Special Disability Trust

We have spent a couple of days investigating Special Disability Trusts. To summarise our findings of them:

They cost a fortune to set up, they are a pain to maintain, the tax law surrounding them is a obscene and they are ridiculously limited in what they can be used for. They are rubbish!

Apparently there are four public servants managing it and there are currently only 10 such trusts set up in Australia.

I am not asking for much. I just want to give some money to my disabled child to ensure that his future is financially secure. But if I do then social security don't recognise that I have done so and the tax office will take the lions share of what I put in.

Mama

Normally I don't talk about things other than Raphael in this blog but I will make an exception for this.

My mother-in-law (Mama) was in a car accident shortly after 2:00pm on Monday 12/5/2008 (the day after mother's day). Annie and I were attending a future planning day for children with disabilities and Mama was getting ready to run some errands and then pick up our daughter from school. It is not known exactly what happened but somehow her unpowered car came loose in her driveway and she probably tried to stop the car which ultimately resulted in her becoming crushed between the car and the house.

Mama was rushed to hospital and we found out about the accident at about 5:00pm when we got home from the planning day. We rushed into hospital only to find out that we had just missed her. She had been taken into surgery with severe internal injuries and a few broken bones. I rushed back home to arrange babysitting for the kids and the hospital kindly agreed to look after Raphael for the night as he was only just out of hospital and our babysitter was not trained to handle the CPAP machine. After I made it back into hospital to be with Annie, I found her talking with the surgeon who had said that they had done all they could and now they just had to hope that her liver would stop bleeding. We went back to waiting but it was only a few short hours with praying before we were told that she had died.

Annie was Mama's only child and they had a very special bond with each other. Mama also provided us with invaluable help by babysitting our other kids while we took Raphael to appointments. The news was a big shock to us and Annie took it particularly badly. Mama was also the sole carer for an elderly veteran who was a close family friend and her passing means that we inherit the responsibility of care. He has no family who can help him and he has been like a father to Annie in Australia. To our children he is uncle Max, he is the only honorary-family-member that we have.

Max has just sold his house that he and Mama lived in and has bought a new house close to where we lived so they could see more of the children and so that we could help each other more. the new house is in the middle of being renovated and Mama has packed up most of the old house but there is still a lot that needs to be done and Annie and I will have to do that because Max is not able to this for himself.

Mama leaves a memory of generosity and kindness but she will also be remembered for her furious rage that would boil over occasionally. The only reason that I mention this is that the time of her accident matched up with the awful earthquake in southern China on that same day. Her anger could be so enormous that I would not be surprised if her raw anger over the accident was a catalyst for this natural disaster.

The photo here is was when she was about 40 and was her favourite photo of herself.

We will miss her.

in hospital again

Raphael went in to have his pre-assessment on 8/5/2008 for his upcoming adenotonsillectomy but as he was getting quite sick we took him around to the PACU area to squeeze in to see a doctor to see what they thought about him. Shipping him back and forth between PACU and the pre-assessment area when doctors were ready to see him filled our day.

I felt a bit guilty about turning up to the packed clinic times with people over flowing in the waiting area only to push in line to see the poor overworked registrar. When I told the registrar that I suspected that an x-ray will probably reveal a pneumonia she said "I think that you should really present to DEM (Eepartment of Emergency Medicine)". I responded with a cheeky "Yes but that will take us ages and you are supposed to make special exceptions for people like us (with complicated children)". She took pity and organised a queue jumping x-ray for us which of course revealed a pneumonia.

This wrecked his chances of having the adenotonsillectomy surgery any time soon and instead landed him in hospital for observation for a couple of days. He was discharged on 10/5/2008.

Everyday appointments

This last month has been very hectic with appointments every day. I have been going to a "magic with music" course to help with Raphael's speech amongst other appointments. I have not had time to have a good scratch and so that is why I have not blogged much lately.

Wife on leave

Annie and our daughter have gone to China for three weeks leaving me with the boys at home.

She left this morning and so far Raphael has already vomited in the car, just as I was turning onto the southern outlet (a kind of free-way). That was really annoying and it was the stinkiest vomit I have ever smelt him do.

I was tired after a morning and afternoon out with the boys and the last thing that I wanted to do was clean him and the car seat up after we got home but there was no-one else and so I had to do it.

A couple of learning to talk classes

We have been recently attending a couple of courses to learn to help Raphael learn to talk.

It takes two to talk and Magic with music. They are both courses developed by Hanen.

I personally find the material dull and repetitive but I recognise the importance in the information that they provide and I can see how it is helping Raphael learn to talk.

Australian hearing and signing playgroup

Another Australian Hearing appointment today confirmed that the grommets are no longer in effect and it looks like he might have more fluid in his right ear. We will have to decide whether we want them to be reinserted during his next operation.

He performed as expected on his hearing tests and new moulds were taken to try to resolve the increasing problems that we are having from feedback (squealing).

It was a long appointment and we arrived at Raphael's new signing playgroup with only 15 minutes left. We had a brash introduction to the teachers there and I was a little disappointed that there was a lot of talking happening there. I was hoping that it might be a speech-free playgroup to encourage Auslan development for the children but there seemed to be more English than Auslan. Maybe all of the administrative work necessitated using a language that most people were fluent with and maybe the quantity of signing will increase next time.

Gross motor skills

Shortly after we found out that Raphael had a completely malformed vestibular (balance organ) and only monocular vision we were told that we could expect problems for Raphael being able to sit.

Then when he was sitting we were told that there would be real trouble for him to be able to pull to stand.

Now he walks!

He now happily chooses between walking unaided, crawling, and pushing a trolley. He is even trying to increase speed, although this usually ends up with a runaway effect followed by a crash that only a father could laugh at while the mother is not present. Turning is a tricky business and sometimes he has a very wide turning circle involving precariously placed footsteps with near toppling consequences, but he usually doesn't fall over during a turn though.

Two year old Ocupational Therapy assessment

Raphael had an Occupational Therapy (OT) assessment today and passed with flying colours.

This gives us even more incentive to focus on his communication and gross motor skills which are lagging behind significantly.

Busy day with appointments

I had a hectic day yesterday, but here I will just talk about Raphael's appointments.

In the morning we had a consultation with a physiotherapist with lots of standing and walking practice. Handling uneven ground and very small steps is the next objective for him now. Also we will try throwing objects while standing up. I think we will be able to find a good game of throwing ping-pong balls at each other while standing.

He is confidently walking with a trolley that I hand customized for him. I added swivel wheels to the bottom of a normal child's walking trolley so that he can swing it around on the ground easily. I made it with some old bed wheels which were quite high and I was planning on going to buy some lower ones becuase initially I thought that it would be too unstable. But now looking at it, I am happy that it is not rock solid as it makes him practice his balance skills more and not just lean on the trolley all the time. He loves it and he seems to be walking with it all the time so I am happy that I spent the time on the modifications.

Later in the day we had an ENT appointment to book in his Adenotonsilectomy surgery for May when his normal ENT returns from being away. One of his grommets has probably extruded (fallen out) and is just sitting in his ear canal. Apparently it can take weeks for a grommet to travel down the canal in normal conditions but putting his hearing aids in all the time might be perpetually pushing it back up the canal and not permitting it to fall out by itself. When he is under anaesthetic for his surgery in May they will have a thorough look and see if they can just be taken away they will also look for any more sign of "glue" in his middle ears.

