A couple more Doctor appointments

We have had a couple more appointments with a Pediatrician and his ENT but apart from Raphael having yet another cold and having his left ear clogged with wax there wasn't much to say.

He has completely recovered from his adenotonsilectomy and now that his latest respiratory infection is subsiding, we have managed to reduce his CPAP level to 4.0 without having any desaturations over night. This weekend we are going to try to put him to sleep without the CPAP on at all (only the O2 monitor).

Adenotonsilectomy

Raphael was admitted to hospital on Thursday 3/7/2008 to have the adenotonsilectomy surgery on Friday.

Thursday night I received a concerned call from a anaesthetist registrar who went to check on Raphael saying that he sounded like he had a severe respiratory infection and that it might not be safe to proceed with the surgery. But after I explained his normal state she understood that this was as good as he ever got. His normal anaesthetist also had a look at him that night and agreed that this was as good a time as any.



Friday surgery went smoothly and he was in NPICU (Neonate and Paediatric Intensive Care Unit) within three hours. He was very dopey coming out of his General anaesthetic with morphine, as would be expected. But after a nap and waking up in the evening, he set about his work charming the nurses in the ICU. Because of his risk factors he was kept in ICU overnight but he was in very good condition.

The next day he was moved to the Paediatric ward and we found out later that the nurse handing him over said that she had fallen in love with Raphael and it was so nice to actually be a nurse to a person rather than to machines (which is what ICU nursing is normally about).

The ENT surgeon gave us a quick run down on how the surgery went (adenotonsilectomy and laryngoscopy) and the only thing that we were worried about was that his right ear had some blood in it. (The last time his left ear came back from surgery with blood in it he lost 50db of hearing in that ear).

Sunday morning 6/7/2008 he was discharged and he was very happy to come home again.

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.

ENT

Our long ENT consultation to day focused on the Adenotonsilectomy and timing for the surgery given that our ENT will be away for a while shortly. We also discussed his hearing loss and further tests.

ENT followup

We had a follow up with Raphael's ENT today. The grommets are still in and his ears are dry and without infection.

We discussed the possibility of repeating an ABR test (an objective hearing test) because the hearing in his left ear appears to have become worse since the grommets. We are not sure if this will proceed though because even if it does, there is nothing that can be done short of inserting cochlea implants. Raphael's left ear is still too good (at 90db loss) to warrant/risk a cochlea implant (usually reserved for 100db+ loss)

I wonder if his hearing will improve when the grommets extrude. The ENT doesn't let us record her consultations unfortunately so I can't remember precisely what she said. From memory I think that the ENT said that hearing loss incurred from ear surgery is not expected to return, but any such hearing loss, in grommets insertion cases, is rare and minimal (10db at most). At the moment I think that the ENT believes that the apparent huge drop in observed left ear function (40 to 90db loss) is actually the result of erroneous readings prior to the grommets insertion.

It is so hard to test Raphael's hearing because there is such a limited time when he is at his optimum (ie when he is not eating or sleeping/tired) and if that doesn't fit into the completely inflexible window that Australian hearing are able to schedule, then it is a battle to get any meaningful hearing testing out of him. We knew it was important for children to be able to hear from a young age, but even with our persistence, I believe that we have still not had all of the hearing tests that could be done to provide Raphael with the best chance of hearing. On one hand it is fabulous to see that our country provides these services for us, but on the other hand it is frustrating that it is not funded adequately to provide the services in the necessary timely manner.

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.

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.

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.

ENT

Our ENT spent over an hour and a half with us today.

She examined his upper respiratory tract with an endoscope to confirm that there is no blockages that could have caused the poor oxygen levels during his last hospital stay. The only thing of note found in this endoscopy was the confirmation of his laryngomalacia.

Examination of the ears revealed a stenosis (extra narrow path) of the external ear canals and a chronic inflammation of the ear drum due to fluid build-up in the middle ear. [Previously in this blog I referred to this inflammation fluid as "otitis media" but I was told that that was an incorrect term.] Inserting grommets is still recommended as an attempt to resolve this inflammation (and associated conductive hearing loss). We were warned that because of the copious secretions in his throat, we may find that his ears may leak like his nose does. This is a potential infection risk that may require frequent ear drops.

