Ophthalmologist and Orthoptist

An Orthoptist visit today confirmed that Raphael is not getting any usable vision from his left eye. But the good news is that his right eye seems to have good vision despite the small coloboma at the base of that eye.

There was not much to talk about with the ophthalmologist, so that was only a quick consultation. We talked about the possibilities of attaching the eye probing procedure to future surgeries because his last surgery was cancelled.

The longest part of this visit was actually trying to pay the bill, the new receptionist was hopeless, when she finally worked out what to do, she messed it up and had to do it all over again. I think it took about twenty minutes to pay the bill. I was not impressed becuase we had a tightly scheduled morning of twelve things to do at nine different locations. We still ended up managing all but two but I was so exhausted by the afternoon that I collapsed in my chair to sleep for two hours. Thank goodness that our ever-ringing phone didn't drill it's ring tone into my head while I was asleep.

Development and weight gain (10.14kg)

Today we weighed Raphael at 10.14kg (a 60g increase from last time).

Raphael has begun cruising to more difficult surfaces of different heights. He still complains about having to do it but is willing to do it if the incentive is high enough.

He has also just managed to say Mama, it is a bit garbled but he clearly pointed to his mum and made this new sound. This is the first new sound in a long time and makes his list of sound:

• "dya-dya-dya"
• "adaadaadaadaa"
• "aiyaiyaiya"
• "mama"

Mama is English and Chinese for Mum and so we are going to claim it as two new words that he has learnt. I think that he knows that am "Dada" and that his mum is "Mama" but I can't be certain about that.

Crazy heart beat

Yesterday we took Raphael to his GP and she confirmed, just as the home care nurse had said the previous day, that this was viral and could be the nasty Influenza that has been going around. We were hoping that he would not get this because he is too young to have had the Influenza vaccination and we knew that it would effect him hard. On the good side, the GP was very happy with how his lungs were sounding.

Last night at 2:30 am his heart rate raised realy high (because of his high temperature). When it past 170 beats per minute his monitor alarm went off and I grogily raised the threshold to 200, when he started to scream and his heart rate past 200 I fought off my deep sleep to see that it peaked at 224 before I disconnected his monitor and CPAP to pick him up.

It was like cuddling a boiling kettle; he was steamy hot and his racing little heart was boiling in his chest. I gave him some Panadol and some hydrolite (becuase he has really thirsty too) and eventually I was able to put him back to bed.

To the doctor

We took Raphael to his GP today because he is still having high temperatures and we also wanted our GP to fill in the medical professional component of the Companion Card form.

She told us the same that everyone else is telling us that Raphael has a viral infections and we will just have to wait it out and give him Panandol and ibuprofen when he needs it.

She is always very supportive of our applications for assistance and this was no exception. If you have a disabled child and your GP does not support you then I would suggest that you find another GP because it makes life so much easier.

Raphael is sick again

Unbelievable! I thought he had just gotten better but tonight we measured his temperature at 39.1 degrees. Some Panadol has picked up his spirits and he is sleeping at the moment.

Yet another contrast study - video fluoroscopy

Raphael had another barium swallow study test today, a video fluoroscopy. This is where they take an x-ray video of him swallowing various different textures to see what his swallow actually looks like.

Sure enough the speech pathologists called his swallow uncoordinated and there was a very clear aspiration when he was drinking thin textures, such as his Nutrini milk. There was no sign of reflux during this study but that doesn't mean that it doesn't happen at all, it just means that it didn't happen during the study.

Notably after he had a drink, later in the day, he did a massive vomit, the largest one I have ever seen him do.

Weighing today (10.08kg)

Today his weekly weighing put him at 10.08kg which is a marginal increase of 50g since last weighing.

Also today, Raphael's Early Learning Teacher and Vision consultant visited us because Raphael could not make it to the last group with his ear infection. His teacher brought a box with her that seemed to contain an endless stream of things that Raphael found intensely interesting. Raphael showed off his ability to see and demonstrated his fine motor skills by posting objects into a box and manipulating small objects with his fingers.

Companion card application

The following are the answers that we are submitting for the complicated question on the application for a companion card.

Describe your need for assistance in the areas of mobility, communication, self-care, learning and planning.
Raphael was diagnosed with CHARGE syndrome on 12/1/2007. Raphael has a number of factors caused by this syndrome that makes it difficult for him to attend community activities and events.

Raphael has respiratory problems that could potentially require immediate first aid and medical aid. Raphael has previously quickly descended into respiratory problems that have required hospitalisation on a number of occasions. Early identification of the warning signs has been important on these occasions in obtaining medical aid in time.

Raphael has propensity to vomit multiple times a week due to nerve problems. Raphael requires assistance while vomiting to ensure that it happens in a socially acceptable way (for instance, not in a public swimming pool as nearly happened on one occasion). But more importantly it is necessary to make sure that his airway is not compromised after vomiting. Equipment to cope with his vomiting (cloths, water, change of clothes, and bags) need to be taken everywhere he goes which he is not capable of carrying himself.

Raphael has no sense of balance, he is blind in his left eye and has vision problems with his right eye.

Raphael's vestibule (sense of balance) is completely malformed and he receives no useful information from this organ. Raphael currently cannot walk and, although it is hoped that physiotherapy and occupational therapy will eventually enable him to do so, his lack of depth perception and decreased field of vision will always make mobility and orientation a difficult task without the aid of someone to guide him.

Raphael has a moderate to severe hearing loss and partially paralysed vocal chords making verbal communication very difficult for him. Raphael uses hearing aids but these cannot be worn on all occasions (for example while swimming or air travel) and his external ear malformation means that hearing aids are not comfortable for him to wear for any length of time. Raphael may be able to communicate with sign language, in these instances when he does not wear his hearing aids, but this will require an interpreter to be with him.

Raphael's paediatrician has said that she only trusts a limited number of people with the care of Raphael because of his complicated medical problems: Royal Hobart Hospital Paediatric Unit, The Royal Children's Hospital (in Melbourne), and us (his parents). Even the Commonwealth Carer Respite Centre refuses to look after him because of his complicated high needs. This effectively means that he cannot go anywhere without one of us being with him.

Please provide the name, date and outcomes of any formal assessments of your condition.

Raphael was diagnosed with CHARGE syndrome by a Geneticist on 12/1/2007.

