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Wiggles!

We had a wonderful family day today. William was given three tickets to the Wiggles Show by Adrienne (the manager) at Ronald McDonald House. We managed to get approval for a gate pass from the cardiac team and the rehab team and he had his antibiotics early before his line was locked off and disconnected from the IV pump and drip.

Wil was very excited just to go in a new wheelchair without all the extra bits attached!

We all piled into the car – the first time in over two months as a family – and we drove into Star City at Darling Harbour. The tickets needed to be exchanged for seats with wheelchair access and so Liza and the kids came with us to the box office at the Lyric Theatre. They were very helpful and even though it was sold out, we managed to buy two extra tickets for the show so we could all go! Even a month ago we could have never imagined doing this as a family so soon! It was wonderful!


William was a little frightened of the possibility of talking to the Wiggles up close. Even sitting in the theatre he was getting cold feet. But as soon as the lights went down and the action started he was hooked! He sang along, he did the actions and he yelled 'Wake up Jeff!” possibly louder than anyone else! At one stage in the show, Sam came up the side of the stalls and our shy little William put his hands over his face. But as he got closer Sam got a big smile and Wil happily gave him a high five!

After the Wiggles we went to the Sydney Aquarium and went for a quick walk around the fish and sharks. He had a lovely time there as well and enjoyed looking for any fish he knew from 'Finding Nemo'.  By the end of the day he was tired but very happy. The same went for his parents.

Steps to recovery

Today was another milestone day – William walked today!. Yesterday we was supporting his own weight in the physio gym. Today he was in the playroom on the Edgar Stephen Ward during the fortnightly HeartKids morning tea. He decided he wanted to get out of his chair so he could play. He later spotted a play kitchen and found it difficult to crawl so with mummy supporting him, he took a few steps. He repeated his efforts in the physio department in the afternoon covering several metres with just some support under his arms. He stood playing holding onto a small bench and was on many occasions standing without any of us touching him. It is amazing to see this change from the little boy who even a week ago could not sit up by himself.

Whilst his rehabilitation is intensive and he sees the play therapist and the physiotherapist daily, the occupational therapist and the speech therapist several times a week, he only has access to the music therapist once a week. Last week he was asleep during the half hour window of time she had available. This week he was chomping at the bit. He loved singing along and banging on the drums. Liza even suggested he might become a drummer but I replied that I thought he would make a better recovery than that! (apologies to our drummer friends)

Drains out

Today was one of those great days. William had his chest drains removed this morning!

The drains are very uncomfortable and are the reason William is still on morphine. They restrict him moving in just about every way and they add a huge load to transferring him to a chair. They would need to be secured carefully on each side of his wheelchair and the tubes carefully coiled out of harm's way. They needed to be attached the his bedside and every time we moved him we would have to ensure the drain was not pulling. They were tapped and banged to keep fibrin clots moving along day and night and were constantly being checked. To have the chest drains out is such freedom for Wil. He sat up unsupported this morning after his drains were out for the first time since his operation!

Liza, Tom and Liv went to Toronga Park Zoo today. Wil and I got out and about around the hospital and grounds with just one trip to x-ray and the rest of the time spent looking for pictures of elephants or playing on the pirate ship in the playground.

The prolonged recovery here in hospital is taking its toll on Liza and I. We are more weary than we would like to be and are burning the candle at both ends a bit. Returning home with William will be such sweet joy for our whole family. It is days like today when we feel that it is definitely getting closer.

William has gradually been improving over the week. Today he had quite a good day. He was trying to hurry his dad up and go for a walk outside - apparently dad was taking to long to get ready getting him into his chair!

He has had his oxygen removed and no longer needs continuous monitoring. This means there are two less things attaching him to the wall and it is just that little bit easier to move him now. His vision still appears to fluctuate a bit. His conversational ability is improving a lot. He is relishing the amount of Wiggles toys and music he has been allowed recently. Today he unwrapped a present that Pos gave him several weeks ago. He was so excited to get his hands on a wiggles guitar!

William's pleural drains are producing less fluid now and hopefully we will have less than a week of needing them (we are a bit careful to make more accurate predictions). Getting his pleural drains out will mark a big step towards his recovery and his discharge. He will be able to start rehabilitation in earnest and we will be able to get his muscles working a bit to try to reverse his somewhat de-conditioned state.

Wil's siblings and Ya Ya went back to Nowra today. There has been a serious outbreak of gastroenteritis in Ronald McDonald House and all siblings have been asked to leave, no-one is allowed to eat in the kitchen or dining areas and the communal areas are empty.

Adam had returned to work and will spend Wednesday night through to Sunday night in Sydney and will work Monday to Wednesday and Friday in Nowra. Liza will continue to hold the fort here and will be on duty by his bedside most of the time. Whilst we are stretching things as far as we possibly can, it would be good to be three people instead of one at times. Everything we are doing is worthwhile though - Wil needs us and it is good that we can share the load. Our other kids also need us and although our family life is dramatically different to what we might consider 'normal', it is still gratifying that we can claim to still have a family life. Work is busy but my patients and colleagues and staff are immensely understanding and supportive, and just to ensure that there are no stress free moments, we are renovating at home and are shortly to start at work as well.

I was discussing building with our project manager and were discussing the rewards of seeing day to day progress, seeing your goals realised and being able to see that all the work that is done actually results in a visible tangible difference. Whilst we have had our share of bad weather and variations to the schedule with William, it is also rewarding to see him improving, growing towards health and being built up again.

Happy Birthday

Today William turned four. His nurses decorated his room with balloons and happy birthday posters and they organised a party for him on the ward. He had volunteers visit him, one of whom was a ballon scuptor and gave him a a lovely balloon teddy bear.

He opened some presents on the ward, had a nap and then we took him to Ronald McDonald House for a birthday lunch with Pos, Pa, Uncle Dan, Aunty Emma, Uncle Dominic and the Kipps cousins.

Will is no longer seeing tigers that are not there. He now has his own soft friendly tiger that is definitely not a hallucination.

Pos made a very hungry caterpillar cake for William with four candles!

Easter Show

William has had a couple of days lying around just draining fluid from his chest cavity after having his drains re-sited.

Yesterday Liza & Adam took Olivia & Thomas to the Royal Easter Show (with Pa, Uncle Dan, Aunty Emma and the Kipps cousins) Wil stayed behind with Pos who looked after him all day while we were out. He did  bit of sleeping in between dressing changes, medications, suppositories and feeds but generally was a little grumpy when awake. Today he has been a little better by all accounts and was lucky enough to get a little bit of the Easter Show come to him. Several of the RAS Easter Showgirls came into the hospital with toys for the kids. Wil has always had an eye for he pretty ladies and he seemed pretty happy to have them visit. I am sure that he was less impressed that the Miss Canowindra Showgirl was an ambassador for rural NSW (and could probably even bake a decent scone) than the fact that she gave him a Wiggles bag and some other play sheets and cards.

We are hoping to be able to rationalise William's antibiotics soon. The sensitivities of his blood cultures was finally reported today and we were pleased to see that the staph is sensitive to flucloxacillin and is not the dreaded MRSA (resistant to multiple antibiotics). He is currently on timentin, flucloxacillin and vancomycin four times a day and gentamycin daily. This is combined with his oral medications including MS Contin, Keppra, frusemide, lisinopril and pyridoxine twice daily, warfarin and melatonin daily and oxycodone and paracetamol as required. It is a never-ending time job just getting him dosed up!

