Back & Forth - Christmas , New Year, Jan - Feb

Over Christmas Harrison had an infection in his central line, and they Used Vancomycin antibiotics to treat this. Now for those of us out there who have immune deficient children, you will know ( and most likely love) this stuff.. However in Harrison's case this is not working. The doctor's are ordered daily cultures to be grown from the central line lumens ( his dangly lines hanging from his chest). They take blood and basically culture it to see if it grows anything, and despite all good effort, this was still happening. So a decision was made to transfer us down the Westmead Children's hospital, so the staff there could acidify the line and hopefully clear the infection.

Yes, this is just as scary as it sounds.Acidification is the process of injecting Hydrochloric acid into each plastic lumen and leaving it for 10 minutes, then aspirating (sucking the acid out of) the line and then flushing with saline to clear the line. This process is then repeated 20 minutes later and then a third time. The plan is to kill any bacteria in the plastic. It is actually a very interesting process.
And so the process began, by cleaning the outside of the line, injecting the acid, aspirating, flushing and then redoing the process 2 more times - 20 minutes after the last time. The whole process takes about 1 and a half hours. The acid when aspirated, comes out as a brown (almost rust coloured) gunge - lovely!!! :-) Then we were transferred back to Gosford, so that 24 hours after the acidification, the lines again had bloods taken from them and cultured to see if any bugs grow. Of course, bugs still grew - Nothing Harrison does is simple after all!! Normally at this point the bug woudl mean central line removal BUT we went for a visit to Westmead again on the 16th Jan for the BMT team to review Harrison. They drew bloods fromthe white lumen as it was uninfected and decided to do a Vanco lock ( Vancomycin antibitic injected into lines and left for 24 hours and changed 2 times) - This which actually worked. Acid can't kill it but antibiotics finally did. In the end we left hospital the week before school started for Marlie, on the 23rd jan but weren't officially discharged til the 25th.

So we were lucky to save the central line as Harrison still has to undergo IVIG monthly - locally at Gosford Hospital. We are becoming almost part of the furniture there these days!!

Of course all of this and some added financial pressure saw Scott and I separate - which was my decision and we have been working hard to see if we can resolve things. Scott is trying very hard as I am - with much counselling on my part to resolve issues I have re Harrison's illness and my guilt over it all. Its amazing how when you are feeling down that you take it out on people closest to you. I'm not saying that I am an angel or Scott either, and we both have things to still work on individually, but we are working things out which is very positive.

Marlie started "big" school on 31st January and so it has been a bit of an effort getting her to and from school with no car - lucky mum moved closer so we often go there and get her before or after school - not such a far walk - which is ok for Harrison as long as I can keep him out of the sun due to the Bactrim antibiotics, or the rain - bad for his chestiness - he has a chesty rattle still since January but it hasn't worsened luckily.

In January they did a lot of testing and found he is about 60% of the way transplant wise but the B cells are very slow to respond ( the importance of B cell s is to make the immunity) And the toxic metabolites went back up a little - which is not so bad but if things don't drastically pick up in April then we are still not out of the woods yet for a second transplant.

Also Harrison has epilepsy - two types actually which is hampering his efforts in development a little ( not much but still...) He has ATONIC seizures - he loses complete muscle tone and literally drops to the floor. This last all of a second and he gets up and keeps going. I thought it was just his funny crawling style for a while but then we had a paediatrician review and he asked about it. Needless to say that when he stared on Tegretol for this it stopped happening and he crawls quite normally now. With the slight exception of hitting his head on walls and furniture - he doesn't look up very well so uses his head to let him know when he has reached a point of no return. not pretty as he ends up with quite a few lumps and bumps and bruises on his head - but he has not given himself any serious damage yet so I will keep an eye on him or just lock him in a padded room!! :)

February was reasonably uneventful - just lots of Chiro and Physio to help Harrison with his development. He carries his head tilted to the left - its very obvious and most of his photos show it to a degree, and all the treatment has helped a little but he is still got a ways to go with this.

He has also learned how to stand. He is getting quite good actually, although he tends to get up on his toes, wobble the hips ( Elvis style) and then fall to the floor very ungracefully) - But he just smiles ( or has a sook, then smiles) gets up and tries again.

The central line has stayed clear now since January and we have come to March. ... Ha ha next post for the March bit! Last lymphocyte count was 0.7 - up from 0.5 but no where near the 4.0 we'd like it to be!!

Testing, testing ... And Christmas in hospital.

Harrison as you now know has epilepsy, but in the investigations to find out if there is changes to Harrison's brain causing this epilepsy, the doctors did an MRI, which showed a lump at the base of Harrison's head, so then a CT scan was done to determine if the lump was on the brain or on the skull.

As it turns out, it is on the skull. So Dr Webster felt it would be wise to follow on with a bone scan (I think just to be safe) but said not too urgently. All this in the last three weeks, and the bone scan (nuclear medicine scan) was brought forward suddenly and conducted Monday 17th December, to see what they could see. They saw plenty....

Now the lump there is visible, showing as a little "hot spot" where the dye they injected showed up. As to what this is we are still unsure, but the doctors feel it is not cancerous - but have said they still need to do more testing to be conclusive. In their words, "we're not out of the woods yet".

So we are next down at the Children's hospital on 16th January 2008.. OMG only really three weeks away, for a Bone Marrow Scan and for the long awaited bone marrow transplant review.

