Busy day with appointments

I had a hectic day yesterday, but here I will just talk about Raphael's appointments.

In the morning we had a consultation with a physiotherapist with lots of standing and walking practice. Handling uneven ground and very small steps is the next objective for him now. Also we will try throwing objects while standing up. I think we will be able to find a good game of throwing ping-pong balls at each other while standing.

He is confidently walking with a trolley that I hand customized for him. I added swivel wheels to the bottom of a normal child's walking trolley so that he can swing it around on the ground easily. I made it with some old bed wheels which were quite high and I was planning on going to buy some lower ones becuase initially I thought that it would be too unstable. But now looking at it, I am happy that it is not rock solid as it makes him practice his balance skills more and not just lean on the trolley all the time. He loves it and he seems to be walking with it all the time so I am happy that I spent the time on the modifications.

Later in the day we had an ENT appointment to book in his Adenotonsilectomy surgery for May when his normal ENT returns from being away. One of his grommets has probably extruded (fallen out) and is just sitting in his ear canal. Apparently it can take weeks for a grommet to travel down the canal in normal conditions but putting his hearing aids in all the time might be perpetually pushing it back up the canal and not permitting it to fall out by itself. When he is under anaesthetic for his surgery in May they will have a thorough look and see if they can just be taken away they will also look for any more sign of "glue" in his middle ears.

We also had a meeting with a home care nurse about his CPAP mask. It is starting to get too small and it is tricky to get on him at night without obscuring his nostrils. We had tried a larger one but it gave him a big blister on his cheek. We will try taping up his face with soft tape and giving the new mask another go tonight but I am not confident in succeeding with this.

We were also planning to see one of his intensivists but she was stuck in an emergency case so we abandoned that plan and will proceed without the consult until we exhaust our current CPAP ideas.

Post grommets ENT visit

Raphael saw his ENT today and she had a look at his grommets. She said that she was able to see both grommets despite the narrow external ear canals and that there didn't seem to be any problem.

We talked about the possibility of doing a videoed endoscope just before his next general anaesthetic, after he has been given drying medicine to clear up his secretions. The purpose of this is to confirm the problematic laryngomalacia around his vocal cords.

The ENT commented that the possible evidence of congenital cholesteatoma was probably just fluid in the ear and not actually skin. Apparently the ear drum is transparent and inspections does not show cholesteatoma. In addition there was no mention of this on the MRI.

During the consultation Raphael made a new sound "nyanyanyanyan". This is the first mixture of consents and vowels that I have heard him make.

The four procedures

Grommets, ABR, CT scan and eye pressure test were the four tests to be done on my boy today.

My wife and I arrived shortly after 7:00am to the hospital. Raphael had already been given some preventative ventolin and the anaesthetis arrived shortly afterwards to give him some a drying medicine (to help with his secretions during the operation) and some other stuff. Actually with the number of syringe fulls of drugs that the anaesthetist injected into his line, I was half expecting Raphael to bulge a bit.

His fluid line was still in and the nurse came and asked us to put him in his baby hospital gown so we stripped his clothes of but of course his singlet could not be removed from the line Annie and I had a good chuckle about all of the possible ways that we could potentially get his singlet of him but eventually resigned ourselves to the fact that he would go into surgery with his grotty singlet dangling from the IV tube

Annie and I prayed that we would see his cheeky smile again.

Finally we brought him to the day surgery area where he was going to start his procedures with the insertion of grommets. We even ran into his normal Audiologist who had postponed her travel just so she could perform the ABR test on him.

After a short wait, I was asked to put on a surgical gown and hat in order to bring Raphael into the theatre. After several embarrassing attempts to correctly put on the surgical gown the nurse said that'll do. I can only assume that she would have a chuckle at my expense later, she did a good job at holding a straight face while I was fighting with it. In hindsight it really wasn't that hard to do. I can only assume that my mind was somewhere else completely, preventing me from completing even this simple task.

