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.

Admitting to hospital

Tuesdays seem to be our days for multiple appointments. After the ophthalmologist Annie zoomed Raphael to his Gymbaroo class for the last half hour of it then back to home while Annie had to go to a dental checkup, then fetching the older kids from one baby sitter to deliver them to another babysitter and school and finally rushing Raphael to hospital to admit him for the procedures on the following day.

We spoke to the Anaesthetist that evening. They weren't aware of the eye pressure test yet but the other three procedures were ready to proceed.

Because he has only just recovered from a cold that he has had over the past week we asked the Anaesthetist about whether he thought it was still safe to proceed. He suggested that we see how he was overnight and then check him again tomorrow.

We were planning to stay until he had his line put in for intravenous (IV) feeding that evening but when we found out that it was going to be very late we went home for the night. He was given IV fluids because he had to be fasted from midnight and the anaesthetist didn't want him to be dehydrated before the operation.

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.

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