An Ophthalmologist stated that Raphael has "very limited or no useful vision in his left eye" (7/2/2007) and on 5/6/2007 another ophthalmologist advised us of another serious eye defect in Raphael's right eye that limits his field of vision in that eye.

Raphael has had a Barium Swallow on 17/5/2006 that identified significant gastro-oesophageal reflux and aspiration.

Raphael has had numerous audiological tests including ABR (Auditory Brainstem Response) and VROA (Visual Response Orientation Audiometry) tests to determine his hearing level. He has been classified as having a moderate to severe hearing loss.

Raphael had an MRI scan on 18/12/2006 that identified severe eye and ear malformations including complete deformity of the vestibular semi-circular canals (sense of balance).

Raphael has 25 medical professionals and 8 education professionals who look after his complicated case. We have a whole folder full of reports on his condition. I have summarised a very limited number of the reports here.

Medical professionals:

• Audiologist [RHH & Aus Hearing]
• Dietician
• Ear Nose and Throat Doctor (ENT) (x2)
• General Practitioner
• Geneticist
• Intensivist (x2) [RHH]
• Occupational therapist [CHC]
• Ophthalmologist (eye surgeon)
• Orthoptist [RHH]
• Osteopath
• Paediatric Anaesthetist (x2)
• Paediatric Cardiologist
• Paediatric home care nurse (x2) [RHH]
• Paediatric Surgeon
• Paediatrician
• Physiotherapist [CHC]
• Social worker [CHC]
• Speech Pathologist [RHH (x2) & CHC]

Educational Professionals:

• Deaf/Blind teacher [RIDBC]
• Gross motor skills teacher [gymbaroo]
• Hearing consultant
• Occupational therapist [ELT]
• Physiotherapist [ELT]
• Speech pathologist [ELT]
• Teacher [ELT]
• Vision consultant [ELT]

Losing power

Last night we had a live test of losing power while Raphael is connected up to his CPAP machine.

For some reason one of our electrical appliances acted up and tripped the safety switch that powers our house. The Oxygen monitor alarmed for just long enough to wake me up before its aged battery died. Floundering up from my deep sleep and trying to find my torch in the pitch black, I could hear Raphael starting to snore without the CPAP operating and so I knew that he was ok.

After calling our electrical provider and resetting our safety switch, I got back to sleep at 3:15am only to be awoken again at 3:45 by one, rather sad sounding, oxygen alarm beep as the power was cut off again. I reset the switch again and this time I was only awarded 15 minutes before the power failed again and now I couldn't reset the power switch without it immediately tripping. The next hour was spent by me hunting around the house turning off all of the power points I could think of, in my sleepy state, and resetting the safety switch. I had to try this several times until I had though of the device which was actually causing the problem.

I checked on Raphael every few minutes during this time but he happily slept through the whole thing, apparently oblivious to the lack of sleep that my concern for him was causing.

Yucky ear

This morning I went to put Raphael's hearing aid in and I found a disgusting brown goo in his ear. Yes he has an ear infection and this brown goo is apparently what you get if you have grommets that allow it to ooze out. So we have more antibiotics to give to the poor lad: eardrops (sofradex) and the regular amoxycillin.

It is also weighing day again so I weighed him while we were visiting the doctor surgery and he weighed 10.03kg. This is a respectable 210g gain since the last weighing but it is still a 270g loss since we started the trial.

Development notes

Raphael is cruising a bit more confidently now and sometimes he is willing to transfer his grip from the low table to another nearby object, but usually he will stay standing around the table until he is tired and then complains to us, because he doesn't know how to get down by himself. We have been trying to teach him how to get down but it is like trying to bend a plank of wood - except this one complains at us.

Cruising transfer:



Can't get Down:



He has become very eager to feed himself with a spoon and eat food that his older siblings are eating. This has posed a bit of a problem becuase he can now reach some of the food that his siblings eat and we have caught him putting food in his mouth on a number of occasions. Of course this makes him choke and vomit.

He is able to draw with crayons a bit now. Well by draw I mean that he can throw the piece of paper off the table and make a stripe and some hammered dots on the table where the paper used to be. This is usually followed by an attempt to chew the top off the crayon which, while isn't desirable in itself, is encouraging that he hasn't developed an oral aversion which is common in tube fed babies.

Sick and hot

We measured his temperature as 38.4c on two occasions today. Panandol made him feel a lot better on both occasions but his fluid intake is well down today. His home care nurse visited and she recommended that we give him some gastrolyte to re-hydrate him a bit becuase he would not drink water or his Nutrini (formula). He is not dehydrated yet but of course we don't want him to become that way so I went to the chemist and bought some Hydralyte (which is a re-hydration fluid like gastrolyte). He was happy to take 60ml of that from a 20ml syringe.

Another long paediatrician appointment

We had another 1.5 hour paediatrician appointment today. I have been quite sick and so it took all of my concentration to follow what was going on during this consultation, at one point the doctor asked if I felt ok because I had gone pale and broken out in a sweat.

Raphael had re-hydrated himself since his last weighing and he weighed in at as respectable 10.18kg in the hospital. Again the dietician and paediatrician are happy to continue the trial without the NGT for another month.

We are also going to have a third in-depth barium swallow to examine Raphael's swallowing mechanism.

During the consultation, our paediatrician said that we are brave parents because we are willing to explore how far Raphael can go without committing him to surgery. I have to say that there is no more effective way to crush someone's bravery by telling them how brave they are. But for me, the safest course of action (surgery and drugs) is not something that I think would make my son's life worth living. So my bravery is not grounded in bold confidence but rather in choosing the long term life benefit over the short term risks. This means that I need to take other courses of action to mitigate the short term risks.

Other topics covered:

• Weight gain, feeding, milk, solids and vomiting
• This last cold that he has had
• Aspiration risks and fundoplication
• Other CHARGE kids swallow development and reflux
• Fundraising for going to Australasian CHARGE conference next year
• List of CHARGE features from UK website. Notably
• Cranial nerves
• Gross motor skills
• Language skills
• Dental issues including teeth grinding
• Sleep sitting parasomnia (like sleep walking except Raphael can't balance standing up up and so he just sits up)

Here is a video of his sleep sitting:

Another weigh-in (9.82kg)

Today I weighed Raphael at 9.82kg. which is a loss of 380g. I am not entirely surprised because he has been sick, vomiting and not eating much over the last week. His appetite is still not good at the moment, so I don't know how the next week will be.

