New mould

We received a new hearing aid mould in the mail yesterday and this has fixed the squealing problems with his left ear. Finally we have squeal free hearing aids; at least for the moment.

Paediatrician

We had a quick paediatrician consultation today to review Raphael after his hospital discharge and to talk about the results from the tests. The tests were all clear btu this still means that we don't realy know what has been giving him the last three high temperatures. We are guessing that it is just cold viruses.

We talked about the possibility of trialling Raphael on oral feeding without using his NGT before we make the current solution more permanent with a G-tube. Our paediatrician doesn't think that it will be successful but is happy for us to try. Her main concern is that the increased volume taken by mouth might result in an aspiration pneumonia and if this happens then we will have to abort the trial.

On another note, even though the latest antibiotics have given him severe diarrhoea, he reached 10.0kg today; Yay!

Cruising

Raphael cruised for the first time today. He climbed up to the arm of an arm chair and then when I offered my hand, just as a stable hand-hold, he grabbed hold and walked sideways toward me. Then he tripped over my shoes that I had left on the floor.

Oh no, high temperature again

After Raphael's snooze yesterday he woke up very groggy, he slumped in my arms all afternoon and evening. We took his temperature at 39.1 degrees and so we called the home care nurse. She came to have a look at him and after further consultation with an intensivist we gave him some Paracetamol and waited till the morning.

Last night was the best night's sleep that we have had in a very long time there were no alarms (apart from the morning NGT of course) and his saturations were 97%-100% all night. He woke up in the morning back to his usual self.

The home care nurse came again to check him out in the morning and after measuring no significant temperature she took a nasal secretion specimen from him for analysis. We expect the results to be available in a couple of days.

Free again

Raphael's regular paediatrician discharged him today so I finally have him at home. He had me worried for a little bit when he coughed and coughed for several minutes. But finally he managed to expel a small flood of mucus and then he was happy again

Nearly escaped from hospital

A paediatrician came around in the morning and was satisfied to send us home in the afternoon after another dose of IV antibiotics. But before midday one of our paediatric intensivists came to see us and said that she was not happy about Raphael going home today and that she wanted him to have close observation tonight. She wanted him to have a night in hospital without any oxygen before sending him home.

Because he has had a spiking temperature over the past few weeks, she is concerned that he will be back again in another couple of days unless they do everything that can be done in the hospital. So he is stuck in the hospital again at least for tonight.

DEM

Our home care nurse arranged the emergency department so that we only had to wait for an hour before Raphael was seen. After the registrar had a poke and prode, she ordered oxygen for him and arranged an xray and a bed on the ward tonight.

Making appointments is always very difficult because we have so many of them and it is impossible for us to be able to remember when we are free. So I bought my wife a new PDA phone to give her access to her calendar wherever she goes. Today I am passing time in DEM (Department of Emergency Medicine) by writing this blog entry on it. I am beginning to wonder whether I bought this phone for my wife or myself.

Back to hospital

Raphael had a high temperature last night and today he has also become grumpy along with maintaining his high temperature. A home care nurse spoke with an intensivist who has advised us to come into the hospital.

So as I type, Annie is driving Raphael to the hospital and I will walk down from work to meet them there in a few minutes.

Disability Services

My wife met with a representative of disability services today about arranging continued respite for a few hours a week after the current temporary arrangement ceases.

This looks like it will go ahead and so we are very happy about this.

No alarms last night

Wow, we had a whole nights sleep last night with no alarms; except for when the overnight NGT feed finished and when our other sick kids got up and needed attention in the early morning.

This is the first full nights sleep that we have had with no CPAP or O2 alarm since we started with the CPAP.

Intensivist followup

This afternoon we went to have a follow up with the intensivist who fitted Raphael's CPAP mask.

We mentioned that our biggest problem was that the alarms go off at night and wake us up. He reminded us that this was not going to be a picnic but then offered to arrange a new massimo(sp?) monitor for us that is less likely to alarm erroneously. We will have to wait for quite a few weeks before this can be arranged, unless we had a spare $8,000 to buy one for ourselves.

One of the home care nurses was at the consultation and as the intensivist was was leaving shortly to catch a plane, and would be away for a few weeks, he asked the nurse to arrange the equipment for us. The look of "this is impossible" crossed her face and the two exchanged a few words about who to speak to to get this done. By the end I think that the poor nurse still felt that it was a monumental task.