More theatre time

William has been out of sorts. He had high fevers last night and this morning both drains were blocked. His chest x-ray showed fluid was compressing both lungs and his oxygen requirements were rising.
The recent blood cultures grew staphylococcus aureus in both samples, one of which is coagulase negative. This means he will be on antibiotics for quite some time and so it was arranged for a long line to be put in while he is in theatre. This is a bit like a central line into his arm which can stay in for a while.

He was fasted overnight and eventually went to theatre at about 5:30pm. At about 7:30pm the cardiothoracic fellow wandered into the ward to tell me he was in recovery.
Will never wakes up happy from anaesthetics but he was not too bad all things considered. We got him back to the ward and the new line kept occluding - probably some kinking of the line which a new dressing may fix but we nursed it through and will address that tomorrow when he wakes.
The new drains have been working well and before he even left recovery he had drained over 200ml from his left side. His right side is draining less as it unblocked itself just before theatre.
Whilst it is not great that Wil had to go to theatre today and it is not great news that he will be on intravenous antibiotics for a complicated infection, he is being treated appropriately and we are all set for further improvement.

Easter Monday

William's left pleural drain is blocked again. There has been little drainage for two days and long periods when nothing comes out. An x-ray done yesterday showed increased fluid building up on the left side again pressing in on his lung again. Greg Rice, the cardiothoracic registrar came in this morning and tried aspirating the fibrin plug out of the drain but it would not budge. Added to this is the fact that the swabs from the left drain site grew bugs called pseudomonas and staphylococcus, and the recent blood cultures also grew staphylococcus.

It all adds up to needing a new chest drain. So tomorrow William goes back to the operating theatre tomorrow.

Easter Sunday

Easter Saturday

William remains somewhat grumpy when his temperature is up but he has his moments when things are looking good. His bowels have still not opened but judging from the amount of aperients we have put in both ends and the sounds gurgling around his tummy, someone might find a lot of something on Easter Sunday that resembles chocolate in colour only.

William's head control has improved significantly and he is now able to sit in the weighing chair by himself. Today  we took the plunge and sat him in a pram, unhooked the suction to his chest drains and hung the collection containers on the back, unplugged his IV pump and got a more user friendly pole, hooked up portable oxygen and right at the end we decided we should pop a sheet over William - It was warm but the bare chested look was not quite what the public needed. We pushed him out of the ward and down to the playground, He had one slow circuit on the roundabout and then we pushed on to Ronald McDonald House where we spent some time looking at the play equipment he had thoroughly tested in the evening when we arrived there four weeks ago. he couldn't get out of his pram but he did enjoy looking at everything. He will love these things so much more once he is not hampered by drips, drains, constipation and fevers and hopefully that time is not too far away!

Good Friday

William had been making slow progress with gradually improving sleep, less hallucinations and confusion and improving sight. Both arms and hands are moving with reasonable strength but his co-ordination and ability to manipulate things is still significantly impaired. The muscle tone in his neck seems to be improved slowly as well. His diazepam has been weaned dramatically, his pain relief is still required but is causing constipation, his drain dressings are causing blisters on his skin but with some dressing changes hopefully that is now under control.

Rehab will commence next week in a bit more earnest but his newest problem seems to be infection again. William has been having fevers for the past 36 hours and his blood count showed a high white cell count and an elevated C-Reactive Protein (which together would suggest something is going on). Swabs from the drains did grow some bugs but it is quite possible  that they are not the source of his main infection.
He had a cannula put back in today (amazingly the anaesthetic registrar found a vein to use!) and he is on intravenous antibiotics again.

Although grumpy and very sad, we are slowly seeing more of our little William as he recovers. He has moments of happiness where his little sense of humour shines through again. Although constipated, he has been passing quite a bit of wind and after one fairly obvious passage of flatus, I turned to him in mock surprise and said "William! What was that?!"
"A frog" he replied, grinning back at me. (Officially, I have no idea where he learnt that, but I will confess to possibly encouraging him by laughing a lot)

Liza drove back home on Wednesday to see Olivia get an award at her school assembly and to see both kids in the school's Easter Hat Parade before bringing them back up to Sydney. It is lovely to be back together as a family again at Easter. It will be hard in the coming weeks as we spend more time with our family separated at times but we do look forward to William's progress and to see how much he will recover as well.

Bad night, good day

William had a bad night, waking at about 1:00am and then not getting back to sleep until 7:30am this morning. During the wee hours he was extremely agitated and for most of the night he appeared delirious. Finally after his third dose of Chloryl Hydrate in a 12 hour period he fell asleep and caught up on five hours sleep. His afternoon was pretty good. He woke up cranky but after pain relief he get much better and was very cheerful this afternoon. We played and he laughed and smiled.
His vision continues to improve... OK, so he did think my hand was a spider at first but when he was given a second go he knew he was seeing Daddy's hand. He was carefully looking at his own left hand today also and remarked as he opened and closed it " 's my fingers!" He has come a long way already from when he could not move that hand at all and could not see it or anything else. He can now clearly make out not only shapes but can even recognise people from their faces at times.

We had visits from a range of medical teams today - The cardiology entourage of over a dozen people stop by every morning on the ward. we were also visited by the rehab team, the pain team, the dietician, the physiotherapist, the social worker, the cardiothoracic registrar, and the neurologist. He had bloods taken (with difficulty) and had all his dressings changed. He sat out of bed for half an hour again as well. Best of all he went to sleep at about 7:30pm - we know how much he needs his sleep at the moment.

Tonight for the first time in three weeks, we pulled out his pyjamas. There are still too many things attached to his upper body but since his chest drains are no longer secured to tapes attached to his nappy, we decided that it was time for his Wiggles pyjama bottoms to make a reappearance. He was pretty happy!

Day 21

William had a good day today.

He enjoyed brief visits from his siblings, who have been fantastic. They go out to the balcony off Wil's room and do colouring and drawings for him. They will spend time talking to him but really understand when we ask them to leave him alone for a while. They completely understand that William's brain was hurt and he is not like his usual self and have taken it all in their stride. William also enjoyed sitting quietly with Ya Ya again today.

He had his IV line removed today - it was only being kept in case it was needed and given that it had probably blocked and was unlikely to be required, it was removed, giving his left hand back. Both hands are now moving well although the left side is still a bit weak and it seems difficult for him to co-ordinate his upper limbs. His head and trunk are floppy but he still has some tone and movement - this will improve with rehab. He sat out of bed a couple of times today - for weighs, linen changes and for about 40 minutes to help his chest continue to recover. He loved his time sitting on mummy's lap today.

His vision has started to improve. He is definitely fixing and following faces now and he may even be able to recognise people by looking at them. He obviously still has trouble seeing but he is definitely getting better and  although it doesn't feel it sometimes, it is still early days yet!

We had plenty of smiles today and even more than a few little laughs. It is amazing what strength we get from each smile.

Settling

William did have a bit of an unsettled day today. Given that when he woke this morning he was already tired - he had had less than six hours sleep in the preceding 24 hours. We let him sleep as much as possible this morning  and tried to keep his stimulation to a minimum. He was reviewed by the pain registrar who increased his MS Contin.

Although he coped fairly well in the morning, he became agitated in the afternoon and was thrashing around a bit. He eventually settled with more pain relief and some sedation and apart from waking in pain at about 10:30pm and then again with agitation and hallucinations at 1:15am, Wil managed to sleep.