Still sick

After a number of evening with high temepratures and a few visits from the home care nurses, I took Raphael to see a GP today. Although his ears were ok, a throat examination showed a pussy adenoiditis. He is on antibiotics now and the good news is that although we don't have the NGT to give him the medicine, he is willing to take it by mouth with no problems.

I assume that this is on top of his cold that his his two siblings and I also have, so even if the antibiotics fix his adenoids, he will still be miserable.

How much does he weigh now? (10.2kg)

I didn't have much hope for Raphael's weight gain today because he has been grumpy and a bit off his food and last night a home care nurse visited and we took his temperature at 38.6.

But today I was surprised by the weighing. His blanket had lost 20 grams and was only 450g but he had gained a little weight and now weighs 10.2kg, that's a 60g increase.

Orientation and mobility presentation

This evening we went to an introduction to orientation and mobility (O&M) presentation done by Raphael's vision therapist at the Early Learning campus. The primary focus was for children with vision impairments but the content was still very relevant for Raphael. I am looking forwarded to working more with the vision therapist to help Raphael learn how to travel safely and take note of his environment to help him learn and play.

Half way through I was supposed to go to my Auslan course but when I got there it was all quiet and the lights were off. Then I checked my mobile phone and I found that the whole course had been cancelled due to lack of members. That was a bit disappointing because I was really enjoying that course and I was learning a lot. I will have to start to place more formalised personal structure in learning from the other resources that I have for signing.

After the O&M presentation we were given the opportunity to wear occlusion glasses that simulate Raphael's vision loss. It was an eye-opening experience (if you will pardon the pun). Even though I was aware of Raphael's vision field losses, this practical experience showed me what it was actually like to experience that vision. My most notable impression is that I can now see why people think that he has good vision by casually observing him; but I am also much more aware of what he is missing out on and how limiting it is to see what can be found in his environment. I found that my hearing would react to things that my vision would normally detect first and so I was frequently in a state of surprise. Raphael of course does not have the benefit of normal hearing and so this sense of environmental awareness is further limited for him.

After my wife and I had both had a turn we put the glasses on Raphael, first in the opposite way to his actual vision which confirmed that it effectively blinded him. He was unwilling to move anywhere and briefly made an unsuccessful groping motion with his hand to find something to touch. When we turned them around to match his vision loss he was happy to crawl around again but eventually signed "finished" to indicate that he had enough of wearing the glasses (caught on the last photo in this blog entry).

Helping with FRACP exams

Today Raphael and I went to the hospital, as requested, to volunteer as guinea-pigs for paediatric doctor candidates to examine as part of their tests to become paediatricians.

It was very interesting to see how these candidates questioned me and examined Raphael to try to obtain a full description of Raphael's condition and our family situation. More specifically it was educational for me to hear the areas that paediatricians need to cover in an initial consultation.

Raphael was classified as a long case and this meant that he was only able to be examined by the examiners and two candidates in the morning that he was there. The examiners asked us to be as helpful as possible and as forthcoming as we could be with regards to my son's condition; but when I brought out my medical summary, the examiners kindly and respectfully asked to have a look and then requested that I put it away and allow themselves and the candidates to conduct the interview and examination without referring to the documentation that I had brought with me. They suggested that it would not be helpful to the testing process if the candidates all came out of this examination with (effectively) a copy of the information that I had pre-prepared rather than teasing the information out my memory at the time. I always rely on notes to recall my memory to Raphael's condition so it was a real challenge for me to be able to describe my son's condition without my notes.

It would be nice to know how the two candidates went that examined Raphael today but I suspect that information is confidential and I won't find out. However, afterwards I spoke to a registrar who said that parents generally have a good idea as to whether a candidate would pass a particular examination or not, based on how well we thought the student got all of the information out of the parent.

Paediatrician, no-NGT review

We had a review with our paediatrician and dietician today. We brought in our ever growing list of topics that we have questions about but after more than an hour we had still only covered one topic - how he is going without his NGT.

Even though he has lost a small amount of weight, his consumption hasn't been that bad. This mixed with Raphael seeming to be very active over the last fortnight has lead our paediatrician to say that she is pleasantly surprised that he has done so well so far and is happy for us to continue the trial.

We are also planning to start to add more energy rich solids/purée to his diet in an attempt to start to introduce more variety.

Weighing again (10.14kg)

Today I took Raphael to weigh him again and he weighed in at 10.14kg. This means that he has lost another 80g. Well I suppose at least he is consistent.

His blanket is still 470g. Today I was secretly hoping that it would have been lighter so that he would have been heavier; but of course this was not the case.

On a different set of scales (at the hospital) he weighed 10.35kg on Friday. I am not relying on this to say that he has put on weight though because I want to make sure that I always weigh him bare on the same set of scales so I can compare his weight to the the previous weighings.

We have been vigilantly recording exactly what he has been eating. The two graphs indicate the volume (in ml) and energy (in kilojoules) that he has been taking each day for the last two weeks.

New CPAP mask and faulty CPAP machine

A home care nurse came today and made adjustments to a new type of mask so that it would fit Raphael and hopefully put less pressure on his face.

We don't know if it will be better or worse than the last mask (in terms of leaks) but there is one thing that is definitely better: the mask makes hardly any noise. The old mask made a "sssssssss" sound where the air came out of the escape vents but this new ones vents are completely silent.

On another note, we have been told that our model of CPAP machine has been recalled due to a manufacturing fault. We were asked to return the CPAP machine to the hospital and pick up a replacement but, on further prying, it may be the case that they won't have a machine waiting to replace the one that we bring back and so we don't know how long he would have to be without one.

We trialled Raphael without CPAP last night while we were away from home and we didn't hear any complete obstructions but we could hear that his airway was very narrow and it was hard work for him to breath while he was sleeping.

Because of this experience, we are not keen on Raphael being without his CPAP machine for more than a night. We are hoping that our home care team will be able to arrange a new machine for us before we return the reportedly faulty machine.

Intensivists

Today we went to have a consultation with two Intensivists and a home care nurse at the hospital. We are still happy to proceed with the CPAP at home even though it does make for very interrupted sleep.