After wheeling him into the theatre and putting him on the operating table the surgeon advised me that the ophthalmologist would arrive in fifteen minutes to perform the eye pressure test. After that they ushered me out of the theatre and back to the waiting room.

We knew the procedures would probably take over 2 hours (if everything went well). After about 3 hours we were told that Raphael was awake and we went to see him in recovery. He was in a little pain and his eyes and lips were swollen from fluid (not an allergic reaction). He was in much better shape than last time he had a GA. We are grateful for the special preparation that the anaesthetist organised for him.

That afternoon he was hooked up to an oxygen saturation monitoring machine and while he was having an afternoon sleep he had two instances when he could not breath properly and had to be turned onto his side to get him breathing again properly. He had been given some codeine to help with his pain and since his episodes I heard a rumour that opioids like codeine can cause resperation difficulties in babies that are already at risk. This made me question why he was given codeine. I was told that the issue was that he had already had paracetamol, and aspirin was a bigger risk after surgery. There was also mitigation for giving him codeine in that he was given the minimum dose and also that there was a nurse constantly in the room monitoring the oxygen saturations of the kids in the room. It all turned out ok and to put this in context he has previously demonstrated that he can desaturate under normal circumstances when he is sleeping on his back so it may have had nothing to do with the opioid anyway.

Later that evening he even gave a smile although he wasn't quite back to his usual self yet.

Consenting to the procedures

I am finding this decision quite stressful, particularly because Raphael's Paediatrician was away at this time. In my mind I kept going over the pros and cons (as I saw them) of doing each of these procedures. The following were the major points that effected my decision on each procedure:

Grommets
Grommets pros:

• Good chance to improve hearing and therefore verbal and communication skills. I believe that Raphael is at a critical age with regards to learning communication and I am doing my best to learn Auslan (sign language) and sign to him whenever I talk to him. But verbal communication could give him so much more in life and so I am very keen on doing all that I can to allow him to do this.
• Tubes are automatically discharged after six to eighteen months. This means that he won't necessarily have to have an operation to remove the tubes when they are no longer needed.
• No longer have to worry about damage to facial nerve due to pressure build-up in the middle ear.
  

Grommets cons:

• Increased risk of ear infection. I believe that these can be adequately fought with antibiotics and, in the worst case scenario, another surgery can be done to remove the tubes should they prove to be causing more problems that what they are solving.
  
• His ear tubes may vent his secretions. Although this may be only aesthetic, this can also lead to an increased risk of ear infection. I don't want to down play the aesthetic nature of this too much; it saddens me to admit that Raphael gets less kisses and slightly different cuddles than my other children received from me because of his copious slimy secretions from his nose. I have to assume that if his ears leak goo, then this will also change the way in which I show affection to my son.
• Tubes are automatically discharged after six to eighteen months. I realise that I also listed this as a pro, but if the middle ear fluid problem persists after this time is up then he may need to have another operation to fit new tubes.
  
• Requires a short general anaesthetic, but this still has higher risks for Raphael than for most children. The biggest disasters that I can imagine coming directly from this procedure are death, brain damage from lack of oxygen, and/or potential irreversible damage to ears. I see the risks of these events as real but very very small
  

Grommets decision:
I believe that the risk of not doing the operation outweighs the risk of doing the operation. We have decided to consent to this procedure.

Video of fitting grommets that I found on the web

ABR (under general anaesthetic)
ABR (under general anaesthetic) pros:

• Achieves a high likelihood of accurate results which can be used to programme Raphael's future hearing aids accurately.
• Doing the test at the same time as other procedures means that there is some benefit in not having to have multiple GAs.
  

ABR (under general anaesthetic) cons:

• ABR can be performed without a general anaesthetic. But in this case there is no guarantee that it will be possible to gain accurate results; and past experience shows that it is unlikely that good results will be obtained while just sleeping.
• The procedure takes about an hour. This is a long time to keep him under GA.
  

ABR (under general anaesthetic) decision:
This was a hard decision. I would prefer to be able to ask more questions from Raphael's paediatrician about possible alternatives before I commit to consenting to this procedure but for now I am happy to trust the ENT doctor and anaesthetists opinion and skills on performing this procedure.

