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Wednesday, July 18, 2012

Annual Review 2012

Ruby's second annual review was today. Sort of like a birthday, but a celebration we would rather not be having. All 4 of us took the trip down to Westmead today. I was very organised and packed a bag full of food and entertainment for the kids.

We got there at 9.30 and started with the normal weigh-in and height measurement. Ruby was very compliant and did exactly as she was told. In fact, she was like that most of the day. It takes a lot to upset her.

Eliza lending some weight to Ruby



Her sweat test wasn't until 10.30 so we started off with the normal nurse checkup. Much to our amusement, Ruby and Eliza burst into squeals of excitement when the suction machine was wheeled in, as it has a huge tin of lollies on it. Ruby had her sputum suction and fir the first time ever didn't cry. It will take a few days to get the results back.  Both kids received a specimen jar full of jellybeans!

Physio checkup was all good too. We discussed different blowing exercises and will be starting off with blowing bubbles in the back with a piece of tubing.  We also discussed getting a vest again. Lot's to think about there especially as a long term study has been completed where it was shown that PEP therapy is more efficient than the vest. 

Then we were off to the sweat test. Electrodes were attached to Ruby's skin to stimulate the sweat glands, then some filter paper was placed onto her skin to collect the sweat for analysis. She left this on for half an hour, but unfortunately not enough sweat was produced and we will have to try again another day. All through the process, Ruby just watched intently what was going on and never got upset. Until it was time to pull the tape off, and then it really hurt her and she had a good cry. 

The electrodes that stimulates the sweat cells to test the level of chloride
Then a consultation with the gastro dr who felt her belly for any enlarged organs and hard parts of the bowel but all was good and squishy. We then chatted to the dietitian which is always painful as I can never actually remember all the things I feed Ruby and I get asked questions like "how much cream do you add" or "how much water does she drink" and my answers of 3 splashes, or showing an amount with my fingers aren't good enough. But dieto is happy with the amount she is eating and the amount of Creon that I am giving her, and no changes need to be made, or supplements added.

Finally, we see Ruby's main dr who checks her lungs (all clear) and we talk about new drugs coming and her general health. We decide to start her on Pulmozyme which is a drug that changes the DNA of mucous, making it shorter, therefore thinner and easier to cough up. This drug is used in many countries as a preventative and works really well. It is try expensive ($25,000 a year) and can only be covered by the PBS if you meet a certain criteria. Which Ruby does, based on her age and recent fight with pseudo. As the doctor said, "we are trying to preserve Ruby's lungs until there is a drug available that will manage all of CF" (referring to the new miracle dug being trialed with different gene types, Kalydeco).

So I feel very lucky that we don't have to push our clinic to try these things, and that we have a health care system that makes this affordable to us. I also appreciate that these things can seem inconsistent between clinics. As Ruby is too young to have the lung function tests, she does not have to show lung improvement to stay on this drug. 


New toys from clinic for being a good girl

So then we pick up a huge bag of meds from the CF pharmacy, pickup the bits and pieces that we have gained (including 2 cute dr teddy bears), make a date to come back in 2 months and treat ourselves to lunch at the hospital cafe.  Oh yeah, then walk to Parramatta Park where we had to park.

A busy day, but both kids did well. Eliza looked out for Ruby and Ruby was charming to the staff as always. 

CF never stops. We are always learning something new, can't rest for  second. 





2 comments:

Allison in AK said...

Hello ~ I just googled "amber teething necklaces cystic fibrosis" and found you! My 7 month old has CF (I also have a 17 year old with it and 5 more without.) and she's having lots of trouble with teething. Don't remember any of the other kids having such trouble. I wasn't sure if whatever the amber does is OK with CF's weird cells. I've benefited from your research here and plan to order one. Thank you for writing!
A new, bloggy friend,
Allison

Allison in AK said...

P.S. ~ your girls are cute, cute, cute!