The Birthday Boy (Day +472)

We had a very exciting day yesterday celebrating Max's 4th birthday. Considering this time last year we celebrated this occasion in hospital, it is another happy reminder of just how far he has come in the last twelve months.

Here are some brief points of his medical issues this past month:

Weight

Max's appointment with Dr KT in early June brought with it the unfortunate observation that he had lost a little weight. It has been a constant struggle over the past year to get him to put weight on, so to find out he had gone backwards since the last visit was a concern. He was ordered to come back to hospital two weeks later where thankfully, like a reverse weight watchers meeting, the scales showed that he had gone back up a little bit. Hopefully the weight loss was just a temporary set back, possibly triggered by the minor cold he had suffered the week prior. 

Ears

It was also observed that Max had an over abundant supply of wax in his ears, so Dr KT ordered it to be, literally, vacuumed out. The ENT (Ear Nose & Throat) registrar was very impressed with how tolerant Max was during the 'clean up' procedure. Under the mountain of wax a small infection was noted, so drops were prescribed to be administered in both ears three times a day for two weeks (a very time consuming exercise). We were pleased to find at the follow up ENT visit that Max's ears remained clear and that the infection had resolved. The ENT registrar also thought that as Max's ears were so clear, it was a good time to have his post-chemo hearing check done. Max went to have his audiology test done on Tuesday, which he passed with flying colours.

Eyes

Bianca mentioned to Dr KT that Max often rubbed his eyes when irritated, to the point were he would flip his eyelids inside out. She asked Bianca whether Max had tears when he cried to which Bianca replied 'No'. To help bring some relief to his dry eyes, Max has been prescribed eye drops, which should be inserted three times a day. However this proves to be impossible. Despite his braveness in handling a variety of treatments to all parts of his body over the journey, Max simply cannot stand having eye drops put in his eyes.

Skin

Max's skin continues to show little signs of improving. Dr KT is currently working through the paperwork to get Max onto a new medication that according to a small study has had some success in treating GVHD. We have just realised that we have mentioned this drug before in the February blog but it wasn't pursued any further as, at that point we all thought his skin may have got better on its own. We should know by the next visit whether this application has been successful.

May milestone madness (Day +442)

In the nick of time we've managed to get a May blog out the door. It has been a pretty eventful month with Max passing many milestones. Health-wise, his skin is still not flash (blotchy, thickened, flakey and still no nails) and his scalp is quite crusty. He was knocked around a little last week by a cold but he seems to be getting on top of that so that's a good sign. Max is still the master procrastinator at mealtimes, making up all sorts of excuses as to why he can't eat. However, that said, when he does enjoy a particular meal...there's no stopping him. Current favourites are spaghetti bolognaise, frankfurts and sausage rolls!

Bolognaise with the mystery hiding vegetables!

As for milestones reached:

No more masks required when visiting hospital! That's right, Max is no longer required to wear a mask in the fashion of a Japanese public transport commuter (for the blogspotters out there that reference is like all my jokes: recycled) when visiting hospital. Not that we had too many troubles getting him to wear it, but is nice not to have to worry about it.

No more nappies! Max has at last levelled up to being a fully fledged member of the nappy free kids crew. For a while now Max, despite being toilet trained still needed to wear nappies at night. After around a month of wee free nappies, Bee made the decision for Max to go to bed sans nappy. And he hasn't looked back.

No more steroids and no more Urso! Max has been on the steroid prednisolone ever since his transplant. Although the steroid hasn't turned him into a beefcake (that's a different type of steroid) they have been important in attempting to keep the peace between him and his new immune system, and have played a part in dampening the impact of the Graft versus Host disease affecting his skin. Unfortunately, like most medications, there are side effects and the steroids were responsible for his lack of growth, osteoporosis, spinal fractures and his cataracts. Max had been slowly weened down to a very low dose of the steroids. With much excitement, at the the last appointment with Dr KT, she said it was time to take the training wheels off and see how Max would handle life without the steroids. She also said we could drop the drug Urso (prescribed to lessen the risk of developing GVHD of the liver as Max was showing signs of impaired liver function) from his regime of medications.

Max is now down to 9 medicines (5 syringes and 4 tablets) in the morning,
1 tablet for lunch, and
8 medicines (5 syringes and 4 tablets) at night.

Re-vaccinations! When they wiped out Max's original immune system, prior to the bone marrow transplant, it meant that all his original vaccinations received since birth counted for nought. At some stage we knew that he would need to take them again but only when his new immune system had built up sufficiently. Well that time has finally come and Max this month received for the second time...Pneumococcal and Meningococcal C vaccines, along with an Influenza shot for good measure.