We also had a meeting with a home care nurse about his CPAP mask. It is starting to get too small and it is tricky to get on him at night without obscuring his nostrils. We had tried a larger one but it gave him a big blister on his cheek. We will try taping up his face with soft tape and giving the new mask another go tonight but I am not confident in succeeding with this.

We were also planning to see one of his intensivists but she was stuck in an emergency case so we abandoned that plan and will proceed without the consult until we exhaust our current CPAP ideas.

Two years old today

Raphael has made it to two years old. Good-on-ya little buddy! After a near disaster only a few days ago, we are proud that he has made it this far.

His fine motor skills are excellent, but his walking is still designed around crashing and he still uses crawling for his main method of transportation.

He is currently on antibiotics because of his inflamed tonsils and this last year seems to have had more than its fair share of hospitalisations.

His communication is disappointing but we are persisting with communication training courses and Auslan. Thankfully, just recently he has started using some more signs.

We now count 11 signs that we are sure that he is using and a further 4 that he might be using:

• finished
• more
• nappy
• ready
• go
• hello/goodbye
• bib
• hearing aid
• time (bed time)
• yummy
• safety belt
  

possible:

• turn around
• car
• pack up
• listen

His vision seems to be stable. He can see most things except if they come from his left and he still short grabs some times.

We have been patching his right eye to try to get as much vision as we can out of his left eye before the window of oportunity passes. He is not keen on having his right eye patched and sometimes hides his face when we tell him that we are going to patch him. But he doesn't complain about it any more and goes about his business as usual while patched. He even tries to walk sometimes, while patched, but he is much less competent this way. I like to think that his left eye's vision is improving but we won't know until we do further vision tests. He is extremely happy when we take his eye patch off.

FACSIA Carer Payment review and stories

Some time ago I filled in a survey on the Carer Payment and was invited to share Raphael's story in a publication to be submitted with the recommendations of the review to the minister.

The recommendations and stories are now publicly available at: http://www.facsia.gov.au/internet/facsinternet.nsf/disabilities/carers-review_carer.htm called the "Carer's Storybook". Raphael has changed a little since then but it is still an interesting read.

Paediatrician and paediatric intensivist

Raphael weighed in at 10.97kg today. This is a good result! His head circumference is big but he is still very short.

He was a little sick and so the paed gave us a script for antibiotics. She reassured us that his vomit in CPAP two days ago doesn't appeared to have had any detrimental effect on him.

We also talked about our concern over Raphael's speech and she gave us some ideas on avenues we could go down to ultimately improve his communication.

After the consualtion we ran into one of Raphael's intensive care consultants and we had a brief chat about his mask getting to small for him. She kindly went and got a batch of masks that were around his size but there wasn't one that was going to fit him. She said that she would contact her interstate colleagues and see if there was anything that might fit Raphael. In the mean time she gave us a maks that we can attack with some scissors to try to get it to fit.

Vomit in CPAP

Raphael vomited into his CPAP mask last night and we were lucky to catch it and prevent him from drowning. A number of events conspired against us last night resulting in a near disaster.

Within 15 minutes of putting Raphael to sleep with his CPAP Raphael vomited into his nasal mask and his mouth filled up with vomit to. For some reason he did not sit up or roll over and instead just struggled with no source of air.

The Oximeter alarm went off when his blood oxygen level degraded below 85% but neither my wife nor I reacted to this alarm immediately. I heard the alarm but I was doing some business in the "little boys room" and I expected Annie to go to the alarm. Tonight however we had forgotten to turn on the baby monitor in our lounge room which we need to be able to hear the Oximeter alarm in the lounge room. Our current Oximeter is only monotone and is diffucult to hear in other parts of the house without the baby monitor to relay the alarm sound. In addition to this we get so many "false alarms" that I had become complacent and was happy to complete the task that I was attending to before going to see what was the matter. I was expecting that it was just Raphael having pulled off the Oximeter probe.

As soon as I entered the room though I realized that the problem was much more serious. The room was dark but there was two numbers glowing on the Oximeter his pulse rate and his Blood Oxigen saturation. The pulse rate was on the high end of normal but the other number burned into my eyeballs as 71. Going over to him I turned on the bedside lamp and saw his nasal mask which was full of vomit and his face covered with vomit. He was pulling at his nasal mask trying to take it off and his mouth was opening and closing trying to expel the pool of vomit that was in there.

Yelling out for my wife, I sat him up and ripped off his mask. He sat there for a moment without breathing and then coughed and vomited a lakes worth into the chuck cloth that I grabbed from his bed.

He recovered quickly and after a mop-up and a change of clothes and bedding he was was ready to go back to sleep. Sitting with him on our bed and recovering from the shock, he lent over and gave both of us a gentle stroke on the arm in turn (one of his ways of showing affection). This picked up our spirits immensely.

After we put him back down to sleep I turned the baby monitor on and then called the home care nursing team to let them know of the problem that we had just experienced. They checked with the registrar and were happy with what we had done. We have an appointment with our Paediatrician in two days and mentioned that any aspiration infections will probably start to show around that time so it is important to talk with the doctor then about the incident.

We were lucky that Raphael didn't drown last night.

Raphael's heart

The "H" in CHARGE - Heart

The following are the details of what we know about Raphael's heart.

Medical Information
Raphael's notable heart conditions consist of the following:

1. "Mildly dilated right atrium" (This was noted in the preliminary report and removed in the final echocardiograph 26/5/2006)
   
2. "Two small low velocity shunts (1.1-1.2 m/sec) across the inter-atrial septum, suggestive of ASD or PFO." (echocardiograph 26/5/2006). "There is a small L-R shunt across the inter-atrial septum, Vmax=1.3m/sec, suggestive of small ASD" ... "ASD/PFO" (echocardiograph 24/11/2006).
   
3. "There is a left sided aortic arch. There is no evidence of a double arch." (MRI 18/12/2006)
4. There is, however, an aberrant right subclavian artery." (MRI 18/12/2006)

Explanation of the Medical Terms
The following is my simplified understanding of the terms and/or concepts listed above:

1. Given that this finding was removed in the final report I assume that this dilation was so mild as not to be worth commenting on.
2. An ASD (Atrial Septal Defect) is a type of defect that is commonly referred to as "a hole in the heart". It has been commented that this may in fact be a PFO (Patent Foramen Ovale), rather than an ASD, which is a common finding which may rectify itself with in the first few years of life.
3. The left sided aortic arch is a blood vessel that pushes on the oesophagus slightly. Fortunately this is not repeated on the other side as well.
   
4. An aberrant right subclavian artery is just a rare configuration in the rout of the right subclavian artery.
   

The Implications of These Conditions

1. No implication unless it is actually dilated
   
2. This reports that Raphael has a small ASD/PFO that seems to have no practical consequences.
3. I wonder whether the pressure on his oesophagus is partially responsible for his swallowing problems. A double arch could result in a ring around his oesophagus and trachea, choking him and preventing swallowing, but he does not have this.
4. There are no practical implications of having an aberrant subclavian artery. It is only relevant to make sure that medial professionals are aware of the condition when procedures are being carried out in the area.
   

Summary:
Raphael does not have any significant heart problems. The minor issues only seem to be relevant to anaesthetists and medical emergencies.