We did notice that William responded very well to Liv and Tom being there. He became irritable if he was stimulated too much but he loved hearing them and seeing them over the weekend.
Adam & Liza actually got to both leave the hospital for a short break today while Pos  & Ya Ya looked after William and Uncle Dan, Aunty Sarah, Uncle Matt and the Jeffery cousins all played with our other kids.

De-intensified

Today was an intensive day of de-intensification:
Wil's arterial line was removed after coaxing one last blood test from it.
His venous line was capped - it was left in place just in case he needs access in the next day or two.
We spoke with the rehab team several times.
We spoke with the pain service doctors.
The Fentanyl infusion was ceased and he was started on MS Contin through his PEG Tube.
We spoke with Prof Ouvrier about the MRI scan.
And we moved out of the intensive care unit!

William's rehabilitation will start slowly next week. The rehab team indicated that he will stay in hospital while he is making good progress and he will be discharged when the gains start to slow down. The actual time will really depend on how he goes but will be in the order of 4-6 weeks more. This time frame gave us a bit of a shock as it meant we will have to confront the reality of a prolonged hospital stay, balancing work, family and hospital commitments. It also reminds us that there is still a long journey ahead of us.

Given that we were changing from one medication to another medication, there was always a chance that the new medication would either make him too drowsy or not give him sufficient pain relief. Unfortunately it was the latter and William was awake until 1:30am. The change of environment and several visitors today (siblings, grandparents, aunt & uncle & cousins) may also have unsettled him a little. It was a rough night as now we are on the ward one of us will stay the night next to his bed to help settle him. We are trying very hard not to overstimulate him - he really needs heaps of rest. It has been more of a problem recently and we are trying to work out the right balance.

It was lovely to see so many of the family though and Wil was definitely enjoying the visit. He was smiling and babbling away, his echolalia was certainly making our kids laugh and when they giggled, he would smile with them, very likely feeling that he was part of the joke.

Among the large number of doctors we saw today was Prof Ouvrier. He told us that the MRI results did not reveal anything new. There was nothing that could not be seen on the CT scan. He felt very encouraged by William's progress so far. His movement in his left arm has improved substantially. He still has some weakness but is moving this hand, wrist and elbow which were showing at the most a flicker of movement only two and a half weeks ago. He appears to be regaining his vision and today he was definitely fixing and following at times. This is of course a huge relief. Prof reassured us that he felt very optimistic for William to improve substantially from his present state. It is always a relief to hear such news.

So while everything that happened today culminated in a very irritable and restless evening and night. we feel we are moving in the right direction. Getting out of intensive care was a great step forward despite the wonderful care we received from all the staff there.

Less tubes

William continues to improve and today he was de-intensified just a little. He had his urinary catheter and one of his venous lines removed. He is still draining a fair bit from his chest drains but his intravenous analgesia will be weaned and he will go onto oral analgesia. He is being weaned from his diazepam as well. His oxygen was almost weaned off but tonight when changing rooms, he desaturated a bit and returned to two litres per minute. Hopefully he will be back to ½ a litre per minute by the morning.
If there is a bed available, we might even make it to the ward tomorrow. The hospital and intensive care seems extremely busy at present so I am not holding my breath but it would be a lovely step in the right direction.
We did not get to see the neurology team today to discuss yesterday's MRI. It was a bit disappointing as we waited around the entire day ensuring one of us was always at the bedside and the other was close enough to be back in under two minutes.
William can have some fairly calm periods but still seems to be having hallucinations at times and at other times is just confused and agitated.
His ability to see seems to fluctuate. This morning he seemed to be able to see my fingers if I wriggled them in front of him but later he showed no signs of being able to see at all. I guess it is still the early stages of his recovery.

Magnetic Resonance Imaging & Magnetic Resonance Angiography

William dozed most of the day, still intubated and sedated. He did get agitated when he had some secretions in his tube but settled well after suctioning. His new chest drain continued to drain well and his chest x-ray this morning, (according to the ICU fellow during morning rounds) was the best it has looked since he has been here.

The MRI and MRA were booked for 1pm. At 1:30pm were were told it was going to be at 3:00pm. At 4:00pm we were told we were just about ready and at 4:30pm we finally left the unit with the anaesthetist.

William did not misbehave with the anaesthetist (apart from opening his bowels for the first time in five days) and before long he was being wheeled back to PICU.

He was settled and reconnected to the machines back at his bedside and then Emma, his nurse, began the appropriate preparations before extubating him and once more he was with out a ventilator and just on nasal prong oxygen. He settled very well and coped with physio without too much problem tonight before falling asleep at 9pm without needing extra sedation.

Before and after

William spent all night off CPAP. He coped well and despite being confused and agitated at times he seemed to be having a reasonable day. As you can see he had a smile and giggle again today.

But his drain was blocked again and his left pleural effusion was getting bigger and compromising his left lung. He needed a new drain. We could not get blood from him this morning. One of the fellows who seems to have amazing success found a vein out of nowhere and managed to get one tube off for his INR but there were still several tests that could not be collected after a significant amount of distress. 

The doctors had been talking and planning and today they decided to take William back to the operating theatre to remove one of his chest drains and replace it. Whilst he was under anaesthetic they would put in a second venous cannula and an arterial line so that we can collect blood easily. The plan was to bring him back intubated and leave him on the ventilator until the MRI scan. The earliest we could get that is tomorrow so Wil is now sedated and ventilated. The up side is that he can get some rest (although at first the sedation at reasonable levels was still not enough to keep him under and he would wake up very distressed - that was fixed as quickly as they could). The major benefit is he will not need a second intubation and anaesthetic for tomorrow's MRI. 

We had an hour to fill while we waited for his to return to the PICU. We mooched around a bit, read and waited - certainly with a lot less stress than last time but it is always good to be told that "You can go in now and see him".

His new chest drain was higher up in the side of his chest - mid axillary line. He had his new arterial line and new venous line - both hands were now back on arm boards.

His postoperative chest x-ray showed the new chest drain in a less than desirable position and the cardiothoracic fellow came in and manipulated it on the unit before securing it again and suctioning out a few fibrin clots that had already formed. His endotracheal tube also required repositioning and re-securing.  After that his nurse gave him a good clean and fresh sheets (the others were wet and blood stained from attaching the drains) It is wonderful to see how the excellent nurses here cope with the intensive stuff of adjusting ventilation, clearing secretions which are blocking his bronchi by suctioning him, adjusting his sedation and analgesic infusions as necessary but they also look at the real issues of keeping him comfortable and clean which might seem inconsequential with so many other things going on, but which illustrate the level of care and dedication they have for their patient.

His blood tests were easily collected from the arterial line and he remained well sedated throughout his physio session. Whilst the visual images look like a step backwards, we know that these things will help William to improve to the point of leaving PICU that little bit quicker.

Wondering about paradoxical reactions?

The MRI scheduled for today was cancelled. Even though William is doing much better, because he was still having CPAP, there was a real risk that once intubated for the MRI, he may have problems coming off ventilation again. That would be a step backwards and the MRI will not change his treatment in the short term. At this stage it has been postponed until Thursday.

The chest drains are still in. The left one blocked again and was again manually unblocked. It only drained for another few hours before blocking off again. They have been in for long enough that the suture holding the left pleural drain in place pulled through his skin today. He had it resutured (with a little bit of fuss and objection from William - apparently he doesn't like local anaesthetic!). He also had his femoral line removed and scored a cannula in his hand.

He spent a long time off CPAP today and this evening he was looking so good respiratory wise that his nurse asked the consultant if he could keep it off while he slept as long as his respiratory effort did not increase.