Only time will tell we guess, as to how the BMT is going and what this next scan tells.

In the mean time Harrison had intragam on Friday 21st December , and two hours after leaving the hospital from this we have been readmitted. Harrison has a central line infection - again. (This time not from chewing on the darn thing though). The line has been accessed by that many different people for that many different reasons over the last month that anything could have happened, and because these go straight into a main vein when they become infected the infection goes immediately into the blood stream. Now for this and Harrison's SCID - well simply it equals a major temperature, and this time a convulsion (which scared the living be-jesus out of me) , then a rushed emergency trip to the hospital for treatment. ...

We don't know why this time, but the bug is fairly resistant and he is on strong antibiotics to hopefully kill off the bug and save the central line. If the central line can't be cleared then we run the risk of having to have this one taken out and a new one inserted.. Which would mean another anaesthetic and hospital stay.. Although we are already in Gosford Hospital - Children's ward for a week of treatment now anyway so what's a few days added on for this? :)

So again we wait, and hopefully Harrison gets better, but I can honestly say this time he is really sick.. He is not the world's best sleeper, and pretty much all he is doing is sleeping now.

So the next blog will be all about Christmas .. I have gone back and added pictures to the last few blogs, and I have added some cool ones of Harrison having his testing done over the last week or two!!

Selling our car... Going, Going, GONE!!!!!!!!!!!!!!

Well the car is sold. It went on Saturday 22nd december.. So we are now officially carless. OMG - have no real idea what we will do without a car ( not that we go very many places) but the freedom of having a car has now gone! It was very sad watching my baby drive away, ut the family it has gone to are lovely so I wish my little AlQ all the best for his future!!







The time has come to sell our car, and so it has been advertised.

I thought I will add a picture or two of it here in case anyone out there reading Harrison's blog wants to buy a good car, at reasonable rates!! :)

Anyone interested tell them to call 0412403739 or email browntl75@optusnet.com.au

A n apple a day didn't keep the doctors away!

Harrison has had quite a lot of testing lately. Due to his being ADA deficient he had a 50 % chance of developing neurological problems - ie brain damage as the enzyme is not there to make the brain function properly. I thought we were looking like things were clear, however Harrison has developed epilepsy. Now I am not upset by the epilepsy part of things but that this means there is damage.

I cannot help now but worry what this means for his future. Harrison seems blissfully unaware or uncaring even. so that helps us stay focussed on the good things happening. but as always we will keep you all posted!

So we headed back to Westmead and have had in the last two weeks, another MRI, Lumbar Punchure, EEG x 3 and a CT scan. The doctors have found something on the CT and MRI at the back of his head. and so they are now wanting to conduct a bone scan. I hope they find nothing but each time he's had a test lately Harrison has then had to undergo another
. Time will tell. It feels a bit like a blow in the guts though - as we seemed to be cruising along and now this. I guess this will be us for a while.

Harrison is now 9 months old. He is almost sitting, and is up on all fours trying to crawl - but usually manages only a backwards push getting stuck against the lounge, or under the coffee table. I spend a lot of my day "rescuing " him. But we are so happy with this progress. He is 8.7 kg and wearing size 0 so he's feeding well.. Although he's not keen on ANYTHING that is not fruit based or custard or sickly sweet oatmeal. Or lasagne which I have to make specially for him to ensure its dairy free - His bum is smelly enough without adding the dairy intolerance to it!! Lol

Ella is in for her tonsils to come out next week.. They are huge she talks funny all the time now and we cannot afford Harrison to get sick. He is still immune deficient and the bone marrow is taking its sweet time to kick in. In January on the 16th we find out how this is really going.
I am sorry for the lack of pictures, these will be updated and added shortly.

Its getting close to Christmas. For the first time we will not have a santa photo with ALL kids in it .. Lucky for me I am getting good at photo shop!!

But we've done our best to make the house look Christmassie. I met a baby in Singapore called Ella - she's doing well but will be in hospital for the holidays.

Anyway Harrison is going great guns barring a few minor technical difficulties!! He's really developed into this happy kid.. Truly one of ours. And he's by far and away the cheekiest one of the lot. Gawd help me when he's three - cause he already tells me in baby talk what he wants now. & I am sure he's mimicking everything I say - no teeth though so he's still got this really gorgeous gummy grin.


Below is a link to another scid kid. Boy called Logan. He's a little cutie. :)



http://littlelogansblog.blogspot.com/

Fundraising

Well we have finished this years fundraising and I have pictures galore to attach -

Firstly the raffles & grandfinal day - we have many friends & family who have blessed us with their help and support. Donna arranged t-shirts for these fundraisers, and they are cute - we have below Lynne & Gabrielle modelling them at a raffle day ....

The t-shirt has a picture of a teddy bear with writing that says - I am helping HARRISON DRAPER with his fight against ADA-SCID.

The raffles were quite a success - and the Grandfinal day raised $30,000 in profit - not a bad days work really!!

The golf day was next on Monday 8th October raising about $10,000 and this will likely become an annual event.Brett Kenny was the guest speaker and Doug Keen of the NRL development MC'd. We were lucky enough to have local business sponsorship for this day..We had about 100 players with a buffet lunch following the golfing.