We also talked about the possibility of facial deformity because of the CPAP straps and discussed attempting to use a different type of mask that reduces the pressure. We are planning to try to fit this mask on Sunday night.

Measuring up for a rail

Today Raphael's Occupational Therapist visited to measure him up to find out what height rails would be needed for our front steps.

Our front steps are quite high and it is expected that his balance problems will cause him great difficulty in getting down them so we are looking at the possibility of installing a rail so that he will be able to get down the steps easily. We are hoping that disability services will fund the installation of the rail for us, but there are no guarantees of this.

Weight gain, development and another cold (10.22kg)

This morning I excitedly pushed Raphael in his pram to the local doctor surgery to weigh him again. Over the last seven days he has taken an average of well over 500ml by mouth (of mostly Nutrini high energy multi fibre) each day. This is only 50ml per day less than his total intake when we were using the overnight NGT feeds so I was confident that he would have put on quite a bit of weight.

I stripped his clothes off like last time to weigh him but this was not very popular this time. Maybe it had something to do with the weather (it was freezing cold). I carefully weighed him, and the blanket that he was sitting on, and then came up with his total weight being 10.22kg. That is 80g less than last time . I can't explain why he has not put on weight considering how much he has eaten! Because he was so distressed, being naked on the scales, I clothed him and balanced him back on the scales to take this photo of him.

On the up side, while we were at our first aid course at early learning today Raphael "cruised" by himself without anyone's hand to hold onto. He did need some encouragement from his mean dad who kept on taking his toys away from him and putting them out of his reach; forcing him to cruise to the toys.

While he has been sleeping, his breathing has been poor over the last day and a half. He seems to be very congested. At the moment his saturations are only 90% and we are waiting for the home care nurse to arrive tonight to have a look at him and possible raise his CPAP to a higher level.

Development observations

Raphael has recently started tall kneeling (kneeling with his thighs perpendicular to the floor) without holding on to anything for a couple of seconds at a time. He seems to be confident with this but then always reaches to grab hold of me before he has any chance of falling.

He has just recently started to sign "time" back to us when we sign "bed" "time" to him and put him in bed. "Time" is a complicated sign but he does a pretty good job of it with a pointed finger and two hands.

On the other hand he uses "finished" less frequently now and is more likely just to turn away from food offered to him. If asked if he is all done then he will enthusiastically sign finished (two handed) to confirm his desire not to eat any more. Also he has begin to use a one handed "finished" sign to indicate that he is having a good time so it can be a bit of a challenge to work out what he really wants. If he is signing "finished" and turns away from food when offered then that is the message that he does not want to eat any more.

Summary so far

The following article was written for the Australasian CHARGE Syndrome Association newsletter. I have included it here for those who do not get access to those newsletters.

My name is Paul Bartlett, my third child was born on 18/3/2006. We already had names selected for a girl or a boy and so we instantly know what to call him. Raphael Shalom Bartlett came into the world with raspy, gurgly breathing (stridor), a crooked mouth and a funny ear but that didn’t stop us from instantly loving him.

Everyone told us that a lot of babies have stridors and facial palsy and that we could probably expect it to clear up in a few days. After a few days he still had the same little peculiarities and people comments turned to "it will probably clear up in the first few weeks". After a few weeks the predictions turned to "a few months" but by this time another problem had become to surface. Before he was three months old we visited a paediatrician because he wasn't putting on weight fast enough. This referred onto an ENT and barium swallow x-ray in the same day. Back then I thought (naively) that having three medical appointments in one day was probably some kind of record.

More work ensued over the following months looking for the reason that he was not putting on weight and trying different things to get Raphael to gain weight culminating in final success with placement of an NGT (NasoGastric Tube) to supplement his feeding by mouth. During this time we had an echo cardiograph that looked for vascular rings around his oesophagus. There wasn't one but it found a small ASD (Atrial Septal Defect) to add to his list of problems. His osteopath, who was trying to therapeutically resolve his facial palsy, noticed a difference in Raphael's eyes which was ultimately diagnosed as being a microphthalmus (small eye) with a large coloboma of the optic disc and retina. Later, a trip to an audiologist revealed that Raphael had hearing problems and the list of issues was beginning to form a small mountain of reports and letters detailing Raphael's problems.

Eventually an MRI was performed on his head and chest to try to find out what was going on inside him. The anaesthetic gave him a rough time but no permanent damage was done. The MRI revealed yet another problem with his vestibular semi-circular canals (which provides a sense of balance). This was enough for the geneticist to make the diagnosis of CHARGE syndrome for Raphael. He indicated to us that this was a very rare condition and according to his database this is the first case in Tasmania.

Researching on the web for any information on CHARGE syndrome, we came across information that seemed to match other issues that we had noticed in Raphael but previously had not considered them to be important. We found excellent sources of information from the CHARGE Syndrome Association of Australasia, the CHARGE Syndrome Foundation (US), the CHARGE Syndrome Listserv (US) and CHARGE Family Support Group (UK). Their websites, published documents and parent contributions have given us excellent sources of information and platforms for questions to our medical consultants.

So the number of appointment started to grow. Adding to the early intervention and therapy appointments came a bunch of tests to look for the other known potential problems with CHARGE syndrome. Three appointments in one day was no longer a record for us and it was becoming the norm. To date our record number of appointments in one day is eight.

I tell Raphael's full story on a blog that I keep about him. You can find it at http://raphaelincharge.blogspot.com/

Early learning and first aid again today

Today was the second first aid lesson covering burns, bleeding and shock.

Our kids had a fabulous time in the early learning centre and Raphael's teacher commented to us today that she still hasn't hear him cry. I think that I have already mentioned that he is a placid child but I thought that I would provide this evidence to my claim.

He does cry from time-to-time but it is rare for him to decide that there is a good enough reason to dedicate the energy to a vocal cry.

Trialling without NGT (10.3kg)

This week we are trialling Raphael without the NGT to see if he still needs the night time feeds. Our paediatrician said that she doesn't think that our trial will be successful but understands that we want to try before committing Raphael to surgery to have a G-tube inserted.

Raphael did not have an NGT feed last night and after his morning milk drink (of 115ml) we took him to our local doctor surgery and weighed him at 10.3kg (bare weight).