Another new sign: "nappy"

Raphael as started to sign nappy. He is very clear and he knows what it means but I don't think that he has put together the sensation of having a dirty nappy ad needing to have his nappy changed.

For instance If I tell him that it is bed time then he might sign "time" or "hearing aid" or "nappy" all things that we attend to at bed time. But he hasn't signed nappy due to having soiled it.

Finally signing "more"

Our first two earnest efforts to get Raphael to sign was with the signs "all done" and "more".

As Raphael always seemed to be "all done" when it came to eating he leared that one very early on. It was his first sign. And so we eagerly kept pushing for him to learn "more" but time dragged on and it didn't look like he was every going to say or sign more.

But all of a sudden here it is "more", spoken poorly, but signed nicely. He has been doing it for a few days now and it is very clear.

Ophthalmologist

We had a quick Ophthalmologist appointment today.

While waiting for the appointment we noticed some people signing in the waiting room and when I saw one of them comment of Raphael's hearing aids I introduced myself and took the opportunity to practice some Auslan on them.

There is nothing new to really comment on from the appointment except that the Doctor offered to examine the articles that I had found with regards to the benefits of occlusion (patching) with a severely malformed eye. I am looking forward to his reply to my email that I sent to him with the articles.

Intensivist consultation

We had an intensivist consultation today straight after discharge to talk about CPAP settings, adenotonsilectomy and future possible sleep study.

Finally out of hospital

Yay, finally out of hospital today with oral antibiotics and an iron supliment. Apparently he has low iron.

Admitted to hospital with pneumonia

Yesterday Raphael didn't eat anything and didn't drink much all day and vomited what he drank. He hasn't been eating anything for a week now and he has been drinking less and less of his formula each day so yesterday when he had practically nothing I thought I should call the paediatric ward and ask them what I should be looking for with dehydration. The head nurse went to ask the registrar and came back with the asnwer "bring him in to DEM (Department of Emergency Medicine) so that the registrar can have a look at him". I wasn't very happy about this, but when I pushed for my original question to be answered the nurse did not respond well so I brought Raphael into Emergency in the hospital.

On arrival to DEM we noticed that it was quite busy. There was a person waiting in front of us with a bucket. She was obviously very unwell and just in case she was contagious, I moved Raphael's pram around to face the other way away from her. At that moment another guy came into emergency, also carrying a bucket. I shuffled Raphael's pram away from him too but became quite sandwiched between these two sick people. After going through the triage desk I was asked to sit in the priority seats but one look at them saw little space between even more people I was beginning to think that I had taken Raphael into hospital just so he could catch another vomiting disease. I sat a short distance away from "vomit row" and sadistically amused myself by noticing that when one of the poor soles vomited others joined in the chorus.

After only 1.5 hours we saw the registrar and his examination showed that Raphael was only mildly dehydrated and he could go home and stay home if his intake improved tomorrow. Before we left though he wanted Raphael to have an x-ray just to make sure that Raphael didn't have a pneumonia. Well and you know the result of this already... he had pneumonia.

Raphael stayed in last night and he was a little better today, they suspect viral pneumonia but are giving him oral antibiotics as a "just in case" measure. He will probably be in for a few days so they can monitor his condition.

Not happy about language development

Raphael has been very slow with his language development lately and his new speech pathologist was very unhappy about him only having only about six signs and fewer spoken words.

We are working on doubling his vocabulary by selecting a number of new nouns that are important to him and encouraging/forcing him to sign them before acting on what he wants.

He has made up a new sign where he taps the back of his hand with his index finger. He is starting to use it quite a lot while looking expectantly to us, but Annie and I have yet to work out what it is supposed to mean. I am not sure if he knows what it means either so I think that we'll just force a translate of "shoe" and give him his shoes along with the correct sign whenever he signs his version.

Sick again

We called a home care nurse out today because Raphael has a bad cough and sure enough later in the day when the nurse arrives his temperature had risen.

Fortunately his breathing is still ok so we wont need to goto hospital... yet...

Walking, wow!

Home care nurse project review

Today we attended a meeting to talk with the hospital about the home care nursing. This was an important meeting with a few families and hospital staff involved with the home care nursing team. The goal was to gather information to achieve a permanent home care nursing programme for high needs children.

Injection and bloods at PACU

Raphael was subjected to some needles this morning involving an intramuscular testosterone injection and taking 12ml of blood for tests (thyroid, haemachromatosis, and CHD7 genetic test).

I am sure that they had previously taken blood for CHD7 genetic testing and I was surprised to see that it had been added to the list of things that blood was being taken for. But when I grilled them about this the kind nursing staff checked the records and could find not find any blood being kept for Raphael's genetic testing so it would have to be done anyway.

He has previously been very difficult to get blood from and I don't think that the registrar who came in to do the job had successfully taken blood from Raphael before. However, this was the most successful time ever and Raphael didn't even vomit when he was distressed about what was being done to him.

Head nodding and shaking

Raphael has begun to nod and shake his head when indicating yes and no to us. You have to have a sharp eye to notice that he does it because it is over so quickly, but we are now convinced that he is using it as part of his everyday language.

ENT

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

New signing skills

Raphael has just signed some two sign sentences:
"This", "there" (put this in there)
"that", "head" (put that on my head)
"finished", "there" (I am all done, put this pencil away)

Paediatrician

A one hour consultation involved discussing Raphael's letters, that we have received from the doctors in Sydney. We also talked about his most recent ear infection and more about the possibility of surgery to have his tonsils and adenoids removed.

First optometrist visit

We saw an optometrist for Raphael the first time today. He spent a bit of time examining the behaviour of Raphael's left eye. We were very happy with the large amount of time that he gave to us but, after careful examination, his summary was the same as what all the other eye professionals have been telling us.

There is little very little research on the benefits of eye patching to improve the vision of an eye with extensive physical defects. Simply there is no chance for the left eye to be visually active while his right eye is so useful to him.

The only benefit that could be achieved in improving the vision in his left eye is that, in the unlikely event of him losing his sight in his right eye, his left eye will at least have the best vision possible.

New communication skills

Raphael has a new sign "here". He uses it to indicate where you should put the thing that is the current focus of his attention.

He also has a new word that is nearly understandable: "helo", he says "aaaaoooo".

Questions people keep asking us (or should ask us)

I will slowly modify and add to this over time

Name: Raphael Shalom Bartlett
Birth date: 18/3/2006
Handedness: Right ( I think)

Chief Problem: CHARGE syndrome

Mother and Father: Paul and Annie Bartlett

home phone: ___________________

mobile: ___________________

Important:
before performing any test or invasive procedure on Raphael (including suctioning) try to call me on my home number and work number. We find that Raphael is often given incorrect types of tests because of his complicated situation.

Don't suction Raphael, just wipe his nose. If the secretions are causing him discomfort then just use the suction hub (don't poke the tube up his nose or in his mouth, as this will cause him unnecessary distress).