Again tonight William was extremely agitated from about 7pm until 9:30pm when he finally went to sleep, again after a dose of chloryl hydrate. He had not just had physio (which I guess I was blaming last night). He was also very agitated with lots of disturbing hallucinations from just after 11am today. He could be having a paradoxical reaction to the diazepam which in each of these three cases was given just before he got significantly worse. We will discuss it further tomorrow but it would be heartening if we can find a easily reversible contributing factor.

Daddy's underpants

William is quite the chatterbox. He talks almost constantly when he is awake. He seems to love being a little parrot and repeating what he hears said in his presence. It is significantly out of character for William and will probably settle down as his cerebral situation improves. He is still having hallucinations, mainly when he is tired and tonight they kept him awake until 9:45pm. He seems a bit more settled at times when he is not to tired. He had a little smile today and I asked him “What is funny William?”
“Your underpants!' he replied with a giggle. It was a joke that was destined to be repeated several times today but we love to see him smile and have not at all been sick of that little quip.

He sat out of bed twice today: Once in my arms as he was weighed and Liza sat with him on her lap for almost 20 minutes in one of his unsettled moments. He did not love sitting up, possibly because he still has two chest drains, a urinary catheter and three IV lines connected to his groin. His head control is not great and that may also give him some discomfort when sitting upright. He is loving cuddles though and he actually asked for one unprompted today. (We have been doing plenty of prompting up until now)

He continues to tolerate reasonably long periods off CPAP (2½ hours today) and we are hopeful that he will not need it for much longer. His liver function abnormalities are improving and his neutrophil count is dropping back towards normal.

Tomorrow he will get his femoral line out if someone can get another cannula in somewhere and he will probably have the MRI. All in all, William has had a much more settled time when compared to a week ago. He still has his hours of agitation but he has been blessed with a few hours of calm as well.

Another day in ICU

William has had a few hours of significant agitation and hallucinations today. There are small signs to hold onto though. He is grumpy and upset but he can recall information in his better times and he does recognise people.

Olivia, Thomas and Ya Ya arrived last night. He was tired when they arrived but knew they were here. He also was interacting with Aunty Sue and Uncle Marco when they were here today as well. Unfortunately Olivia woke this morning feeling unwell. Because we are staying in Ronald McDonald House, we must be very careful of any risk of passing on infection so 'Liv had to go straight home. We did not even let her have breakfast here but took her straight out the side door to the car. Ya Ya (Liza's mum, Marea) took the kids back home. Poor Olivia & Tom were really upset. Olivia was fine again tonight but we would never take the risk. We are all missing each other and I know that William is certainly missing their love and attention.

Wil had some more fluid drained out of his left pleural space today as his drain was again manually unblocked. The fluid is still coming away and will need to be less before the drains can come out. He has had his midazolam infusion ceased and he has been put of diazepam to replace that for sedation. He can have the diazepam orally (or at least via his gastrostomy) He has also had his captopril increased. His blood pressure did dip for a while today but he recovered with a bit of chest physio (nothing like stimulating Wil's lungs to get his blood pressure up!). It is looking like a long road still but we are looking forward to it leading out of the woods soon.

Wires out

William has been making slow steps in the right direction.

His chest x-ray looked a lot clearer today and given the left chest drain seemed to yield another 15ml over the course of the day. He was finally fed at 4pm and theatre was cancelled. No new chest drains today!

William also had his pacing wires removed today. The wires which are inserted into the heart muscle during the operation usually do not bleed but sometimes can. If they do not come out early (before the mediastinal drain), then they are left in until they form tracts that any bleeding will follow back to the skin. Even though William is mostly not in sinus rhythm, he is coping well and has not needed pacing for a while. The INR was low enough today to take the wires out and he is now minus three more things protruding from his chest.

He spent an hour and a half off CPAP this afternoon! He was tolerating it very well! There was some early hallucinations but they settled and then we listened to some Wiggles, made some animal noises ("cock a doodle doo!") and even had a few giggles and smiles. He has been referring to anyone who comes to touch him for a clinical reason as a doctor. ("No more doctor" he will insist) Today he learnt a new word - "Physio". His parents' favourite words from Wil are "I love you" and "mmmwah!" which is how he gives us a kiss from behind the CPAP mask.

Whilst Will is still having hallucinations, he has not been quite as bothered by them today as yesterday. He seemed to have a longer time awake with less hallucinations. He certainly seems to be more susceptible when he is tired. This can be a problem because they keep him awake. He would like to go to sleep but cannot with spiders or tigers bothering him. Prof Ouvrier gave these visions a name today - Charles Bonnet syndrome. Hopefully we are close to seeing them settle substantially.

We are feeling very supported here and wish to thank all of you who have sent us things (we certainly loved all the pictures from William's preschool friends and Tom and 'Liv's classmates as well that we received today). Thank you for the messages, the offers to help out and the prayers. We especially appreciate the time you take to read about his progress, to learn of his steps forward and those inevitable setbacks he has had to overcome as well. Thank you for sharing in this stage of William's journey.

Effusions and Delusions

Wil has had a tough day. He has been very unsettled today with more hallucinations and generalised delirium than yesterday. His chest infection (sputum cultures grew haemophilus) seems to be improving with gradually decreasing white cell counts, no fevers and decreased work of breathing. Then there was this morning's chest x-ray...
His x-ray this morning was terrible. The left side of his chest was very cloudy and there was a huge effusion. The drain on the left had been blocked for some days now but previous x-rays were only showing minimal collections of fluid. He had an ultrasound to estimate how much fluid he had in his lungs. It is hard to be accurate about volumes but there was definitely enough to drain. We were consented to go to theatre for a new drain and were told it would happen at about 8pm. At about 7:30pm the cardiothoracic registrar came in to let us know there would not be theatre time until about midnight and he would try to unblock this drain first. He gave a few very big pulls with a large syringe and managed to pull through a bit of fibrin clot followed by about 100ml of fluid. We were thrilled - maybe this would work! Over the next hour about another 10ml drained and then we had an x-ray performed. Much better but still some fluid left. The next question to answer was whether he needed a new drain (probably still does) and if so do we go to theatre at midnight or tomorrow during the day. Eventually it was conceded that William was not going to suffer by waiting until daylight for the procedure and that we will see how things are in the morning.
Maybe it was because of the increased fluid but Wil was certainly having a few more hallucinations today. He was seen by Robert Ouvrier (Wil's neurologist) again today who reinforced his opinion that these would settle. He also felt that William was blind at present but that he thought there was a good chance of recovery. The pathways to the appropriate part of the brain are all intact, it is just that the visual part of the brain which was damaged is not recovered sufficiently yet.
William had an EEG again today. This time he was hallucinating some of the time, generally unsettled for some of the time, co-operative for a tiny bit and asleep at the end of it. This was great! We had good EEG readings during all of these times and we could confidently see no epileptic activity associated with the hallucinations. That seems to rule out visual seizures. We are still playing the waiting game though and Wil will improve as quickly as he improves. Time frames are still impossible to pin down.

Counting

William is gradually improving.

His sedation has been weaned a little and so he is a bit more agitated and he does take a lot longer to settle. He did have some more hallucinations about tigers and spiders today when he was given some time off CPAP.
But he is also showing some promising signs. He was a bit distressed during his echocardiogram today. When it was nearly over we started counting with him. He started saying a few numbers along with us. We got to seven and paused. William didn't - we clearly heard "eight.., nine..." come through his CPAP mask. We were very happy to hear that our little boy was still counting.
He has also opened his eyes a couple of times briefly today. Not long enough for us to tell if he is looking at us but nonetheless he is getting there!