Later that night the home care nurse dropped off a new mask for us becuase the old one has a small split in the nose cushion that is starting to widen. But unfortunately it was the wrong mask. The device that she brought was actually connected to the sort of hat that we were using and so this is where the confusion must have happened. The current mask still works so we will keep using that one until the replacement arrives. The nurse also adjusted the CPAP level back down to 8.0. It was raised to 9.0 when he had his last cold but since he is getting over it the intensivist advised lowering it back down again.

Early learning

My wife took Raphael to Early Learning this morning I believe that the general advice was that the Physiotherapist was happy with Raphael's development but the OT (occupational therapist) thought that he was lagging quite a bit.

The problem with his fine motor skills might be becuase other children his age are picking up small pieces of food with their fingers to eat but of course he doesn't get this experience; he can't even eat mashed potato, let alone a whole sultana. So we are going through our toys looking for things with small things to grab hold of. Of course we can't just give him tiny things because of the risk that he might put them in his mouth and choke on them. The only thing that I can think of at the moment is baby puzzles that have the little knobs on the pieces to pick them up by.

My wife believes that Raphael has made attempts at verbalising a few words:
All done: "Ada"
Zài jiàn (Chinese for goodbye): "Dyadya"
Gěi: (Chinese for give): Ge

She claims that the evidence is that:

• the sound is approximately correct
• he has said these things more than once; and
• he says them in conjunction with sign language (or action in the case of give)
  

I think that Annie is still being a bit optimistic about this being speech. I suspect that this might turn out to be another "head scratching incident" as he has not signed mummy in a very long time. But regardless of this we will use his sounds back to him (and with the correct pronunciation) to reinforce this behavior and with any luck he will learn that he has actually said an understandable word and not just a coincidental grunt.

Hearing aid adjustments

We visited Australian Hearing this afternoon for some adjustments to Raphael's hearing aids. The first task was to fit a new right ear mould. This latest mould fits far better than the previous attempts and so we were hoping that this will stop the dreaded squealing.

We then tested his hearing with another VROA test but this time he was tested with one hearing aid in at a time. This showed that his right hearing aid is boosting his hearing ability down to about 25db which is good enough for conversational hearing. His left ear however still behaved poorly even with his hearing aid.

The Audiologist further amplified the relevant frequencies of Raphael's left hearing aid but noticed that he mould is getting a bit loose. she took another mould of Raphael's left ear to get another mould made (that sentence is a bit confusing, I wonder what the real terminology is). This new mould will be posted to us when it is ready.

Sure enough, by the time we got Raphael home, both hearing aids were playing a chorus of squeals. Annie will be going to Aus hearing on Friday for the RIDBC teleconference and so she will ask them to do something about it then.

The factors are working against us:

• His right ear doesn't have enough cartilage in it to hold a hearing aid properly and so any movement, jogs the aid out slightly and starts a feedback squeal. The new mould is better but still not perfect.
  
• His left ear requires more amplification but because the aid is a little loose, this amplification has resulted in the left aid squealing.
• The combination of: cold weather, low set ears and not much neck to speak of means that his clothes are usually pressing the bottom of his ears when he is sitting and this of course dislodges the moulds slightly causing more squealing.

Development observations

He currently has seven teeth and they look ok at the moment (not that I am a qualified dentist).

He is now able to manipulate two objects in one hand. We can demonstrate this by handing him three objects one at a time. The first he takes with his right hand, the next he takes with his left hand and when offered the third, he sometimes transfers an object from one hand into, the already occupied, other hand before taki9ng the object.

Raphael is learning his bed time routine well. Last night Annie and I were going about the business of setting up of of his night time equipment when he lifted his leg in the air when we went to put his oxygen monitor on his foot. This isn't the first time he has done this and we are now convinced that he has learned this behaviour. He has only been on CPAP for a very choppy/interrupted month and so it took us by surprise that he has learned to help us, by doing this, so quickly.

On the down side, he has vomited three times today so far. He is still not very well.

Incidental trip to the ENT

My other son was at the ENT doctor today and it turns out he is definitely going to need surgery as well (sigh).

We took this opportunity to get the ENT to have a quick check of Raphael's grommets becuase we are a little concerned that he might be getting some wax build-up. But the ENT said that it all looked good. There was some wax in there but it was not in the way and it would be unnecessary to clean it out.

