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.