Allergies:
No known allergies but:

• Codeine is a candidate as a cause for a particularly bad oxygen desaturation while sleeping in hospital once.
• Raphael is at high risk of aspiration (and aspiration pneumonia) because of his swallowing dysfunction and reflux/vomiting. Do not feed Raphael foods that cause him to vomit (see "Feeding" below)

Sleeping:
Raphael has twosleeps of a day:

1. Afternoon sleep from about 14:00 to 16:00
2. Night sleep from about 20:00 to 06:00 with CPAP
   

Raphael sleeps without a pillow. His airway is less obstructive if he sleeps on his side but he may roll onto either side and/or his back during his sleep. Raphael is used to falling asleep by himself and will do so by himself at his sleeping times unless there is too much stimulation. Before his sleep time, remove his hearing aids and it is generally a good idea to drape sheets over the side of his cot (if in hospital) until he is asleep so that he does not get distracted by the other activity in his room. With the exception of his night time sleep, if he is woken up then it is unlikely that he will go back top sleep.

It is important that he gets these sleeps as they interact with his feeding schedule (see below).

Feeding:
Raphael has been diagnosed with failure to thrive (FTT). He does not voluntarily take enough food to sustain his growth and he is prone to vomiting if he eats too much or the wrong types of foods. For this reason it is important to stick as close to the feeding schedule as possible.

He has two types of feeds:

1. Puréed/smooth solids with fortification (eg Paediasure)
2. Milk (Nutrini high-energy multi-fibre) by straw cup
   

Puréed solids
Raphael can only successfully swallow fluids and highly puréed foods. Other foods will collect at the back of his throat and cause him to cough and vomit. Raphael can manage smooth yoghurt, custard, fruit purée, and puréed sweet potato. Mashed potato is too lumpy and will guarantee a vomit. blend in one scoop of Paediasure for each 40g of purée that you feed to him for fortification.

If Raphael starts to cough then it is possible that he is about to vomit. Always be prepared with a decent sized cloth nearby to catch it.

When Raphael has eaten enough he might sign "finished" by twisting his fists slightly. He may even vocalise "A'da" (all done) as well and refuse food by turning away or pushing the food away.

When Raphael is sick he is less likely to want to eat puréed foods and usually prefers Nutrini.

Milk (Nutrini) by straw cup
Raphael drinks milk throughout the day as he wants it but we offer it to him specifically in the morning, at lunch time and in the evening. He could drink anything from nothing to 200ml depending on his mood.

For the milk feed, allow Raphael to sit up and hold the cup himself. He would like to be able to put the cup on a flat surface that he can reach to drink again at a later time.


CPAP:
Raphael sleeps at night with his CPAP machine to reduce his sleep apnoea. Normally his setting is 8.0 but when he is sick the intensivists usually order this to be raised to 9.0 to improve his blood oxygen saturations.

Our CPAP routine is:

1. Attach oxygen saturation probe.
2. fit CPAP mask. Note that the top three straps should be left alone as they don't need to be adjusted and if they are then it makes it harder to fit later.
3. Turn on CPAP.
   
4. When removing the CPAP mask, turn off the airflow and then disconnect both lower straps. Leave the top three straps alone.
   

Blood Oxygen Monitoring:

• Raphael's normal awake blood oxygen saturation is usually between 95%-99%.
• When deep sleeping he can desaturate down to 75% and his airway obstructs often. Raphael's obstructions appear to be worse when sleeping on his back and/or if his head is tilted forward.
  
• When sleeping with CPAP his saturations are usually between 97%-100%, sometimes as low as 95%
  
• When Raphael is sick his saturation fluctuates between 85%-93% while he is awake. while he is asleep this can be worse.
• When sick and on CPAP his saturations have been measured at around 91%. Adding a small amount of oxygen to the flow (even as low as two litres/minute) can bring his saturations back up to 95%.
  

Comforting:
If we (the parents) are not on the ward then we will not be far away. Common causes for when he is grumpy:

• Been held for too long
  
• Nappy needs changing
• Tired
• High temperature
• Wants something he can't reach
• Wants to go down (he may be pointing to the ground if you can find his hand)
  

Raphael might throw mini tantrums if he does not get something that he wants and is indicating so (by pointing). Distraction may work but this is getting harder as he becomes more stubborn.

Raphael loves to crawl around and always enjoys exploring the playroom.

We recognise that in hospital television is a useful tool to distract children from their problems and it is possible to distract Raphael with television but keep in mind that his moderate to severe hearing loss and left eye blindness can reduce his interest. The following are some tips to overcome this:

• Fit his hearing aids.
• Make sure he is sitting squarely towards the visual stimulation.
• Do not amplify the sound to a level that you think is too loud for yourself; this may distort the sound that Raphael hears.
• Even Raphael's good eye (right eye) has a problem that makes it difficult for him to see objects higher than eye level. It would be better if the visual stimulation was provided at his eye level rather than requiring him to look up to see it.
  

Communication:
Raphael has a moderate to severe hearing loss in both ears which is manageable with hearing aids. With or without hearing aids Raphael understands and uses some sign language. Useful signs that he uses include:

• "bed time"
• "drink"
• "hungry" and "eat"
• "Medicine"
  
• "Finished"
• "More"
  
• "Tissue"
• "Bib"
• "Shoes"
  

Raphael is effectively blind in his left eye and his right eye may have a reduced upper field of vision. Engage him front on and at his eye level to get his attention.

Daily Routine:

• ~6:00-7:00: Raphael wakes up. We disconnect his CPAP when he wakes.
  
• ~8:30: Offer fortified puréed fruit in a bowl with a spoon and Nutrini Energy Multi Fibre (in a straw cup)
• ~10:30: Maybe hungry again, offer left over purified fruit or fortified custard.
  
• ~12:00: Offer fortified yoghurt in a bowl with a spoon with Nutrini Energy Multi Fibre (in a straw cup)
• ~14:00-15:00: afternoon sleep (sleeps for 1-2 hours)
• ~16:00-17:00: Offer puréed pumpkin in a bowl with a spoon and Nutrini Energy Multi Fibre (in a straw cup)
• ~20:00: Night time sleep
• Offer Nutrini Energy Multi Fibre (in a straw cup)
• Brush teeth
  
• Connect CPAP mask and then turn it on (8.0 normally but sometimes raised to 9.0 when he is sick). Raphael usually falls asleep within a few seconds of the CPAP turning on)
  

Bathing:
We normally bath Raphael once per week on a Saturday. You can bath him more if you want (eg in case of a huge vomit)

Previous Hospitalisations:

• 20/11/2008-21/11/2008: Sleep study in Monash
  
• 3/7/2008-6/7/2008: adenotonsilectomy
  
• 16/6/2008-20/6/2008: admitted to hospital for high temperatures with unknown reason.
  
• 8/5/2008-10/5/2008: admitted to hospital for pneumonia.
• 11/2/2008-13/2/2008: admitted to hospital for pneumonia.
  
• 12/10/2007-13/10/2007: admitted to hospital because of upper respiratory infection and swolen tonsils. Constant observations because of possible risk of tonsils closing airway while sleeping.
  
