Getting milk and bags

I just realised that there is another regular appointment that I haven't included yet.

Once per month we have to arrange to go to pick up Raphael's milk supply from the RHH. Each month we load up ten boxes of milk and a large box of feeding bags in the back of our small car. Then we drive them home an download them onto the floor kitchen where we trip over them for the next four weeks.

Hearing aid adjustment

This morning we took Raphael to have his hearing aids adjusted to try to stop the awful squealing from the feedback.

They reduced the high pitch gain and this seems to have improved things a lot. It still squeals if he leans up against something but at least it isn't squealing all the time now.

Putting things on things

Finally he has got the hang of putting things in a designated area. For example he has a music box with magnetic dancing pieces (bees) and he has finally got the hang of putting the bees on the music box so that they dance. Admittedly they don't stay there long becuase he likes to pick them off whenever he sees them dancing on the music box.

It is still difficult for him to do and although he can put things on other things, I have still not seen him intentionally drop something in something else. Or to word it another way, I have not seen him drop an object into a container.

More language development and a game

His "mmmmm" sounds have actually developed a little more. They can vary in pitch and duration and he uses them a lot more than he used to. He used to only use them when he was excited, but now he will make the sounds while playing by himself as well.

He has also started to play peek-a-boo by raising a cloth between us and then bringing it down expecting me to say boo. This is usually followed by uncontrollable little giggles (and Raphael thinks that it is pretty funny too).

Two new notable developments for language

Raphael has started making a new sound "aaaaa". So this has added to his vocabulary of "eeeee" and "mmmmm".

He has also just started to use sounds to get out attention when he wants to be picked up. He will crawl up to one of us and raise his hand, if we ignore him for a little while then he will "eeeee" to get our attention.

Finished beginners Auslan course

Tonight was the last night of the eight week Auslan course. I really enjoyed it and I am using it as much as possible at home. I am sure that Raphael understands some of the common signs that I sign to him. Some common phrases that I think he understands:

• come here
• drink time
• bed time
• finished

When I started the course I was unsure whether Raphael would ultimately use Auslan for communication but I wanted to get started early in case he would. I still don't know know if he will so I might continue onto a level one Auslan course. I have not decided if I will do this yet but I have expressed my continuing interest to the teacher.

Hearing aids

After the morning's gymbaroo, Raphael went to Australian Hearing to have hearing aids fitted. As usual (after gymbaroo) he was exhausted but he performed well for the practical hearing tests and these actually showed an improvement in hearing low frequency sounds.

They customised some digital hearing aids and fitted the ear moulds that had arrived since our last visit. Raphael was well and truly sick of being held by then and so we put him down on the floor where he was happier. While the audiologist explained the use and care of the hearing aids to us Raphael explored the room and the corridor outside the room.

The mould doesn't fit very well in his wonky ear becuase the cartilage isn't in the right places to hold the mould in properly. Apparently this can be a common problem for kids with CHARGE syndrome. Because of this poor fit he seems to get a bit of feedback on the device. The audiologist fitted some kind of feedback protection but now that we have got it home and have put the aids on him I am not convinced that the feedback protections does anything. In the end I just resorted to hold him down and jamming the hearing aid in as hard as I though safe to do. This used much more force than any mother would endorse, it was lucky that Annie wasn't home at the time.

Results of post grommets ABR

We received the results from the post grommets ABR today so we eagerly opened the email to look at the results. We were disappointed with the results, they did not show any improvement at all. The results actually demonstrated significantly worse hearing than previously tested. This was attributed to the blood and "debris" in his left ear.

As far as we are concerned we think that his hearing has actually improved. Annie and I both think that he is much more likely to turn to look for us if we call his name. This is much better than before when we had to yell his name to get a response.

Discharged after the four procedures

He is even happier today and after a quick consultation with his ENT and a paediatrician they were happy for him to go home.

His left ear still has dried blood in it but we are not to wash his ear with water becuase of the grommets. Apparently there is a risk of infection if water gets into his ears.

They prescribed some ear drops (Sofradex) that we are to drop in each ear three times a day for three days. I believe that this is to treat the chronic infections and also help dissolve the wax and blood in his ear.

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.

Ophthalmologist

We had a long ophthalmology appointment today where we bombarded our ophthalmologist with a page of questions that we had based on research that we had done into charge syndrome.

I will add the list of topics that we covered here after I have had a chance to listen through the audio recording, I think that the most important thing to note was that the eye pressure of his right eye (the good one) is still at the upper limit of the acceptable range.

When we advised the doctor of the general anaesthetic that we are having tomorrow, he commented that this would be a good opportunity to get a more accurate eye pressure reading and we hastily arranged a consent form and someone to actually perform the test.

Another sign

He can now sign "up", well actually it is nothing like the Auslan sign for up, but I believe that it is a generic baby sign for "up".

This crawl speed is now quite fast when he wants it to be and if my wife or I give him a little smile then he responds with his huge lopsided smile and then he tears across the room all fours, booldozing any toys that may be in the way and finishing with him lifting one arm in the air indicating that he wants to be picked up.

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.

Pre-admission time booked

Today we received a letter advising us to present at the peri-operative at 12:55 on 11/4/2007 for pre-admission, and if we miss the clinic time then the procedures will be cancelled. Yikes! that's less than 24 hours away.

It is good news that we'll have the chance to talk to someone about Raphael's procedures. I am looking forward to this.