Finally the benefit night at Gosford Racecourse. We had a lovely view of the floodlit racecourse... 170 guests, 3 course meal, flowing beer & wine and a great night.
Guest speaker Stuart Dickinson of the Australian Rugby Union - he is a ref, and recently is back from the World Cup. Doug Keen MC'd again and was also part auctioneer with Ashley Knight a local real estate agent.

David Proust did a fantastic comedy act warming up the crowd for the auctions.
Rather than uploading hundreds of pics though I did a video of the night.....


We have truly been blessed by the help & support of friends, family and business alike and without this support our success in these events would not have happened.

THANK YOU...... :)
next post will be an update on Harrison's bone marrow transplant progress - we are just waiting on some test results.

Autopilot

Its been a while between posts. I've been living life on the freeway in autopilot mode. I think the car drives itself to the hospital now - and I can do it sleeping - or in a major traffic jam when trucks collide with cars and you have to leave 2 hours earlier than expected, just to get there in time!!

Harrison has had quite a few visits etc to the hospital. So in order to bring things up to date I will list what we've been up to - then I will add pics next time!

Events: Golf Day on 8th October was a great success - Brett Kenny was the guest speaker for the day. Lots of pics from this to add later

Benefit Night at Gosford Racecourse - 10th November was a massive night. I got to go to this one too - and I can honestly say we had a fantastic night. We had Stuart Dickinson from the ARU Referee's guest speak, a fantastic guy called David Proust do a comedy act and Doug Keen from the Rugby League - Referee's development officer as MC. Ashley Knight a local real estate agent helped with the auction. Also lots of pics to come!!

Hospital stuff:
Last Intragam was 26th October - next one due 23rd November at Gosford Hospital - which will also be the next set of blood tests to see how things r going.

*31st October - Operation on abscess at Westmead Children's hospital - was ok but they could not find where the abscess started so it may reform - so far so good though - it seems to be healing .
*6th November - Eye Dr Rowe review. Retinitis is now confirmed with a minor change in the retina since Dr Rowe saw Harrison on the ward in July. He is short sighted so will need glasses at some stage, and the nystagmus continues but is intermittent. Review now set for 9 months time unless he has a dramatic change before then.
*9th November - Hearing test @ Westmead under sedation. Harrison did ok with this but they couldn't test full range. No change with some hearing loss just outside normal ranges , so hopefully this won't change anymore and he'll go on to lead a pretty normal life hearing wise! 3 monthly reviews will continue though to be sure!! ( Did that sound Irish? - Sorry!!! )
*13th November - review from surgery - all ok & seems to be healing - only need to see Dr Harvey again if things don't heal properly.
*16th November - review with BMT (Bone Marrow Transplant) team - I am excited about this one - definitely want to know how things R tracking.. Will keep you all posted too.. :)

So things are steady at the moment - we are watching his ears as they are both a little red, so hopefully no infection occurs. & I can have a little rest from planning events. Although I had such a good time the other night I sort of can't wait for it to happen again! (Next year anyway!! :) )

Harrison has also reached a milestone - He is now rolling from back to front ( still one way though) and pushing himself backwards. The beginning stages of crawling - which I am very impressed by and no doubt he will continue. I am watching carefully and working him hard to keep his left side moving as he is a not using his left arm or hand as much as the right - but so far so good.

And finally - Ella had chicken pox. And luckily for us Harrison has shown no sign of contracting this.. Now don't get too excited though, as it is only through the miracle of Intragam (plasma product with antibodies) that Harrison stayed well - as this would have contained chicken pox antibodies - and he had the last dose of that the week before Ella broke out. So we are very lucky that this happened when it did and Harrison ( and Marlie & Sterling too) stayed well.

Progress - slow but steady wins the race.

My recent posts have talked about Harrison's blood test results

I asked the Dr's to clarify what these are, and what this means in terms of a normal person.

So... Lymphocytes - a healthy 7 month old baby would have a cell count of 2.0 lymphocytes (or 2000 per ml3 (milli litre cubed) of blood. This would be the lower end of normal blood count.

Harrison's recent lymphocyte counts were 0.5 - or 500 cells per ml3 blood.

Also the chimerism (ky-mer-ism) test done to determine Harrison's bone marrow transplant function showed that t-cells were 45% female( Ella's cells) and the B-cells were at 22%.

So the bone marrow is starting to take over from Harrison's cells - but has a ways to go yet and unfortunately no great improvement in the lymphocyte counts etc.

Having said that, Harrison's count did climb a little from 0.2 to 0.5 (or 200 cells to 500 cells) so that's something.

Also the Intragam ( plasma product that has some antibody properites) has really helped too. His levels are staying up pretty well at normal levels with each months transfusion, which is such a great thing. Although it doesn't stop him getting sick, it does mean that he can have the best fighting chance at keeping well. To anyone again who reads this and donates blood , THANK YOU. It is a great thing we live in the age we do - and that the wonderful donations made can keep our baby so well.

I am so proud of Harrison and Ella - and the achievement being made with Ella's bone marrow. Of course, we are not out of the woods yet (so to speak) Harrison still has a long way to go before he is normal and can join the outside world. It is reall y hard at the moment cause he seems so well. And he is leanring to do things really well, but I worry daily that he is going to be receovered, and I worry that whole"what if the bone marrow doesn't make things right? What then?" Its a very uncertain time for us, and as summer encroaches I would love to take all of the kids to the beach and swimming - but it is still not safe for Harrison to go out yet - and so we stay home.