• 22/6/2007-25/6/2007: admitted to hospital for upper respiratory tract infection. High temperature was noted on 12/6/2007 but not hospitalised at that time as Raphael appeared to recover.
• 1/6/2007-3/6/2007: admitted to hospital for bronchiolitis (upper respiratory tract infection).
• 4/5/2007-19/5/2007: admitted to hospital for possible aspiration pneumonia and CPAP fitting and training.
• 17/4/2007-19/4/2007: CT scan, grommet insertions, ABR test and eye pressure test under general anaesthetic. Good result from anaesthesia. This hospital stay noted the Codeine incident (see Allergies above).
• 25/3/2007-27/3/2007: hospitalised for two night sleep study. Study showed frequent and severe blood oxygen desaturations.
• 21/3/2007-24/3/2007: admitted to hospital for possible aspiration pneumonia.
• 28/2/2007-1/3/2007: admitted to hospital for possible aspiration pneumonia.
• 18/12/2006-19/12/2006: admitted to day surgery for MRI scan. Reacted poorly to anaesthetic and hospitalised for one night.
• 4/12/2006-8/12/2006: hospitalised for NGT placement and training.
• 18/3/2006: Born in Calvary hospital. Normal full term delivery.

Immunisations:
Up-to-date (as of 11/2/2008).
(he has had his 18 month old vaccinations).

Drugs Previously Taken:

• temp list:
• Ciprofloxacin (ear drops) - antibiotic for middle ear infection (first taken 31/1/2008)
  
• Prednisolone (oraly) - anti-inflamitory? (used to reduce the swelling of Raphael's tonsins)
• Demazin (oraly) - cough management
• Paracetamol (oraly) - pain relief
• Ibuprofin (oraly) - pain relief
• *Codine (iv[?]) - pain releife (*see allergies section)
• Amoxicillin (oraly intramuscular[?] and iv[?]) - antibiotic
• Ceftriaxone (iv) - antibiotic
• Clamohexal Duo (oraly) - antibiotic
  
• Sofradex (ear drops) - antibiotic
  
• local anaesthetic applied along with intramuscular antibiotic
• Drying medication for general anaesthetic
• General anaesthetic and associated drugs
  

We lost a hearing aid

We went to Hobart's summer festival on the 30th of December 2007 and when we were about to go home we noticed that Raphael only had one hearing aid in. I was really tired but I couldn't just go back to Australian Hearing and say that I lost the hearing aid but didn't even look for it.
I didn't think that there was any hope for finding it. The summer festival stretched from parliament house lawns all the way along the docks. I think that I heard that there was an average attendance of 30,000 people per day to the festival but because that was the Sunday I think that there were more. I reckon that there were well over 50,000 people there and some areas were totally packed.

So with this impossibility of finding a needle in a haystack I trudged back along the path that we had walked scanning the ground hoping to see a scuffed, kicked or trodden on hearing aid. I asked stands along the way to locate the areas where lost property was kept. The first lost property that I found didn't have it, only an umbrella, a hat and a coat.

I was just about to give in but there was another lost property place to check and so I knocked on the the little temporary police hut's door. As I stepped in asking "has a baby's hearing aid been turned in..." I saw it sitting all by itself in the middle of the desk. It was in perfect condition, not stepped on, scuffed or kicked about; amazing! I had found some of my family during my search and they had joined in the hunt; when I showed them my success they were also amazed.
Apparently it was found on the parliament house lawns where we started our day in the festival. Interestingly I did not even look on the lawns because I thought that I had seen him with both hearing aids after we had left them but thank goodness someone picked it up and handed it to a policeman.

It was much quieter on the lawns by the time I took this photo as all of the activity had moved down to the docks.

Wow new skills

Raphael loves to show off and when there is a big audience he is at his most adventurous. He has to get over the initial shy factor first but then watch out for exciting new things from him.

On this one extended family gathering Raphael signed Hearing aid when it was whistling.



He then demonstrated how to push it in to stop the feedback.







And then to everyone's amazement he stood up! Well only for a split second, but it was the first time that he had even tried to do it. He tried again and again until he managed to be on two legs for two seconds. After that it became a game to intentionally fall over and then laugh so that was the best he managed for that day.

I have finally updated the sydney trip post

For those of you who have been waiting patiently for the Sydney trip post to be updated, your lucky day has come. http://raphaelincharge.blogspot.com/2007/11/trip-to-sydney.html has been updated and I ave added a number of photos for the last three months of posts.

Computer expert

Check out his developing strength and balance allowing him to push up onto his tipi-toes. The Paediatric CHARGE specialist, that we saw on our Sydney trip, referred to his gross motor development as remarkable.

Dear Teacher

An example of Raphael's vision and gross motor development

Another leaking ear inefction

Raphael's chronic ear infection is leaking scum again, this time it is particularly thick, dark and bad smelling but he is still in a good mood and so does not appear to be in any pain at all. His hearing aid is not clogging up with this because I think that it is too thick.

General status summary

I think that it would be a good idea to have a general update for those people who want to know what is going on with Raphael.

He used to have a sticky eye problem with narrow or blocked tear ducts but that seems to have gone away now. He uses his vision well even though his left eye has no central vision at all. His vision has been tested as 6/30 in his right eye (where 6/6 is the equivalent of 20/20), this is normal for his age and his right eye is expected to improve. Every ophthalmologist has told us that his left eye will not improve even with patching "occlusion" but we are trying it anyway (for about an hour a day) because I have read a couple of articles that suggest that there might be hope even when there is severe eye malformations.

He still has frequent copious secretions from his nose that need regular wiping attention.

He is still unable to swallow anything lumpier than a fine purée. Anything lumpier will send him into a choking coughing fit that ends in a vomit.

He still suffers from sleep apnoea at night (stopping breathing). But when he is connected to his CPAP machine this entirely prevents the problem.

It is planned for him to have surgery to have his adenoids and tonsils removed which is expected to reduce, or even possibly cure, his sleep apnoea. This is still waiting for our ENT doctor's approval but this is expected to be a formality. We have our next ENT appointment on 17/1/2008.

Raphael is no longer using a feeding tube and has been eating enough to keep his paediatrician very happy, even though his dietician isn't always in the same mind.

His hearing seems to be worse than first expected but his hearing aids are serving him well at the moment and he is starting to try to copy words that we say. We have finally found a way to keep his hearing aids in and have much less feedback which is good. He has been having very frequent colds and these infections can cause his grommets to extrude gross puss from his right ear. This is his best ear for hearing but unfortunately his hearing aid keeps getting clogged and so does nothing for him.

Raphael's talking seems to be poor for his age, he still mainly only vocalises grunts. Having said this, his communication skills are excellent. He always makes his desires clear with body gestures, noises and props. In fact I would go as far as to say that his communication skills are so good that we can probably start to work on some of the more detailed skills of communication such as negotiation. This is a good thing because it is safe to say that Raphael's negotiation skills are extremely poor.

An early intelligence assessment test has shown that Raphael has cognitive abilities in line with his age. This is not a precise test but can give some indication of his mental skills.

Raphael can crawl very skilfully now, climb up on furniture that is not too high, cruise around furniture and transfer to different objects, walk with someone holding onto him, or using a trolley, and stand as long as he has a third point of reference (whether it be holding something or leaning up against something). I think that the next stage of standing unaided will be the real challenge because he has no depth perception and his balance organ (vestibular semicircular canals) are completely malformed.

Raphael's fine motor skills have advanced considerably. Initially he was diagnosed as "delayed" then after some time "slightly delayed" and recently his OT said that he was now "advanced".

