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.