Unfortunately his femoral line is not bleeding back and he will need more jabs to collect blood.

His CPAP is being weaned back and he will have short periods of time without it in the coming days. He tolerated 30 minutes this morning and was really good for over an hour this evening. The physio also seems to be helping. He is getting his chest physio four times a day, the last one at around 11pm at night. It is a big day for all and especially for William as he puts in this huge effort to get better.

Chest infection

We have had a relatively stable day.

After leaving William last night, he spiked a fever and was commenced on antibiotics for what is presumed to be a lung infection. He needed more oxygen overnight but this was weaned back to his previous levels this morning. He still requires reasonable pressures and fairly significant oxygen but it looks like he is not working as hard to breathe today.

One of the blood cultures taken yesterday did grow staphylococcus but I suspect it was a contaminated sample. His white cell counts were high but did not get worse as the day progressed.

He is getting physiotherapy four times a day and he is not loving that too much. He is a brave little soldier though and although he cries, often he will sob “OK” to our requests to do just a little more. We know it is helping him breathe easier.

Having had a whole day with no major events has left us to recharge a little emotionally. Even when he was sleeping peacefully it was nice to sit by his bedside and take that all in. It was reassuring to experience William resting and healing.

Things will get better

Today Will has been pretty stable but it has not been an uneventful day.

He is still on CPAP and he still needs it. The lungs looked a bit better on the x-ray this morning though.

He was still dropping his oxygen saturations a bit, his respiratory rate was often sitting quite high and since we were going to have to monitor his blood gases and chemistry regularly there were a few tries to get an arterial line in. The registrar and the fellow both had several goes and eventually we got enough blood for a gas and blood cultures but it was not going to give us a sampling line. This afternoon he had a triple lumen femoral line put in. He now has more monitoring but at least he does not need multiple punctures for his blood tests and he could also get rid of the drip in his right hand which had been in for eight days. We did have one vein on his foot we were saving for his next cannula but now that will not be needed either.

His urine output seemed to drop off and a urinary catheter was put in. He must have had a little retention because his kidneys were still pumping it out and his bladder was very full. That seems to be going well now.

We were seen by Prof Ouvrier, the neurologist this afternoon. He felt that in view of the EEG, seizure activity was now a less likely explanation for the hallucinations and that we were more likely looking at just general damage to his brain plus all of his other medical stuff contributing to a delirium. He is confident they will settle soon. He is also confident that William will regain his vision. He did say that he thought it unlikely that Wil would get away scott free, but he did feel that he would have a significant recovery from the things that are currently affecting him.

In the short term though he will need to rest, we will need to get his lungs better, and he will need ongoing sedation for a while. When he is a bit better and not so 'intensive' he will need an MRI.
While there have been some hallucinations today, he has not been distressed to the same degree as over the weekend. We were also able to talk to him during them and he would respond. He is pretty unhappy but he recognises our voices and will respond to questions a bit in his more awake moments.

We will take each day and hour as it comes but at the same time we are confident of his ongoing improvement.

A not so lazy Sunday

William resumed his distress again this morning. By 8:15 this morning Liza rang me to see if I could come up and get someone to mind the kids. I rang Pa and he and uncle Dan came straight to the rescue. They were on the doorstep in less than 30 minutes.

The sedation with chlorpromazine was keeping him from getting too active and prevented the severity of what he was experiencing the night before but he was still distraught and his head still was soaked with sweat, salty to our kisses. Richard Webster, the neurologist on for the weekend came to visit and he felt that as the swelling was not worse on the CT from 12 hours before, and as there was no bleed and no biochemical imbalance, the most likely thing that was occurring was that William was having focal seizures in the visual part of the brain. There were watershed stokes evident on both sides in the part of the brain responsible for vision and apparently it is an area which is quite sensitive with a high seizure potential. Focal seizures in this area will cause William not to be able to see anything at the time but will cause him to have distressing visual hallucinations. This seemed to make so much sense and of course we were grasping for answers.

An urgent EEG was arranged and huge dose of chloryl hydrate was given to him so that he would be co-operative enough to have the EEG. It certainly made him co-operative (you will not struggle when unconscious)! He could not be woken at all during his EEG, he was sedated so heavily. It would have been nice to catch one of these episodes but at the same time a relief to see him comfortable, to see him sleeping, not tortured by the distress of these visions and confusion which had possessed him for the previous day and a half. After the EEG he was commenced on a midazolam infusion which will probably keep him unconscious until tomorrow. He was also commenced on an anti-seizure medication – Keppra.

After he had been asleep for several hours, Liza and I finally felt that we could leave him in the capable hands of his ICU nurse (Sarah who was looking after him today also regularly nursed him in the cardiac ward when he was a baby). We knew he was comfortable and safe – it was the first time since his operation we felt confident that this was going to last for a while and we could leave together. We joined Liv and Tom, Pa and Uncle Dan, Aunty Emma, Pos and Ya Ya (who had been in Brisbane and flown back this morning) and Uncle Dominic, Jordan, James and Laura. It was Laura's birthday and they had relocated to the play area between Ronald McDonald House and the hospital so that they could all visit Wil and so Liv and Tom could stay with us but still have their little birthday party. William will not remember his visitors today. We will not forget this day, despite the fact that we are so tired that stringing a sentence together is a herculean effort.

I was upset this morning and had a big sob in front of Olivia and Thomas. They both sprung to cuddle me as comfort, and Olivia patted my back and said “It will be all right Daddy. How 'bout instead of crying we say a prayer for William”. She then prayed that “William will get better, he will see, he will walk and talk and will play again very soon”
I felt grief that my seven year old daughter should have to be exposed to such life experiences that she felt comfortable in her role of comforter, I felt proud of her abilities and I was humbled by her quiet strength and faith. “Suffer the little children... for such is the kingdom of God”

William is comfortable now though and it looks promising that this will continue for most of the night. The days to come are unknown but again we are confident we will see steps forward...

Think in hours ahead not days. As I typed the above lines the phone rang. "William is awake, can you come up." He became too heavily sedated and the midazolam was stopped. When he woke he was mildly upset but his oxygen saturations began to drop. We gave him suction, got him coughing and gave him extra oxygen but nothing was working. The cardiology fellow came in and performed an echo in ICU. The heart was still working well and the new anatomy is performing the job nicely. An ultrasound performed by the ICU fellow showed only minimal pleural effusions. He had an urgent chest x-ray which showed the left lung partially collapsed with some fluid both in the lung and around it. We tried high flow nasal oxygen but this did not work. We ended up putting Will on CPAP.

He eventually stabilised with adequate oxygen levels but will need the CPAP mask ventilation support overnight.

Delirium

We had hoped that yesterday would be William's worst day. The timing of events suggested that he should start improving from here on in. He was certainly more awake but still very irritable this morning.
Emotionally we were spent. We have too much to think about, to cry about and to worry about, but at the same time answers rather than fears give a certain freedom.
Olivia and Tom came to Sydney today with Pa and uncle Dan. At about 11:30am just after they arrived they came to see William and he smiled! It was his first smile since the operation! He talked to them, he responded to questions, he definitely looked at us. He even asked some questions. He was certainly still confused and he was not terribly appropriate in all situations but he was still in intensive care on fentanyl so that was forgiven. He could talk, he could smile, he could hear, he could respond. We were still so worried about him but were so thankful for the improvement. This was definitely the best he had been.
His central line was removed and his urinary catheter was also taken out. Things were getting better.