Carer payment

I (or rather my wife) have recently received a letter from the Carer Payment (child) Review Taskforce with a questionnaire about our experience with the carer payment. I thought that it had some interesting questions so I am sharing here my answers to those questions. I don't want to breach their copyright so I will not quote their questions but I will summarise the gist of them.

1. The first question is about the general issues that we face as carers.
Our son's disability has had a number of major influences on our lives and family lives.

• Large amounts of time are consumed looking after our son. We have a large number of long medical and educational appointments. At the moment we have four weekly appointments and countless numbers of approximately quarterly appointments which means that not a week goes by without a couple of them. We use eight educational professionals and twenty two medical professional. Our busiest day so far involved eight appointments finishing at 11:00pm. Often the appointments are so important that we spend the whole preceding evening preparing questions and information for the consultant; and after the appointment I go through the audio recording to make sure I have understood everything that we have talked about, doing follow-up research when necessary.
  
• The complexity of his disability means that it is necessary for both of us (parents) to attend his medical appointments to completely grasp what is going on with our son. This creates a problem for babysitting for our other two children. Fortunately my mother-in-law is very helpful for baby sitting for us but she is also caring for an old gentleman and so it can be very difficult to juggle priorities some days.
  
• Both of us (parents) have opted to work part time to care for our family. As we are both public servants this has been relatively easy for us to achieve but severely limits career and superannuation prospects.
• We would like to travel to visit relatives but our son's medical requirements mean that one of needs to stay home with him.
• I find that our son's frequent hospitalisations to be very stressful and I am often sick during or after his hospitalisation.
• His medical equipment, that he is attached to at night, alarms multiple times each night requiring one of us to get up and attend to him or the equipment. He sleeps though the night but only on two occasions in the last two months have we had a whole night of undisturbed sleep. We are expecting that this will be the case for a number of years to come.
• Our paediatrician recently told us that, at this stage, she only trusts three groups of people to monitor/care for our complicated son. Royal Hobart paediatric unit, Melbourne Children's Hospital, and us as parents. While it is good to know that she trusts us, it also highlights that, practically speaking, we cannot leave Raphael with anyone and he must always be with us unless he is in hospital.
  

Receiving the carer payment has actually been negligible compared to the benefits that come with it. The concession card and the maximised family tax benefit (because we receive the carer payment) far outweigh the meagre amount of money that we actually receive as carer payment. We are acutely aware that if we earn too much then we will exclude ourselves from receiving the carer payment (and related benefits) and so I have stopped applying for promotions. We have calculated that it will actually result in a significant financial loss of I earn to much money.

We have found centrelink very difficult to deal with. We often find that if we call centrelink multiple times about the same issue, we receive many different answers. It seems as though there is a vast difference in experience and even though we ask very specific and well defined questions such as: "can you please tell me how my carer payment figure is calculated?", the first two times that we called the person told us that they could not tell us how it was calculated and that we are not allowed to know how it is calculated. The third time that we called we spoke to a very knowledgeable person who was very helpful and was able to explain the calculations completely. We have had very similar experiences in the past when we have called centrelink about family tax benefit. I also find that the fortnightly estimate system to be completely inadequate for our financial situation but I don't have time to go into the details here. I would much rather that it be calculated by financial year like family tax benefit.

2.The second question is about applying for carer payment.
Applying for carer payment was quite stressful and frustrating because of the tight deadlines involved.

After we found out how severe our son's condition was we spent a lot of our time going to medical and educational appointments.

When I found out that we might be eligible for financial aid from the government I called centrelink. It took many tries to get though because the number was usually engaged. After I managed to speak with someone they advised me that we would definitely be eligible for Carer allowance (because of my son's diagnosis of CHARGE syndrome) and we might be eligible for carer payment pending a doctor filling in a form about the severity of Raphael's disability. Then I was told that the payments are only backdated to the time that I made my first enquiry; and then only if I return the completed form within a fortnight from the time that I contacted centrelink. It is not like Raphael only needed additional care from the date that I called centrelink so I can't think of a good reason that it should not be backdated to when he was born.

In addition, a fortnight might sound like plenty of time but when you have to allow a couple of days for the form to arrive in the mail, a couple of days to book an appointment with a doctor, and a couple of days to post the letter back; it doesn't leave much time to fit the GP doctor appointment in amongst the myriad of other doctor and educational appointments that it was necessary for us to attend.

We only just managed to fit the appointment in on time with our GP and fortunately the actual process for her to fill in the form was quite straight forward. I can't say that I remember all of the medical requirements although I remember that I did understand what they were about at the time.

From my memory questions on the form to apply for carer payment seemed to be mostly based on medical requirements and very little based on educational requirements. Even though we maintain private health insurance, Hobart's only hospital for children is the public hospital and so we have relatively few medical expenses. On the other hand many of our special educational expenses (especially physical therapy and language/sign-language training) are not subsidised and so these are very expensive for us.

The final thing to note on this topic is that once we had submitted the form, a person called us to say that it was the best/most completed form that this person had ever had received and thanked us for our attention tot he fine detail. Even though this may have been the case, we received a call a few days later asking us to submit more information.

3. The third question is asking for comments on the eligibility criteria.
I can't say that I have any firm opinion on whether the eligibility criteria are appropriate. I don't want to profit, financially, from my son's disability but I am grateful for the money because it has gone towards therapies, technology, and conferences that we would had had to pay for by ourselves otherwise.

The Cynical bone in my body notices that if I spend a lot of money and time on my son then he may improve as he gets older and possibly void my eligibility for the carer payment, and all the other more substantial benefits that come along with it. Of course my intention is to do what is best for my son regardless of whether I receive or don't receive any aid from anyone.

The income assessment for carer payment is absurd. For instance, I have a house loan and money in an account offsetting the interest payable on the house loan. Under the current calculations the money in my offset account is deemed to be earning me interest (which of course is not true) and an arbitrary amount of money is calculated as income earned from this money but the interest payments on that loan are not considered. If I paid off the loan, with the money, then we would receive more carer payment; even though we are not worth more or less than before. The only difference is that we would be less flexible with regards to our cash flow in the event of an emergency needing a large sum of money.

4. The final question is just asking about any extra information that I might wish to include.
The only other thing that I have not included is the extra brain power and emotional strength that I need to draw on.