Relaxing of isolation restrictions! Dr KT has further relaxed Max's restrictions for interacting with the open world. Max celebrated by attending a couple of small birthday parties, a quiet christening, and giving his mum a hand at the local supermarket.

Emma's Christining

Our Stud at Griffins Birthday!

First time Shopping in over 1 year!

When not out enjoying a bit of the world, Max is quite content occupying his time at home with Mum doing beautiful artwork, drawings and painting!

Self Portrait

Upon asking Max what the big red splodge in this picture was, he replied "It's your big heart Mummy!"

Another portrait of Mum

and Painting...

Thank you to Paul and family for all our goodies!

Party tiiiiime! (for the most part) Excelleeeent! (Day +410)

Hello all,

It has been a while since we last updated the blog. Max has been quite well recently and continues to impress us with his feats of mental and physical dexterity. Since we last spoke, we have had many reasons to celebrate including the anniversary of Max's transplant, a special family birthday and, depending on your point of view either the holiest of christian holidays or a celebration of cheap chocolate, Easter.

The one year anniversary of Max's transplant was on the 15th of March. This was quite a momentous occasion for us and we partied accordingly. Oma, Mawa, Opa and Mapa all helped us celebrate the milestone with presents and a magnificent feast! Needless to say, Max got into the spirit of the evening and very much enjoyed being the centre of attention.

THE  'BIRTHDAY'  CAKE!

Excited 'birthday' boy

The Basket of Goodies

It's still a long way away but once Max has fully recovered, we are planning on having a massive party for all of his family and friends to celebrate his overcoming of Wiskott Aldrich syndrome. Stay tuned.

A couple of weeks later, Max had a particularly full on lip-blowing-up episode, which we managed to capture for posterity...  

Nice lips huh!?

Later that evening, as Tim had endured a big evening at the footy and had fallen asleep on the couch, I decided to let Max sleep in our bed...just incase...

As it turned out, I was so thankful that I had decided to do this, as during that evening I awoke to noise coming from our back room....as I went to investigate...I noticed the back door was wide open...and Tim still asleep on the couch! To cut a long story short Tim and I startled the (...insert rude derogatory terminology here...) from attempting to disconnect our computing equipment and they fled....leaving only with my laptop! The immediate concern for us was whether I had backed up my thesis...which thankfully I had!!! Police arrived within 10 minutes, sniffer dogs managed to track a scent to a few blocks away, and finger print officers did what they do best (but unfortunately were unable to detect any prints). Through all of this excitement, Max remained sound asleep in our bed!  

The next weekend we celebrated Auntie Lou Lou's birthday. It was also the first time that Max and his five cousins, including the twins, had all been together. It took a little while for Max to feel relaxed and comfortable with so many other 'little' people, but by the end of the day he was enjoying himself very much!

Cousins

And then a couple of weeks later it was Easter. The Easter Bunny came to visit Max, the difference this year of course is that he visited at home and not hospital. He (or was it a she???) pulled out all stops for Max, building an obstacle course with an Easter Car Hunt and the prize of a scrumptious Chocolate bunny at the finish line! Just how clever is that Easter Bunny?

The Easter Bunny trail....leading to surprise Cars...

Surprise Car number 3...

...and the big chocolate bunny!

Analyzing his surprises

Happy Boy!

When not partying or eating chocolate, Max has been spending his time:

Filling out activity books designed for children of much greater ages, which he thrives on!...

Jammin' with Dad...

Playing a lot of soccer and golf....

And watching a lot of television (Current Favs: Dora, Mickey Mouse Clubhouse and Big Bang Theory).

Medically there is nothing much to report. Max's skin is stable; it's not getting any better but it's not getting any worse either. He had another (overdue) infusion of biphosphanate to strengthen his bones. Apart from the stress involved with trying to get the canular in, the infusion went along well with no dramas. Hopefully Max will only need to have one more infusion (in about 4 months time), and then depending on his bone density results we can say sayonara to them.

Chillin' in the Day Oncology Suite!

His muscles are ever so slowly but surely gaining definition. His weight is still low, but rising nonetheless...he broke the 14kg mark at the last weigh in! He still has a way to go to match his pre-transplant weight of 18kgs. Unfortunately Max's 6 week stint of 'in-home' physiotherapy sessions came to an end a few weeks ago. But some great times were had and I now have excellent ideas put in place of how to keep up with Max's strengthening regime.

