Early learning - hearing consultation

This morning we had a consultation form a hearing specialist at early learning.

We talked about a general plan to help Raphael develop his hearing skills and came away from the session with a few things to do:

• Speak in sentences but emphasizing the key word to him, trying to include it in a sentence fifteen times if possible.
• Start communication with Raphael by vocalising before adding sign language. The idea is not to deprive Raphael from understanding by sign language, but rather to encourage him to use his hearing for communication as well as his vision.
  
• Continue to repeat back vocalisations to Raphael
  

Help from a home care nurse

It was extremely helpful having the home care nurse come tonight. She dropped off a replacement suction pump and a bunch of equipment manuals. She further helped us by:

• Inserting a new NGT for Raphael.
• Going through resuscitation with us again on an infant dummy; better than the adolescent one that we practised on before.
• Explaining to us how best to clean the CPAP equipment
• Talking about how best to manage Dr appointments while being an inpatient
• Talking about how to deal with being an outpatient and having appointments close together.
• Offering to help consolidate appointments to be closer together rather than have them spread over an awkward amount of time
  

Another tube out

I went to get Raphael up after his afternoon nap only to find that he had managed to soak the tube tape loose with his secretions and then cough the tube out half way.

I pulled it out the rest of the way (rather than risk reinserting it) as I know we have a home care nurse coming tonight who can put a new one in.

"The elephant is dancing"

Our code phrase to indicate that Raphael is awake

Oxygen probe showing poor saturation

Last night we had problems with the blood oxygen saturation monitoring machine. It kept on showing results that were clearly too low for what was actually going on with Raphael. We tried a few things:

1. We tried retaping the probe to his foot a few times. If anything this seemed to make the problem worse. At one stage it was showing a "reliable" signal but only reporting a O2 level of 15%.
2. We tried using an old probe. This was even worse, at one stage reporting as low as 3%.
3. We tried reseating his mask again but this had no effect.
   
4. We then attached a brand new probe but this still showed saturations dropping as low as 60%.
5. Eventually the computer techie side of me took over and I checked all cables only to notice that the machine end of the probe cable was loose.

Finally after reseating this cable everything sprang back to normal, well nearly normal anyway. It only showed 92% O2 level which isn't great but it was good enough for us to call it a success and we finally got to sleep at around 12:30.

Healing service

We brought Raphael to Citylight church tonight for a special healing service. The guest(?) preacher prayed especially for Raphael, and later the church pastor and a number of the congregation prayed around him.

A few people told us (and I believe also) that Raphael will be healed. The guest preacher prophesied that within three months he will receive miraculous healing and that the medical professionals will be surprised.

And just to let you know what was going though my own mind for Raphael, here was the list that I brought with me

• Nearly completely blind in left eye and other minor eye problems with both eyes
• Moderate to severe hearing loss in both ears
• Balance sensory organ completely malformed
• Uncoordinated swallow resulting in no weight gain without feeding tubes
• Vomits easily and is prone to pneumonia
• Breathing difficulty, especially when sleeping
• Heart defect

I certainly don't mean to limit the healing to this list. I will continue to pray for my boy; please pray for him also.

Another new way to extract an NGT

Well this one was a ripper.

I was in the middle of taking a few opportunistic photos of my family on a relaxing Saturday morning when Raphael started to cough. After three decent goes at coughing he started to turn the tell tale red of pending up-chuck.

I carefully threw the camera onto a couch and grabbed the nearest chuck cloth. lunging in front of him, I was just in time to catch the unpleasant contents of his stomach as it heaved out of his mouth. But something was different. Apart from the different type of thick lumpy vomit that the Nutrini causes (rather than the Infantrini), there was something else wrong. The NGT tube had formed a loop, I have seen this before when he has managed to vomit the tube out his nose so I grabbed the loop and intended on pulling the tube all the way out. This did not work exactly to plan. Somehow I ended up with the tube still securely taped to his face and the stomach end poking out his mouth. Argh, he had managed to vomit the tube out of his mouth, not his nose.

After cleaning him up we pulled the tube all the way out of his nose. Fortunately the home care nurses have previously offered to come to reinsert Raphael's NGT to save us going to the hospital should he pull it out again. So I have called them and I am now waiting for someone to come to fix him up again.