Also very proud of him that he makes progress every single day . As I previously noted in my last blog that he babbles now - a great achievement in itself - and today he did a new thing again (Previously he learned how to roll one way) but ... as the newspaper people arrived here doing a story about Harrison for the benefit night, he suddenly rolled the other way for the first time! What a star is my little man? I am trying to capture footage of it - so taht you can all see it - but of course most of the time I get the after shot!! Lol

Also a great deal of thanks have to go out to our friends - for all the calling and emailing to make sure we are ok, and keeping us sane when the isolation would have otherwise driven us nuts! You know who you are - and you are worth your weight in gold.

Another day another doctor

Another week has come with two hospital visits this week.

The first was tues23rd Oct - to see the surgeon about the abscess on Harrison's bottom. Harrison has had this abscess since 5 weeks of age. He is now 7 and a half months old. It keeps closing over, refilling, bursting and then closing over again. It is often painful to him for me to touch it - as he mostly screams if I do.

After the bout of hospitalisation with his central line infection adn the 4 medicines he got for that the abscess seemed to be healing. However I noticed Monday that the little heads are forming again and his bottom is quite red again. Dr Harvey took a look and has decided to lay it open. I am worried what they will find when it is done, particularly if it is something more than just the surface abscess they seem to think it is.

Further, Harrison's lack of immune system can lead to serious infection - as the operation will leave a large open raw wound area right next to his anus. Not a great spot at the best of times, let alone for a kid with Harrison's immunity issues. He healed ok when they did the surgery to place his central line. But that was a 1 cm cut and not a great deal of open wound. I am frightened this will not be so successful, but the surgeon (who has delayed this surgery now 4 times) feels it is a calculated risk but worth doing now.

So this Friday we go to Gosford hospital for IVIG (Intragam - antibodies transfusion) again & then next Wednesday 31st October we go into hospital again for the surgery.

Tehn as I think I've said on the 6th we have the eye test review - which I am also worried about and then 9th hearing tests.

On a plus Harrison is rolling from side to tummy with assistance and he is babbling dada - why is it that dad always gets the first "word" - Ho Hum!

I will add a picture of him in the next post. Kids are all screaming at each other and I am close to having a melt down. Its been a very stressful week - one that I don't see getting any less stressful. ( That and we haven't yet had as many ticket sales to hte benefit night, and that is starting to worry me too. )

Thank you to all of you for reading this site. I love to see that we get lots of returning and new visitors alike.

Hospital visit number...

I have actually lost count already as to how many visits to hospital Harrison has had. Wednesday was a check with the immunology team who a happy with the weight and height he is. Even though to me he seems small in comparison to Marlie, Sterling & Ella, Harrison is holding his own.

The formula concentration has been dropped back to normal, he is now eating solids really well - But I tell you it was hard yards getting him to eat them at all.

Dairy is a no go zone for little H, and his eyes and ears, well I'll get to that in a minute.

Once we saw the immunology team, Where Harrison was given the royal treatment - Sam ( Dr Sam Mehr for the uninitiated) is my main Dr contact and he is always up for a cuddle. But then he called Dr Alyson Kakakios ( Alyson) a cuddle nazi - which I thought very funny cause he likes cuddles just as much as Alyson.. But hey she has a nickname now that she'll never live down!

It makes for a fun visit when we see the Immunology team.

Then a nearly 4 hour wait to do the eye check. They gave him some eye drops and we sat and waited in a dark room for 20 minutes once he had them in to get him "dark sensitised". Marlie was with me for the day and she was a little unnerved by being awake and in the dark for so long. The staff gave her this little pen torch with a red cap on it and she was happily shining red light everywhere.

Harrison's eyes did what the team expected with Retinitis - didn't respond all that well to the light impluses. Then they left him for 5 minutes with lights on and repeated all the tests. To me it didn't seem like there was a very big difference in the dark vs light testing.. In fact the lines that they look at seemed pretty flat ( not much up and down range from the horizontal line)

So I guess he has retinitis confirmed from this but we'll know more on the 6th November. ( Another hpspital visit)

So I now have 23rd Oct - Dr Harvey for the check on his abscess. Since he was hospitalised for the central line infection, Harrison's bum seems to have cleared up? Will know that for sure next week too. Then 26th October for Intragam AGAIN!! then 6th November eye Dr appointment to discuss the results and further testing then 9th November for the hearing test under sedation . 4 appointments, in three weeks is pretty good going and all bar one at Westmead. Lucky the car drives down there on autopilot now.

Hopefully we'll still have a car then? Trying to sell it so that we don't have to pay for a lease with the money we currently bleed from a stone! I am now, you see, on unpaid leave so MONEY is tight. Very tight, but then you don't need to be a rocket scientist to work out that we were gonna get tight on one income when we were used to living on two.

Anyway will keep you posted (LOL) as to how things r going with Harrison. The physio was happy with him the other day - so that is a plus.

benefit night flyer

I thought I should add this flyer for better benefit night info - Click on the picture to open up a larger version for printing and distribution. Plus it's a record then of the flyer and the work we put into Harrison's fundraising efforts.

Golf Day

We had a really good golf day for Harrison on 8th October. I wanted to post pics from this.