We also discussed other procedures that could be done under the same GA:

• PEG insertion
• CT scan
• ABR test

The ENT also told us that getting two procedures done at once was very difficult, getting four together may be extremely difficult. The nurse in charge of booking the theatres and surgical teams gave us some of the reasons for this impracticality and didn't think that it would be possible to achieve. I still don't know if this is a good idea to do all of these procedures together as these other procedures will add a considerable length of time to Raphael's anaesthetic. I don't know what the best thing to do is anymore with regards to all of this, but I do know that want to improve his hearing as soon as possible.

We have another Australian Hearing appointment on Tuesday 3/4/2007 which I believe will be an opportunity to fit hearing aids.

appointment day

Today was a busy day. This is my (Paul's) day off but Annie had to take the morning off aswell because of the number of appointments that we had lined up. Today we had:

1. Consultation with PEG nurse,
2. Gymbaroo (gross motor development for Raphael),
3. Consultation with ENT,
4. "Let's sign" sign language course, and
5. Tonight Raphael's Early learning teacher will come to our house to discuss a learning plan for Raphael.

Sleep study

Raphael spent two nights in hospital (Sunday and Monday night) to monitor his oxygen level while sleeping.

The first night showed very low oxygen levels, sometimes down in the 60s. The second night was much better only sometimes going down into the 80s.

Because his oxygen was so bad on Sunday night we had an appointment this morning with an ENT doctor in the hospital. As the oxygen was much better last night, the ENT visit was very short; really only to say that we don't need to do anything right at the moment. We have another appointment with the ENT on Thursday where we will have more time to ask questions about his throat and ear problems.

The nurses supposed that his position seemed to effect his oxygen intake. One said that when his head was tilted forward, his O2 levels dropped. I don't know if we will get a formal report from the sleep study. I suppose I'll have to wait and see.

Another medical visit (five this week so far)

Saturday afternoon the ENT registrar at RHH asked us to bring in reports of all of the medical findings because the ENT wanted to go over all of the reports that she could get her hands on to see if there is more that can be done for Raphael. So this morning we dropped in to the hospital with a stack of freshly photocopied reports and letters for them to look over.

We are so glad that Raphael is being looked after by people that genuinely care.

When we first knew that Raphael had a few problems with him, I was concerned that we were in a small state with not many doctors. But now I am growing in confidence because even though we may lack quantity, there seems to an abundance of quality.

Compiled the blog history up to now

phew that took a while!

I know that I missed detailing several paediatrician appointments, at least one ENT appointment and I have barely mentioned the speech pathologist and dietician appointments but there were many in this time as well. I haven't even touched on the number of times that we have been to the hospital to reinsert his NGT after he has extracted it (3 times last week alone).

On average up till now we have had about two appointments per week. Sometimes four sometimes only one. I can't remember the last time that we had a week without an appointment of some kind for Raphael.

ENT appointment in RHH

See the end of "A CT Scan story" 15/1/2007 for a description of this consultation

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)

ENT

The ENT did another laryngoscope. This time his nose was big enough to be the entry point (so it should be, I have an enormous schnoz). He said that he no longer thought that Raphael had laryngomalacia but that there was definitely something going wrong back there.

He said that if more information was required on his condition then it might be necessary to see a paediatric ENT Doctor (possibly in Melbourne) but he was going to let the paediatrician decide on whether there was any value in doing this.

First ENT visit

Later that same day we visited the ENT. Annie and I carried in our three month, old gurgling like a jack hammer, to the Dr’s office and briefly explained the reason that we had been referred to him. He did a laryngoscopy (sp?) through Raphael’s mouth because his nose was too small for the scope to fit through. Raphael hated it. He sat on my lap with one hand pushing his head back into my chest and the other holding his arms down. Every now and then I had to remind myself to only hold his head firmly and that I didn’t have to squeeze the life out of him with my other arm across his abdomen. This is what fathers are for. Mothers seem have too much heart to torture their sons for a prolonged period of time. After what seemed an eternity the ENT reported that it looked like there was some signs of laryngomalacia but also that he was producing a lot of secretions that were pooling at the back of his throat that he was unable (or unwilling) to clear himself (hence the gurgling sound). He said that all these secretions made it difficult to see what was going on. He recommended a Barium swallow test to try to work out if there was anything basically wrong with his oesophageal plumbing (not the term he used).