It is necessary that I learn Auslan to help my son communicate and have a significant amount of medical knowledge to be able to cope with his every day needs and emergency situations. I obtain this knowledge from both informal sources and formally from training courses (such as first aid training). My opinion is that the costs associated with purchasing training and educational material does seem to have been considered in the way that the carer payment is made as the focus only on the medical requirements.

You can read in detail about my son's life so far on a detailed blog that I keep at:
http://raphaelincharge.blogspot.com/

Turning CPAP back down again

Raphael has finally been deemed well enough to have his CPAP turned back down from 9.0 to 8.0. A home care nurse came to do the honours for us tonight and Raphael slept though the minor adjustment without a stir. He is still happily sleeping now with blood oxygen saturations between 97-99%.

I am hoping that tonight will be better than last night, which woke me up nine times from various alarms going off.

First aid course

This afternoon Annie and I went to the first of four St John's first aid sessions covering resuscitation and Choking. These topics are particularly relevant for us because of Raphael's breathing and swallowing problems.

The course has been subsidised and held at Early Learning, who also provided child care in an adjacent room for our three kids which was fabulous. The kids all had a great time and we didn't have to worry about being too far away from Raphael for too long.

Circus Quirkus

While waiting for our last paediatrician appointment one of the nurses gave us tickets for out whole family to go to a show (Circus Quirkus). We went last night and had a great time (It is the first time that we have been out as a family for something other than a medical appointment for quite a while). While we were there we saw Raphael's paediatrician across the crowded hall. (I don't know how may people were there but if I asked I might guess five hundred). We used the opportunity to say hello and disguise a medical question in amongst some small talk.

Raphael, with his hearing aids in, seemed to really enjoy the music which is very encouraging as far as his hearing skills go.

New mould

We received a new hearing aid mould in the mail yesterday and this has fixed the squealing problems with his left ear. Finally we have squeal free hearing aids; at least for the moment.

Paediatrician

We had a quick paediatrician consultation today to review Raphael after his hospital discharge and to talk about the results from the tests. The tests were all clear btu this still means that we don't realy know what has been giving him the last three high temperatures. We are guessing that it is just cold viruses.

We talked about the possibility of trialling Raphael on oral feeding without using his NGT before we make the current solution more permanent with a G-tube. Our paediatrician doesn't think that it will be successful but is happy for us to try. Her main concern is that the increased volume taken by mouth might result in an aspiration pneumonia and if this happens then we will have to abort the trial.

On another note, even though the latest antibiotics have given him severe diarrhoea, he reached 10.0kg today; Yay!

Cruising

Raphael cruised for the first time today. He climbed up to the arm of an arm chair and then when I offered my hand, just as a stable hand-hold, he grabbed hold and walked sideways toward me. Then he tripped over my shoes that I had left on the floor.

Oh no, high temperature again

After Raphael's snooze yesterday he woke up very groggy, he slumped in my arms all afternoon and evening. We took his temperature at 39.1 degrees and so we called the home care nurse. She came to have a look at him and after further consultation with an intensivist we gave him some Paracetamol and waited till the morning.

Last night was the best night's sleep that we have had in a very long time there were no alarms (apart from the morning NGT of course) and his saturations were 97%-100% all night. He woke up in the morning back to his usual self.

The home care nurse came again to check him out in the morning and after measuring no significant temperature she took a nasal secretion specimen from him for analysis. We expect the results to be available in a couple of days.

Free again

Raphael's regular paediatrician discharged him today so I finally have him at home. He had me worried for a little bit when he coughed and coughed for several minutes. But finally he managed to expel a small flood of mucus and then he was happy again

Nearly escaped from hospital

A paediatrician came around in the morning and was satisfied to send us home in the afternoon after another dose of IV antibiotics. But before midday one of our paediatric intensivists came to see us and said that she was not happy about Raphael going home today and that she wanted him to have close observation tonight. She wanted him to have a night in hospital without any oxygen before sending him home.

Because he has had a spiking temperature over the past few weeks, she is concerned that he will be back again in another couple of days unless they do everything that can be done in the hospital. So he is stuck in the hospital again at least for tonight.

DEM

Our home care nurse arranged the emergency department so that we only had to wait for an hour before Raphael was seen. After the registrar had a poke and prode, she ordered oxygen for him and arranged an xray and a bed on the ward tonight.

Making appointments is always very difficult because we have so many of them and it is impossible for us to be able to remember when we are free. So I bought my wife a new PDA phone to give her access to her calendar wherever she goes. Today I am passing time in DEM (Department of Emergency Medicine) by writing this blog entry on it. I am beginning to wonder whether I bought this phone for my wife or myself.

Back to hospital

Raphael had a high temperature last night and today he has also become grumpy along with maintaining his high temperature. A home care nurse spoke with an intensivist who has advised us to come into the hospital.

So as I type, Annie is driving Raphael to the hospital and I will walk down from work to meet them there in a few minutes.

Disability Services

My wife met with a representative of disability services today about arranging continued respite for a few hours a week after the current temporary arrangement ceases.

This looks like it will go ahead and so we are very happy about this.

No alarms last night

Wow, we had a whole nights sleep last night with no alarms; except for when the overnight NGT feed finished and when our other sick kids got up and needed attention in the early morning.

This is the first full nights sleep that we have had with no CPAP or O2 alarm since we started with the CPAP.

Intensivist followup

This afternoon we went to have a follow up with the intensivist who fitted Raphael's CPAP mask.

We mentioned that our biggest problem was that the alarms go off at night and wake us up. He reminded us that this was not going to be a picnic but then offered to arrange a new massimo(sp?) monitor for us that is less likely to alarm erroneously. We will have to wait for quite a few weeks before this can be arranged, unless we had a spare $8,000 to buy one for ourselves.

One of the home care nurses was at the consultation and as the intensivist was was leaving shortly to catch a plane, and would be away for a few weeks, he asked the nurse to arrange the equipment for us. The look of "this is impossible" crossed her face and the two exchanged a few words about who to speak to to get this done. By the end I think that the poor nurse still felt that it was a monumental task.