The procedures are scheduled to start at 7:30am on Wednesday 18/4/2007.

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.

Yet another NGT

Raphael extracted his NGT again this evening. After his evening oral milk feed he rolled over to play and after a very short time stated to vomit out everything that he had drunk. I was too slow to catch this one with a chuck cloth, but fortunately it was caught by the blanket that we have laid on the ground to catch just such an occurrence.

One of his expulsions pushed out part of the tube. In the heat of the moment I was in two minds as to whether it was still in far enough for me to push it back in again. My indecision gave him the chance to reach up and he tugged it out further. That became the deciding factor and I pulled the rest of the tube out.

If he would have managed to hold onto the tube for just two more weeks then he might not have had to have another one fitted. We have tentatively got a surgery date to have a G-tube fitted (along with CT scan, grommets, and ABR) in two weeks time - more on that later, as we are not exactly sure what is going on with that yet.

Another sign

He has learnt a new sign: "Finished" and this one is very interesting because he is actually using it at the appropriate times. As with all of his signs, he is not signing accurately, but I am not worried about that.

This was actually the first sign (along with "more") that I tried to teach him so it is very satisfying that he has finally picked it up.

Some development

He can now confidently move from sitting to crawling position without cracking the side of his head on the ground.

He has also worked out how to use a hammer to try to hammer little pegs in holes. This is definitely a small step up from his previous attempts where he would swing the hammer and miss the toy completely. He usually ditches the hammer after a short time and then just uses his palm to hammer at the pegs. The pegs usually don't move at all with either method so he then resorts to just pulling the pegs out of the top and chewing on them.

ELT consulation

Raphael went to an Early Learning Tasmania session today with Annie. Annie said that he was a bit bemused with the six or so women with varied specialities giving him their full attention.

• General teacher
• Vision and mobility specialist
• Speech pathologist
• Physiotherapist
• Occupational therapist
• Session recorder
• and of course mummy
  

Another dodgy plug on the NGT

Argh, this is the second NGT tube that we have had where the plug has come loose by itself. you might have notice that it has been over a week since he has had the tube replaced so we thought we were doing well; and then this happens.

A loose plug manifests itself in an devious way. The first sign that you might get is when you pick him up you might put your hand in a gooey slimy patch of vomit on his clothes, becuase this isn't that uncommon anyway we just change him and continue on our merry way until we find yet another vomit patch somewhere in a very short time. After another change of clothes we realise that his new change of clothes is already contaminated. That is when the alarm bells ring and we look for the offending plug. Sure enough when plugging it back in again it just slips straight back out again and the smelly part digested contents of his stomach start to leak out once again.

My wife has discovered that to perform a temporary fix we just need to snip of a plug from an old NGT and plug up the hole with it. And then do a load of washing.

The first photo shows the unplugged tube and the extra piece that we used from a previous tube. The second photo shows the extra piece plugged in, making the normal yellow plug redundant.

You might also be able to see dust stuck to the outside of the tube. This is just because it gets sticky-tape residue on it from where we tape the tube to his clothes during the day.

Hi five

There was lots of fun to be had this evening by encouraging Raphael to give "hi fives" to mummy and big sister.

Also on the development front he has also been known to sign "hello mummy" a few times recently. Two words together; wow! Or maybe it was just "hello" followed by him scratching his head again.

Four appointments today

The four appointments today were: Gymbaroo, Australian Hearing, Let's Sign and a meeting with Raphael's future primary school principal.

I have talked about Gymbaroo before so I won't elaborate further here.

At today's Australian Hearing appointment, Raphael had another functional hearing test. He was seated on his mother's lap and sounds were played through speakers to his left or right and if he responded by turning towards the sound then he was rewarded with either a puppet in a window or a toy elephant drumming on the other side. The results of the test were the same as previous tests with regards to what volume and pitch he could hear. Ear moulds were then taken of his ears canals for the purposes of fitting hearing aids. He wasn't very keen on having his ears filled up with putty; but I have become quite adept at holding his head still.

Today was the last day of the 4 week Let's Sign course. I am a little disappointed that it is finished but I am very grateful to the teacher who went to the trouble of preparing extra signs just for me and giving me one-on-one help with the signs.

After the Let's Sign course we met up with the state coordinator of ELT (Early Learning Tasmania) and the principal of our local primary school to discuss Raphael and his older siblings in the schools. They are extremely supportive and have seem to have fabulous resources to help educate disabled kids and their families. I felt tears of gratitude well in my eyes when they told me what they thought they would be able to do for Raphael and his older siblings.

Crawling to explore

Crawling is now the preferred method of getting around; and he is using it to start to explore our home.

We have learned a few things because of his new found desire to explore. Such as:

• Even if we can't see him, we can work out roughly where he is just by listening for his noisy, gurgley, wheezy breathing.
• Shoe laces might be the most interesting things on the planet.
  
• We know what rooms he has discovered because he has marked his explorations with small slimy patches of nose goop.
• There is no such thing as the "silence warning" with him. For those who don't know what the silence warning is, let me paint you a small picture. Your children are playing out of sight when all of a sudden you look at your spouse and say "I can't hear the kids"; this can only mean one thing... they are up to no good. Because Raphael is quiet all of the time, except for the gurgle and wheeze, we can't hear the quiet patches where he has found a power chord to chew on.