To hospital again & support

Harrison has again been to hospital. We went on Thursday a week ago, then came home the following Tuesday - but went back daily to hospital for IV antibiotics. He got a serious infection in both the lumens (the lines that you use to get bloods etc from) in his central line. So he's had 5 days of antibiotics in hospital and we got to come home - only to have to go back for two more days of IV but hey that is so much better than living at the hospital again :) They did his next lot of intragam, so he's all IG'd up for another month - but even so I see delays in development now. He seems to have stagnated in his progress.

As always the support of our family and friends has been sensational - we are truly blessed. I am also very lucky to have found a wonderful forum for PID on the web in Essential Baby. There are a few mums who chat about their lives with Primary Immune Deficiency. I am very lucky to have this sort of support to. Cause not only can we discuss our sick children, but about life in general - and this keeps me sane.

We are both (Scott & I) stressed as I am now on unpaid leave - and the future is so uncertain. I just have to hope for the best. But I am making the calls this week to the banks and mortgage and hopefully we'll get some sort of relief. I am also looking for some night work to help make ends meet. What a world.

Harrison is doing ok though otherwise and he's booked in now for a physio assessment next week to see his progress and what it is I have to do with him to help him along. He has such poor arm and tummy muscle strength. But he tries to sit up and even though he's wobbly he tries which is good enough for me.

I popped a pic of him recently wedged into the corner of the lounge. As you can see his head drops to one side and he doesn't sit "up" but he tries.

We are also meant to be writing up all the thank you's from the grandfinal day and Golf day, but I find this task increasingly overwhelming. So many to do but just not enough time in the day. I hope that those that have helped and supported us know that it's late only because I have 4 children to look after, a million calls and emails to follow up on each day and try to sort out a way to keep our house and car. But if you happen to read this site and are one of those people that have donated gifts, cash, time or support then please hear me when I say thank you. It is only with such generous support that we can help Harrison get the best possible care and treatment he deserves.

GENETIC TESTING IS BACK !

Genetic testing came back from America yesterday too. This is different to the enzyme test previously done.

This test was to see if they could find the specific ADA genetic mutation. Sometimes they cannot find it or it has not been reported before . In our case a few other cases have been reported with our same mutation.

Now for those who wish they were doctors and or who understand genetics, I have included below the doctors email re this. From my research into the genetics part of it I have identified four other children ( Worldwide) that carry the exact same genetic coding we have for ADA deficiency. Pretty scary stuff - although I cannot say for sure that these would be the only 4 others,but it just shows how rare this thing is. We knew that anyway given Harrison being only the fourth child diagnosed in Australia with ADA deficiency in the last 10 or 15 years, but this sort of confirms it for me.

The number 4 seems to be a common theme here - Harrison is my 4th Child, he is the 4th diagnosed in Australia with ADA-SCID, and only 4 others I can find in the world with the same genetic mutation, and according to statistics we have a 1 in 4 chance of having a child with ADA deficiency. Very Scary Stuff!!!

I am going to ask for a more detailed breakdown of what this means exactly when we visit the hospital next week - and of course Google myself to death to find answers to my questions that I can then convert the below to normal speak - for translation to you all. :)

Email detail from Doctors: "Harrison (patient) is homozygous for the A329V mutation in exon 11 (c.986C>T); both of his parents and his sister Ella are carriers for this mutation.

A329V has occurred in a number of ADA deficient patients with SCID,. When expressed in E. coli, human ADA cDNA carrying A329V has about 0.05% of ADA activity expressed by the wild type cDNA (Arredondo Vega et al AmJ Hum Genet 63:1049, 1998). "

An Exon is a very small part of a gene. It is the part that holds the particular coding if you like for your genetic makeup. They are numbered and so that is how the genetics Doctors can identify where a particular trait comes from – like this ADA gene. So I think it goes that - in Harrison’s case on chromosome 20 is where the ADA gene lives. The mutation that causes the ADA deficiency appears on Exon 11 within that ADA gene. Because of this mutation, (which both Scott I carry), if a child is born who gets both sets of these mutated genes, then they have the disorder. Harrison is this child. Luckily our only child AFFECTED. Ella carries the disorder ( and so only got 1 set of the mutation and the other set she got is OK) and it looks as if Marlie & Sterling are ok and probably not carriers.

It is known as autosomal recessive genetic disorder. It is a genetic trait we both carry - with no prior family history on either side, so we are just unlucky that Harrison has this - but ... We as a family are very lucky that all kids aren’t affected – as we have a 1 in 4 chance of having a child with ADA deficiency, the way genetics really works means that ALL of the kids could have been affected. We just got lucky. Like we got lucky that Harrison and Ella were a perfect match for Bone Marrow.

I have since found out that 80% of patients who need a bone marrow transplant don't end up getting one from a sibling. So we are truly very lucky indeed.

The World According to Marlie

I thought I would re-introduce you to my 5 year old, Marlie.

She came up with a whole new baby theory yesterday - and it is well worth sharing :)

Marlie told me and I quote: "You know when you go out for dinner, and that's when you get big and fat and pregnant."

All I could think was GAWD HELP ME!! If that is how easy it is I am in trouble !! ha ha - Scott & I went to dinner when Harrison got home?

I just couldn't help adding this - ah the mind of a 5 year old. So uncomplicated :)

I have added footage of Marlie & Sterling dancing to "That's Gold" - she is unique - ballet dancing at that!