Later that night the home care nurse dropped off a new mask for us becuase the old one has a small split in the nose cushion that is starting to widen. But unfortunately it was the wrong mask. The device that she brought was actually connected to the sort of hat that we were using and so this is where the confusion must have happened. The current mask still works so we will keep using that one until the replacement arrives. The nurse also adjusted the CPAP level back down to 8.0. It was raised to 9.0 when he had his last cold but since he is getting over it the intensivist advised lowering it back down again.

Early learning

My wife took Raphael to Early Learning this morning I believe that the general advice was that the Physiotherapist was happy with Raphael's development but the OT (occupational therapist) thought that he was lagging quite a bit.

The problem with his fine motor skills might be becuase other children his age are picking up small pieces of food with their fingers to eat but of course he doesn't get this experience; he can't even eat mashed potato, let alone a whole sultana. So we are going through our toys looking for things with small things to grab hold of. Of course we can't just give him tiny things because of the risk that he might put them in his mouth and choke on them. The only thing that I can think of at the moment is baby puzzles that have the little knobs on the pieces to pick them up by.

My wife believes that Raphael has made attempts at verbalising a few words:
All done: "Ada"
Zài jiàn (Chinese for goodbye): "Dyadya"
Gěi: (Chinese for give): Ge

She claims that the evidence is that:

• the sound is approximately correct
• he has said these things more than once; and
• he says them in conjunction with sign language (or action in the case of give)
  

I think that Annie is still being a bit optimistic about this being speech. I suspect that this might turn out to be another "head scratching incident" as he has not signed mummy in a very long time. But regardless of this we will use his sounds back to him (and with the correct pronunciation) to reinforce this behavior and with any luck he will learn that he has actually said an understandable word and not just a coincidental grunt.

Hearing aid adjustments

We visited Australian Hearing this afternoon for some adjustments to Raphael's hearing aids. The first task was to fit a new right ear mould. This latest mould fits far better than the previous attempts and so we were hoping that this will stop the dreaded squealing.

We then tested his hearing with another VROA test but this time he was tested with one hearing aid in at a time. This showed that his right hearing aid is boosting his hearing ability down to about 25db which is good enough for conversational hearing. His left ear however still behaved poorly even with his hearing aid.

The Audiologist further amplified the relevant frequencies of Raphael's left hearing aid but noticed that he mould is getting a bit loose. she took another mould of Raphael's left ear to get another mould made (that sentence is a bit confusing, I wonder what the real terminology is). This new mould will be posted to us when it is ready.

Sure enough, by the time we got Raphael home, both hearing aids were playing a chorus of squeals. Annie will be going to Aus hearing on Friday for the RIDBC teleconference and so she will ask them to do something about it then.

The factors are working against us:

• His right ear doesn't have enough cartilage in it to hold a hearing aid properly and so any movement, jogs the aid out slightly and starts a feedback squeal. The new mould is better but still not perfect.
  
• His left ear requires more amplification but because the aid is a little loose, this amplification has resulted in the left aid squealing.
• The combination of: cold weather, low set ears and not much neck to speak of means that his clothes are usually pressing the bottom of his ears when he is sitting and this of course dislodges the moulds slightly causing more squealing.

Development observations

He currently has seven teeth and they look ok at the moment (not that I am a qualified dentist).

He is now able to manipulate two objects in one hand. We can demonstrate this by handing him three objects one at a time. The first he takes with his right hand, the next he takes with his left hand and when offered the third, he sometimes transfers an object from one hand into, the already occupied, other hand before taki9ng the object.

Raphael is learning his bed time routine well. Last night Annie and I were going about the business of setting up of of his night time equipment when he lifted his leg in the air when we went to put his oxygen monitor on his foot. This isn't the first time he has done this and we are now convinced that he has learned this behaviour. He has only been on CPAP for a very choppy/interrupted month and so it took us by surprise that he has learned to help us, by doing this, so quickly.

On the down side, he has vomited three times today so far. He is still not very well.

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.

Higher temperature

last night a home care nurse visited us again to check on Raphael's progress. His temperature had risen to 39 and he was quite placid. Normally when he has high temperature he is grumpy. Being a little concerned, the nurse contacted our paediatrician again and she sat with Raphael while he slept for an hour to make sure that he was going to be ok at night.

He was fine all last night and the only equipment alarm that went off was the NGT pump which just an easily fixed kink in the tube that stopping the flow at one point.

Without this fabulous service and care from the home care team we would probably have had to admit Raphael to hospital last night so I am very grateful to the people that make this service possible.

Bronchiolitis

Home care nurse visited again this morning to check up on Raphael. She is concerned about him and thinks that he is a borderline case of needing oxygen overnight tonight because of his respiratory difficulties. We are not equipped at home to supply oxygen to Raphael with his CPAP so he would have to go to hospital if he needed this.

We put him down for a morning sleep with his CPAP machine and agreed to take him into the ward tomorrow for a Paediatric resident to check him. I personally didn't think that he needed to go to hospital but I was not going to reject the advice.

He slept very well with the CPAP and I took him in after he woke up. I believe that the resident was a bit overwhelmed by Raphael's condition. Raphael always appears to be in slight respiratory distress, even when he is well, so it is hard for doctors to decide whether he needs close monitoring in hospital. In the hospital his temperature was measured as 37.5 and it was noted that he had more respiratory distress than normal but was well hydrated. The resident called the on-call paediatric consultant, who just so happened to be our paediatrician, and returned to us with the same opinion. Everyone agreed that he is a borderline case for hospitalisation.

This places the decision on us and so I brought him back home with the advice to bring him into emergency if we think he heeds to be hospitalised.

Stupid machines!

Last night a home care nurse visited to see if anything could be done about the pressure sores that Raphael has been getting on his forehead from the CPAP mask.

Fortunately when she was visiting she also noticed some of the problems that we had been having with the oxygen monitor. She ended up going to get another one and taking the faulty one away.

Last night was not a good night in terms of alarms. Even with the new Oxygen monitor the alarms came thick and fast from the machine last night. Raphael is sick with a cold but was not having bad desaturations; he was coughing a lot which caused him to move which, in turn, resulted in faulty readings for the Oxygen monitor.

At about 3:00am we gave him some infant cough medicine (Demazin). We also turned him onto his other side and within thirty minutes his saturation monitor showed better readings and he did not wake up for the rest of the night.