Last night CPAP

Last night at about 10:30 Raphael's oxygen sensor alarmed and we witnessed the desaturation spike as low as 77%.

Unsure what was causing the problem we called a home care nurse who advised us to try suctioning him and also changing his Oxygen sensor probe (in case there was something wrong with the one we were using).

During the process we noticed that one of the air out-flow ports from the mask had become blocked with a dry goober and so we cleared the hole with a skewer and wiped out the mask. Suctioning did not get anything out of him but after we had put everything back on again we did not have any more problems for the whole night.

The nurse called again a short time later to see if we needed her to come but we were sure we were fine; gee they are dedicated!

Raphael's bed setup

Here is a brief description of how we have setup Raphael's bed in our bedroom while he is on his CPAP machine. At this stage we don't know how long we will need to keep him with us in our bedroom but I suspect that it will be in the order of months at least.

The first thing we attach is the oxygen monitor probe. The probe is taped to his foot and feeds back information to the main unit (in the picture). When Raphael is not wriggling it shows the rhythmic pumping of blood on a graph and displays his oxygen saturation level and heart rate. The majority of O2 alarms are due to Raphael wriggling rather than actually having a low blood oxygen level. In fact there hasn't been a low O2 reading since we have had him at home on CPAP. We have set the low blood oxygen alarm level to be 90%.

This picture shows the oxygen cylinder that we use when we are suctioning and fitting the CPAP machine to Raphael. Raphael can become quite distressed when we are suctionin him and fitting the mask, so much so that sometimes he can turn a bit blue from fighting. The oxygen helps him maintain his colour until he has calmed down. At this point we can turn it off. At least in theory this is how it works. I forgot to turn it off last night and so it was drained empty by the morning (oops). We have ordered another one and will have to pick it up some time today.

Above the cylinder bag is a Laerdal mask. This mask is for use in an emergency if Raphael stops breathing. We have received some training in how to use it and we are hoping to get some more practice on Tuesday when a home care nurse will hopefully bring an infant dummy for us to attempt to resuscitate.

We use the suction machine the suck out Raphael's secretions immediately prior to attaching the CPAP mask. The theory behind this is that we don't want the CPAP machine to blow his secretions into his lungs when it starts up. Disappointingly it isn't as powerful as the suction in the hospital, but realistically it is powerful enough to do the job that we need. It probably isn't necessary to have enough suction to suck the contents of his stomach out his nose.

The CPAP machine is set to 8.0 (centimetres of water) pressure. This machine is effectively an air pump that keeps Raphael's lungs and airways open, preventing them from collapsing while he sleeps (obstructive sleep apnoea). This is a noisy machine even when the alarms aren't going off

This picture shows the whole right hand side of his bed. The things to note here are:

• Kangaroo pump on the pole. This is the pump that is used to pump Raphael's feeds into him at night. You can even make out the rate on the photo (35ml/hour)
• The bed is raised at this end by an old bassinet placed upside down underneath the legs of the cot. This is to hopefully reduce the reflux of the overnight pump feed.
• The O2 monitor isn't actually there any more, we moved it closer to our bed so that we can silence the alarms quicker when they go off.
  
• Suction machine.
• Oxygen cylinder.
• Box of tissues to help manage the copious secretions
• rubbish bin on the chest of drawers for the packaging rubbish that is left over from the disposable medical pieces.
• Hearing aid bags (yellow and navy/black) on the end of the chest of drawers.
• Small bucket of disposable equipment on the chest of drawers containing syringes, litmus paper, tape, suction heads, foot probes. Realistically we use some of the disposable equipment many times before it becomes unusable.
• Towel under the pole is to catch any mistakes or drips made while setting up the feeding pump each night.

The left side of Raphael's bed looks like the following:

• CPAP machine, and of course hose, connected to his face.
• O2 monitor. This is actually where we have it stationed.
• Box under the chair is actually full of the feeding bags.
• Towel behind the bed is jammed between the cot and the wall to prevent damage to the wall.
• Under the cot is a big bag full of more disposable equipment.
  

And of course the baby:

CPAP and other alarms

We think that we are finally getting to know the tricks of strapping the CPAP mask to Raphael. We have had a couple of nights where different alarms went off at us without mercy only allowing a few hours sleep for the person who was on night duty (we have a roster based on who is working the following day).