Raphael still experiences parasomnias where he sits up in his sleep. He has started to also do other things in his sleep which are more annoying such as incessantly screaming (presumably night terrors), he sometimes stops if we turn him to another side or turn his CPAP off and back on again, but not always. He also sometimes pulls off his oxygen monitor in his sleep, this is a real pain because then an alarm sounds and inevitably he makes such a mess in getting it off that it takes us quite a while to get it sorted again before we can put it back on him again. It is nice that it is summer though, it is easier to get up at least three times a night when it is warm rather than cold. The frustrating thing is that he happily sleeps through the whole night, it is only becuase of his "beeping" equipment (pun intended) and sleep screaming that we are woken up.

Speaking a word for a goal

Today Raphael said "U! u! u! u!" (as in "Up up up up') indicating tghat he wanted to be lifted up to his high chair.

This is the first time that he has said an understandable word (not just a grunt) to ask for something that he wants.

Advanced fine motor skills

Raphael has been improving his fine motor skills lately. So much so that this Occupational Therapist has gone as far as to say that his fine motor skills are now advanced for his age.

This is amazing. He started off with people calling him delayed, then slightly delayed. We were then warned that his development will be adversely effected by him not being able to participate in eating finger foods. But now his OTs say that he is advanced for his age.

We have ready that early intervention is very successful for CHARGE kids and it is nice to be able to say that Raphael is turning out to be one of these success cases.

Well done my boy!

Meeting a lovely CHARGE family

By chance, someone at our church was taking with one of their work mates and found out that they had a child with CHARGE syndrome over 10 years ago. Unfortunately the child only lived four months but he has left a lasting memory with the family.

Today we were blessed with the opportunity of meeting with the family. They were lovely and showed us an album of photos that they had of their child. We talked for hours and we look forward to an oportunity to see them again.

I believe that the probability of knowing someone, who knows someone that has CHARGE syndrome are quite high. If I assume that I know one hundred people and each of those people knows another one hundred unique people then I calculate there to be 64% chance that one of those people will have charge syndrome. Calculated as follows

n=assumed number of unique people an individual person knows
=100

c=assumed chance of having CHARGE syndrome
=1/10,000

h=chance of knowing at least one person who has CHARGE syndrome
=sum[i=1 to 100] c*(1-c)^(i-1)

k=chance of knowing at least one person who knows at least one person has CHARGE syndrome
=(sum[i=1 to 100] h*(1-h)^(i-1))+h

I hope you can forgive my make-do mathematics without access to the necessary symbols and positioning.

Quick visit from a new home care nurse

We had a quick visit from one of the new home care nurses just to check up on Raphael. It was nice to meet someone from the new team.

High temperature, vomiting and a little surprise

Last night at 10:30 Raphael became extremely restless while sleeping and it turned out he had a fever (39 degrees).

we gave him some panadol and he was so thirsty that we gave him some hydralite but after only 15ml of the hydralite he vomited everything up. We wanted to give him another dose of panadol because he had ejected it all but we weren't sure if it was safe, we didn't want to overdose him on paracetamol.

To find out if it is ok to give another dose if the first one is vomited out we called the new home care nursing team but we knew that they closed well before 10:30 and so we weren't surprised when there was no answer. We then called the 24 hour doctor telephone service available in Hobart. This was the first time that we had called this service for Raphael and after the nurse gathered some information from us over the phone, she asked us if we wanted an ambulance or to take him into the hospital emergency room. We didn't think that he needed to go to the hospital but this is the usual reaction for medical professionals who hear/see Raphael when he is a little sick. After getting a very brief medical summary, she referred us to a doctor and he told us that it would be ok to give Raphael another dose of panadol if he had vomited the previous dose out within 25-30 minutes of it being given.

He said that it was "refreshing to hear" when we said that we didn't want to bring Raphael into hospital whenever he is sick or we would be going there every week. From looking at him this time though I quietly thought that we might end up bringing him into hospital later that night; he had slight respiratory distress and he oxygen saturations were 90-91 when on CPAP instead of his normal high 90s. If he got any worse then I was going to bring him in. The previous night I was woken up 5 times by Raphael. I needed sleep but it didn't look like I was going to get much sleep that last night either so I mentally prepared myself for another sleepless night in the emergency room. I got to sleep at about 12:30 after monitoring him for a while and he woke up at 5:30. That was much better than I expected.

He is bright as a button now and this morning while watching me prepare his breakfast he even said and signed "more" without any prompting from me. His nose is runnier than normal but I don't think that we will be taking him to the emergency room today.

Development and vomit

Raphael has done some new things. He is getting over his sickness finally and has been trying out a new sign and a new sound. He can now say "ooooo" and can copy us when we sign "more" although I dont believe that he knows what it means.

On another note I have somethign to complain about. As a general rule we try to feed Rapahel as much food as possible, but if he eats too much too quickly then he vomits. So it has become an art form to determine whether he has eaten too much recently. Today, recently, we decided that he had eaten enough for the time being but for the first time I can remember he wanted to eat more. We didn't want to risk giving him more and so we refused to give him anything. Unfortunatelly this made him upset and when he gets upset he can go into a coughing fit and when he goes into a coughing fit he is very likely to vomit; this is exactly what happened. So in an attempt to prevent him from vomiting, we caused him to vomit. That was so frustrating!

Some developmental skills

Raphael has learned two new signs:

• "yummy" and
• "hearing aid"

He uses yummy usually when we feed him something from a spoon, particularly if it was something from our plate/bowl rather than something from his own bowl. He uses "hearing aid" whenever we are about to put his aids in. The problem with this is that his hand is in the way.

He is responding quite well to verbal communication while he is wearing his hearing aids but will only verbalize language with grunts rather than pronounceable words or syllables.

On the down side his left ear's hearing seems to have become significantly worse since the gromet insertion (90db loss) this places his left ear in the severe to profound hearing loss category. It is our fear that because it seems to have become worse, it may continue to degrade in the future.

Also at the moment his right ear's grommets are leaking quite a bit of goop which keep on clogging up the hearing aid molds making them useless. We have become quite proficient in cleaning them but it is not a pleasant task to extract a thick glob of puss from the mold tube.

The boiling tube

Last night a peculiar rattling sound emanating from Raphael's equipment woke us up. It turned out to be condensation in the tube that had built up over night into a small puddle in a loop of the tube. The air blasting past the puddle of water was making it gurgle.

We emptied the tube and turned the humidification setting down a notch to see if that solves the problem. I guess we'll find out tomorrow night.

Video conferencing at home

Today two guys have come to install a video conferencing unit in our home so we don't have to go to Australian hearing to use their unit. This will be really good for us because we wont have to get baby sitters every Friday and it is one less time per week that we have to go out for our appointments with Raphael.

RIDBC are fully funding the unit. I can scarcely believe that they are willing to fund this. It is costing them a fortune in start up and ongoing costs.

Paediatrician and paediatric intensivist

Today we had a paediatrician review to discuss the Sydney trip but we weren't really ready. We only had two letters and we had not gone through any of the recordings yet to consolidate our understanding of what we had learned in Sydney.

The paediatrician was impressed by the size of Raphael's tonsils and wants us to see our ENT to discuss removal again. She would like to wait until Raphael is over his current infection though.

While we were there we managed to see Raphael's intensivist who showed us how to setup the humidifier on Raphael's CPAP machine and also gave us the ability to make minor changes to the pressure if Raphael needs it. We also discussed the possibility of using the "AutoSet" feature on the CPAP machine but decided against.