In Home Physio session

Tim and I managed a small getaway to the city for the night (including massages and dinner....courtesy of my wonderful group of friends - Thank you so much for your generous gifts). Tim and I had an absolutely wonderful time! We have also made the decision to renovate our humble abode so there are plenty of exciting times ahead.

You've come a long way baby!...(Day +365)

Just a short blog update to commemorate the delivery of Max's new immune system.

On this day last year Max received a cord blood transplant which cured him of his Wiskott Aldrich syndrome. I expect this day will be a special day for us for the rest of our lives....it really is another birthday for Max.

March 15th for his immune system.
June 29th for the rest of his body.

Max receiving those golden cord blood cells March 15th 2010

The cord blood...the donor to which we will be forever grateful

Avid followers of our blog will know of the many ups and downs and twists and turns we have endured this last year...although we are not out of the woods just yet, boy have we come a long way....

From the fear of Max having a brain heammorage if he was to bump his head.....
....to now rejoicing in the fact that his body can now actually produce a scab!!!


Ahhhh platelets....we do love thee!

Today!

A more detailed blog of this months happenings will follow in the next few weeks...

King of the Cubby House (Day +349)

Hello everybody,

Welcome to the better-late-than-never February edition of the Max Der Starke blog. You will be glad to hear that despite a couple of small setbacks, Max continues to impress us with both his physical rehabilitation and his mental advancements. 

Max wrote this all on his own!

Medically speaking, there have been a couple of developments. Max had his appointment with the Immunologist to explore the possibility that he had developed an allergy. If you remember from the last blog, Max has recently experienced on a few occasions after eating, a spontaneous swelling of his lips. Despite much amateur detective work, we had been unable to narrow down the cause to any particular type of food or additive. Well, according to the immunologist, the most likely cause of Max’s reaction is idiopathic angioedema, which for those unversed in latin or medical terminology translates as ‘the cause of the swelling is unknown’. Given all that he has gone through, the immunologist said that it would be difficult to try and pin the root cause down to any one thing and as no other more serious effects were experienced, we were told just to continue to keep an eye on it, and if he has the reaction again, apply ice and/or administer anti-histamines - and avoid that food.
  

As for Max’s skin, this continues to be a concern. The bout of hot weather experienced in Melbourne a couple of weeks ago seemed to knock him around a bit and his skin flared up worse that it had been for a while. Whether it was a good thing or not, it was probably at it’s most fiery on the day of Max’s appointment with Doctor KT. It had been hoped that over time Max's skin would settle down all on it's own, but according to the Doctor, it might be time to try a new course of action. In the past, they have investigated Photopheresis as a treatment option but this has pretty much been ruled out due to Max’s size (or lack thereof). We are expecting that Max will soon be prescribed a medication traditionally used in the treatment of cancer but that has recently been used with some success in treating GVHD. We'll let you know more after his next visit to Doctor KT.

Feet looking pretty red and flakey.

In the past year, due to the steroids Max has been on, he has not grown in height and, as mentioned in previous posts, has dropped a couple of kilos in weight. Hopefully in the long term he will catch up some of this lost time in his physical development. The more immediate concern is his weight which continues to hover around the 13.5 - 14 kilogram mark. He has gone a little backwards in his enthusiasm for food. An unfortunate side effect of him becoming more expressive is that he is quite brilliant in articulating long winded excuses as to why he is not quite ready to eat. This can be simultaneously hilarious and exasperating. As was the case in hospital, his tastes continue to change on a frequent basis; yesterday's favourite (eg pork dumplings) can easily become today's "I don't like it any more / I am off them" meal. The current favourite is Spaghetti Bolognaise, but we'll see how long that lasts.

On a more positive note, with each day, Max becomes more physically active and capable. It looks like all of Bianca and the physio's hard work is starting to pay off. On top of the physiotherapy session Max has on each regular trip to the hospital (once every two weeks), the hospital have arranged for Max to have, for six weeks, a weekly at home physiotherapy session, which needless to say he enjoys immensely. With the improvement in the weather of late, Max has been able to spend a lot more more time out in the backyard, running around, kicking the soccer ball and playing in his cubby. The other weekend, while Bianca and I were cleaning out the garage, we took our eyes off Max for just a couple of minutes. Before we knew it, Max was demanding that we should come out from the garage to the backyard where we were shocked to see that he was standing on the landing of his cubby house. The amazing thing being that he had climbed up the steps of the cubby house all on his own.

Amazingly, yesterday it was a year to the day from when Max had his Hickman line put in. With the next month marking the one-year anniversary of his transplant look out for a special edition anniversary update.


Cheers,


Tim

On the road to recovery.... (Day +312)

Happy New Year!