The thing is that we don't want to strap the mask to tightly to his face because we have heard that tight strapping can prevent children's faces from growing properly.

Here are some profile photos of Raphael as he is now to compare in the future to see if the mask is causing any compression on his skull.

Choosing to sit

Raphael is finally moving to a sitting position more frequently to play with toys. Previously he had moved from crawling to sitting about twice in his life but I have seen him do it about five times today.

This is good news as he now has the confidence to sit without falling. His other senses must be making up for lack of balance sense.

New hearing aid moulds

Raphael finally received his new hearing aid moulds today and the Audiologist turned up the volume on the high frequency sounds of his hearing aid again in the hope that he new moulds would stop the really annoying feedback. Of course it sounded good at Australian Hearing but as soon as we got him home his right aid started to squeal relentlessly. On the bright side though, his left aid seems to be fine.

Next operation scheduled for 14 June 2007

I have just been in contact with Raphael's Ophthalmologist who has found the scheduled date for Raphael's next operation which is on 14th of June 2007.

This procedure is currently planned to include

• Installation of a "mic-key button" g-tube
• fundoplication
  
• probing and flushing tear ducts

My wife is still unsure about the open surgery and fundoplication and so she would like to speak to our paediatrician again before the operation.

More on this topic to later.

First night at home with CPAP

Last night we had a visit from a home care nurse who made sure that we had setup all of the CPAP equipment correctly and also went through resuscitation with us in the event that Raphael stops breathing.

Finally escaped from hospital

Raphael is finally home. We have been issued with three machines that go ping, an oxygen cylinder, sticky things, slippery things and enough plastic tubing to pump gas into and out of every house in our street. We are still waiting on the suction machine to be delivered though.

The night before last we tried a prototype headpiece but this did not work very well as it kept on slipping down. Last nights CPAP was very good, we finally had a customised five strap headpiece that fitted well and did not slip off at night when he moved around. At one point he even got up and crawled around the cot. He knocked off some of his sensors (sticky things) but the mask stayed on and maintained a good seal the whole time.

Over the last two nights they also measured his CO2 levels. The first nights results were ambiguous but the second nights results encouragingly showed no CO2 build up.

Paediatric cardiologist

Our 4:30pm paediatric cardiologist appointment started at around 7:00pm. I was disappointed to find out that earlier in the day a paediatric registrar had filled in the cardiologist on Raphael's condition and had missed out some very vital pieces of information. This meant that when Raphael had his echo cardiograph, ahead of time when we were not there, the cardiologist did not look at the particular heart defect that we wanted him to look at.

This all could have been avoided by calling us and I was very disappointed when it was suggested to me that it was my fault that I was not contacted because I did not provide a mobile phone number. I find this especially insulting because they did not even try to call me on my work telephone number (which incidentally forwards to my mobile).

Amidst my frustration at the situation, I felt sorry for the cardiologist who's time was wasted because of the lack of one administrative phone call.

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.

Yesterday, what a day!

Yesterday started with my wife waking up with a moderate allergic reaction. So I:

• Rushed my daughter off to school leaving my wife next to the phone with strict instruction to call 000 if she started to have any problems with breathing.
  
• Rushed onto hospital to pick up Raphael
• Realised that he didn't have his hearing aid and so rushed back home to pick up his hearing aid
• Zoomed down to Early Learning Tasmania where he got some one-on-one work with physiotherapists. The next lot of gross motor practice for him will be to practice getting down from standing (that is standing while holding onto something). In general they said that although he is still a little behind in his gross motor skills, he should not be doing more at this stage anyway because of his other impairments.
  
• Flew back home so Raphael could have a sleep before his Australian Hearing appointment (to get new hearing aid molds).
• Received a phone call saying that the molds weren't ready yet (phew, that gives me an hour break)
• Took Raphael along to a parent teacher interview (for our elder daughter)
• Rocketed back to hospital to meet with a orthoticist(?) (someone who practices orthotics) and occupational therapist to see what they might be able to do about sorting out a customised hat to secure the CPAP on Raphael at night.
• Immediately afterwards we met with intensivist and paediatric home care nurse about arranging necessary equipment that we will need to have at home to maintain the CPAP on Raphael. One extra piece of information that came out of here is that it is not the air pressure that causes gradual facial squashing but rather the pressure of the straps that hold the mask on. Another interesting point was that the intensivist was going to alert the ambulance service that if we call about Raphael needing emergency transportation, then we really do need it. I imagine that there is some kind of list of people that we have been added to, titled These people aren't kidding if they call.
  