Things got worse.
In the afternoon, William became more agitated. Initially it seemed that perhaps he was just feeling pain when he passed urine. He became periodically very agitated and was calling out “That's enough!” It got worse and became virtually continuous. He was at times screaming. He was not looking at us – he could not see. He was distressed, he was so anxious and he did not look comfortable. This escalated, he was sedated but it did not work. The doctors could see that he had become so much worse and because his blood pressure remained pretty good and urgent blood tests did not show any cause for the delerium, we had to consider that something like a bleed into one of the areas in his brain was a potential cause for this. The radiologist was called in and we went down for an urgent CT scan at about 10pm. He was upset but we managed to keep his head still enough to scan him. Liza was with Liv & Tom, I sent her at text at 10:02pm: “No bleed” It was sent with a wave of relief.
Liza replied “What then?” and it was about then the answers reached a hiatus. We did not know what had caused this delirium but with more sedation at about 11:30pm, William finally went to sleep for the first time since 6:00am that morning. He stayed asleep for about an hour then woke agitated. He got worse again in about 20 minutes, hallucinating in the same way he had been all evening. He was so distressed, “No more wake up Jeff!, No more incy wincy spider!, no more Henry, No more tigers!, No more Sponge Bob, No more Pop go the Wiggles!” He would yell these things and many more with such absolute distress and with no possibility of comforting him. His head was sweaty and he could not see anything but these hallucinations. He was thrashing around. It was more than a parent should have to bear to see their child like this. Eventually at around 12:45am and after lots more sedation (including a bolus of midazolam) little William fell asleep. He slept with no more distress while his father tried to catch a bit of sleep at his bedside.
At 5:30am I crept down to Ronald McDonald house to tag Liza who came up to sit with him while I tried to get some sleep before our other kids needed breakfast.

Rock Bottom

The cardiac kids can earn beads for their experiences in hospital. Basically each time something happens like a drain out or a scan, discharge, surgery, or a new diagnosis, they are able to collect a bead. There are different types of beads for each event so that one can associate these beads with what has happened. We were discussing the various beads with Jody, our nurse yesterday. We had looked at the beads we did not want to earn - readmission to PICU, ECMO and the 'Bad Day' bead. The 'Bad Day' bead is big, bumpy and black and that is what we earned today.

My concerns about William's left hand not moving much, as well as his reduced communication and increasing confusion were confirmed to be amongst my worst fears. It appears that William has had significant ischaemic brain damage to multiple areas. It looks like what is called a watershed ischaemic event - the type of stroke you can get if the brain is deprived of blood flow for a significant time. We have no idea when it happened. Apparently he never had any significant periods of inadequate blood pressure during his operation but given the weakness in the left hand was something I noticed only hours after the procedure finished it seems that it must have occurred around this time.

William was asleep for most of the day. He got quite irritable a couple of times today and on those occasions would call out for Mum and Dad. He was staring straight ahead and did not seem to see anything. He has not asked for water and has not been able to even reply yes or no to questions in the last 36 hours. His most agitated was during the CT scan. At least when he is agitated he manages a few words. "I don't like it!" did come out today as well as "It's sore!"

But he is not 'with it'.  He is confused, we don't know if he can see and he does not recognise us. We are gutted, distraught, and so sad that is is beyond description.

We have been taking turns to sit with William. Liza wakes at 5:30am and makes her way to the ICU by about 6am while I shower and breakfast before getting there around 7:00am. Liza left today after the morning rounds at about 10am to come back the Ronald McDonald house and get some stuff done. She came back at lunch time so that I could go to eat and get some stuff done but today I could not leave. I told Liza that we should both be with him at the moment, I was not ready to go.

I should have been happy. Although the chest drains had yielded only a small amount overnight, they drained too much in the morning to consider removing them today. But this was no big thing. We actually expected them to be in for a week to ten days and we knew that we could still leave ICU with them in. The INR needed rechecking. It had got to 4.8 yesterday, a morning reading showed it getting better being down to 3.7.  A test at about lunch time came back with an INR >14 (that is definitely not a good thing!) the test was repeated immediately and the second sample retuned a reading of 3.2. Much better and confirmation that the first test had been an error. This meant we could start to 'de-intesify' him.

He had his arterial line removed from his left hand. Even though he had really not been moving his left hand much, there was some spontaneous movement and I thought maybe we would see an improvement when that line came out. But he was still floppy on that side and even though his left leg was moving well, we asked if the registrar could review him before we were moved to the ward. (The ward bed was ready and they were expecting us - we were almost out the door of ICU) The registrar confirmed that there was a problem (which we knew and feared). She arranged a CT scan and it was then I saw the images. I could see several areas which were not normal for a brain scan on the screen as I came and stood behind the radiologist. I felt sick. I still do.

We have been visited by the neurology fellow. Prof Ouvrier (our neurologist) had already seen the scans. It was likely a very significant brain injury. There was swelling and it was (in the word of the neurology fellow), "not very good news". We had already been seen by the cardiologist and cardiology registrar and following that the intensivest and ICU fellow. They were all very caring, no one could say how this happened, no one can say how it will turn out. We just have to wait and see. I know how they feel as a doctor - they see us, crying and distraught. There is the overwhelming desire to do something, do anything that could make it better. There is nothing they can do. We have no further questions that they can answer. Time is needed before we can do anything else, before we can see what will happen.

We are confident he will improve. What he will return to is the question. At the present time his brain is swollen as a result of the injury to the areas which have not received enough oxygen being inflamed. This is causing other brain cells which will recover to be temporarily out of action. What is temporary and what is permanent is not known at this stage.

If you read this post tonight or in the next few days, please pray for William. If you are reading this months down the track then pray as well and give thanks for the improvement that we have faith he will have experienced.

Each day we will take as it comes. Each hour is a new hour.

William is a fighter. William is a conqueror.

Another Day

Another day in intensive care.

William was in and out of nodal rhythm today.

The amiodarone seems to be helping and today it was changed from an intravenous infusion (which has to go through his central line) to tablets. The rationale is that he can have his central line out if the medication can be given through his gastrostomy instead. The problem however is that William's INR got to 4.8 today. The INR is a measure of how thin the blood is, or how hard it is for the blood to clot. A higher INR means easier bleeding. An INR of 1 is normal if you are not on medication. We are aiming for an INR of 2 for Wil. So an INR of 4.8 is too high to risk leaving reasonably large holes in the jugular vein and the radial artery. So there we are - William still has his central line and his arterial line in place and they will stay there until his INR looks a bit safer.
He does seem to be in less pain this evening. He is not talking much and responding with little grunts. "Ugh" has become the universal answer to each question today. I suspect that as his pain is a little better, he is feeling a bit more spaced out on the fentanyl. He will still need the fentanyl until his chest drains come out though. The drainage from these two tubes is slowing down which is great. It means we are a little closer to being able to get them out.

Pacing ourselves

William is still getting better but we had a minor setback today. He went back into a nodal rhythm again. While his blood pressure seemed to be coping with that, his lactate levels were rising which indicated that something had to be done. He is back on the pacemaker and on an amiodarone infusion. His surgeon, cardiologist and intensivist have all reassured us that mostly these arrhythmias will completely settle down and that this is more nuisance value than anything else.
On the plus side he had his mediastinal drain (next to his heart) and his peritoneal drain (in his abdomen) removed today. His pain seems well controlled and he now is only on a fentanyl infusion and paracetamol for pain as his clonidine infusion was also stopped last night. His dopamine infusion was also weaned off last night. His chest drains are still yielding a reasonable amount of fluid and they will have to stay in for a reasonable bit longer.
All in all we are moving forward slowly. It is not really a surprise, we knew we were in for this and we are always happy when progress is being made.