Overall it has been a good start to the year...two big positive events occurred since our last update:

Positive One) Restrictions have been lifted on healthy adults, although Max would love to be able to see people his own size and age; he is still loving the fact that 'other' adults can now enter our home and play with him.....as am I!! Max was always such a social being and loved mixing and meeting people.....this is once again strongly evident!

Christmas was lovely albeit very quiet, and Max enjoyed receiving all the attention from his parents and grandparents (Oma, Opa, Mawa and Mapa). Max’s other Aunts and Uncles dropped by to say “Ho Ho Ho”, but a special mention must go out to Max’s cousin Jack. Although technically Max is not yet allowed to mix with children, shhhhh....we made an exception on this occasion, given that we knew that Jack was free of illness (and having a nurse as his mother, I knew she would be able to spot any signs in an instant!). Max had not been in contact with another child for over 10 months, so understandably he was a little cautious at first, but after a while he was enjoying playing with his cousin immensely….even telling him what to do (and what not to do!)!

Since this time we have also made another exception.....again shhhhh....for Max to see his other 4 cousins (all in one family....and all under four years of age....Oi !) Again, Max had a blast!!!

Positive Two) No more ‘clean/sterile diet’! Those in the know may be wondering why Max has been on the ‘clean diet’ for so long? A ‘clean diet’ is usually only required for the first +100 days post transplant, as during this time BMT patients have severely compromised immune systems which could not fight off potentially harmful bacteria that naturally occurs in most food. Because Max acquired Graft vs Host disease (GVHD) post transplant, he has had to take a large number of immunosuppressive medications to keep the GVHD under control, and has therefore been required to remain on the ‘clean diet’ for all this time. Although Max is still taking these immune suppression drugs, on my request, Dr KT decided that it would be ok to relax his diet somewhat to include other food. This means Max doesn’t have to eat food which has only been either freshly prepared, or which has come from individually sealed packets. It also means he can have Maccas!...a great way to stack on the fat…more on that later. Being off a ‘clean diet’ also means that we are no longer required to boil water for Max to drink, it can now come straight from the tap! Ahhhh the luxury.....

As mentioned above, it was on my request that Max be able to eat foods other than those acceptable for a ‘clean diet’. Up until very recently, Max's eating has been a serious issue. He was continuing to dramatically loose weight - despite our attempts to lather the fat on his foods wherever possible! The Drs and dietician warned us that if Max’s weight was still dropping after Christmas we were going to have to start specialized feeds, as a way of forcing the weight back on. This meant having to reinsert either a NG tube (aka Mr Nose) or a port directly into his stomach. As you can imagine, I was extremely nervous and upset about this prospect.

Relieved that Max could now eat a wider range of foods (with the lack of ‘clean diet’ restrictions), I swung into party food mode – there was chips, dips, and all things harmful to the waistline and cholesterol level! Things were going 'great guns' until a few Sunday nights ago (and of all times, the night before we were going away to the beach), whilst Max was eating a chilli rissole and some ‘Easy Mac’ (packet Macaroni and cheese), he began complaining of a sore mouth and stomach….. Within 5 minutes Max’s lips had swollen up to three times their normal size. We didn’t muck around in case his throat started to swell up also, so we headed straight to the emergency department of the RCH. By the time we arrived, his lips had started to deflate, but we were still rushed into an isolation cubicle. The Dr assumed that Max must have an allergy of some sort to something that was in the ‘Easy Mac’ meal; so after a few hours we were sent home with an appointment slip to see the immunologist in a few weeks time. We were told to just “steer clear of eating ‘Easy Mac’, as if Max were to have another episode, it would certainly be much more severe”. This is due to the fact that, depending on the type of allergy, after the first reaction the body ramps up the production of the antibodies, increasing the risk of a more severe reaction the second time around. 

With my detective hat on, I thought the trigger of this mysterious lip flaring incident could be put down to either MSG ‘food enhancer 621’ or the natural food colouring ‘Annatto’…. the handy internet and Google tools aiding in my detective mission! Chuffed with my expert investigation skills, I thought we could just avoid those additives and relax. Not so. Upon eating some chips (which don’t contain MSG), and a cheese toasty, (which he had consumed many times previously), Max’s lips began to flare up again! As we were at the beach - and hence a couple of hours away from the RCH - we applied ice to his lips and were relieved to see the swelling eventually go down. (So much for the Drs claim that a ‘much more severe reaction would occur if it were to happen again’!). Max’s lips have swelled four times since, each time after something he has eaten many times previously, but each time his lips go back to normal within 30 minutes or so and no other symptoms arise. Very bizarre indeed. I have spoken to our Dr KT about it, to which she prescribed some anti-histamine…..but she didn’t seem overly concerned?! Our appointment with the immunologist is on the 8^th of Feb, hopefully she can shed some light on the situation. So being off the ‘clean diet’ is great, but as always - with us - there is bound to be some hiccups on the way! Thankfully, at our last hospital appointment Max had gained a little weight (0.2gms), so fingers crossed his weight can steadily increase and we can avoid that dreaded NG tube?! A trip to McDonalds is now a regular event after each hospital visit...