• Drove to an extended family dinner at my parents place, stopping at a bunch of shops to buy supplies along the way. While at this event Raphael learned a new Auslan sign: "applause". He would raise his hands in the air and, in response, the rest of the family shook their hands in the air accompanied by a "yay", eventually he got the idea of vocalising as well and he would raise his hands, take a deep breath and say "aaaaa" with a big grin on his face. (photo courtesy of my mum).
  
• Finally after saying a long goodbye to some relatives who were going back to the mainland, we zipped back home for me to pick up my work clothes and then I went to the hospital to fit Raphael's CPAP mask and sleep next to him in order to gain experience with fixing any problems that might occur overnight.

Last nights CPAP experience was pretty good with the new blue gel mask. This blue gel mask had a ridiculous clear membrane on the inside that was frequently blocking Raphael's nose (that was until I attacked it with a pair of scissors the night before last), it is much better now. Once the mask was on and strapped down it only took half a minute before he was fast asleep. The headpiece needed to be adjusted four or five times last night but apart from that there were no problems. I am looking forward to getting the custom made headpiece when it is completed.

I got about four hours of interrupted sleep last night in the hospital. I am looking forward to tonight when it is my wife's shift in the hospital and I get to crash at home in a nice comfy bed instead of an armchair recliner that can't be made flat.

Five teeth

Another tooth has fought it's way through Raphael's gum and another is very close. In true Raphael style he didn't complain at all and just keeps on charming people with his smiles.

Pulling to stand

The last few days have been much the same with regards to conencting him to the CPAP machine at night.

He has finally had his ECG (electrocardiogram) for the upcoming paediatric cardiologist consultation.

He is able to stand now for a number of minutes while holding onto the wall or a rail. He has also even pulled himself to stand a few times over the last couple of days.

An informative day in the hospital

Our paediatrician talked to me this morning. All the relevant consultants now agree that it is a good idea to proceed with inserting a g-tube and performing a fundoplication. We have a tentative date booked in three weeks for the operation but this is dependant on there being an intensive care bed available after the procedure.

I also finally managed to get copy of a stack of reports that I was missing. The consultants are generally too busy to chase down reports so the best thing to do is to ask a nurse (or intern/resident/registrar) to get copies of all the reports ready for the consultant so all the consultant has to do is say "give the reports to them".

Still in hospital

Yesterday we saw a paediatric surgeon about the possibility of performing a laparotomic (open) G-tube insertion and fundoplication.

The ENT registrar also visited to ask about the mysterious neck which had been ordered for Raphael. Like us, they seem to be unsure why this has been ordered. I actually think that there was a miscommunication somewhere and that it was supposed to be ordered for our other son Zachariah. He also commented that the CT scan showed some evidence of congenital cholesteatoma but he probably does not have this. It will continue be monitored and we will talk more about this at the next ENT consultation.

We attempted to connect for his afternoon sleep but he would not tolerate it again. It seems at though we are not going to have any success with his afternoon sleeps. I was exhausted and so I went home to sleep and while I was out I missed our paediatrician who came around to see us (rats).

Last nights CPAP was good. The intensivist made some adjustment to the mask by hacking at it with a pair of scissors becuase she said that it was probably the mask blocking his nose that was causing the unknown desaturations the previous night. He woke up four or five times last night, panicking with the air flow across his face but had no desaturations. His blood oxygen saturation level was consistently around 97%. They said that they will not send us home with this mask because it is not safe now. They have tried three masks so far but they will keep trying to find better ones.

The intensivist has increased the CPAP level to 9CWP (centimetres of water pressure). This seems to be enough to overcome his obstructive snore. This level of pressure is getting up to the level where we start to have to monitor his facial development. High levels of CPAP can push the front facial bones back during growth. The intensivist is interested to know how much the NGT is effecting the efficiency of the CPAP. We may have to do further adjustment of pressure after the NGT is replaced with a G-tube. This is what his profile looks like now

First attempts with CPAP

Yesterday after his Gymbaroo and Australian Hearing appointment (to get new moulds made) we rushed back to hospital to try his CPAP at 3:00pm. The intensivist tried a couple of different masks but Raphael was in a fighting mood and so this first attempt was abandoned.