Raphael does not like to touch grass

And my wife needed a way to remind me that it was time to mow the lawn

Trip to Sydney

We traveled to Sydney from 16/10/2007 to 31/10/2007 to get some second opinions from medical paediatric specialists and educational specialists who had dealt with CHARGE syndrome.

It would have been nice to be able to say that we had a nice vacation but in reality it was really hard work. The hardest bit by far was transport. We were kindly hosted by RIDBC in a fabulous four bedroom house that they allowed us to use while we were there. But this location is not near a train station and to get to our appointments, that weren't at RIDBC, we had to catch a bus to the nearest train station, catch a train to the city and then transfer to another train or bus to get to where our appointment was. This usually took between 2.5 to 3 hours one way and after over five hours travel each day we were exhausted. The furthest we travel in Hobart is 20 minutes so this was a bit of an adjustment for us. When I have previously stayed in Sydney I have been near a train line and I have only traveled into the city which was really easy and fast. Because of this past experience, I wasn't prepared for long travel times. We had even made some 9:30am appointments; I don't know how we managed to get to them on time.

While we were there we saw:

• Medical Specialists:
• Paediatric Cardiologist
• Paediatric ENT
• Paediatric Ophthalmologist
• Paediatric Sleep Specialist
• Paediatrician
• General Practitioner
  

• Education and Allied specialists
  
• Teacher for the deaf and blind
• Specialist teacher for learning to hear
  
• Orthoptist
• Educational Psychologist
• Occupational Therapist
• Preschool teacher for the hearing impaired and Auslan signing
• Preschool teacher for the vision impaired
• Playgroup leader for hearing impaired children
  

• We also met with some "CHARGE families" (families with a member who has CHARGE syndrome). Thanks to Stephanie and Angela who made an extra special effort to meet with us. Stephanie also has a blog. Pictured to the right are photos "Oscar and Raphael" and "Stephanie and Raphael".
  

(I hope I haven't missed anyone out)

We selected all of the Medical specialists based on recommendations from other CHARGE families. It was comforting to see doctors who said "I have seen children with CHARGE syndrome before" rather than "CHARGE association... hmm, I've heard of that". Actually there was one exception to this, the GP that we saw in Sydney hadn't heard of CHARGE syndrome at all. We weren't planing on seeing a GP but of course Raphael (and the rest of us) got sick while we were there. It didn't matter that the the GP didn't know about CHARGE syndrome; he checked Raphael's lungs, airway and ears which was all that we needed.

The following is a compilation of the topics that were covered with the relevant specialists. This has taken me ages to compile (I actually only added this list to the post in very late December)

Topics covered with the ENT (Ear, Nose and Throat surgeon)

• hearing (and getting worse), current hearing aid solution good at this time.
  
• CPAP, sleep study and airway issues, also associated with tonsils
• poor feeding and regurgitation
  
• possibility of seeing gastroenterologist
• good nasal passages
• good teeth
• medications
• improvement over time
  

Topics covered with the Ophthalmologist (eye surgeon)

• extensive left eye coloboma
• right eye coloboma
• lots about patching (ocular occlusion)
• eye pressure
• retinal detachment
• photographing colobomas
• Raphael's general vision capabilities
• eye conditions associated with CHARGE syndrome

Topics covered with the Cardiologist (heart doctor)

• PFO/ASD
• aberrant left subclavian artery

Topics covered with the Paediatrician (CHARGE specialist)

• Raphael's complete medial and developmental history, conditions and diagnosis including:
  
• Notable test results
• Stridor
• funny right ear
• hearing loss
• poor weight gain
• low set ears
• short neck
• facial weakness
• hearing loss
• physical skills
• speech/communication
• ear infections
• pneumonia, choking and aspirations
• CPAP and sleep
• PFO
• Need for thyroid test
  
• Infections
  
• Grommets
• Vestibular dysfunction (balance)
  
• How to go about getting testing for the CHD7 gene
• Need for continued early intervention including total communication (signing and verbal)
• Specialists we are seeing in Sydney
• Other incidence of CHARGE in Tasmania
• Good prognosis

Topics covered with the Thorasic Surgeon (and sleep specialist)

• CPAP pressures and variable pressures
• Tonsils and adenoids
• Need for a sleep study (better after removal of tonsils and adenoids)

The most notable medical outcome of the trip is that were are almost certainly going to have Raphael's tonsils and adenoids removed to help with his breathing while sleeping.

The education and allied health specialists at RIDBC gave us excellent information while we were there including:

• how to test Raphael's responsiveness to the ling sounds
• how to incorporate a communication programme to teach both signing and verbal skills
• visual skill testing

We are still waiting for the written combined services report (as of 27/12/2007) but my wife has talked with them about their observations over the video conferencing facility.

We did have a couple of days when we could do some sight seeing for the kids. But don't think that Raphael got to fully appreciate the 3D IMAX theater, given his monocular vision. Even 3D glasses won't let him see in 3D.

Discharged

Raphael was fine last night and he was discharged this morning. We took him home and will go back later in the day to pick up his prescription of Amoxycillin and Prednisolone.

Endocrinologist, dietician, paediatrician and hospitalisation

Raphael had his first endocrinologist appointment today. There were no surprises, but it is good for us to hear from the specialist. We will have a thyroid blood test at some time but this is not urgent.

While we were waiting for the the endocrinologist we managed to see Raphael's dietician and we were able to adjust his feeding script that we receive from the hospital to reduce the amount of Nutrini and increase the amount of solids fortification (PediaSure).

Raphael had lots of coughing yesterday afternoon so while we were in the clinic we also asked to see a paediatrician to just check his lungs and make sure that he was ok. He had a temperature of 40.9c! He didn't seem to be that sick to look at him but apparently he was cooking inside.

The paediatric registrar had a look at him and his lungs sounded clear, which is good, but his tonsils were really swollen. Any other child would have been sent home but because he is complicated the registrar phoned Raphael's regular paediatrician and when she came in to have a look at him, she ordered him to the ward for the night. His tonsils were so swollen that she was concerned that may exacerbate his sleep apnoea and so wanted him to be constantly monitored overnight.

He was written a script for Penicillin (to treat any possible bacterial tonsillitis infection) and Prednisolone (to reduce the swelling of his tonsils). Of all the drugs in the world I would have thought that penicillin was probably the most well known, well maybe apart from Viagra. But the hospital didn't have any Penicillin. Isn't that kind of like the cheese shop sketch? Oh well the doctors said that Amoxycillin would be fine in this instance and so he was given that instead.

Organisations that we have approached for support and/or concessions

(originally compiled: 24/5/2007, added to: 8/8/2007 and 12/10/2007)

If having a disabled child wasn't enough work for you, then trying to find support and services to help your child adds to that load.

Initially it was hard to find any organisations that provide support because we didn't have a starting point. Then near the end of our journey of searching for support we came across an organisation that provided a large list of support organisations. It took Annie days to go through the list to try to decide which ones she would focus on calling. It then took days for each of these organisations to assess whether we qualified for the support that they would provide. We still haven't contacted all of the organisations yet and I think that we are still waiting to hear back from some of them that started their assessment of Raphael weeks ago.

Here is the list of organisations that we are utilising services and/or receiving concessions from.