CT scan
CT scan pros:

• This will be able to reveal more information with regards to Raphael's cochlea and middle ear ossicles. It would be nice if something could be found that can be corrected; I am sceptical that this will be the case though.
• Having the CT scan now means it is combined with other procedures under a single general anaesthetic.

CT scan cons:

• He needs to have a general anaesthetic.
• He needs to be transported from radiography to the surgical area while under anaesthetic.
• He will be subjected to another dose of radiation.
  

CT scan decision:
We have already consented to having this test done in the past and my opinion hasn't changed. I would like to do as much as we can to improve his hearing and if the specialists believe that the risks are manageable then I am still in favour of this procedure going ahead.

G-Tube
Thank goodness I don't have to make a decision on this one any more. I still have lots of unanswered questions about having a procedure to fit a G-tube. The main outstanding questions that I have are regarding what type of g-tube is best to be fitted, the method used to get it in place, and whether a fundoplication is also necessary for him.

I have found a video of someone performing a PEG (one of the three ways that I am aware of inserting a G-tube)


Summary:
I would like the three procedures (grommets, ABR, and CT scan) to go ahead. Even if the outcome is the worst result, I believe that I will be able to say that I carefully considered my son's future life and made the decision to the best of my ability with the best advice that I could find.

Prayer is the only thing left for me to do now.

Pre-admission clinic

After hastily arranging a baby sitter for our older kids we turned up to the peri-operative unit just on time. The administrative staff confirmed the details that they had about us and then we were advised that we were to see a nurse, anaesthetist, and surgical doctor.

We spent a few minutes with the nurse who has some administrative questions to ask and some simple medical questions, eg if he has any allergies.

Then we had a wait before an anaesthetist saw us. He wasn't the anaesthetist that we were expecting to see and the more answers we provided to his questions, the more puzzled he appeared to become. Eventually he worked out that we really shouldn't be speaking to him because we had already had the consultation for the special case that had been arranged for Raphael. We asked him about Raphael's cold that he has had for the last week and he said that we should bring up his issue when we see the anaesthetist on the day of the operation so that the anaesthetist can assess the situation then. He said that the complexity of Raphael plays a big part but also made the comment that Raphael's anaesthetic risks need to weighed up against the long term risks of not repairing the hearing loss as early as possible.

After another long wait we met with an intern who asked us a lot of questions. She apologised in advance because she knew that she was going to ask a large number of questions that we had already answered today. The staff are always so nice and understanding of our situation. After she had finished gathering information we pulled out our list of questions but as she was only filling in for the normal surgical ENT intern, she couldn't answer many of them. She kindly offered to arrange appointments with the relevant specialists.

On our way out to try to find someone who might be able to help us with our questions about the potential fitting of the G-tube we ran into the anaesthetist who is going to perform the GA on the day. Fortunately he could shed some light on the G-tube questions that we had but we still didn't know who had ordered the G-tube surgery, what type of G-tube surgery had been planned and if our paediatrician knew anything about the plans.

After this fortuitous, informative meeting we managed to speak with the person who had managed to arrange the line up of the procedures. From this we found out a few interesting things:

• Our ENT is prepared to give up their entire morning list for Raphael's multiple procedures.
• Our normal RHH audiologist will be away at the time of the procedure so a different one will do the work on the day.
• It seems that no-one actually requested the G-tube. It appears to have purely stemmed from our conversations with various specialists. We mentioned that we thought that our paediatrician will arrange for Raphael to be fitted with a G-tube in the future some time and we questioned whether this next GA would be the right time to do this.
• They seem to have started to organise the G-tube procedure but are still trying to contact our paediatrician. The hospital got a tentative OK to proceed by contacting our GP but I don't know what will happen if they can't contact the paediatrician before the scheduled operation.
  

We are not confident about the G-tube aspect because we seem to have been the driving force where as we would rather the recommendation from from a consultant (not us).