Getting better

William has improved a lot today. He was still uncomfortable and at times quite cranky but he is much better than last night. He continued to improve over the day and this afternoon he was able to catch up on some sleep. He has been drinking water very well and although upset that he is still sore and has so many tubes still attached, he is getting better at coping. When he is upset he moves his legs around but still keeps his arms (both of which are still attached to lines) quite still. He tells us in sobs "It is finished, the operation is finished!" The poor little bloke, he thinks the operation is a bit like his tube feeds and once it is finished we can disconnect him and let him go. Still, he is getting calmer and tolerating things much better as the hours tick on.
Today his pacing was gradually turned down until his own rate took over, and the infusion of amiodarone was stopped. His clonidine infusion has been weaned down and will stop in an hour or so. Significantly his atrial catheter (a tube going directly through his chest into his heart to measure pressures) was removed without incident this morning also. We were able to replace his oxygen mask with nasal prongs this afternoon. And all the numbers on the machines are looking good!
All in all William is having a very good day.

The Fontan

Major heart surgery on your son - it certainly does not get easier with repetition.
After a restless night, we woke at about 6am to shower and dress and get William ready to go up to the hospital.  We were supposed to be there by 7:00am and Liza & I were both looking at each other with that knowledge that we were feeling the same thing: That dread of seeing our little boy suffering, the fear of the unknown, the effort of looking to the positive without being totally able to ignore the possibilities of the negative.
William of course was much better than us.
"Come on, let's go to the hospital!" he cheerfully encouraged us as soon as he had his slippers on. He wanted to play.
We carried him up to the day surgery admission suite and after checking in with the ward clerk, we waited for about an hour. He had been here for pre-admission last Friday so there was little to do until the anaesthetist arrived. He had a fantastic time playing with all the toys and having a few stories read to him. He was not in the slightest bit perturbed about this morning.


At 8:00am The anaesthetist came out and I carried him into the operating suite as he waved goodbye to mummy. Our wonderful anaesthetist sang to him as he edged the mask closer and William remained very calm. Eventually he started to turn up the gas and Wil struggled a bit but not for long. It is a hard thing to walk out on your unconscious son but he was in the best of hands.
Liza and I ate a breakfast which we barely tasted (probably a good thing). We sat, and talked small talk, read the paper, wandered around a bit getting a few little tasks done waiting for the pager to signal that we had imminent news.
At 11:40am it sounded out and we made our way once more along that unfortunately familiar path to the paediatric intensive care unit. Our first news on arrival was that he was on his way back, to please take a seat and wait. We complied and after about half an hour, two of the ICU nurses emerged to introduce themselves. Emma and Sarah would be looking after William, who was still not back yet but they knew he was stable. A little wait longer and Prof Chard came by and told us that everything went according to plan. We were really waiting for that news! The only complication was that William was in a junctional rhythm and needed atrial pacing (an external pacemaker with wires triggering off the atrium to contract) He assured us that this was not uncommon and it usually settled in a day or two. We saw Richard Chard again as he came back out of ICU and he told us that William was now on the ward and  that they had extubated him! We had honestly expected him to be ventilated for at least the first day. If he was coping with that then we were indeed very lucky.
Intensive Care is never that much fun. Whilst we are reassured by the level of care, he is there for a reason - he is still not very well.
This afternoon was tough. For the first couple of hours he moaned with each breath. He could not focus on anything when his eyes were open, his respirations were splinted and he was often really upset and in significant pain. His morphine was increased to very high levels but his pain was still not controlled.
He would hold our finger in his right hand and squeeze it tight but when I noticed that his left hand was still with no spontaneous finger movements, I really felt my heart in my mouth and my intestines in knots. He kept his eyes and head over to the right and in between painful cries he would listen to us and wiggle the fingers of his right hand when we asked him but his left hand stayed still.
Dear God, please do not let this be another stroke I prayed.
After about half an hour (but it felt like an eternity) he was moving his left thumb spontaneously and several hours later he was gripping with his left hand. The relief, inversely in proportion to the fear, was wonderful!

Later that afternoon and several times during the night William became very agitated and started thrashing both of his arms and legs around violently. He eventually settled with pain relief and sedatives and luckily he did not pull out any drains or lines. Given that the morphine was not helping enough, he was changed to Fentanyl and Clonidine which seemed to be much better. He was still not terribly comfortable but who could blame him? He still had two chest drains, a mediastinal drain, a peritoneal drain, pacing wires and an atrial line all competing for space at the bottom of his chest. He had all the tubes of his jugular central line taped to his neck and one arm was boarded up with his venous lines and the other with his arterial line. His urinary catheter, gastrostomy tube and oxygen mask completed the tangle of tubes that are part of intensive care.
By the time I left after midnight to catch a few hours of sleep, William was sleeping for longer periods and was getting easier to settle.
Despite the pain, William was doing well haemodynamically. His blood pressure stayed pretty good and the pressure difference across his lungs stayed low - just as we wanted. In all the concern about his pain, we forgot momentarily to marvel at his pink lips and oxygen saturations in the high 90's.
This was the new William and he was continuing to improve.

William, The next instalment

William's progress has been gradual but steady over the past couple of years.

He is certainly growing! Wil has been steadily following the average rate of growth and now is only 1cm shorter than Tom was at the same age. He is now 20kg and 102cm tall!

His speech has been coming along gradually. He still attends speech therapy weekly and usually can make himself understood. His pronunciation still needs a bit of work but he is definitely getting there.

Eating is still the bane of our life but there has been some progress. While William still requires four tube feeds through his gastrostomy button each day, he is eating a little. We get two to three teaspoons of custard into him every day and he will have a go at an arrowroot biscuit every now and then (although more goes down his front that in his mouth). He drinks water reasonably well now and will now ask for water when he is thirsty. He still baulks at taking milk or other drinks from a cup but will often have a 'taste' of other foods, especially if the other kids are eating them. We have no doubt that out genes will shine through and William be be a great eater.

Of course the other area in which we hope William will emulate his parents is toilet training. Unfortunately he is still in nappies but we are hopeful that this will not be for much longer.

Wil does often get short of breath and tired at the moment. From his last operation until about January this year he had maintained oxygen saturations of around 80% but since January he is more often between 67-71%. He loves to be active in short bursts though and often will run as fast as he can (especially if cuddles are at the other end of the run!) He loves to rumble with his brother and sister and for William a big treat is being carried upside down. Surprisingly he loves it! One of William's favourite pastimes is teasing his older brother, Tom. He is very accomplished at it, the cheeky little thing!

William goes to swimming lessons every Saturday morning with 'Liv & Tom. Recently he graduated to the tadpoles class which means that Dad does not have to get in the water with him. Wil loves swimming and he copes reasonably well.

He also loves preschool. He has been going to preschool for almost two years now. His first day of preschool was his first day of walking. We went through so many pairs of trouser with his little bum shuffles, it was great to see him up on his feet! Preschool was also great for peer pressure and it was then that Wil started to put more food to his mouth to be like the other kids.

Wil is also going to Noah's Ark early intervention centre every week in school term. He really loves that almost more than anything else. He spends two hours in a playgroup with a physio, speech therapist, occupational therapist, early childhood educator and other various therapists. It has been absolutely wonderful to have this available locally.