Harrison's Development

Well last week Harrison's eyes started to act up again. And I saw a friends baby who is younger and doing probably the same things as Harrison. So I was a little depressed - cause its that next step - seeing the reality of how Harrison is not as far developed as he should be for his age. I am none the less very proud of my little man. He is very cheeky, Many Many smiles and coos and gas - however this aspect is not so quite and cute - rather more loud and demanding - like HELLOOOOOO Look at me!! He was never destined to be a wall flower though, that and we have the loudest other 3 children too - so he will definitely be loud anyway - bad hearing or not!! Lol

So I popped a pic of Harrison trying to sit up - He is clever enough now that he wriggles down the bouncer gets his bum on the floor and then wriggles enough that he can almost sit up. Not very well - cause literally after this picture was taken he slide down to the left side of the bouncer, and then had a wriggle on the floor, but he is doing it! I am happy that he does this much.

Also he has worked out how to roll from one side to the other . Mostly it is from lifting up his legs and his chubby tummy helps the whole rolling thing happen, but he is trying - and sometimes ends up doing a little circle work and moving to a slightly different section of his play mat.

What a little star!! And to prove he is smiling - and chatting here is another pic. He most especially likes a chat at 4 am .. Great for him - crap for me - cause by the time he finishes his conversation, feeds, has his bum changed and then gets back to bed its nearly 5am and I have only 1 more hour's sleep then I get up to Marlie & Sterling ( the early risers of the family :) )

What else is news.. Well I'll update his weight in a couple days once he sees the pediatrician, then next week we go down for the next Intragam and the all important hearing test. It'll be good to have some answers as to how his hearing is.

Marlie sterling and Ella are really doing well now I am home ans a bit more settled, and they are listening to me (Well most of the time) when they are asked to do something - like cleaning away toys :) I am proud of them though as they really have had to take a back seat to Harrison - and have done it with very little complaint.

Footy Show- That's Gold! segment on Harrison

We had a segment on Nine's Footy Show about Harrison - I am trying to get a copy to load onto this site - however, I can tell you they did a lovely job - It may even still be on their website.

Now I know the quality isn't the best - and we are getting better copies done - but for those who missed it go to the following link if you 'd like to see it...

That's Gold Grand final segment

What a wonderful job those guys do and we were very privileged to be one of the selected families.

It's still hard to believe we are a family that need help. But help we do need, and the footy show certainly did this!

A big hug to The Chief and to the key organiser for this segment Danny Keen. And to nine's footy show & NIB.

Harrison's Eyes

Harrison's eyes have gone funny again. This is what they (the Doctors) call nystagmus and it is getting worse again. If you try to do what he does, you will find it most disorienting, and he really does struggle with it from time to time. But he is still soooo cute - so I thought I would start to share some video of him. I hope you can all see it ok. (And yes I am VERY biased as to just how cute he is - but I really don't care!!!

Eels Pictures - Finally !! Us and Harrison with the Eels players :)

I finally got the Eels Pictures downloaded - There they all are ..

This is the photo we are using for the fundraising. It reminds me every time I see it just how sick he was then and how careful we have to be not to get back to that. I also see how far he has come. He was sitting on my chest today while I laid on my back, and he was looking at me while I blew raspberries on his feet and then he'd crack up laughing. Such a gorgeous kid. How lucky I am to have him. And Scott has become so much more comfortable picking him up now too. He'll not worry so much about the central line anymore and grab him for a hug. I even got out a couple of times the last week - on my birthday no less!!

I am back off to hospital with Harrison this week. IVIG time again, and see the "cast of thousands" as I like to call it , when we get there. Harrison is doing pretty well, although I cannot keep food up to him , and seriously for the amount the little man has he should be at least double the size he is.

I started solids with him this week. Which makes no difference - except to the output of poos. They have increased too!!

I sat helping last weekend with our first fundraising raffle at the football. So many people there with babies in prams, and most of them bigger than Harrison yet half his age. I really thought he was holding his own in terms of looking his age, but I guess I am used to seeing what Harrison looks like now. He is still so little - my tiny man!! :)

But it makes me wonder at how much I took for granted before with the other kids. I put them in the pram walked around the streets and shops with them and never thought twice about it. Now it is careful planning or make a call to someone to get me something I am in short supply of. I cannot wait to be able to just go for a walk with Harrison in the pram and not worry that someone will sneeze on him and make him sick again.

and even though he has 10% of his cells showing as female cells there is no signs of immune function. He has been putting weight on at the expense of me feeding him 2 and a half hourly and it breaks my heart that I cannot take him with me every where I go - I hate leaving him behind. I also have found how bad I am at the moment emotionally. I find talking to strangers about him hard, and I really worry how he'll be long term. Those test results from the US have still got me in a knot. Luckily we do go back to hospital this week - I have a list of questions to ask the doctors when I get there.

Harrison will be six months old soon - and he is a little behind in his development. Being home may be driving me nuts - but I am thankful I am home. I have said bugger off to the housework - eventually the clothes will get put away - but for now I am enjoying the kids, and their new things they do - and them with Harrison, although they still want to pick him up, at least he is holding his head just slightly better which is enough that I can feel more comfy with them "having a turn" with Harrison ( or for those un-initiated with this "having a turn" it means having a cuddle). Such a great fight solver that one. All I say is who wants a turn of Harrison - Fighting all stops - well at least about the toy they were fighting over, because then its a tackle-a-thon to the lounge to see who gets a "turn" first!