After four hours we got home we called our paediatrician's rooms and even though our Paediatrician is still on holiday; they had just arrived back in the country and so we managed to pass a few messages back and forth. The end result is that we think that our paediatrician is currently recommending against getting a g-tube fitted at this time. There are a number of possible reasons for this decision and although we don't know what they are yet, we trust our paediatrician and so will cancel this procedure.

Four procedures under the one GA

Last night we received a phone call from the surgical unit at RHH saying that they have somehow managed to line up the four procedures that we had been preparing for:

• Grommets
• ABR
• CT scan
• G-Tube (or PEG)
  

I was excited to hear this news but later that night my sleep was very restless, I kept going through the whole thing in my mind and wondering whether it would be the right decision to go ahead with all of these procedures at once. For CHARGE syndrome kids I have read that it is a good idea to combine procedures under a single general anaesthetic. But having said that, consultations with specialists reveal that long anaesthetics have their own considerable risk.

Anaesthetist consultation

We had a long consultation with another anaesthetist today (he is a very patient man fortunately). He will be the anaesthetist who will perform Raphael's next general anaesthetic.

I am not going to cover everything that we talked about. He fortunately let us record his consultation so Annie and I can refer to it again before our next anaesthetic consultation.

The next general anaesthetic will be for a potential mix of the following procedures:

• Grommets
• Taking blood
• CT scan
• Endoscope of the larynx
• Auditory brainstem responses test
  

We talked with our paediatrician about this as well and there is some question over which procedures would be a good idea.

Grommets
Grommets are still the high priority to get his hearing improved. Hearing is so important for language development that this will happen as soon as possible.

Taking Blood
While he is under it is a good opportunity to take some blood for tests. There will be some blood taken to test for DiGeorge sequence (I think that is what it is called - to do with immune response), and some blood taken for possible future gene testing. I think there is another vial to be taken too but I can't remember what that one was for.

CT scan
Now that we have an official diagnosis of CHARGE syndrome there doesn't seem to be need to do this scan. Originally it was going to be used to clarify some of the findings from the MRI. the paediatrician will talk with the ENT as to whether there are any additional benefits, from a hearing perspective, that would be gained from doing a CT scan.

Endoscope
The ENT said that the next time Raphael has a GA it would be a good time to do a thorough examination of his throat with an endoscope. When we questioned the anaesthetist about this he said that it can increase the risk of complications because the extra probing from the endoscope may induce more swelling than otherwise might happen. Effectively this would just be another risk that would need to be managed at the time.

Auditory Brainstem Responses test
Initially Annie and I were keen on the possibility of having another ABR immediately after the grommets were inserted, while still under GA but since:

• The anaesthetist said that this extra long time that he would spend under GA (at least another half an hour, could be an hour) would add risk to the procedure,
• The ABR can be performed on a sleeping baby, and
• He might have to be moved to another theatre for that particular procedure,

We aren't so keen on the ABR happening during the GA anymore and Annie has called the paediatrician's rooms to leave a message for her to cancel that part of the procedure.

(edited 10/3/2007):
Here is a list of the topics covered in this consultation:

• Previous Anaesthetic for MRI
• Proposed Endoscope
• Risks of procedure/s
• Method of Anaesthesia for his condition
• Heart defects
• Kidney abnormalities
• Feeding frequency
• Cold viruses
• When and how long to admit to hospital
• Anaesthetic versus sedation
• Resistance and unexpected reaction to anaesthetics
• What procedures will be performed
• Access to anaesthetist notes
• CPAP usage

A CT Scan story

Raphael had his CT scan in rather strange circumstances. He was originally booked to have his CT scan on a Tuesday but then on the Friday before we received a phone call from the hospital saying that they weren’t going to have a Paediatric Anaesthetist available on that day and wanted to change the booking date to the Preceding Monday.