If you ask William what his favourite thing is he will invariably answer "The Wiggles". We are constantly bombarded with music requests for "Wiggles!?" every car trip no matter how short. He will re-watch videos a million times over and he loves his wiggles shirts and other paraphernalia. Last year we went on a driving holiday up the coast to Queensland. As far as William was concerned everything we did was just incidental to the trip to 'Wiggles World'! He was so excited. when we got there. He still talks about who's seat he sat in on his various rides in the big red car.

In 2008 we took the kids on holidays to Fiji. It was fantastic! Whilst our kids were very content to swim in the resort pool and saw no need to swim at the beach or see coral, they loved the tropical environment, the friendliness of the Fijians and of course the plane, bus and boat trips we made were also huge highlights for the little ones. It was a wonderful opportunity to just go somewhere beautiful and to really relax.

We have been making our annual pilgrimage to Charlotte Pass each year too. Last year after an early start and a long drive, when we finally arrived and got suited up to take the kids up to the beginners area they were all more than a little tired but could not be dissuaded from heading out into the snow. I had to duck into the surgery for literally 30 seconds and I asked Wil to sit down on the snow. When I came out he was lying supine, arms outstretched, fast asleep in his little hooded snow suit. With his lips their usual blue, I'm sure it made a few people look twice and it made me look very neglectful!

We spent time with our extended family at Murramurang near South Durras last year. Again the pool was a real hit, as was the nearby Mogo Zoo. I think Wil found the wildlife right outside our cabin just as appealing though.

Last year William had a cardiac catheter performed by Dr Cooper at Westmead Children's Hospital. Everything looked pretty good and his pressures and valves were all fine. We had assumed that his next operation would follow soon after - he had reached the 15 kg mark and after all, the catheter was basically to check pressures in preparation for the next round of heart surgery. It was a strange mixture of relief tinged with the prolongation of the tension associated with waiting when Stephen Cooper told us that William could wait another year for the surgery. He would cope with it better emotionally when he is almost four rather than almost three. It was reassuring that he was doing well enough that he was not in medical need of this operation immediately and of course we were not wanting to put him through all that the surgery entails before he needed it, but we knew it was only a postponement.

And so here we are, on the eve of William's next big operation. Tomorrow William will have the third of his planned surgical procedures at Westmead Children's Hospital. He is having a 'Completion Fontan' procedure. Currently the deoxygenated blood coming from the lower part of his body via the inferior vena cava goes straight back into his heart without travelling through his lungs. It mixes with the blood from the upper part of his body which has come through his lungs and is pumped around his body again by the right ventricle.

This procedure will disconnect his inferior vena cava from the heart and divert it into the vessel going into the lungs. This will essentially mean that all the blood coming back from his body will flow through his lungs and he will have normal oxygen levels in his blood for the first time in his life. Unlike the rest of us though there will be no pump to push the blood through the lungs (normally the right ventricle does this job). His blood will flow passively through the lungs but from there the fully oxygenated blood will be able to be pumped around his body, hopefully improving the way he feels significantly.

Stay tuned for more updates...

Small steps

William has been doing quite well.

His heart has been doing all the right stuff with the cardiologist remaining very happy with how things were going at his last review. He even extended the time until the next check-up to six months - a great endorsement of Williams stability at the moment.

On the feeding front William has been making some progress. He has been having intensive speech pathology and has actually swallowed two teaspoons of water! He will put a spoon into his mouth now, and will even accept tastes of things (if he is in the mood). He has a few foods which he will repeatedly put onto his tongue himself!
He loves to imitate his brother and sister and he really enjoys sitting up at the table at mealtimes with his bowl and spoon.

Will's mobility is improving with an increasing amount of time spent on his feet. He will walk holding on to someone's hand, will cruise around furniture and just over a week ago he took his first two steps on his own!

He is a gorgeous little boy with a beautiful nature (even though that tends to being a little too cheeky at times). He is a joy!

Hello, I can stand!

William has been pretty good in the last few months. His heart is going well and Steve Cooper has pushed out out reviews to six monthly!

His eating is still a major issue (or rather a major non-issue!) He has improved to the point of putting non food objects into his mouth at times and at meal times he will happily put a spoon up to his mouth. The moment it has something on it though, it goes nowhere near his face.

He is seeing a speech pathologist on a weekly basis now.

William still does not really speak. He does say 'hello' to anyone and everyone (unless you ask him) He says 'bye-bye', 'mum', 'dad' and 'moo' (the latter in response to the cattle he can see from his window)

The bum shuffle has evolved. When excited, William gets air between bum touches – he actually bounces along. If he is allowed to spend some time outside and manages to slide along on a rough surface, he will wear the seat of his pants out very quickly. Recently when we were on holidays, William was going through a pair of shorts every day for a while with his exploring!

He has been standing up recently though so the days of the bum shuffle could be numbered. At present he will pull himself up and hold on to furniture but will not yet cruise around the place on his feet. He knows that he gets plenty of praise when he stands so sometimes he will sit then stand four or five times in quick succession. After that he gets too puffed.

We recently took a week off to go to Vincentia for a beach holiday. All of the kids loved the beach and swimming. William at first did not really like the sand. We deduced that by the way he would balance on his bottom with both his feet and hands held up in the air while crying. He soon got used to it though but the water was his real love. I would place him on the beach and after a while he would put his feet down and shuffle off towards the water at quite a pace, his bottom leaving a round groove all the way to the waves. He would then sitting in the whitewash absolutely loving the little surges that would hit him. Occasionally he would almost be pushed backwards completely, only saving himself by getting a hand behind him just in time, giggling and laughing the whole time.

He is such a precious little boy.

Holidays with Pos and Pa

We have had a really good month. We are still amazed by the difference to our lives with William not vomiting. He still gags quite a bit which is still distressing. It is also of concern because it may contribute to loosening of the fundoplication.

William has really started to grow though. He was last weighed on Monday at an appointment at Westmead Feeding Clinic. William has finally burst through the 10kg mark! He has really been catching up on some growth now that he is keeping all the nutrition in his body!

In August, we spent some time down at Charlotte Pass where I do a locum with a bit of work and a bit of skiing and the family get a holiday. William's 'Pos & Pa' took care of him for these two weeks to try to keep him safe from the germs that were lurking. As Liza and Olivia both got influenza down there, and Thomas and I also got viral infections, it was a very good thing. William still caught something after we got back but he coped pretty well and got over it quickly.
He loved staying with his grandparents but we did miss him so much! Pos bought him a teddy bear which ended up with its own tummy button so William could feed and 'bubble' him as well. He loved having the run of their house but he especially loved playing in the garden.



William is very happy with his bottom shuffle and shows no desire to walk or even stand just yet. He saw the neurologist last week who was happy with some aspects of William's progress but advised us to get a little help with some of the other stuff. Certainly his talking is behind the average for his age but he is starting to say a few things quite appropriately. With a bit of speech therapy I am sure that the day when we can't get him to stop will be upon us sooner than we know it!

The area of William's development in which we have seen really exciting improvement in the last month is his progress towards feeding. He is still not taking any food by mouth but he will let us touch his mouth now and will even put a spoon up to his lips himself. It sounds like small stuff but the progress he has made in a month and a half is far more than he got in the preceding nine or ten! And the wonderful thing is that he is enjoying the games we play trying to get his feeding happening.

He is a cheeky little boy at times but very lovable! He certainly does not give up the opportunity for a cuddle when he can get one and we love to indulge him.