Higher temperature

last night a home care nurse visited us again to check on Raphael's progress. His temperature had risen to 39 and he was quite placid. Normally when he has high temperature he is grumpy. Being a little concerned, the nurse contacted our paediatrician again and she sat with Raphael while he slept for an hour to make sure that he was going to be ok at night.

He was fine all last night and the only equipment alarm that went off was the NGT pump which just an easily fixed kink in the tube that stopping the flow at one point.

Without this fabulous service and care from the home care team we would probably have had to admit Raphael to hospital last night so I am very grateful to the people that make this service possible.

Bronchiolitis

Home care nurse visited again this morning to check up on Raphael. She is concerned about him and thinks that he is a borderline case of needing oxygen overnight tonight because of his respiratory difficulties. We are not equipped at home to supply oxygen to Raphael with his CPAP so he would have to go to hospital if he needed this.

We put him down for a morning sleep with his CPAP machine and agreed to take him into the ward tomorrow for a Paediatric resident to check him. I personally didn't think that he needed to go to hospital but I was not going to reject the advice.

He slept very well with the CPAP and I took him in after he woke up. I believe that the resident was a bit overwhelmed by Raphael's condition. Raphael always appears to be in slight respiratory distress, even when he is well, so it is hard for doctors to decide whether he needs close monitoring in hospital. In the hospital his temperature was measured as 37.5 and it was noted that he had more respiratory distress than normal but was well hydrated. The resident called the on-call paediatric consultant, who just so happened to be our paediatrician, and returned to us with the same opinion. Everyone agreed that he is a borderline case for hospitalisation.

This places the decision on us and so I brought him back home with the advice to bring him into emergency if we think he heeds to be hospitalised.

Stupid machines!

Last night a home care nurse visited to see if anything could be done about the pressure sores that Raphael has been getting on his forehead from the CPAP mask.

Fortunately when she was visiting she also noticed some of the problems that we had been having with the oxygen monitor. She ended up going to get another one and taking the faulty one away.

Last night was not a good night in terms of alarms. Even with the new Oxygen monitor the alarms came thick and fast from the machine last night. Raphael is sick with a cold but was not having bad desaturations; he was coughing a lot which caused him to move which, in turn, resulted in faulty readings for the Oxygen monitor.

At about 3:00am we gave him some infant cough medicine (Demazin). We also turned him onto his other side and within thirty minutes his saturation monitor showed better readings and he did not wake up for the rest of the night.

While we were having problems with the probes at night we tried something new. Rather than move a probe when it was causing problems, we simply added another one to another foot or hand and connected it to the machine to see if the new location would be any better. In the morning I discovered the problem to this approach: Raphael had manage to tightly bind his feet together and was working on tying his hand probe tail to the NGT chord. It took me ages to get him untangled and at one point I too managed to connect myself to the octopus of chords and Elastoplast tape.

Coming surgery

We currently don't have a date for Raphael's next surgery. We are waiting for Raphael's anaesthetist to return and then, presumably, a date will be arranged for the surgery.

We expect the following will be performed:

• ENT endoscope (performed after the pre anaesthetic medication but before the GA is issued)
• tear duct probe
• G-tube insertion
• fundoplication (here is a video of a fundoplication performed by laparoscopy. Raphael will actually have his performed by laparotomy to reduce the risks of complications.
  

One appointment becomes four

This morning We had an appointment at the hospital for our other son. This just so happened to be with the same surgeon who is scheduled to perform Raphael's coming surgery.

We had Raphael with us and at one point the Surgeon stopped and commented on the nasty cough that Raphael has barking. He said that this might be an issue for his surgery next week. When we said that we were happy for Raphael's preferred anaesthetist to make that decision on the day before the surgery the surgeon replied by saying that the anaesthetist in question would be on leave, and would not be back for seven weeks. Annie and I were dumbfounded. We thought that we had already confirmed that Raphael's anaesthetist would be available and so we didn't know what to say. Later the surgeon confirmed for us that particular anaesthetist would definitely not be available.

Due to an administrative error we still had a paediatrician appointment today that should have been cancelled. So we took advantage of this appointment and had a consultation with our paediatrician about what we were going to do. She was a little concerned about his current cold symptoms and so this, mixed with the absence of Raphael's anaesthetist, resulted in her suggesting that we wait until after his anaesthetist is back. Annie and I were relieved with this assessment.

While we waited between the two appointments we made two impromptu appointments with the hospital social worker (to ask about respite options) and dietician (to talk about his weight gain and dietary requirement).

Radio Interview

Today I gave an interview to a local radio station about how good the Royal Hobart Hospital has been for Raphael. It was very short and superficial but I hope that it is enough. It is for an appeal to raise funds for the "give me 5 for kids" programme. The money raised will go towards purchasing medical equipment for the Royal Hobart Hospital to enable kids to go home rather than be forced to stay in the hospital.

The presenter thought that the programme would go to air on Tuesday coming but he was not sure about that. He offered to contact me closer to the date to let me know when it will be on.

More notes on development

Raphael has started to make more sounds:

• "dya-dya-dya"
• "adaadaadaadaa"
• "aiyaiyaiya"

I think that his hearing aids are doing him a lot of good!

He is also proficient at getting hold of things and pulling himself up to his feet, via the correct half kneeling position. He has even just started to stagger a couple of steps while being supported. He hasn't learned how to get back down to the ground elegantly though.

Ophthalmologist 2nd opinion

This afternoon we took Raphael to RHH to see an orthoptist and to get a second opinion from an ophthalmologist, who has specific experience in genetic and paediatric ophthalmology.

The orthoptist tested Raphael's vision with rather peculiar grey boards with black and white stripy lines on one side and a tiny hole in the middle to look through from behind. She span the board around till I felt dizzy and then stopped and looked though the little hole from behind to see if Raphael looked at the side of the board with the stripes. She repeated this with boards that had finer and finer stripes until she declared that Raphael's vision appears to be quite good; at least the vision out of his good eye is quite good anyway.

The ophthalmologist consultation was very interesting. The result from the pressure test on the 18/4/2007 was that the pressure in his right eye was 15mmHg (this is well within the safe range). He examined Raphael and said that Raphael had a coloboma in his right eye as well as the left. This right eye coloboma was small and very low down which meant that it would probably only effect the very top of Raphael's vision. He also recommended that Raphael's tear ducts only be probed and not flushed when they are examined in his coming surgery.

Topics covered:

• Raphael's general vision
• Colobomas
• Eye pressures
  
• Probing and flushing tear ducts at next surgery
  
• What can be done to help his left eye
• Retinal detachment
• Critical developmental ages for eyes
• Eye sizes
• Optic nerve hypoplasia and micropthalmia
• Type of eye specialists
• potential treatments in the future

Hearing aid mould modifications

I took Raphael to Australian Hearing this morning for another attempt to resolve the squealing right hearing aid. The Audiologist cut excess material from the existing mould and took a new impression to make yet another mould.

Out of hospital

Raphael has finally escaped from hospital today. We have a course of antibiotics to give to him over then next five days.

In hospital again

Raphael had a hard night on Thursday 31/5. He coughed up mucus into his CPAP mask and we think that he may have inhaled some back into his lungs. He didn't have a temperature but he was starting to cough, reminding us of previous times when we had to admit him to hospital. After taking the mask off and putting him on his stomach he coughed up a lot more mucus. Annie and I couldn't agree on whether to refit his mask again and so we called on a home care nurse again. The nurse visited us and had a listen for any problems in his lungs and we eventually refitted his mask. The rest of the night was generally uneventful (apart from a few "high leak" mask alarms)

Early Friday morning (1/6) we called on the home care nurse again because we found that Raphael had a high temperature. Sure enough we packed up and went into hospital again and dumped our other kids on one of our baby sitters. He was inspected by a doctor and a chest x-ray and blood was taken. The verdict was that Raphael probably just has a viral infection that isn't causing pneumonia yet but given his history, and condition, it would be wise to admit him over night to monitor him if he gets worse.

Our paediatrician was on the paediatric ward and so we managed to ask her our two burning questions about the surgery coming up on the 12/6 (it has been brought forward from the 14/6). We wanted to know what people can eat after having fundoplications and we wanted to make sure that our preferred anaesthetist was going to be able to perform the anaesthesia on the modified surgical date. Her answers put our minds at rest and we are all ready to proceed with the surgery on 12th of June.

Later that day a registrar tried unsuccessfully to insert an intravenous line to give him antibiotics (just in case). Eventually he was given an injection into his muscle to slowly release antibiotics over a period of twenty four hours. Apparently this is much more painful for the child and so it is blended with a local anaesthetic to make the pain go away quickly. The irony is that the anaesthetic causes more pain while the injection is being given.