While we were having problems with the probes at night we tried something new. Rather than move a probe when it was causing problems, we simply added another one to another foot or hand and connected it to the machine to see if the new location would be any better. In the morning I discovered the problem to this approach: Raphael had manage to tightly bind his feet together and was working on tying his hand probe tail to the NGT chord. It took me ages to get him untangled and at one point I too managed to connect myself to the octopus of chords and Elastoplast tape.

Coming surgery

We currently don't have a date for Raphael's next surgery. We are waiting for Raphael's anaesthetist to return and then, presumably, a date will be arranged for the surgery.

We expect the following will be performed:

• ENT endoscope (performed after the pre anaesthetic medication but before the GA is issued)
• tear duct probe
• G-tube insertion
• fundoplication (here is a video of a fundoplication performed by laparoscopy. Raphael will actually have his performed by laparotomy to reduce the risks of complications.
  

One appointment becomes four

This morning We had an appointment at the hospital for our other son. This just so happened to be with the same surgeon who is scheduled to perform Raphael's coming surgery.

We had Raphael with us and at one point the Surgeon stopped and commented on the nasty cough that Raphael has barking. He said that this might be an issue for his surgery next week. When we said that we were happy for Raphael's preferred anaesthetist to make that decision on the day before the surgery the surgeon replied by saying that the anaesthetist in question would be on leave, and would not be back for seven weeks. Annie and I were dumbfounded. We thought that we had already confirmed that Raphael's anaesthetist would be available and so we didn't know what to say. Later the surgeon confirmed for us that particular anaesthetist would definitely not be available.

Due to an administrative error we still had a paediatrician appointment today that should have been cancelled. So we took advantage of this appointment and had a consultation with our paediatrician about what we were going to do. She was a little concerned about his current cold symptoms and so this, mixed with the absence of Raphael's anaesthetist, resulted in her suggesting that we wait until after his anaesthetist is back. Annie and I were relieved with this assessment.

While we waited between the two appointments we made two impromptu appointments with the hospital social worker (to ask about respite options) and dietician (to talk about his weight gain and dietary requirement).

Radio Interview

Today I gave an interview to a local radio station about how good the Royal Hobart Hospital has been for Raphael. It was very short and superficial but I hope that it is enough. It is for an appeal to raise funds for the "give me 5 for kids" programme. The money raised will go towards purchasing medical equipment for the Royal Hobart Hospital to enable kids to go home rather than be forced to stay in the hospital.

The presenter thought that the programme would go to air on Tuesday coming but he was not sure about that. He offered to contact me closer to the date to let me know when it will be on.

More notes on development

Raphael has started to make more sounds:

• "dya-dya-dya"
• "adaadaadaadaa"
• "aiyaiyaiya"

I think that his hearing aids are doing him a lot of good!

He is also proficient at getting hold of things and pulling himself up to his feet, via the correct half kneeling position. He has even just started to stagger a couple of steps while being supported. He hasn't learned how to get back down to the ground elegantly though.

Ophthalmologist 2nd opinion

This afternoon we took Raphael to RHH to see an orthoptist and to get a second opinion from an ophthalmologist, who has specific experience in genetic and paediatric ophthalmology.

The orthoptist tested Raphael's vision with rather peculiar grey boards with black and white stripy lines on one side and a tiny hole in the middle to look through from behind. She span the board around till I felt dizzy and then stopped and looked though the little hole from behind to see if Raphael looked at the side of the board with the stripes. She repeated this with boards that had finer and finer stripes until she declared that Raphael's vision appears to be quite good; at least the vision out of his good eye is quite good anyway.

The ophthalmologist consultation was very interesting. The result from the pressure test on the 18/4/2007 was that the pressure in his right eye was 15mmHg (this is well within the safe range). He examined Raphael and said that Raphael had a coloboma in his right eye as well as the left. This right eye coloboma was small and very low down which meant that it would probably only effect the very top of Raphael's vision. He also recommended that Raphael's tear ducts only be probed and not flushed when they are examined in his coming surgery.

Topics covered:

• Raphael's general vision
• Colobomas
• Eye pressures
  
• Probing and flushing tear ducts at next surgery
  
• What can be done to help his left eye
• Retinal detachment
• Critical developmental ages for eyes
• Eye sizes
• Optic nerve hypoplasia and micropthalmia
• Type of eye specialists
• potential treatments in the future

Hearing aid mould modifications

I took Raphael to Australian Hearing this morning for another attempt to resolve the squealing right hearing aid. The Audiologist cut excess material from the existing mould and took a new impression to make yet another mould.

Out of hospital

Raphael has finally escaped from hospital today. We have a course of antibiotics to give to him over then next five days.

In hospital again

Raphael had a hard night on Thursday 31/5. He coughed up mucus into his CPAP mask and we think that he may have inhaled some back into his lungs. He didn't have a temperature but he was starting to cough, reminding us of previous times when we had to admit him to hospital. After taking the mask off and putting him on his stomach he coughed up a lot more mucus. Annie and I couldn't agree on whether to refit his mask again and so we called on a home care nurse again. The nurse visited us and had a listen for any problems in his lungs and we eventually refitted his mask. The rest of the night was generally uneventful (apart from a few "high leak" mask alarms)

Early Friday morning (1/6) we called on the home care nurse again because we found that Raphael had a high temperature. Sure enough we packed up and went into hospital again and dumped our other kids on one of our baby sitters. He was inspected by a doctor and a chest x-ray and blood was taken. The verdict was that Raphael probably just has a viral infection that isn't causing pneumonia yet but given his history, and condition, it would be wise to admit him over night to monitor him if he gets worse.

Our paediatrician was on the paediatric ward and so we managed to ask her our two burning questions about the surgery coming up on the 12/6 (it has been brought forward from the 14/6). We wanted to know what people can eat after having fundoplications and we wanted to make sure that our preferred anaesthetist was going to be able to perform the anaesthesia on the modified surgical date. Her answers put our minds at rest and we are all ready to proceed with the surgery on 12th of June.

Later that day a registrar tried unsuccessfully to insert an intravenous line to give him antibiotics (just in case). Eventually he was given an injection into his muscle to slowly release antibiotics over a period of twenty four hours. Apparently this is much more painful for the child and so it is blended with a local anaesthetic to make the pain go away quickly. The irony is that the anaesthetic causes more pain while the injection is being given.

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: