After a long time on no apparent speech progress we change strategy about a month ago and we have had some success.
We have stopped giving him what he asks for when he signs for something and have demanded that he also have a good try at saying the word/sentence also. This seems to have paid off because in the last couple of weeks he has started using multi-word sentences and in the last couple of days his pronunciation has improved a lot. He is even occasionally letting a "t" slip out.
Q: So what has been happening lately? A: The regular drudge of appointments of course.
In addition to the occasional doctor appointment, this is what a typical week looks like at the moment:
Monday: Signing with a deaf adult followed by the early intervention playgroup
Tuesday: Speech Pathology
Wednesday: RIDBC (Royal Institute of Deaf and Blind Children) videoconference
Thursday: Teacher of the Deaf - teaching listening skills.
Friday: Childcare in a mainstream childcare centre. We are hoping to give him some real world exposure rather than the world of therapy and "special needs" that he is immersed in for the rest of the time.
Raphael had two more sleep tests in Hobart and he continued to maintain excellent saturations over night. So with this information we organised another full Polysomnography test in Melbourne for 29-30/4/2009.
To cut a long story short, this latest test confirmed the good results that he appeared to be having in Hobart.
The Doctor told us that the previous test in Melbourne showed 23 apnoea events each hour. To put this in perspective 1 is normal and 5 is extreme so Raphael was really struggling then. This recent test showed that Raphael had improved down to just 3.5 events per hour. This result is good enough to put a stop to CPAP. We were warned not to get rid of the machine though because if he comes down with a respiratory infection then he may need to temporarily go back on it again.
We also still need to continue to monitor him because since he has stopped CPAP we have noticed that his stridor (gurgly/raspy breathing) has come back again strongly during the day. If this is still there after six months then it may be worth another full sleep study in Melbourne to make sure that nothing is getting worse with his sleeping.
As for Annie and I, we are celebrating the opportunity of getting uninterrupted sleep for the first time in three years.
Raphael had a simple sleep study performed last night in hospital. This is mainly to monitor his blood oxygen saturation while not on CPAP.
The results were good in that he maintained good oxygen levels all night without a single desaturation. He even has a slight upper respiratory infection at the moment so this was an excellent effort.
We will leave him off CPAP at home for the next few nights and he will go back into hospital on Monday night to see if things are still the same.
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 necessary.
Raphael has a very sensitive Gag reflex, touching the top of his tongue with a tongue depressor is usually enough to trigger a vomit.
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. Close observation is necessary while Raphael is drinking and eating.
Sleeping: Raphael has two sleeps of a day:
Afternoon sleep from about 14:00 to 16:00
Night sleep from about 19:30 to 07:00 with CPAP
Raphael's 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 turn off) 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. If he is woken up during his afternoon sleep then it is unlikely that he will go back to sleep.
Feeding: Raphael is transitioning from fortified foods to family meals.
Currently he has a number of sources of nutritian intake:
Milk (Nutrini high-energy multi-fibre) by straw cup
Slice of bread spread with "paediasure butter" (three scoops of paediasure mixed with small amount of water to make a spreadable texture).
Sustagen Icecreams (Sustagen made up to a thick consistency and then frozen on a paddlepop stick to make an icecream).
Offered family foods at meal times. He still has occasional trouble with fibrous meat (eg beef/pork) and crunchy foods (eg raw apple) so it is neccessary to observe him while he is eating these things.
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 foods and usually prefers Nutrini.
Milk (Nutrini) by straw cup Raphael drinks milk throughout the day as he wants. He could drink anything from nothing to 400ml 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 at 5.0.
Our CPAP routine is:
Attach oxygen saturation probe.
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.
Turn on CPAP.
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%-100%.
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:
Nappy needs changing
Tired
High temperature
Frustrated about being unable to do something
Wants something but is not getting it. Common wants include drink (Nutrini), bread, TV, or whatever he is pointing at.
Raphael might throw mini tantrums if he does not get something that he wants. If he can't have it tell him no. We don't mind if he chucks a temper tantrum.
Raphael loves to walk around and always enjoys exploring the playroom. Cars, aeroplanes, books and puzzles are usually winning toys.
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 and turn them on.
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 Nutrini Energy Multi Fibre (in a straw cup) and bread spread with "Paediasure butter" (see above). He may also want a tiny amount of cereal with milk.
~10:30: Maybe hungry again, offer left overs from breakfast.
~12:00: Offer whatever is going for lunch. He is usually fond of bread, so you can't go wrong with that.
~14:00-16:00: afternoon sleep (sleeps for 1-2 hours)
~16:00: Offer Nutrini Energy Multi Fibre (in a straw cup)
~17:00: Offer whatever is going for dinner.
~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 9/4/2009). (this includes an influenza vaccination for 2009 winter). Drugs Previously Taken:
temp list:
Ciprofloxacin (ear drops) - antibiotic for middle ear infection (first taken 31/1/2008)
Prednisolone (oraly) - anti-inflamitory? (used toreduce the swelling of Raphael's tonsils)
I mentioned to another family that I have been learning Auslan and they asked me to publish my revision videos so here they are. Of course this does not replace doing an Auslan course but I hope you can get something out of them.
Raphael turned three today (yay). We had a party in the park and some present opening inside. He a very full day and ended up totally exhausted, falling asleep in the beanbag.
As you can see from his charts, we have had a very successful nutrition campaign with him that we are all very happy about. However we are now entering a new era of food with him because he is now starting to eat family food. He is now rejecting his old puree's which is unfortunate because they were very easy to fortify with extra nutrition, such as Paediasure. We still have other methods that we can use though. He still likes to drink his Nutrini milk and he loves to eat icecreams that we make out of Sustagen pudding.
we have tried osteopathy and acupuncture point massage but neither of these has made any difference.
Currently we are not doing anything to overcome this.
Antisocial behaviour
Now that we have the vomiting and aspiration under control most of the time we are starting to put firm boundaries in place for Raphael. He is no longer in charge of everything.
Poor swallow, aspirating fluids and choking on lumpy foods
Main food is pureed fruit, custard, yoghurt, and pureed pumpkin
gradually trying to give Raphael different textures, so far he can eat soft crumbly cake in small amounts.
Slowly developing gross motor skills
Early intervention playgroup once per week.
Auslan playgroup once per week.
Added a double rail to our front steps so that Raphael can hold it while climbing and descending the steps.
Lots of trips to the local park and playground equipment.
Sleep Apnoea
Sleep testing about once a year
CPAP while he sleeps overnight with an oximiter.
Heart defect
Echo cardiographs occasionally to ensure stability.
No current action.
Funny looking right ear
Nothing.
It would have been possible to mould his ear within the first few weeks of life without surgery, but unfortunatelly we did not know about the ways of doing this then.
Aspirations
It has been recommended that thickening fluids that he drinks would be a good idea to reduce the chance of aspirating fluids.
We are ignoring this as it is just too much trouble along with everything else we have to do.
Vomiting
When he has periods of vomiting we limit his fluid intake and feed him10ml of water at a time every 10 minutes to try to keep his fluids up.
We have to make sure that there are no drinks sitting around for him to find or he will guzzle them and then vomit.
Cognitive skills
Formally testing him yearly to make sure that we are addressing any shortcomings.
I work with computers for a living (a systems administrator for those of you who know a little about the industry). This gives me an advantage with working with computers to make my life easier and the ability to give me information at my fingertips. However there is always a cost of having increased information, and that is increased time required to absorb that information and turn it into knowledge.
On Thursday night I spent three hours writing a script (programme) to download all of the video signs from http://www.auslan.org.au for my own personal use in educating myself more in Auslan. If this is in breach of the copyright of the site then I do apologise. I also downloaded the synonyms and meanings of the words and grouped them all appropriately together so that I can easily look them up. I then loaded them onto my phone with appropriate free software to be able to display them. Now I can lookup the signs for over 7000 words quickly wherever I am. For instance I just timed myself and from a cold start it took me 20 seconds to look up the word "Whatever" and so now I know how to sign it.
The next part involved getting a set of pictures that Raphael can look at so I can then teach him the sign for the word. The reason that I was thinking of doing it this way is because of the outstanding success that I had previously with teaching Raphael signs from pictures. He loves looking at the pictures on my phone and then signing it.
So my wife and I started scanning/photographing the pictures from a childrens' picture dictionary of 1500 words. Then I spent another couple of hours writing another script to compare the list of video signs with the list of picture signs that I had and creating folder of all of the correlating pictures.
So now all I have to do is stay one letter ahead of Raphael to teach him a stack of signs. So far I have gone through about 60 signs and reviewed or learned all of them. This has brought me up to about half way through the letter b so there is plenty more to do. I have only scanned up to the letter D, so I will need to do more photographing of the dictionary aswell.
That is my plan but there is one serious flaw to the plan. Raphael learns faster than I do.
This is the sort of thing that I have access to now:
Click on image to replay the sign.
crash
smash
accident
As a Noun
An event in which a moving car or other vehicle hits something and is badly damaged or destroyed. English = crash, smash, accident.
