Still in hospital

Raphael is still on oxygen when sleeping otherwise his oxygen level maxes out at 85% and drops to the mid to low 70's frequently. The paediatricians say that they want him to be above 90-92%.

Early today we had a visit from a paediatric surgeon resident to have a quick chat about having a G-tube surgically placed via a laparotomy and also possibly performing a fundoplication. This initial consultation was mostly fact finding for the paediatric surgeon with some very basic information for us.

At 2:30 pm a session was arranged to give me an introduction to CPAP (Continuous Positive Air Pressure) and how we are going to introduce it to Raphael in the hope that it will overcome his obstructive sleep apnoeas. One thing which really stuck in my mind was when they said that applying CPAP for a child is a life altering decision becuase of the large amount of work involved in maintaining it at night through the alarms and potential issues that itself can cause. The meeting had our paediatrician, two intensivist consultants (intensive care doctors) and two home care nurses who are going to help us maintain this system long term.

Unfortunately (or one could argue fortunately) some of the equipment that was set aside for Raphael's CPAP was intercepted in an emergency for another child so we were not able to start CPAP on Raphael tonight. They pointed out that although CPAP is used widely, it is very difficult to find a mask that fits an infant well becuase the major market for these sorts of devices is for "fat men who drink too much".

We are planning to start the CPAP tomorrow at 3:00pm during Raphael's afternoon sleep.

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.

Confirmed not doing G-tube

Raphael's Paediatrician's rooms called us to confirm that our paediatrician does not want to proceed with the fitting of the G-tube at this time.

Not doing G-tube now

Surgical bookings managed to contact Raphael's Paediatrician and have received advice to hold off on performing the G-tube surgery. From another source I heard a rumour that it is very difficult to perform a PEG on a child this young, keyhole surgery for this procedure is not offered at RHH, and a laparotomy procedure could take 6 hours and so entails considerable risk for a child with general anaesthetic problems.

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.

Appointment with PEG nurse

A specialised nurse came to see us this morning, before the ENT appointment, about the procedures and practices involved with Percutaneous Endoscopic Gastrostomy (PEG) tubes.

She talked to us about:

• how the ways in which the procedures can be carried out,
• how to care for the tubes or "buttons" (including the regular maintenance),
• what to do in certain unexpected circumstances, and
  
• how feeding occurs through the tubes

Now it would be good to speak to Raphael's paediatrician again to find out exactly what they have in mind for Raphael with regard to the type of procedure and possible additional fundoplication.