Annie has been really worried about this General Anaesthetic (GA) for him and has been wanting to have the test done while he is sleeping instead because she read that it is possible to conduct CT scans on infants that are sleeping and it may not be necessary to give them a GA. But every doctor that we saw dismissed it as not worth doing because any movement renders the CT scan worthless and it would have to be done again. (The radiation Dose from a head CT scan is about 2 millisievert (mSv) which is about the same dose as an Australian would get in normal life (background radiation) over a year)

On the Monday we went in and were admitted to day surgery, we hung around for a little while and then an anaesthetist came to us (we have met her before, but I can’t remember her name) and she said that the Paediatric Anaesthetist was not available today because he was unwell (actually he had walked into a pole and injured his eye). She suggested that we go home and make another booking.

So at this time we had a CT scan machine and crew booked and a really sleepy baby but no anaesthetist. Annie saw this as her opportunity and so she begged the Anaesthetist to arrange the CT scan with a sleeping baby instead of having a GA. The anaesthetist was hesitant but eventually agreed to try to help us have the procedure done without anaesthetic. She then made a long phone call to the X-ray department to try to convince them of performing the procedure. While she was on the phone Annie and I wondered whether God had hit the Anaesthetist with a pole so we could do this without a GA. We laughed about the possibility but then felt sorry for the poor guy who had hurt his eye. Eventually she returned and advised us that “they” agreed to do the procedure with a sleeping baby.

The radiographer met us down in the X-ray department and ushered us to a waiting bed behind a curtain where the lights could be dimmed to help Raphael get dozy. We had a short wait while another patient was having a CT scan and then just as we were about to go in the radiographer came out to tell us that the radiologist did not want to perform the scan on a sleeping baby because there was practically no chance of the image turning out as babies of Raphael’s age move in their sleep a lot. Not to be deterred we asked to speak to the radiologist and he kindly came out to talk to us. When we told him about our fears of a high mortality rate of CHARGE babies under GA he agreed to perform the CT scan.

The normal high intensity lighting of the CT scan room was turned down only to leave the ambient light coming in from behind the lead glass shield for the operators and the LED lighting of the science fiction like CT scan machine (shaped light a donut) including a laser light cross on his head. We didn’t take any photos in there as we did not want anything to risk anything to disturb Raphael. It is doubtful that a photo could have captured the mood in the room anyway. Raphael’s tiny body was gently strapped down to an adult sized bed and Annie stood over him feeding him a bottle of hot milk coaxing him off to sleep. It was a very different send off than the MRI anaesthetic where he was held down kicking and screaming by three people while the anaesthetist and his assistant unsuccessfully tried to get him into a GA sleep.

He fell asleep after one and a half bottles of hot milk. At this time the radiographer decided that it was a good time to insert the head supports (why she didn’t do this before was a bit of a puzzle to me) but he didn’t stir at all and finally everyone left the room except for me in a lead apron and Raphael lying on the bed. The room was dark and quiet and even Raphael was not making his normal snoring sound (for those of you who have not heard him, he is always making a snoring sound even when he is awake). The bed whizzed his head back and forward through the donut hole and finally slowly passed his head through the machine that seemed to take about a minute. Through all this time he was perfectly still.

After it was all done we took him back up to the day surgery and waited to hear whether he had been still enough for the CT scan results. I was sure that he must have been because I was convinced that he had not moved at all, but unfortunately the message finally came up that there had been some movement and they weren’t sure whether the resolution was good enough.

After a subsequent visit to an Ear, nose and throat doctor (25/1/2007) more than a week later (in the Hospital) about his hearing problems the ENT said that they could see from the CT scan report and from clinical inspection that he had fluid in his ears that is almost certainly interfering with his hearing and that he could need an operation to insert ear grommets in his ear. Apparently this operation requires a General Anaesthetic (sigh). We again told her about our concerns about General Anaesthetic and asked her if the repeat CT scan could be performed at the same time. She thought this was a good idea and said that she was sure that she could arrange it. In addition to this she also thought it was a good idea to do a thorough examination of his throat while he was under GA. So at least now we are going to have several procedures performed at once under the one GA.

(I still think that it was a bit extreme that God had to hit the Anaesthetist with a pole to prevent Raphael from having another GA)