plain old slow - but that is enough of my griping.

Harrison has a lymphocyte count of 0.8 (800 cells) so still a tad off the 4.0 we are aiming for - which means he is still quite badly immune deficient, and susceptible to infection. Hi white blood cell count had been low for some time - very low in fact, but this seems to have risen to 5.3 from 2.2 - and although this count is still low(and therefore not close to being normal) its a step in the right direction!

His neutrophils which have been up and down like a roller coaster lately have settled back down to a nice healthy (and in normal ranges) number - which is a good thing as these cells help fix you up when you cut yourself, and boy oh boy Harrison has been on a one man self destruction mission of late! He has been very wobbly lately - more so than normal on his feet and this is partly due to ear infection but I am not sure this answers the problem completely. As a result of his almost drunken walking "style" he has split his lip open in four separate places, cut above his eye - which had to be glued, had his fingers trapped in a door as it was being shut - he was falling and grabbed onto the door jam & was caught right down to the knuckles on his hand, and he has chipped off a good sliver from one of his front teeth. To say he is in the wars, well that would be an understatement.. I am beginning to think about bubble wrapping him for protection.. Then he could be a bubble boy in more than one sense! :)

And lastly the all important T cell count. Was 76% now 3 months later only 83% - that is donor cells in his blood system. So while it is positive that this number is increasing , its still aways off being classified as a success. URRRGGGHHHHH.. Hopefully the B cell numbers will have grown somewhat, as if they are still around the 25% mark then things may not be so good - and these we need so he can make his own antibodies and get off having the immunoglobulins infusion every 4 weeks.

So we wait - and also the test for the toxic cell % is still being done in the USA - which may or may not be back in time for our 1 year post transplant BMT review on 22 August. If this figure goes up again, then this could also indicate a few problems. Hopefully things will be all good, cause honestly I don't know how any of us would fare with Harrison having a second transplant done.

Now we get to the eyesight review - the first one in 9 months and so far not too bad. We found out that Harrison's retina has only changed very slightly, and there seems to be no swelling at the back of the eye causing issues, and both eyes have the same visual acuity - ie they can see the same. Harrison's left eye though, has a turn in it and it has gotten so bad that he no longer uses the eye, instead turning his head so his right eye takes off the strain. We are now desperately patching the right eye to get the left eye muscles to correct, and on 22 August we will find out if the eye patching - and the utter upset that this has caused Harrison has worked. At the moment we have to patch the right eye for up to 3 hours a day - in short sessions.

Harrison won't walk around a whole lot when he has the patch on, and so far the only way I can keep him distracted enough to leave it alone is to put on Dora the Explorer on the DVD. He at least leaves the patch for up to 30 minutes, but the poor thing struggles. His eyes both flicker an twitch and his nystagmus (shaky eyes from left to right) gets so bad he just has to sit.

I really don't see much improvement yet, but I will keep on keeping on, until we have the review. If this does not work it simply means Harrison will be given glasses - a little earlier than the Dr wanted to give him but he definitely needs them already anyway, and so if its a bit early - well, what's the difference really? The Dr said Harrison's eyeball shape has a bit to do with his poor vision, they are more football than soccer ball shaped like most of us have, so his vision will naturally be affected anyway- The Dr said that Harrison's eye were between 3.5 and 4 "clicks" and most kids are a 1 - 2 measure - so he is a bit outside the norm anyway:) We knew that already but hey!

Also the whole SCID and RP thing has never really been dealt with before - both conditions are usually a genetic inherited thing - RP is a dominant thing - ie parents usually have it or carry it on dominant genes and pass it on, where as SCID is recessive and far less frequent. Never has there been a SCID kid with RP - it's just not heard of, and so we heard the word SPECIAL a lot at kids. We knew that of course, even from conception and his pregnancy surviving the implant of an IUD after I was already pregnant, that he was special, we just didn't know how "SPECIAL" he would turn out to be. !!! The Dr's are now running on the theory that Harrison juswt had something special and the RP is not in fact, a second genetic inherited disorder, but just a side affect of the SCID - Lucky him :)

His neurologist has cut down one medication to twice a day instead of three times a day - and we have a review and overnight stay on 1st Sept to assess his epilepsy more then.

Anyway that said in true Harrison fashion he is now awake at 11.00pm just as I was hoping to go to bed. I will now go get him and try again to settle him down.. Ahh kids - don't you just LOVE them? Hmmm ... Hope this all makes sense - if not drop an email and I will try to explain better.

Harrison driving me nuts while trying to blog

Harrison seems to think he is the next Bill Gates - microsoft would nmot know what hit them if this happened - something like a cyclone methinks....

fbSo Harrison is going to type and when he goes to bed v
i will update ... see how helpful hhe is !!!!


s
v bfdsscxxcszcdnvfv g v fc ff fcv m ty n yuy uyxnn h fde54vbb ffd v czvvcfvvb frfvxscA aw2dsrAQVSFDX I will leave it to you to translate!!!!!