National and international services:

• Australasian Charge Syndrome Association of Australasia. I have recently become a director of this organisation. The dedicated volunteers that work for it, organise events for awareness, fund-raising, conferences, and activities that can enrich the lives of people/children with CHARGE syndrome. While not wealthy, this organisation finds a way to maintain free membership for CHARGE affected individuals (and parents) while even providing some small financial assistance for its members in certain circumstances. Contact details for your local representatives can be found http://www.chargesyndrome.org.nz/contacts.html.
• Association for Children with a Disability. The website seems to be primarily for the Victorian branch but there is a Tasmanian branch and a Hobart office in Bathurst Street. I am sure that I have not fully realised the benefits that this organisation has to offer but the one thing that we have used from them was their fantastic list of service organisations. It is worth contacting them to obtain this publication called "Through the Maze - A guide through the maze of services for parents and children with a disability".
• Australian Hearing. Australian hearing is a national government service that provided the hearing aids that Raphael is using. These devices cost thousands of dollars so I am very happy that we get them for free. Look for your local branch here: http://www.hearing.com.au/findus.
• Centrelink. CHARGE syndrome automatically qualifies the carer of that person to receive a carers allowance from the Australian government. If the person in question has a disability severe enough and the carer's income is low enough then they may also qualify to receive carers payment. I have found that dealing with this organisation is a significant challenge. One tip that I have is if you don't like the answer that you are given when you call them, then call them again another day and ask the same question to see if you get a new answer that you like better. Keep trying this until you get an answer you like. The financial assistance that they provide is fabulous. If you qualify for Carer's Payment then you also qualify for a concession card.
• RIDBC (Royal Institute for Deaf and Blind Children). For us this service has taken a teacher consulting role with experts in deaf and blind children available to give advice for Raphael's development. They also send us toys to borrow that are tailored to get Raphael's interest and extend his development. The teaching/consultation sessions are done via teleconference at Australian Hearings Hobart campus. RIDBC's contact details can be found http://www.ridbc.org.au/aboutus/contactus.asp.
• Gymbaroo. This is a privately run pay-for-service activity that is like a playgroup with specific activities and equipment designed to stimulate a child's development. There is a national website here: http://www.gymbaroo.com.au/
• The Companion Card programme. To quote their website: "The Companion Card allows people with a profound disability, who require attendant care for the rest of their lives, to participate in community activities and events without discrimination. Companion Cards can be presented at participating organisations where cardholders will not be required to pay an admission fee for their companion who is providing attendant care." Unfortunately not all states and territories participate in this programme at the moment. Only Victoria, Western Australia and Tasmania currently issue and honor the card.
  

Tasmanian Services:

• Department of Infrastructure, Energy and Resources Transport Division. This Tasmanian government department is the managing body for disability parking permits. We have found that the disability parking permit has been very helpful with regards to parking near the hospital when Raphael needs to be brought in multiple times per week. They also have a reduced fee for car registration and MAIB (Motor Accidents Insurance Board) premiums for concession card holders.
  
• Early Learning Tasmania. Early Learning is part of the Tasmanian Department of Education and provides an amazing service for children below school age. They have an excellent array of services from therapies to consultations with specialist teachers and all with excellent flexibility with regards to the time that they provide the service and the location. Incidentally they have had a name change ad are now called "Early Childhood Intervention Service".
  
• Calvary Health Care Children's Therapy Services. This is an outsourced service from the Tasmanian health department. They provide physiotherapy, occupational therapy, and speech therapy for Raphael. This service is quite different from the Early Learning service in that Calvary is geared up from a health and physical development perspective where as Early Learning has an education focus. There is some peculiar rule that means that we are not supposed to utilise both services at the same time so we are going to have to choose one of them over the other. The only explanation that I can think of for this strange rule is that the governing bureaucrats must have little idea of what is actually happening in the therapy services for children.
• Home Care (home and community care - HACC). HACC is jointly funded by federal and state governments. Here is the website for federal information. We get domestic cleaning assistance from here which frees us up to spend more time with Raphael.
• Community Based Support (south). This organisation receives funding from HACC. We receive limited respite assistance from here because this organisation is low on funding, but it has been very good for Annie. We have also utilised a gardening and spring cleaning service from this organisation, which was well needed, because cleaning was one of the first things to be dropped with Raphael's time demands.
  
• Disability Services. This is part of the Tasmania's government health department. Raphael has been accepted as a client of Disability Services and they have been instrumental in organising ongoing respite. We are also in the process of applying for a home improvement to help Raphael once he starts to walk (a rail for our front steps)
• Early Support for Parents. This is a volunteer home visiting service that helps "anyone with the care and responsibility for children, especially families experiencing stress". They don't seem to have a web presence but they have some contact details here: http://www.police.tas.gov.au/community/child-protection/parenting-support
  
• Aurora Energy. Aurora is the electricity service provider Tasmania. Holders on a concession card receive a power bill concession if you apply for it. They also provide a concession for people who use certain types of medical equipment in their home.
  
• Department of Treasury and Finance. This Tasmanian government department claims to provide land tax exemptions to concession card holders. We have not realised this benefit yet becuase we did not hold the concession card at the time that our last land tax was payable.
• Royal Hobart Hospital (RHH). Raphael was born in Calvary hospital which actually made it a little difficult with regards to leveraging some of the RHH outpatient services. Fortunately our Paediatrician pulled some strings and made it possible for Raphael to use the services from RHH. The professional staff (consultants, registrars, therapists and nurses) devote an amazing level of care and time for Raphael and us.
• Hobart City Council. Our local city council provides parking concessions in the city car parks for holders of concession cards. This has saved us a lot of money because the Argyle Street car park is the only practical parking solution for when we go to the Royal Hobart Hospital. HCC also give property rates concessions to concession card holders.
  

I suspect there are more services that we receive that I have forgotten about while making this list.

First dentist visit

Raphael had his first dentist visit today. They talked about:

• the possibility of brown adult teeth because of repeated high temperatures,
• he has an obvious under-bite
• seems to have a flat nose ares (possibly due to CPAP mask?)
• any major dental work will probably have to be done under general anaesthetic because of the risks associated with his possible ASD (heart defect).
• to reduce the chance of major dental work it will be a good idea to apply a protective layer over his teeth when he is older.
• frequent visits (every 3 to 6 moths) will be beneficial to make sure that we catch any problems early and also to get Raphael comfortable at the dentist.
  

Successful applciation for companion card

Our previous application for a companion card for Raphael has been accepted and granted. We have an interim letter and we are just waiting on the official card to be printed and sent to us.

(yay)

Paediatrician and travel help

We went through a good list of questions today with Raphael's paediatrician. Just like me she is very happy with his weight gain and general appearance. Fortified feeding is still required but tube feeding is no longer being considered (which is great news). I believe that this marks the end of the "no NGT trial" and that we can call it an outstanding success.

We are giving ourselves a great big pat on the back for all the hard work that we have done but also not forgetting to praise God for this amazing turn around that no-one predicted.

After the paediatrician appointment we went to see the guy in the hospital who manages the travel support for assisting us to travel to Sydney. We were careful to clearly explain the travel and accomodation support that we will receive from other places because we don't want to "double dip". The hospital is going to be very helpful for us financially as they are willing to cover some of our taxi trips which looked like they were going to be more expensive than our air tickets.