At 9:00pm we tried again, we had a new headpiece (a nice soft one) and even though we had an initial struggle to get it on him he finally went to sleep with it on. All seemed good for the first hour but after that he would "desat" (blood oxygen level would drop) periodically with no apparent reason. This seemed to happen more when he was on his back or right hand side.

During his sleep he would occasionally move and the mask would slip a little. This resulted in air escaping and blowing across his face. This made him panic and struggle making the leak worse. This meant that I didn't get much sleep last night.

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.

Another day in hospital

Annie and I zoomed in and out of hospital today amongst other commitments.

Raphael's line became blocked in the evening but, because he was so much better already, the registrar was happy to happy to change Raphael over to oral antibiotics instead of putting another line into him.

He still needs oxygen when he is sleeping to keep his blood oxygen saturation above 90%.

Next day in hospital

Last night Raphael spiked up to 40 degrees but Paracetamol helped a lot. During the morning his temperature rose above 38 a couple more times in the morning but Nurofen and more Paracetamol helped keep this under control.

Unfortunately we weren't there for part of the day to manage the taping of his NGT. This gave him the opportunity to pull out his NGT... twice. In desperation the nurses resorted to making a little boxing glove for one of his hands to stop him from pulling out the tube a third time in one day. He had actually kept his tube in for a record five weeks before pulling it out twice today; one more week and it would have been actually due for a change.

He is still not eating much and still coughing a bit but it was good to see that his temperature stayed normal all afternoon.

Pneumonia, third time

Last night Raphael coughed all night. Of course I slept right through it all as normal and so when I woke up in the morning I went to work. I thought it was a little strange that he wasn't connected to his feeding pump in the morning but women do strange things some times so I just thought this was one of those occasions for my wife.

Annie called me at work in the morning to tell me of his coughing problems last night. This sounded like the same thing that happened to him last time he was admitted for pneumonia so after a bunch of phone calls and left messages we finally had an appointment to bring Raphael into the hospital at 2:30pm.

Annie had been checking his temperature all day but it was fine up until our paediatrician checked it at 3:30pm when it was 38 degrees. At one stage I thought the paediatrician said "you are very cruel parents, so if you are concerned when you call then he will probably need antibiotics". When I questioned her about the "cruel parents" comment she quickly clarified "cool parents!". To be honest though it is hard not to feel cruel when I have to hold him down to shove tubes in him or stick him with needles or even replace tape on his face.

He had an x-ray taken which showed pneumonia and tenting of the diaphragm (although I am not entirely sure what that means). By the end of the night he appeared to be chained down with tubes and taped up like a mummy to keep all of them attached. He was attached to:

• Blood oxygen saturation monitor attached to his foot
  
• Oxygen tube prongs in his nose
• Antibiotics in an IV in his foot (Here's a tip for kids that aren't walking yet, get IV lines put in their feet instead of their hands so they can still play.)
• Feeding tube down his nose

After ten minutes he was completely free from the goop saturated tape that was holding the oxygen tube to his face and so Annie and I taped up his face thoroughly to hold it all in properly.

The CPAP equipment wasn't ready for him yet so this will still start on Monday night when they can get the most appropriate equipment for him. Our paediatrician thinks that we will still be in hospital then anyway.

Marathon paediatrician effort

We spent well over an hour and a half with Raphael's paediatrician today and also spent some time with an intensive care consultant (intensivist) discussing Raphael's need for some intervention regarding his sleep apnoea.

After Raphael's simple sleep study, his ENT examined his adenoids and tonsils but didn't find that they were obstructive and therefore would be no benefit in having them removed. Because of this, the next step is to admit Raphael to hospital on Monday for some days for all of us to get used to operating a CPAP (Constant Positive Air Pressure) machine while he sleeps.

As usual with long consultations, I will need to go over the recording that we made to pull out all of the information and to make the list of topics covered.

Early Learning session today

Annie took Raphael to an Early learning session today and was pleased to note that Raphael was finally able to drop objects into a box. I am sure that more was achieved during the session but I was not present so I cannot give a full account.