As a Verb or Adjective
Of a car or other moving vehicle, to hit something and be badly damaged or destroyed. English = crash, smash, have an accident.
all professionals say no useful vision from this eye but recent patching (27/9/2007) shows that he can distinguish objects at least as small as peas. 1/6/2008 he can walk with his right eye patched. 31/7/2008 vision improved in left eye, receives peripheral vision from left eye even with no occlusion of right eye.
Cranial Nerves:
left facial palsy
left optic nerve hypoplasia
vestibulocochlea nerve malformations
Uncoordinated swallow resulting in frequent aspirations (he has a good clearing cough though)
Heart:
Small ASD (has also been described as a patent foramen ovale by different cardiologist)
Aberrant right subclavian artery
Growth and development:
fed by NGT from 4/12/2006 till 9/7/2007 because he would not put on weight fast enough.
development slightly delayed, receives early intervention
Poor swallow also means that he is unable to swallow anything lumpier than a purée without coughing and gagging. January 2009, has started to cope with bread and soft fruits. Currently main nutrition comes from Nutrini and purées (fortified with Paediasure).
Ears:
Bilateral vestibular malformations; "only 1 hypoplastic semi-circular canal is identified". Walking is preferred mode of travel at 26 months.
Bilateral grommets inserted to deal with chronic ear infection. These extruded around May 2008.
Bilateral hearing aids to address hearing loss
Right ear:
characteristic CHARGE external right ear
moderate (60db) hearing loss in right ear
Left ear:
severe (90db-100db) hearing loss
vestibulocochlea nerve malformations
Airway:
experiences frequent obstructive sleep apnoea has CPAP when sleeping to overcome this (pressure: 8-9). September 2008, post adenotonsilectomy, reduced CPAP to pressure 4.0. November 2008 sleep study in Monash recommends CPAP to be set to 5.0.
aspirations are common while drinking ("significant gastro-oesophageal reflux" and "excessive pharyngeal milk and secretion residue")
His laryngomalacia has not been noted for some time now. Laryngomalacia noted during adenotonsilectomy 4/7/2008.
Mucus and food that he has recently eaten frequently runs from his nose.
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 with pneumonia, suspected viral, temperature peaked over 40.
11/2/2008-13/2/2008: brought into hospital because of concern about possible dehydration; admitted to hospital with pneumonia.
12/10/2007-13/10/2007: admitted to hospital because of very large tonsil airway obstruction concerns.
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.
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 (post intubation stridor).
4/12/2006-8/12/2006: hospitalised for NGT placement and training.
18/3/2006: Born in Calvary hospital. Normal full term delivery.
We are yet to get the report from the cardiologist but the sonographer showed us that there is still a hole on the ultrasound display.
We had a training paediatric sonographer perform the test on Raphael with an experienced sonographer sitting behind her. Raphael was acting as a perfect child while he was being scanned and lay perfectly still for about 45 minutes, holding his favourite toy ambulence in his hand until therey were all finished.
We talked with the sonographers while they were doing the scan and they often commented on Raphael's amazing compliance (especially considering he is nearly three year old). I asked how they did the test with uncompliant children and they just said "quickly". It is nice to know that they had time to be very thorough. At home Raphael isn't nearly as compliant, but there is something about being in the hospital that sedates him.
I have had this blog post in draft for a very long time. These were references to papers that supported patching (occlusion) to improve vision in eyes that have serious physical defects. They didn't appear to be very well written but they gave me hope for patching in Raphael and my hope has been vindicated. I only provide the references here in case any other parent in a similar situation needs them:
Well I didn't think he'd be able to handle an m&m but my wife thought otherwise and as today marks the start of us starting Raphael's potty training, Annie wanted to the food reward system to be something new.
He handles the m&ms well, he eats them by waiting for them to disolve in his mouth and then they are no problem for him.
The potty training on the other hand hasn't been entirely successful yet but we have only be trying for a couple of hours so far and it took our other kids a week before they were potty trained.
Raphael has made moderate speaking progress this year but has been picking up Auslan signs extremely quickly. I can no longer count how many signs he knows because there are too many to keep track of.
Today he spontaneously used a two sign phrase that we have not used with him before. He signed "Brother" then "Sit" then pointed to his back. This was shortly after his mean father put a stop to a game where Raphael's older brother was bouncing on Raphael's back.
So that Raphael can participate in eye chart tests, we trained him to sign the eye chart pictures. I did this by photographing the chart with my phone and sitting with him and going through the pictures. Here is the result of our work:
You'll notice that I have been a bit slack in blogging lately. I have stopped blogging every single appointment because it was getting silly with at least four a week.