Devouring a cheese burger....

.....minus the pickles of course!!

As Max is now feeling more like himself, his dramatic acting and unbelievably well developed negotiation skills have become the major frustration for us. He can be a bit of a 'sookie-la-la' at times, and meal time (including administering all his medicines) can last for more than an hour at a time, which is oh so frustrating…….


An example of our morning routine:

Me: "Come on Max you need to have some breakfast"

Max: "No I don't!....I want to watch Top Gear"

Me: "Ok, I'll put on Top Gear once you have a mouthful of your egg...."

Max: "No! Top Gear first, THEEEENNN mouthful..."

Me: "NO! mouthful, THEN Top Gear...."

A bit of a forced cry occurs and a cuddle is requested, then, just when I think I have succeeded by eventually managing to get the fork to his mouth ... Max quickly turns his head away....
Max: "Oh nooooo! It's too cold!...it needs to go in the microwave......"

Another frustration of ours is that Max is often spontaneously struck down with an ailment I have coined "BLS : broken limb syndrome".... or BoLlockS!! It is clearly mood dependent, and manifests itself by his hands or legs suddenly becoming incapable of performing any task I ask of him, as they have either "fallen apart", are "broken" or are "lost"! This usually occurs around meal time when I ask him to feed himself, or if I ask him to go and do a task....

Example:
Max: "I caaaaan't walk.....", 

Me: "Why Max?"

Max: "...because my legs are broken...they don't work anymore!!!"

When, however, Max is in a good mood (or he has forgotten about his 'poor limbs') he not only walks; but jumps, runs and even gallops!!! "Look Mummy look, my legs are working today!!!"

Hospital visits are now down to fortnightly, and in some respects they are getting a lot easier. The rewards of a new matchbox car after his blood tests... a trip to Maccas for a cheeseburger, and the promise of a ride on the firetruck makes things all worthwhile for Max...

Blood tests have never been pleasant; but as Max now knows what to expect, he proceeds to instruct the pathologist on what to do! As he pulls up his sleeve, he whimpers “I don’t like it when you take my blood, but you need to put the butterfly (type of needle) in this arm, and you have to do it veeeeeery slowly….ok?”.  

He is also getting much more tolerant with Dr KT. He no longer shrieks out “No, I don’t want to see you…..I don’t like you….at all!” Dr KT would always exclaim “Gee, lucky I’ve got thick skin”. Nowadays, it’s a little more friendly.... “You’ve got poo poo on your head!’.  

As Tim mentioned in our last update, Photopherisis may have been on the cards if Max's skin was not improving. I initially was unfazed by the prospects of Max having to have this treatment. I knew it would be a lengthy process, weekly at first, then fortnightly, then monthly…..for possibly many years. But naively I thought it would just require Max to have a canular inserted, blood treated, then the canular removed for each treatment session. After speaking more in depth to Dr KT about it at our last visit, boy was I wrong! Max would require a central line, similar to the Hickman line (aka Mr Hose) but much more uncomfortable, stronger and less flexible due to the large amount of blood flow that has to pass through it. Having a central line will also once again bring the threat of more infections and the regular dressing changes and bathing hassles.....so heres hoping something 'magical' can happen and his skin can resolve its issues with his new immune system......to avoid this having to happen......soon!

It continues to be an effort for me to do much around the house, or talk on the phone, as Max is always demanding something of me...whether it be to feed him, play with him, or watch some special maneuver he has devised with his cars. The only proven way I can get at least 30 minutes of 'me' time, is by turning on Top Gear....thank God for Foxtel!!!  

Thank you to:

• Lis B for the cool 'stars and astronauts' torch.
• Amy M for all the Cars, macaroons and lovely visit and play.
• Nurse Helen for looking after Max while we celebrated Mapa's special birthday, and the remote control lamborghini!
• Eph B for the 'Top Gear' mags and Ute, and Mike for the 'ride' in his pick-up truck. 
• ...and everyone else for the wonderful X'mas presents for Max.