Friday, 10 April 2009

Simple Sleep Study

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.

Thursday, 9 April 2009

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: ___________________


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.

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.
Raphael has two sleeps of a day:

  1. Afternoon sleep from about 14:00 to 16:00
  2. 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.

Raphael is transitioning from fortified foods to family meals.

Currently he has a number of sources of nutritian intake:
  1. Milk (Nutrini high-energy multi-fibre) by straw cup
  2. Slice of bread spread with "paediasure butter" (three scoops of paediasure mixed with small amount of water to make a spreadable texture).
  3. Sustagen Icecreams (Sustagen made up to a thick consistency and then frozen on a paddlepop stick to make an icecream).
  4. 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.

Raphael sleeps at night with his CPAP machine to reduce his sleep apnoea. Normally his setting is at 5.0.

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%-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%.
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.
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)
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.
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 to reduce the swelling of Raphael's tonsils)
  • 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

Wednesday, 8 April 2009


Well it looks like that trampoline that we bought has been doing good therapy for us.

Raphael jumped for the first time today. Both feet leaving the ground at the same time. He did this in our lounge room, not on a trampoline.