Progress

I thought I'd add a cute pic of the kids - they have been so good this week, even with me being a cranky pants!!

Well this has been an interesting week. We saw the pediatrician on Monday, then the community nurse on Wednesday, by Thursday Harrison had developed a cold.
So he is ok, but has a runny nose and a cough. I called Westmead Childrens and spoke to Sam, (Dr Sam Mehr ) and they are happy to watch Harrison, but any development at all and I call back down to them and discuss.

As I said in the last blog Harrison now has a 10% count of Female cells which should mean he has something there that may fight off this virus he has. He already has Bactrim antibiotic which he takes daily to ward off any chest infections. So we will see how things go with this.

I also changed Harrison's central line dressing. I did it last week too - while it did the job it looked all wrinkly. This week- it looks so much better, I felt like a real pro!!!

Here's a pic of that too...

We also got told about some of the enzyme tests coming back from the USA that were done in line with the genetic testing they are doing for us too. This enzyme count confirmed what we already thought. Scott & I carry the rotten ADA gene!! Ella is a carrier (and so given Sterling's low counts of ADA enzyme he would be too) - but Marlie is not - her count is 1.6 aka perfectly normal and Harrison is ADA deficient.
They also tested the toxicity levels of Harrison, which is an indicator of sorts for how badly affected these kids are. Harrison's toxic count was quite high, and as much of the literature I have read indicates- it is these kids that then go on to have the neurological problems. So we knew this already with the eye problems and hearing loss, but this is a bit more of a complication - we will now have to wait and see exactly what Harrison has in stall for us!!

The cold will certainly give us a clue if things are moving though, as we hope he'll be able to fight it off. ( and we hope that its not some bacterial infection just starting!! )

Anyway that's about it this week. That and I had a chat to John Laws about Harrison praising the hospitals and staff that gave us such incredible care, and the difficulty we find ourselves in without much help from Centrelink. I was very nervous talking to him but I think it helped, and if in the long run it benefits Harrison then I would do it again in a heart beat.

Ciao

Reality

Forgive the dark nature of this post. I am struggling with life this week. The pressures of being at home full time are starting to mount. The only bright point in all of this is that Harrison's cell count at 30 days post transplant showed signs of female cells - about 10 % of his blood count in fact. This is pretty damn good, and means that the bone marrow is grafting- now we wait to see exactly how well this is.

The reality of life has hit home. Yesterday I went to see the pediatrician. Dr Buckmaster - a god in his own right, as far as I am concerned. He did such an amazing job with caring for Harrison and getting him transferred to Westmead. I really feel that he saved Harrison's life. I was really emotional about seeing him. At the end of my appointment with him I told him thank you - for all you did in saving Harrison's life and being so thorough, because he wouldn't be here now if you hadn't done what you did. I then cried all the way home. You see, Dr Buckmaster was quite real with me - and we talked about Occupational therapy and physiotherapy and setting up the things Harrison will need. Harrison's eyes are also doing a new side to side movement as well as the up and down stuff too now. I cry because I don't know why this is happening, and if this is going to be a serious problem in the future. I cry because at the moment life seems a little hopeless. I cry for the life I once had, without the massive burden we now face financially.

But it is not just that that I am emotional about. Giving up work is hard, not that I value work over the kids, but that work is my time, selfish - yes, but I value the time and space away from the kids, house and Scott to just be me. I miss not being out there and earning my income. Work has always been my tool for coping with life.

Whenever I got stressed out or had a problem - I'd work. Now that avenue is no longer an option. I have had to give up work, purely to care for Harrison. It seems strange that I should miss this so much, and it pales in comparison to being with my son but it was my outlet. That and I feel that I'm not contributing as much financially now, which I have always been able to do.

Scott's income alone is not enough to support us and when my maternity leave entitlements are done, well that's just it. No more help. Harrison has a few problems, yet he is not considered to be sick enough that we get Carer's Payment. However- he can't leave the house, has multiple little side issues and we have no other means of an income.

Further adding to my depressed state is the fact that we will have to sell the car. This puts us out of pocket just another couple of thousand dollars, that we also don't have - and leaves us without a car. They joy of the novated lease was great - while I was still working. No income means no money to pay off a car. But what do we do? And I am concerned that the fundraising that is happening won't quite be enough to cover Harrison's medical bills as time goes on.

I am so scared about the future. I don't want to lose our family home, but if interest rates keep rising and we have only 1 income we may well lose the house. No house, no car and a sick child.

It really sucks that if a person sticks a needle in their arm - the government bend over backwards to help them out, but those in genuine need? Not a hope.

Then add my stupid sister to this already difficult equation. She decides to run off the rails now. Why, probably cause the focus of attention is no longer on her. So instead of stepping up to the plate, she stuffs things up completely. And I feel guilty because Mum, while she is here helping me, then has Hope to deal with. Mum does so much for us - practically putting her life on hold to be here helping us, and I really love her being here and helping, yet I feel that I would happily throttle my sister right now for giving Mum so much extra stress. So instead of falling apart - like I truly want to do at the moment, I don't - Because Mum has enough to deal with right now.

There are days, where I wish that I could also choose my family as I can choose my friends. It would soooo make life much , much more simple.

Making Friends

We have been put in contact with another family in Queensland (QLD). The IDF of Australia are a foundation for parents and families to have contact with like families suffering the same disorders. Our Harrison as you know has ADA-SCID, while Harrison from QLD has 'IL7 Alpha Receptor Deficiency'. Another form of SCID and also a genetic inheritance.

We were lucky enough to meet and I have had the absolute pleasure of speaking to Tammy, Harrison's mum (Harrison is pictured left this is from around the time when he was diagnosed at around 11-12 months old. ) Strangely yes, he is a Harrison too.. and it gets even more coincidental that QLD Harrison's dad (Ivan) works for a telecommunications company as does Scott.

Our little friend left has had a BMT also, and was so much sicker than our Harrison, and really showed even on his sickest days strength and courage. He is smiling in just about every photo Tammy has sent me. I thought it would be great to put them on this site as encouragement. You see, this little boy went through an amazing time, and was critically ill for a time, but has pulled through remarkably well. He is now a 2 year old boy, who looks like any other, attending preschool too!! I am so excited by this - It gives me hope that our Harrison will do just as well.

To show you how well he is doing...















He's a picture of health here, playing and with his brothers.

What an inspiration and reminder what a lot of positivity can do to help along a situation.

I find it so comforting to have another family to speak to who have been through more than we have, but also through so much of the same experiences. What a lovely and amazing world we live in.

I look forward to a long and happy new friendship with little Harrison and his family and I wish them all the very best for the future.

Many thanks to Tammy who allowed me to pop a picture or two of Harrison on this site, such a valuable thing to do!!

Home... Sweet, Sweet, Home!!!

Home.. what a lovely place to be. The kids have been so well behaved and were so excited to see Harrison and myself as they walked through the door.

Harrison is very much not used to the chaos that goes with 3 children running around and making noise, but he is settling well. So far so good, no sniffles or disasters occurred in our first weekend. Harrison has taken to our old net bouncer, and I have a head roll under his head and shoulders to keep them forward so he is reaching in front of him. He is very happy to sit and kick, and has quickly learned to watch TV here when he's grumpy. As usual the Wiggles feature heavily in his TV viewing!! I still can't believe how little he is even now.

We had a couple of articles published this week to do with Harrison and his disorder. The local newspaper - The Express Advocate, and in the Saturday edition of the Sydney Morning Herald. The herald link is below ..
"A Rocky Start - but Harrison's Off Home "

It a lovely article and the picture is gorgeous.. I will eventually scan in teh picture for the site too.

There's no place like home ...

Well.. we didn't click our ruby slippers together, but we are home.

The amazing nursing staff... What does one say ? We were gifted with a bunch of amazing and talented people. Our small gifts and words of thanks will never be enough to really say thank you for all that was done for Harrison and our family.

It has been a very trying journey and this photo is representative of how far Harrison has come, having a picture near the nursing station with no gloves, gowns or masks in sight.

The Children's Hospital at Westmead have outstanding staff and facilities and we were just damn lucky to have been in the right place.

Home time was a very emotional time, Scott and I got very emotional saying our thanks, and I burst into tears after exiting to the hallway from the ward. 10 weeks of worry, stress and sleepless nights filled with questions swelled to overflowing and came out as tears. I remember saying I can't believe we are actually going home, and I am still as emotional today as I was the day we left. We are taking Harrison home, and there was a time where I thought that it may never happen.

Strange what you get used to - I became reliant on the nursing staff and have to say I am worried at being home and keeping Harrison germ free. But I know I have great people that I can call at a moments notice and ask.

The house looked so different. I felt like I was stepping for the first time into a new home again. Everything was so clean and tidy, not the house I remember leaving !!

Scott put up the ISOLATION sign at our front door. It's funny watching people who walk by read the sign, like we are in quarantine. But it's still so true, cause a bug will make him sick again and so we keep telling the kids to wash their hands, not go too near Harrison and cover their mouths if they cough, just in case.

The weekend will be different - I will be on my own with the kids most of the time, we'll see if I survive!!

When we came home there was so much stuff in our room , the car was full to the brim.. I still have to unpack all of it, scary how much you collect in 10 weeks!!

Reflections before home time

We are finally going home. I thought I'd do a little review...

Harrison before getting sick. 5 weeks old






Then Harrison gets sick and swells and needs oxygen.

Then he is off oxygen, still swollen and losing weight.

11-12 weeks old





IVIG and albumin help restore his system a little, skinny but looking a little better.

He learns to smile, he has the transplant and starts to gain weight.

16 - 17 weeks

Playing with the lumens of his central line .. his new in-built toy!! Its got the added advantage of keeping him still while weighing him :)

He develops new skills and enjoys tummy time. I think he forgot he is actually sick!!


20-21 weeks old



Little Peek- a - boo games start to appeal.. Plus he just looked damn cute there in his little bib!!

A Milestone is achieved. On 1/8/07 Harrison gets to leave his isolation room for the first time in 10 weeks. He is in his brand new pram that he used about 6 times pre-hospitalisation and it has a funky rain cover over it to keep him protected, but hey its freedom..

We had lots of photos today, with a video crew who came in for the hospital and captured this first milestone of leaving the room.


Then the local newspaper captured his image for a Jeans for Genes Day article to be published on 3rd August.

Then I took lots of photos and thought it would be lovely to include our wonderful "Ward Granny", Lyn - who looked after Harrison and showered him with love and attention.